Rotation- Pediatric (Pre-Test Questions, Blueprint, And Usmle World))

Front 1

At this age an infant will typically regard the face, follow items to the midline, begin to vocalize, respond to a bell, and lift it's head from an examining table. How old?

Back 1

1 MONTH (4 weeks)!!!

Front 2

At this age a child should at least be able to smile and coo, when smiled at or talked to. How old?

Back 2

2 months (8 weeks).

Front 3

Not until this age would an infant be expected to follow a moving toy from side to side and also in a vertical plane. How old?

Back 3

3 months (12 weeks).

Front 4

Skills such as maintaining a seated position occur in what infant age range?

Back 4

6-8 months.

Front 5

At what age can children begin to copy a square? How old is a child that can balance on one leg for only 2 seconds? 5 seconds? At what age are children able to dress themselves independently?

Back 5

4-5 years old. Children younger than this have trouble with this task. A 4 year old can only balance on one leg for about 2 seconds. A 5 year old can balance for about 5 seconds. At age 5 a child should be able to dress herself independently.

Front 6

By what age should the language of a child be fully understandable and the child be able to name four colors, define five words, and know three adjectives?

Back 6

This all occurs by age 4.

Front 7

Pauciarticular juvenile rheumatoid arthritis asymmetrically involves large joints, especially the knee, and often has no other symptoms. 20% of girls, however, that have the monoarthritis, or pauciarticular form of juvenile rheumatoid arthritis have what other symptom as their only other sign of disease? What examination is recommended to avoid morbidity of this condition?

Back 7

These girls are found to have Iridocyclitis (Anterior Uveitis). Because this eye disorder can result in blindness and can require treatment with local or systemic steroids, it is recommended that children with this condition have frequent slit-lamp eye examinations.

Front 8

What is another name for Staph Scalded Skin Syndrome? What age group does this disease most commonly occur in?

Back 8

Ritter disease. This is most commonly seen in children under 5 years of age.

Front 9

Patient under 5 years of age presents with a rash that was preceded by fever, irritability, erythema, and extraordinary tenderness of the skin; circumoral erythema, crusting of the eyes, mouth, nose, and blisters on the skin can develop. Intraoral mucosal surfaces are not affected. Diagnosis? Concerns of care for this patient are likened to care for that of what other type of patient?

Back 9

Staph Scalded skin syndrome. Peeling of the epidermis in response to mild shear forces (Nikolsky sign) leaves the patient susceptible to problems similar to those of a burn victim, including infection and fluid and electrolyte imbalance.

Front 10

What would the recommended treatment be for a patient who had an injury from a human bite that became red, indurated, and swollen, and who has a temperature of 39.4C (103 F)? How should the wound be "allowed to heal"?

Back 10

Hospitalization fro appropriate drainage procedures, Gram stain and culture of the exudate, vigorous cleaning, debridement, and appropriate antibiotics. The wound should be left open and allowed to heal by secondary intention (healing by granulation tissue rather than closure with sutures due to possilbe infection with anaerobes from the oral flora).

Front 11

What generalized acute vasculitis classically presents with arthritis, usually of the large joints, colicky abdominal pain, vomiting, and melena; renal involvement occurs in a significant number of patients and is potentially most serious manifestation of the disease; papable purpura. Diagnosis?

Back 11

Henoch-Schonlein purpura (Anaphalactoid purpura).

Front 12

Can leukemia cause pupura?

Back 12

YES!!!

Front 13

What do the serum compliment and IgA studies look like in a patient with Anaphalactoid purpura (Henoch-Schonlein purpura)? What about coagulation studies and platelets?

Back 13

Compliment and IgA can be normal or elevated. Coagulation studies and platelets are normal.

Front 14

A 6 year old child who was toilet trained at age 2 begins to develop ongoing constipation over the course of a couple of months. Later the child begins stooling in their underwear without the ability to control the occurrence. On physical examination stool is found in the rectal vault. Diagnosis? What variations of this condition exist?

Back 14

Encopresis is the main diagnosis; this is defined as the passage of feces in inappropriate locations after bowel control would usually be expected (usually older than 4 years of age). Encopresis is seen with chronic constipation and overflow incontinence (retentive encopresis), and without constipation (non-retentive encopresis). Retentive Encopresis with liquid stool leakage around a large fecal impaction, is more common.

Front 15

This condition of the tibia has a juvenile form (1-3 years) and a toddler form (4-10 years). This is Genu Varum (bowlegs) that continues to progress and worsen, and is in some cases unilateral. Radiographically there is a prominent step abnormality and beaking at the proximal tibial epiphysis. Bracing can be affective up until the age of 3; later correction may require surgery. Diagnosis?

Back 15

Blount disease.

Front 16

Avascular necrosis of the femoral head, cause by an unknown interruption of the blood supply by a currently unknown cause; onset is usually between 2 and 12 years of age and classically presents with a painless limp, although mild pain of the thigh is common. Diagnosis?

Back 16

Legg-Calve-Perthes disease.

Front 17

Repeated microfracture of the tibial tubercle at the insertion of the patellar tendon in a child. This is an overuse injury, and presents with swelling and knee pain localized to the tubercle. Improvement occurs with rest. Diagnosis?

Back 17

Osgood-Schlatter disease.

Front 18

This condition typically occurs in overweight adolescents, and presents with a limp. Radiographically, the capital femoral epiphysis is separated from the neck of the femur and remains in the acetabulum as the rest of the femur moves anteriorly. Diagnosis?

Back 18

Slipped capital femoral epiphysis (SCFE).

Front 19

What are the diagnostic criteria for Diabetes mellitus in terms of a fasting glucose level, 2-hour plasma glucose during an oral glucose tolerance test, or symptoms of Diabetes mellitus plus a random plasma glucose?

Back 19

Fasting glucose >126 mg/dl

2-hour plasma after oral challenge >200 mg/dl

Symptoms + random plasma glucose >200 mg/dl

Front 20

What glucose levels indicate impaired glucose tolerance in terms of fasting values and 2-hour post oral challenge values?

Back 20

Fasting value 100< x <125 mg/dl

2-hour post oral challenge value 140< x <200 mg/dl

Front 21

This is a rodenticide that is still found in some rural communities; more common poisoning with this compound is found in fire victims; patients may present initially with headache, but then become agitated and confused. Seizures and dysrhythmia may follow; blood gases will show severe metabolic acidosis. Diagnosis?

Back 21

Cyanide poisoning.

Front 22

What is the cause of neonatal acne and what is the peak age of incidence? How long does it take to resolve?

Back 22

Neonatal acne results from maternal hormone transmission and peaks at about 2 to 4 weeks of age. It resolves in a few weeks to months, and is occasionally severe enough to require treatment with agents such as tretinoin or benzoyl peroxide.

Front 23

What is the description of Milia found in a neonate?

Back 23

Milia are benign, tiny white bumps found on the nose.

Front 24

What are the main criteria for diagnosing ADHD in terms of age and duration of symptoms?

Back 24

Symptoms of inattentiveness and hyperactivity must be present for more than six months and must occur before seven years of age. The symptoms must cause significant impairment in functioning and must occur in at least two different settings.

Front 25

What is the major cause of end stage renal disease in children?

Back 25

Renal scarring secondary to vesicoureteral reflux disease.

Front 26

How is the diagnosis of VUR (vesicoureteral reflux) best made?

Back 26

Via cystourethrogram or radionuclide cystogram.

Front 27

What is the recommendation from the American Academy of Pediatrics for a child that has a first UTI from 2 months - 2 years?

Back 27

That child should undergo a VCUG or RNC to detect VUR.

Front 28

Patient presents with inattentiveness and hyperactivity-impulsivness. His symptoms have been present for mote than six months, began prior to age seven, and cause significant impairment in functioning both at home and at school. Diagnosis?

Back 28

ADHD; all these criteria must be met for a child to have a diagnosis of ADHD.

Front 29

What s the major cause of end stage renal disease in children?

Back 29

Renal scarring secondary to reflux; Reflux is present in almost all children with renal scars, and renal scars are present in up to 60% of children with gross reflux. The growing kidney is very prone to scarring; therefore, scarring secondary to reflux is more commonly seen in children than adults.

Front 30

What anatomical defect of the urogenital tract is one of the most common causes of hypertension in children?

Back 30

Vesicoureteral refulx disease.

Front 31

Physical findings in an infant show apathy, weakness, hypotonia, large tongue, sluggish movement, abdominal bloating, and an umbilical hernia. Patient has poor appetite, choking spells during feeding, constipation and excessive sleepiness. Diagnosis? Most common cause? 1st choice of screen? Treatment?

Back 31

Hypothyroidism; this is most commonly due to thyroid dysgenesis. Screening is done by measuring serum T4 and TSH levels. The treatment is Levothyroxine (initial dose of 10 mg/kg, then titrated accordingly).

Front 32

This is an autosomal recessive disorder that involves degeneration of the anterior horn cells and cranial nerve motor nuclei. It is a cause of "floppy baby" syndrome (the other cause is infant botulism). Diagnosis?

Back 32

Werdnig-Hoffman syndrome.

Front 33

This is an autosomal dominant disorder characterized by muscle weakness and atrophy (most predominant in the distal muscles of the upper and lower extremities), motonia, testicular atrophy, and baldness. Diagnosis

Back 33

Mytonic congenital myopathy.

Front 34

Children present with this rash which is typically pupuric and distributed symmetrically over the lower legs, buttocks and arms. Arthralgias most commonly affect the knees and ankles, with these symptoms being transient, and no permanent damage to the joints. GI symptoms are present in the majority of patients, and most commonly include colicky abdominal pain (presumably due to local vasculitis) that is frequently associated with vomiting. IgA deposition in the skin or kidney confirmed by immunoflourescence microscopy is necessary for diagnosis. Diagnosis?

Back 34

Henoch-Shonlein Purpura.

Front 35

The most common mutation that leads to this disease is a deletion of a three base pair codon encoding for phenylalanine in a gene located on Chromosome 7; it is believed that this mutation prevents the protein product of this gene from trafficking to the correct cellular location. What is the disease?

Back 35

This is a defect of DF508 in the CFTR gene located on Chromosome 7, which leads to the development of Cystic Fibrosis.

Front 36

This vitamin has been shown to reduce the morbidity and mortality rates of patient with measels through immune enhancement. Is also helps the GI and respiratory epithelium to regenerate. Which vitamin is it?

Back 36

Vitamin A.

Front 37

What is the most common test used to evaluate a patient for Phenylketonuria? What is an alternative test?

Back 37

Blood phenylalanine levels; however, Guthrie test, which is a qualitative (coloration) test of the urine, can also be used as it detects the presence of metabolic products of phenylalanine in the urine.

Front 38

Anemia with an RDW 20%< in a child is suggestive of what type of anemia?

Back 38

Iron deficiency anemia.

Front 39

A normal RDW </= 20% in a child with anemia is indicative of what type of anemia?

Back 39

A Hemoglobinopathy like Thalassemia.

Front 40

What is the drug of choice for Thrombocytopenia in all age groups < 30,000/mm3?

Back 40

Corticosteroids.

Front 41

What diagnosis should you suspect in children who develop isolated thrombocytopenia after a viral infection?

Back 41

Immune Thrombocytopenia.

Front 42

A displaced anterior fat pad found on X-ray after a child has fallen on an outstretched hand, presenting with minimal swelling on the elbow, but tense skin on the forearm and sever pain is indicative of what injury with what complication?

Back 42

Displaced anterior fad pad is a radiographic sign of suprachondylar fracture, which may be complicated by Volkmann's ischemic contracture

Front 43

Supracondylar fracture, with an anterior fat pad shown on X-ray can be complicated by Volkmann's ischemic contracture. How should this type of compartment syndrome be treated?

Back 43

This condition is a type of compartment syndrome, and treatment should consist of immediate compartment release fasciotomy to prevent further damage.

Front 44

Classic presentation of this disease involves a young boy with eczema, thrombocytopenia, and recurrent infections with encapsulated bacteria. The initial mainfestations often present at birth and consist of petechiae, bruises, bleeding from circumcision, or bloddy stools; low IgM, high IgA and IgE, poor antibody responses to polysaccharide antigens, and moderately reduced numbers of T cells and platelets. Diagnosis?

Back 44

Wiskott-Aldrich syndrome, caused by a defective gene encoding the Wiskott-Aldrich protein.

Front 45

This syndrome is characterized by decreased degranulation, chemotaxis, and granulopoesis. It is a multisystem disorder with mild coagulopathy, peripheral and cranial neuropathy, hepatosplenomegaly, pancytopenia, partial oculocutaneous albiinism, frequent bacterial infections (usually S.aureus), and progressive lymphoproliferative syndrome. The findings of neutropenia and giant lysosomes in neutrophils will confirm the diagnosis!!! Diagnosis? Treatment?

Back 45

This presentation describes Chediak-Higashi syndrome; treatment includes prevention of infection with daily TMP-SMX and daily ascrobic acid.

Front 46

This syndrome is characterized by chronic puritic dermatitis, recurrent staph infections (skin and respiratory tract), markedly elevated serum IgE levels, eosinophilia and coarse facial features. Recurrent staph infections involving the skin, lungs and joints, with other features such as distinctive facial appearance, dental abnormalities and bone fractures are essential for the diagnosis. Diagnosis?

Back 46

Hyper-IgE (Job's) syndrome.

Front 47

What disease should be considered in any patient with recurrent or unusual lymphadenitis, hepatic abscesses, osteomyelitis at multiple sites, or unusual infections with catalase-positive organisms (e.g. S.aureus, Serratia marcescens, Aspergillus, and Burkholderia cepacia)?

Back 47

Chronic granulomatous disease.

Front 48

Marfan's features + thromboembolic events = what classic presentation?

Back 48

Homocystinuria.

Front 49

Describe the differences in lens dislocation that occur in patients with Marfan's vs. Homocystinuria.

Back 49

In Marfan's syndrome, the lens is typically dislocated upward, and in homocystinuria it is dislocated downward. Other eye complications of homocystinuria are cataract formation and retinal detachment.

Front 50

This type of neonatal conjunctivitis is the most common cause of a red eye that presents within the first 24 hours of life. Approximately 80% of patients who receive prophylaxis with this medication (used to prevent gonococcal conjunctivits) experience mild conjunctival irritation and tearing that generally resolve within 24hours. What is the cause?

Back 50

Silver nitrate drops.

Front 51

This neonatal conjunctivitis presents on the second to fifth day of life as a hyperacute and highly purulent conjunctivitis. It is the most destructive neonatal eye infection, since it may result in corneal perforation and permanent blindness if left untreated. The treatment is Ceftriaxone. Diagnosis?

Back 51

Gonococcal conjunctivitis.

Front 52

This neonatal conjunctivitis usually presents on the fifth to fourteenth day of life as congestion and discharge that may be scant, mucoid or frankly purulent. It may appear even in patient who received prophylactic drops. The treatment is Erythromycin, which has to be administered systemically to decrease the risk of pneumonia with this bug. Diagnosis?

Back 52

Chlamydia trachomatis conjunctivits.

Front 53

What is most commonly the first test that is done in a child that is suspected to have lead poisoning? What is the confirmatory follow up test that should be done if the first test is found to be positive?

Back 53

Capillary blood specimens (using a fingerstick) are widely used in the screening for lead poisoning. However, false positives are common for various reasons. Therefore, further work-up with a confirmatory serum venous blood level is required if a screening capillary lead level is >10 ug/dl. If the serum lead level is >10 ug/dl as well, intervention is needed.

Front 54

What condition of the kidneys is Impetigo most commonly associated with?

Back 54

Post-streptococcal glomerulonephritis.

Front 55

Recurrent impetigo with Staphylococci is most commonly due to carriage of the organism in what part of the body?

Back 55

The nares; nasal carriage.

Front 56

What are the two treatments/antibiotics of choice when treating impetigo?

Back 56

Topical Muropirocin or Oral Erythromycin are the treatments of choice for local impetigo.

Front 57

Why do patients with Turner's syndrome have a higher risk of Osteoporosis?

Back 57

Patients with Turner's syndrome have a higher risk of osteoporosis due to lower estrogen levels and only having one copy of X chromosome genes involved in bone metabolism.

Front 58

The finding of a palpable olive-shaped mass in the upper abdomen pathognomonic of what condition, and is sufficient evidence to warrant surgery?

Back 58

The finding of the olive-shaped mass is pathognomonic of Pyloric Stenosis.

Front 59

Dilation of the entire ventricular system with distinct enlargement of the subarachnoid space over the cerebral cortex is very suggestive of what condition?

Back 59

Nonobstructive or communicating hydrocephalus secondary to a subarachnoid hemorrhage (SAH). Subarachnoid hemorrhage is actually the most common cause of a communicating hydrocephalus.

Front 60

What is the most common cause of a communicating hydrocephalus in children? What is the mechanism by which this creates hydrocephalus?

Back 60

Subarachnoid hemorrhage is the most common cause of communicating hydrocephalus in children. Accumulation of blood in the subarachnoid space may lead to destruction of the arachnoid villi and cisterns (whose function is to absorb CSF fluid), thereby blocking the flow or decreasing the absorption of CSF, and leading to hydrocephalus. SAH can be caused by intraventricular hemorrhage which is common in premature infants.

Front 61

What study is essential to making the diagnosis of Acquired Aplastic Anemia?

Back 61

Bone Marrow Biopsy.

Front 62

Pure red cell aplasia which occurs in healthy children between 6 months and 5 years old. There is a gradual onset of symptoms such as pallor and decreased activity. Physical examination is unremarkable except for pallor and tachycardia. Typical laboratory findings are normocytic normochromic anemia, with hemolobin levels ranging from 3 to 8 g/dl, and extremely low reticulocyte count. Diagnosis?

Back 62

Transient Erythroblastopenia of Childhood (TEC).

Front 63

Pure red cell aplasia that presents in the first 3 months of life with pallor and poor feeding. CBC reveals a normocytic or macrocytic anemia with reticulocytopenia. WBC and platelet counts are normal. Diagnosis?

Back 63

Diamond-Blackfan anemia (DBA), or Congenital Pure Red cell aplasia

Front 64

Patient presents with pancytopenia and characteristic congenital anomalies, such as hyperpigmentation on the trunk, neck and interiginous area and/or eyelid changes, and renal malformations. Blood counts start to decrease between 4 and 12 years of age, and the initial manifestation is usually thrombocytopenia, followed by neutropenia, then anemia. Diagnosis?

Back 64

Fanconi's anemia.

Front 65

What is the peak age of incidence for a child with intussusception?

Back 65

8 months; the range is from 3-36 months

Front 66

What segment of the bowel is intussusception most commonly found in?

Back 66

Intussusception is most commonly found at the Ileocolic junction, with the Ileum telescoping into the Colon.

Front 67

What are associated lead points for children outside the age range of 3-36 months that present with intussusception?

Back 67

Meckel's diverticulum, foreign obdy, polyp, lymphoma, or hemangioma.

Front 68

Classic presentation of this condition is an infant with colicky abdominal pain, vomiting, and red currant jelly stools. Between abdominal pain episodes, the child appears well. On physical exam, the abdomen is usually soft, nontender, and nondistended. A sausage-shaped mass is often palpated. Abdominal ultrasound may show "targetoid" lesion. Diagnosis?

Back 68

Intussusception.

Front 69

Why do we see hyperinflation of the lungs in a neonate with meconium aspiration? What state does this hyperinflation lead to?

Back 69

Hyperinlfation occurs secondarily to the valve-like mechanism by which meconium obstructs the airways, keeping air from exiting the lungs after each inspiration. This state of hyperinflation consequently leads to a barrel shaped chest (increased AP diameter).

Front 70

The typical chest radiograph of a neonate with this condition includes flattening of the diaphragm, hyperinflation, coarse streaking and patchy opacities over both lung fields. Complications include pneumothorax, infection, pulmonary hypertension, residual lung problems and permanent neurological impairment due to asphyxia. Diagnosis?

Back 70

Meconium aspiration of the newborn.

Front 71

Transient tachypnea of the newborn is cause by fetal lung fluid which remains in the lungs and causes respiratory difficulty. It is a self-limited condition that generally improves after a few hours. Ascultation DON NOT show rales or ronchi. The lungs are usually very clear and patients usually do not require more than 40% FiO2 to oxygenate adequately. What is the most common setting for this to occur?

Back 71

This occurs most commonly occurs in fetuses delivered by Cesarian section.

Front 72

Umbilical hernias most commonly occur in what pediatric demographic? What are the criteria for observing for spontaneous resolution and referring for surgical management?

Back 72

Umbilical hernia is due to an imperfect closure or weakness of the umbilical ring. It is especially common in low birth weight, female black infants. Most umbilical hernias disappear spontaneously by 1 year of age. Surgery is advised if the hernia persists to the age of 3-4 years, exceeds 2 cm in diameter, causes symptoms, becomes strangulated, or progressively enlarges after the age of 1 to 2 years.

Front 73

What is the recommended first line treatment for pertussis? Can you give it at any time during the course of the disease?

Back 73

The recommended first line treatment for pertussis is a macrolide antibiotic (erythromycin, azithromycin, or clarithromycin) regardless of the age or the immunization status. Treatment is given whenever pertussis is suspected or confirmed, and regardless of the stage of the disease. This treatment is of benefit because it shortens the evolutino of the disease and clinical improvement, and it limits the spread of infection.

Front 74

Me(T)anephros is the embryologic precursor of the renal parenchyma and it gives rise to what tumor of children?

Back 74

Wilm's (T)umor!

Front 75

What embryologic structure gives rise to the seminal vesicle, epididymis, ejaculatory ducts and ductus deferens?

Back 75

Mesonephros.

Front 76

Paramesonephros gives rise to what structures and in which gender?

Back 76

Paramesonephros gives rise to the fallopian tubes, uterus and part of the vagina.

Front 77

These four clinical criteria have been shown useful in differentiating what two conditions: WBC >12,000/mm3, Temp >39C (102F), ESR >40mm/h, and REFUSAL to bear weight.

Back 77

These criteria are used to work up Septic Arthritis vs. Transient synovitis associated with URI. If at least thre of these four criteria are met, further work-up is indicated to rule out septic arthritis.

Front 78

1 month old infant brought in for routine visit; histories are unremarkable, vital signs normal; examination reveals a harsh, loud holosystolic murmur over the left lower sternal border; palpation reveals a thrill over the left precordial region; no cyanosis and pulmonary ascultation reveals no rales, CXR reveals a heart of normal size and a slight increase in pulmonary vascularity; EKG normal. Diagnosis? Treatment?

Back 78

All this is characteristic of a likely small VSD, which is the most common type of congenital heart defect. In small VSDs, the volume of the left to right-shunt remains small even after the postnatal decline in pulmonary resistance. Thus, the pulmonary circulation is protected, and symptoms which wouls otherwise appear due to the increased blood volume in the lungs are absent. Small VSDs close spontaneously in 40% of cases by three years and in 75% of cases by 10 years, so reassurance and surveillance are all that are needed.

Front 79

This is an autosomal recessive condition characterized by an excessive number of trinucleotide repeat sequences, resulting in an abnormality of tocopherol transfer protein. It is associated with necrosis and degeneration of cardiac muscle fibers leading to myocarditis, myocardial fibrosis and cardiomyopathy; cardiac arrythmia and congestive heart failure contribute to a significant number of deaths. Diagnosis?

Back 79

This all points to a diagnosis of Freidriech's ataxia.

Front 80

What are the developmental milestones of copying images for years 3,4,5,6?

Back 80

3 year- copies a cross and a circle
4 years- copies a square and a rectangle
5 years- copies a triangle
6 years- copies a diamond

Front 81

This is a rare syndrome characterized by precocious puberty cafe au lait spots and multiple bone defects (polyostotic fibrous dysplasia). It is responsible for 5% of the cases of female precocious puberty, and may be associated with other endocrine disorders, such as hyperthyroidism, prolactin- or GH-secreting pituitary adenomas and adrenal hypercortisolism. This condition is sporadic and has been recently attributed to a defect in the G-protein cAMP-kinase function in the affected tissue, thereby resulting in autonomous activity of that tissue. Diagnosis?

Back 81

McCune-Albright syndrome.

Front 82

What are the 3 P's associated with McCune-Albright syndrome?

Back 82

(P)recocious puberty, (P)igmentation (cafe au lait spots), and (P)olyostotic fibrous dysplasia.

Front 83

What is the most common posterior fossa tumor in children?

Back 83

Cerebellar astrocytoma is the most common. Medulloblastoma is the second most common.

Front 84

Where do >90% of medulloblastomas develop?

Back 84

In the Cerebellar vermis, which leads to posterior vermis syndrome (truncal dystaxia).

Front 85

If there is a mass detected in the cerebellar hemisphere of a child, what is the likely diagnosis?

Back 85

Either an abscess or an astrocytoma.

Front 86

This cerebellar syndrome consists of arm leg, and gait dystaxia, with ipsilateral cerebellar signs. Diagnosis?

Back 86

Hemispheric cerebella syndrome.

Front 87

A child presents with headaches prominent in the morning; lethargy, decreased appetite and decline in school performance; unbalanced gait, trunk dystaxia, horizontal nystagmus, papilledema. Diagnosis?

Back 87

Posterior cerebellar vermis syndrome; likely due to Medulloblastoma.

Front 88

A child falls on a pencil or stick in the mouth. What injury is this child at risk for?

Back 88

The picture will present with the patient having symptoms of an acute stroke, which will be due to Internal Carotid artery dissection, which may be followed by thrombosis. Interestingly, the onset of symptoms may be delayed up to 24 hours after the traumatic episode.

Front 89

Ascending paralysis in a child after a gastroenteritis with CSF analysis revealing significantly elevated protein levels and mildly elevated cells (ALBUMINO-CYTOLOGIC DISSOCIATION). Diagnosis? Treatment? What type of nerves are involved?

Back 89

This is Guillan-Bare syndrome and the treatment is plasmapheresis or human immunoglobulin. Recovery follows the inverse order of the initial progression of the disease. The underlying pathology involves mainly the peripheral motor nerves, although sensory and autonomic nerves may also be affected.

Front 90

A large thymic shadow on CXR can be misdiagnosed in children less than what age, as a right upper lobe infiltrate, right middle lobe infiltrate, hilar lymphadenopathy, or Cardiomegaly?

Back 90

A large thymic shadow is a normal finding on a chest radiograph in children less than 2 years old.

Front 91

What is the most common type of congenital heart defect? What is the type of heart sound that it is associated with? What is the diagnostic test that should be done?

Back 91

ASD is the most common congenital heart defect. It is usually detected incidentally in school children, and has a characteristic fixed splitting of the second heart sound. An Echocardiogram is diagnostic.

Front 92

How can PDA be treated percutaneously?

Back 92

PDA can be treated percutaneously via placement of coils in the PDA.

Front 93

What is the most useful test for diagnosing Pyloric stenosis?

Back 93

Abdominal ultrasound is the test used for diagnosing Pyloric stenosis.

Front 94

Are Preterm infants given vaccinations according to their chronological age or their gestatinonal age?

Back 94

Chronological age; the American Academy of Pediatrics recommends that vaccinations for pretem infants be given according to their chronological age because prematurity does not markedly change the immune response to vaccines and the risk of infection and complication in preterm infants is greater than term babies.

Front 95

Newborns should be given the Hepatitis B vaccine at birth no matter their gestational age. When should this vaccine not be given?

Back 95

Only if the infant weighs less than 2 kg.

Front 96

What vaccines should a child be given at two months of age?

Back 96

Hep B, Rotavirus, Tetanus, Diphtheria, Acellular Pertussis, HiB, pneumococcal, and inactivated polio vaccines.

Front 97

Can live attenuated vaccines be given to infants that were Preterm?

Back 97

YES!!!

Front 98

What is the primary use of Carotid Ultrasonography?

Back 98

This is used to evaluate Carotid stenosis.

Front 99

What hemoglobinopathy can cause stroke in children?

Back 99

Sickle cell anemia!!!

Front 100

What are the clinical tests that help to establish the diagnosis of Hyaline Membrane disease in the newborn? What are the definitive treatments?

Back 100

The clinical course, CXR, blood gas and acid base values help to establish the diagnosis of hyaline membrane disease of the newborn. Treatment includes early mechanical ventilation and surfactant administration.

Front 101

This type of respiratory distress in the newborn usually follows an uneventful normal term vaginal or cesarean delivery. It is characterized by early onset of tachypnea, sometimes with retractions or expiratory grunting, and typically cyanosis that is relieved y minimal oxygen. The lungs are usually clear without rales or ronchi and the CXR show prominent pulmonary vascular markings, fluid lines in the fissures, over aeration, a flat diaphragm, and occasionally pleural fluid. Diagnosis?

Back 101

Transient Tachypnea of the newborn.

Front 102

This type of respiratory distress of the newborn is associated with universal hypoxia that is unresponsive to 100% oxygen. The CXR may be normal or may show parenchymal opacification in the chest, depending on the etiology. The presentation of this condition is often indistinguishable from Hyaline Membrane disease.

Back 102

This is Persistant Pulmonary Hypertension of the newborn and should be suspected in all term and post-term infants with cyanosis, with or without fetal distress. Persistence of the fetal circulatory pattern of right-to-left shunting through the PDA and Foramen ovale after birth is due to very high pulmonary vascular resistance.

Front 103

Partial airway obstruction of some airways may lead to pneumothorax or pneumomediastinum; patchy infiltrates, coarse streaking of both lung fields, increased anteroposterior diameter, and flattening of the diaphragm characterize the typical CXR of the newborn found with this condition.

Back 103

This describes a newborn with Meconium aspiration syndrome.

Front 104

This condition of the newborn classically present as with periods of apnea, pallor or cyanosis, poor suckling, abnormal eye signs, high-pitched, shrill cry, muscular twitching, convulsions, decreased muscle tone or paralysis, metabolic acidosis, shock, and decreased hematocrit; the fontane may be tense and bulging. The condition is rarely present at birth and is diagnosed on the basis of the history, clinical manifestations, and trans-fontanel cranial ultrasonography or computed tomography (CT). Dianosis?

Back 104

Intracranial hemorrhage!!!

Front 105

A CXR description of fine reticular granularity of the lung parenchyma for a preterm infant most likely describes what condition?

Back 105

This describes Hyaline Membrane Disease.

Front 106

This congenital infection presents in the first year of life with clinical manifestations of intermittent fever, osteitis and osteochondritis, mucocutaneous lesions, lymphadenopathy, hepatomegaly and PERSISTENT RHINITIS. Diagnosis?

Back 106

Congenital syphilis.

Front 107

Patients with this congenital infection can present with microcephaly, microphtalmia, and meningoencephalitis; but the classic triad consists of sensorineural deafness, cardiac anomalies such as persistent ductus arteriosus and atrial septal defects, congenital glaucoma and cataract. Diagnosis?

Back 107

Congenital rubella syndrome.

Front 108

Chorioretinitis, hydrocephalus, and intracranial calcifications make up the classic triad for what congenital infection?; there can also be hepatomegaly, diffuse lymphadenopathy, jaundice, and diffuse petechiae.

Back 108

Congenital toxoplasmosis.

Front 109

Injury to what organ system in a patient with HUS results in the highest morbidity and mortality?

Back 109

The Kidneys!!!

Front 110

Pediatric patient presents with abdominal pain and bloody diarrhea for the past two days; she is lethargic and weak; poor skin turgor, face and extremities are swollen; skin is yellow; labs show anemia, thrombocytopenia, and elevated total bilirubin. Diagnosis?

Back 110

Hemolytic Uremic Syndrome!!!!

Front 111

Patients with deficiency of the carbohydrate degrading enzyme most often only present with the symptom of cataracts. Diagnosis?

Back 111

Galactokinase deficiency

Front 112

Patients with this disease show the signs and symptoms of a patient with Galactose Urydyl-transferase deficiency, but have the additional findings of hypotonia and nerve deafness. Diagnosis?

Back 112

Uridyl diphosphate galactose-4-epimerase deficiency.

Front 113

What is the most common age range for febrile seizures to occur in?

Back 113

3 months-6 years of age

Front 114

Describe the time period and quality of a simple febrile seizure.

Back 114

A simple febrile seizure is a generalized tonic-clonic seizure that lasts less than 15 minutes and occurs only once in a 24 hour period.

Front 115

Do simple febrile seizures cause permanent brain injury or increase the child's future risk of epilepsy?

Back 115

NO!!!; febrile seizures are not associated with either of these.

Front 116

What are the criteria for diagnosing a complex febrile seizure? What is the time span of the seizure; quality of the seizure

Back 116

A complex febrile seizure is diagnosed if the seizure is FOCAL, lasts >15 MIN, or if the total duration of seizure activity lasts longer than 30 MIN.

Front 117

Are children with complex febrile seizures at greater risk for recurrence or afebrile seizures?

Back 117

YES!!! Children with complex febrile seizures are at an increased risk for febrile or afebrile seizures in the future, especially is there is a family history of epilepsy in a parent or sibling!!!

Front 118

What type of illnesses are febrile seizures most commonly associated with?

Back 118

Viral illnesses or Otitis media.

Front 119

What procedure should be considered in a patient less than 18 months of age that is not considered in patients older than that age, when the child presents with a report of what seems like a simple febrile seizure?

Back 119

A lumbar puncture should be considered in children less than 18 months old (especially less than 12 months old), as a neurological exam may be unreliable in working up the cause of the seizure-like activity and in ruling out possible sepsis.

Front 120

Is VUR more common in girls or boys? VUR is present in what percentage of children with UTI? How is diagnosis of this condition made?

Back 120

GIRLS; VUR is present in 35-40% of children with UTI; diagnosis of this condition is made with a Voiding Cystourethrogram (VCUG) followed by renal imaging.

Front 121

Low serum C3 and CH50 levels with normal C4 levels indicate activation of which branch of the compliment pathway?

Back 121

The Alternative Compliment Pathway.

Front 122

The hypocomplimentemia that occurs with poststreptococcal glomerulonephritis should resolve in how many weeks? What diagnoses should you consider if it does not? How long does it take for renal function to return to normal?

Back 122

Hypocomplimentemia usually resolves in 8 to 12 weeks. Other disorders such as SLE and Membranoproliferative glomerulonephritis should be considered if hypocompplimentemia persists for at least 4 months!! Renal function returns to normal in 1-2 weeks as the inflammation begins to resolve with clearing of infection.

Front 123

Hematuria, which is usually microscopic, may persist for 6 months following and episode of acute post-streptococcal GMN. Persistent hematuria beyond that period is suggestive of what other condition?

Back 123

IgA nephropathy.

Front 124

Proteinuria involved in Post-Strep GMN resolves very slowly. How long could proteinura last potentially?

Back 124

Up to 15% of patients may have proteinuria for a period of 3 years.

Front 125

What is the course of ASO titers found in PSGMN during days to month after the infection?

Back 125

Antibody titers tend to rise after 7 days, peak after one month, and return to normal in 3-4months.

Front 126

What are 5 long-term neurologic sequelae associated with bacterial meningitis in children?

Back 126

1. Hearing loss
2.Loss of cognitive functions (due to the neuronal loss in the dentate gyrus of the hippocampus)
3. Seizures
4. Mental retardation
5. Spasticity

Front 127

Eosinophilia, thrombocytosis, leukopenia, bleeding, skin rash, and increased creatinine, AST, ALT and bilirubin levels have all been associated with the use of what antibiotic used to treat pediatric meningitis?

Back 127

CEFTRIAXONE!!!

Front 128

The main treatment for Tricyclic antidepressant intoxication presenting with seizures, hypotension, and prolonged QRS complexes on EKG, after management of ABC's involves what medication? Why?

Back 128

The treatment for tricyclic antidepressant intoxication includes SODIUM BICARBONATE. This drug not only helps to correct the acidosis, but also helps to narrow the QRS complex prolongation! Benzodiazepine is given when the patient presents with seizures that require treatment, but PHENYTOIN IS AVOIDED due to its ability to increasd the frequency of ventricular arrhythmia.

Front 129

What are the major milestones that must be met by children in order for the child to become potty trained? When will a child demonstrate readiness for toilet training?

Back 129

The child must be aware of bladder filling, be able to consciously tighten the external sphincter, have normal bladder growth, and be motivated to stay dry. They must also have met motor milestones to be able to walk to the toilet, sit upright on the toilet, and the language to indicate the need to use the bathroom. The child will demonstrate readiness to begin toilet training when they communicate the need to urinate or defecate prior to passing urine or stol, and when they can withhold the urine or stool for a brief period of time.

Front 130

Newborn with history of jaundice in association with light colored stools, hepatomegaly, and direct (conjugated) hyperbilirubinemia is indicative of what neonatal cholestatic condition? What other conditions that are more urgent could present with this picture?

Back 130

Biliary atresia; in the real clinical setting priority should be given to conditions that require prompt diagnosis and treatment such as sepsis, endocrinopathy (hypothyroidism), and nutritional hepatotoxicity caused by metabolic diseases (galactosemi, tyrosinemia). After these diagnoses are ruled out, the final step is to differentiate biliary atresia from neonatal hepatitis.

Front 131

Conjugated hyperbilirubinemia greater than what level is an indication for evaluating neonatal jaundice?

Back 131

A level >2mg/dL

Front 132

What are the main 5 indications for the evaluation of neonatal jaundice?

Back 132

1. Jaundice that appears in the first 24-36 hours of life.
2. Serum bilirubin rising at a rate faster than 5 mg/dL/24hours
3. Serum bilirubin greater than 12 mg/dL in full term (especially in the absence of risk factors) or 10-14 mg/dL in preterm infants.
4. Jaundice that persists after 10-14 days of life.
5. The presence of signs or symptoms associated with the jaundice.

Front 133

At what age does breast milk jaundice occur? Is the jaundice associated with Breast-milk jaundice conjugated or unconjugated?

Back 133

Breast-milk jaundice appears in the second week of life and the hyperbilirubinemia that occurs is indirect (unconjugated). The unconjugated bilirubin level may rise as high s 10-30mg/dL. Levels drop rapidly as soon as breastfeeding is stopped, and the infant is given formula for 1-2 days. After 2-3 days the breast feeding can be resumed, and usually there is no return of the hyperbilirubinemia. Although it is a benign condition, in some instances, phototherapy may be indicated.

Front 134

How long does it take for physiologic jaundice of the newborn to subside? How high do te unconjugated bilirubin levels get? What demographics is it most common in?

Back 134

Physiologic jaundice consists of moderate unconugated hyperbilirubinemia (<12-14 mg/dL) that appears after the first 24 hrs. of life and resolves before the end of the first week. It is a benign condition and is more common in preterm infants, infants of dabetic mothers, and infants of Asian or Native American descent.

Front 135

What is the enzyme deficiency associated with Crigler-Najjar and Gilbert's syndrome. Is Crigler-Najjar a severe disease?

Back 135

Crigler-Najjar and Gilbert's syndrom are due to deficiencies in UDP-Glucuronyl Transferase that result in unconjugated hyperbilirubinemia. In Gilbert's syndrome, the deficiency is mild, and patients are asymptomatic due to the mild indirect hyperbilirubinemia. In Crigler-Najjar syndrome, the enzyme is ABSENT and patients present early in life, and die within their first few years.

Front 136

What are the infections, drug, medication and newborn illness contraindications to breast feeding?

Back 136

Newborns should not be breast fed by a mother who has herpes simplex lesions on the breast, active tuberculosis, or HIV. Maternal use of medications such as radioactive isotopes, antimetabolites, and chemotherapeutic agents is a contraindication to breast feeding (as is the maternal use of drugs of abuse). Finally, infants with certain inborn errors of metabolism such as galactosemia, phenylketonuria, and urea cycle defects, should not be breast fed.

Front 137

At what stage of sleep do night terrors occur in children? At what ages do night terrors occur?

Back 137

Night terrors occur during the non-REM stage of sleep. Night terrors occur in children between the ages of 2 to 12 with a peak incidence at 5-7 years old.

Front 138

A newborn male has oliguria and a midline mass in the lower abdomen. What is the most likely diagnosis?

Back 138

Posterior urethral valves; the infant has a congenital lesion that is causing him to pass only small volumes of urine. Posterior urethral valves are the most common congenital obstructive urethral lesion in males. This patient's midline lower abdominal mass probably represents a distended bladder and further supports this diagnosis.

Front 139

This is an abnormal fold in the distal prostatic urethra that obstructs urine flow out of the bladder. Affected infants may develop hydronephrosis, azotemia, and failure to thrive. What is the diagnosis and what is the diagnostic test of choice?

Back 139

This describes a Posterior Urethral Valve and a Voiding cystourethrogram (VCUG)is the diagnostic test of choice.

Front 140

What is the peak age range that we find Wilm's tumor in children?

Back 140

This is found at a peak between ages 2-5.

Front 141

This condition is a congenital anomaly where the penile urethra opens on the ventral surface of the penis rahter than at the tip. It results from incomplete fusion of the urethral folds on the ventral penis. Diagnosis?

Back 141

Hypospadius.

Front 142

The classical presentation of this condition is a male infant with a distended palpable bladder and abnormally low urine output. Diagnosis?

Back 142

Posterior Urethral valves.

Front 143

Nocturnal enuresis is due to a developmental disorder or maturational lag in bladder control while asleep. The condition is more common in boys and more than 20% of the cases spontaneously resolve before the patients are school age. For patients with persistent nocturnal enuresis what is the first drug of choice? What is second line medication?

Back 143

Desmopressin (DDAVP) is the first line therapy; Imipramine is a second line medication.

Front 144

What is a typical antipsychotic that is used to sedate children for conditions such as Tourette syndrome and schizophrenia?

Back 144

Haloperidol.

Front 145

What is the peak age at presentation for children with Kawasaki disease?

Back 145

18-24 months, with 80% of cases occurring in children less than 5-years old.

Front 146

A patient must have fever >39C for five days as well as 4 out of 5 defining criteria. What are these criteria?

Back 146

1. Extremity changes such as erythema, edema, or desquamation of the hands and feet
2. Conjunctivitis, which is usually BILATERAL and nonpurulent
3. A rash on the trunk
4. CERVICAL LYMPHADENOPATHY, which must be >1.5cm and is usually UNILATERAL
5. Oral changes, which can include erythema, fissured lips, or a strawberry tongue

Front 147

What supporting lab tests might you see along with a presumptive diagnosis of Kawasaki disease?

Back 147

Sterile pyuria, a C-reactive protein level >3, an ESR >40, an albumin level <3, and elevated ALT, thrombocytosis, and leukocytosis (>12,000). Other findings may include urethritis, orchitis, arthritis, or hepatitis. Induration and erythema of a previous BCG vaccination site can also occur.

Front 148

Similar symptoms of strawberry tongue, a red sandpaper-like rash over the trunk and extremities, and cervical lymphadenopathy are shared by Scarlet fever and Kawasaki disease. What are two distinguishing features?

Back 148

The fever of Scarlet fever has a shorter duration than Kawasaki disease (usually remits by day3-5) and the rash begins to fade by day 5-6.

Front 149

The rash associated with the skin infection is erythematous and is warm and tender to palpation. The rash is accentuated in the flexor creases and patients can develop bullae and exfoliation as well. Diagnosis?

Back 149

Staphylococcal scalded skin syndrome.

Front 150

This is a multi-organ system disease that is diagnosed based on clinical criteria. The patient presents with fever and a rash that can progress to desquamation of the hands and feet, systolic BP <90 mmHg, and involvement of at least three other organ systems (GI, mucous membranes, renal hepatic, central nervous system, or hematologic- specifically THROMBOCYTOPENIA). Diagnosis?

Back 150

Toxic Shock Syndrome.

Front 151

What is the percentage of all patients with Kawasaki disease that develop coronary artery aneurysm? What is the baseline test that all patients with Kawasaki disease should have done?

Back 151

About 10-15%; all patients with Kawasaki disease should have baseline and follow-up echocardiograms to rule out the presence of coronary artery aneurysms.

Front 152

What are the main treatments given for the management of Kawasaki disease?

Back 152

IVIgG and Aspirin. Aspirin is usually given at high doses (80-100mg/kg/day) initially and then decreased to a low dose (3-5mg/kg/day) for maintenance anti-platelet therapy for another 6-8 weeks or longer depending on echocardiogram results. Early treatment is associated with better outcome.

Front 153

A newborn has a rash that has appeared on his abdomen and chest; he appears otherwise healthy and has no fever or risk factors; there are erythematous papules and vesicles surrounded by patches of erythema; microscopic examination of the pustules reveals Eosinophils, which is diagnostic. Diagnosis?

Back 153

Erythema toxicum; this is a benign, self-limited condition usually found in newborns after the first 2 days of birth. Treatment is not necessary.

Front 154

Small pearly white cysts found on the face of a newborn. Diagnosis?

Back 154

Milia.

Front 155

A young boy with symptoms of increased intracranial pressure (e.g. headaches, vomiting), bitemporal hemianopsia, and a calcified lesion above the sella has what lesion until proven otherwise? How is diagnosis made?

Back 155

Craniopharyngioma; which is an epithelial remnant of Rathke's pouch. The diagnosis is usually made by computed tomography (CT) or magnetic resonance imaging (MRI). The presence of a cystic calcified parasellar lesion on MRI is almost diagnostic of Craniopharyngioma. Surgical removal is the treatment of choice.

Front 156

What are some of the associated endocrine symptoms that are found with Craniopharyngioma?

Back 156

Diabetes insipidus, growth failure associated with either hypothyroidism or growth hormons deficiency.

Front 157

At what age does a child develop a social smile (language) and At what age does a child begin to recognize parents (social)?

Back 157

2 months!!!

Front 158

At what age does a child begin to babble (language), sit up well unsupported (gross motor), have raking grasp (fine motor), and recognize strangers/have stranger anxiety (social)?

Back 158

6 months!!!

Front 159

At what age can a child roll back to front and front to back (gross motor)?

Back 159

4 months!!!

Front 160

At what age can a child say 2 words and obey 1-step commands (language), walk alone (gross motor), throw objects (fine motor), and imitate action/come when called (social)?

Back 160

12 months!!!

Front 161

At what age can a child hold their head up (gross motor)?

Back 161

3 months!!!

Front 162

At what age can a child build a tower of two blocks (fine motor)?

Back 162

15 months!!!

Front 163

At what age does a child speak in 2-3 word phrases/obey 2-step commands (language), walk up and down stairs without help (gross motor), build a tower of 6 blocks/turn pages of books (fine motor), parallel play (social)?

Back 163

24 months!!!

Front 164

This syndrome is characterized by an accessory pathway between the atrium and ventricle resulting in preexcitation and an increased risk for tachyarrythmias; affected patients can either be asymptomatic or present with chest pain, palpitations, syncope, or cardiac arrest. Diagnosis?

Back 164

This describe Wolff-Parkinson-White (WPW) syndrome in which an accessory pathway conducts antegrade from the atria to the ventricles faster than the conduction through the AV node, which allows part of the ventricle to depolarize early. This results in the classic electrocardiographic findings of a SLURRED INITIAL PORTION OF THE QRS (the delta wave), and a widened QRS complex.

Front 165

A slurred QRS (delta wave) defines what childhood cardiac arrhythmia syndrome?

Back 165

Wolff-Parkinson-White syndrome.

Front 166

Shortened PR interval, delta waves, and widening of QRS complex. Diagnosis?

Back 166

Wolff-Parkinson-White syndrome.

Front 167

In the pediatric population, CNS tumors are the most common solid tumor and the second most common malignancies (after leukemias). 60% are infratentorial tumors, 25% are supratentorial tumors, and 15% arise in the midline. What is the most common histology of the infra- and supratentorial groups?

Back 167

Benign astrocytomas are the most common histologic type of tumor in both groups.

Front 168

This tumor develops in the dorsal aspect of the midbrain; symptoms consist of endocrine syndrome, intracranial hypertension, Parinaud's sign (paralysis of vertical gaze), and Collier's sign (retraction of the eyelid). Diagnosis?

Back 168

Pinealoma.

Front 169

This disease is AUTOSOMAL DOMINANT; the classic example is the inability to release the hand after a handshake; the pathology is distinct in that all types of muscles (i.e. smooth, striated, cardiac) are involved; abnormalities of the endocrine, immunologic, and nervous systems can also occur; endocrine manifestations include diabetes mellitus, testicular atrophy, frontal baldness, and hypothyroidism. Diagnosis?

Back 169

Myotonic Muscular Dystrophy (Steinert disease); the second most common muscular dystrophy in the U.S.

Front 170

This is an X-linked recessive disorder characterized by a defect in tyrosine kinase in B cells; classically male patients are asymptomatic until 6-9 months of age, after which he begins to experience recurrent pyogenic (S.pneumoniae and HiB) infections. The maternal IgG antibodies are responsible for keeping the infant free from infections in the first 6-9 months; diagnosis is confirmed by decreased serum concentrations of total Ig's or markedly decreased B cells on the smear. Diagnosis?

Back 170

Bruton's agammaglobulinemia.

Front 171

Patients with this condition can have somewhat similar presentations to Bruton's agammaglobulinemia. This condition is distinguished, however, because pts. usually manifest with less severe symptoms at a later age (~15-35 years old); serum concentrations of total Ig may be decreased, but there is no absence or decrease in the number of circulating B cells; there is also no sexual predominance in this disease. Diagnosis?

Back 171

Common Variable Immunodeficiency (CVID) or Acquired hypogammaglobulinemia.

Front 172

This condition is characterized by decreased IgG levels, normal IgA levels, and variable IgM levels. These Ig levels all normalize by 6-11 months of age. Diagnosis?

Back 172

Transient Hyopgammaglobulinemia.

Front 173

This condition is characterized by decreased IgA levels (less than 50mcg/ml) with normal serum concentrations of other immunoglobulins (although IgG2 subclass deficiency may occur); there is no evidence of any immune defects; patients commonly present with respiratory, GI, and GU infections. Diagnosis?

Back 173

Selective IgA deficiency.

Front 174

What is first line treatment for an acute exacerbation of lung disease in a cystic fibrosis patient when trying to provide double antibiotic therapy for double coverage for Pseudomonas areuginosa? What is a single second line therapy?

Back 174

Treatment of this condition is rapid administration of empiric antibiotics, and the usual choice is a combination Ceftazidime or Penicillin derivative such as Ticarcillin + an Aminoglycoside such as Amikacin or Gentamicin. Imipenem/clastin is effective, but is used as a scond-line drug. Double coverage (combination abx) is important because of the remarkable resistance of this organism to most abx, and its ability to develop further resistance during the course of therapy.

Front 175

This is a disabling complication of sickle cell disease and is relatively common; it has also been described with other hemoglobinopathies, such as SC hemoglobin disease. Other non-traumatic causes include chronic corticosteroid therapy, SLE, alcoholism, and Gaucher's disease. Individuals have progressive hip pain that gradually limit activities; pts may have elevated temp (but AFEBRILE), with normal vitals; there will be NO LOCAL TENDERNESS, but restriction to ABDUCTION and INTERNAL ROTATION of the hip. Diagnosis?

Back 175

Aseptic Avascular Necrosis of the femoral head.

Front 176

What differentiates Osteomyelitis and Avascular Necrosis of the femoral head pt presentations?

Back 176

Osteomyelitis is usually accompanied by HIGH FEVER, and typically has an ACUTE ONSET.

Front 177

What differentiates joint effusion in the hip joint versus avascular necrosis of the femoral head?

Back 177

A joint effusion usually has characteristic LOCAL WARMTH AND TENDERNESS.

Front 178

What differentiates septic arthritis and avascular necrosis of the femoral head?

Back 178

Septic arthritis has fever, SEVERE RESTRICTION OF MOVEMENT IN ALL DIRECTIONS, inflammatory signs, and bacteremia.

Front 179

A newborn infant has paralysis of the left hand and ipsilateral Horner syndrome (miosis and ptosis). These features result from injury to C7, C8, T1, which is a rare form of brachial palsy. Diagnosis? How is diagnosis confirmed? Treatment?

Back 179

Klumpke paralysis; this diagnosis is confirmed with MRI, which demonstrates nerve root avulsion or rupture. If by three to six months there is no improvement, neuroplasty, neurolysis, end-to-end anastomosis, and nerve grafting may be attempted.

Front 180

A newborn infant presents with an absent Moro reflex, but intact grasp reflex of the affected arm. Patients present with a characteristic postion, which consists of adduction and internal rotation of the arm with pronation of the forearm (waiter's tip). Diagnosis? Nerves injured?

Back 180

Erb-Duchenne palsy; C5, C6 injury.

Front 181

An infant presents with irregular and difficult respiration, and an abdomen that does not bulge with breathing. Diagnosis?

Back 181

Phrenic nerve injury involving C3,4,5.

Front 182

The presence of 46 XY chromosomal profile in a patient with this syndrome is associated with higher risk of gonadoblastoma. Diagnosis?

Back 182

Turner Syndrome (chromosomal variation)

Front 183

What is the primary amenorrhea seen in Turner syndrome due to?

Back 183

This is due to ovarian failure.

Front 184

When a Turner's patient height falls below the 5th percentile, what is the method of treatment offered and for how long?

Back 184

In this case the patient can be placed on Growth Hormone, and therapy is continued until the bone age is greater than 15 years, and the growth rate falls to less than 2 cm.

Front 185

Anti-Smith and anti-dsDNA antibodies are the confirmatory tests for what disease?

Back 185

SLE

Front 186

A patient presents with Parinaud's syndrome (paralysis of VERTICAL gaze that may be associated with pupillary disturbances and eyelid retraction (Collier's sign)). The involved lesion is likely on the rostral midbrain at the level of the superior colliculus and CN III. Endocrine syndrome can result from interruption of hypothalamic inhibiting pathways, and sometimes beta-hCG secretion and consequent Leydig's cell stimulation (precocious pubertal signs result). What are the two most likely tumors to cause this?

Back 186

Pinealomas and Germinomas.

Front 187

What tumor is the third most common malignancy in the pediatric population (Hint: it arises most frequently from the adrenal glands and sympathetic paraspinal ganglia)?

Back 187

Neuroblastoma.

Front 188

Children with HSP are susceptible to what GI complications?

Back 188

Intussusception and GI bleeding.

Front 189

A child presents with peripheral edema and scrotal swelling; hematuria and proteinuria are present; patients can also have abdominal pain with a large amount of blood in the stool, which is indicative of complications of this condition. Diagnosis?

Back 189

Henoch-Schonlein pupura; this patient likely had palpable purpura on the legs and buttocks and the blood in the stool could be indicative of a GI bleed or intussusception.

Front 190

What is the only condition in children that has an association with Cholecystitis (normally rare in children)?

Back 190

Sickle Cell disease.

Front 191

When giving intravenous boluses for the purpose of fluid resuscitation what are the only two fluids that you should use?

Back 191

Normal saline or Lactated Ringer's solution.

Front 192

What is the reason why hypotonic solutions such as Half Normal Saline and 5% Dextrose should never be used for initial fluid resuscitation?

Back 192

This is because as hypotonic solutions they will quickly exit the intravascular system and lower the sodium too rapidly. Precipitous drops in sodium levels can cause CEREBRAL EDEMA!!!

Front 193

How is Congenital Adrenal Hyperplasia associated with 21-hydroxlase deficiency diagnosed and confirmed?

Back 193

The diagnosis is suggested by increased levels of 17-alpha-hydroxyprogesterone (serum), and diagnosis is confirmed with ACTH stimulation test.

Front 194

An infant presents with signs of meningitis and a petechial rash that is prominent on the axilla, wrists, flanks, and ankles. Diagnosis?

Back 194

Meningococcemia.

Front 195

This is a component of multivitamins that, when ingested in large amounts, has a corrosive effect on the GI mucosa, leading to abdominal pain, nausea, vomiting, diarrhea and hematemesis. This compound accumulates in the mitochondria and tissue, thus impairing energy production and resulting in cellular damage and toxicity. Hypotension then occurs due to increased capillary permeability and venodilation. The resulting peripheral hypoperfusion and mitochondrial damage lead to accumulation of lactic and citric acid, and consequently metabolic acidosis. Other complications include liver necrosis, coagulopathy, drowsiness, seizures and coma. Diagnosis? Treatment?

Back 195

Iron poisoning; treatment depends on level of severity, but Ipecac syrup can be used to induce emesis in asymptomatic patients with an intact gag reflex. Intravenous deferoxamine, an iron chelator is used in moderate to severe cases of intoxication.

Front 196

Acute ingestion of this vitamin causes anorexia, bulging fontanelle in infants (pseudotumor cerebri), hyperirritability, and vomiting. Diagnosis?

Back 196

Vitamin A overdose.

Front 197

Acute ingestion of this vitamin causes anorexia, nausea, vomiting, diarrhea, headache, polyuria and polydypsia. Diagnosis?

Back 197

Acute Vitamin D ingestion.

Front 198

This vitamin may cause hyperbilirubinemia in infants.

Back 198

Vitamin K.

Front 199

Symptoms of this condition in infants include poor feeding, irritability, vomiting, and decreased activity. On examination, the fontanelle may be tense and bulging, the scalp veins may appear prominent with shiny, tight skin overlying the vessels, and the cranial sutures may be widely spaced; head growth is at 97th percentile. Diagnosis? First step in evaluation?

Back 199

Hydrocephalus; a CT scan of the brain is the best initial choice to evaluate for intracranial pathology and will reveal dilated ventricles.

Front 200

What is the main treatment for an infant diagnosed with Hydrocephalus?

Back 200

Treatment consists of a shunt that is placed from the venticle to the peritoneum, pleura, or right atrium, which allows the excess CSF to drain rather than continue to collect in the ventricles.

Front 201

Define Todd's paralysis.

Back 201

Todd's paralysis represents a POSTICTAL (HAPPENS AFTER SEIZURES) condition that usually rapidly improves with restoration of motor function within 24 hours.

Front 202

At what age does a child begin to play with other children?

Back 202

18 months!!!

Front 203

What are the three most common organisms to cause pulmonary infections in patients with Cystic Fibrosis?

Back 203

Haemophilus, Pseudomonas, and Staphylococcus.

Front 204

Severely neutropenic patients develop pneumonia due to what three main organisms?

Back 204

Staphylococcus, Psuedomonas, and Aspergillus.

Front 205

What two acid-fast organisms are common sources of infection for patients that receive chronic glucocorticoid therapy?

Back 205

Nocardia and M.tuberculosis.

Front 206

CMV causes pneumonia in patients with deficient cell mediated immunity and a CD4+ count less than what level?

Back 206

CD4+ count <50 micoL

Front 207

Mycobacterium avium intracellulare causes fever in HIV patients, epecially when the CD4 count is less than what number?

Back 207

<50 microL

Front 208

Systemic Carnitine Deficiency (SCD) is an inherited defect in fatty acid oxidation that can present with hypoglycemia, hyperammonemia, hypoprothrombinemia, and acute episodes of encephalopathy, thus making it look similar in presentation to Reye Syndrome. What is the main distinguishing factor of SCD?

Back 208

Elevated Acyl-carnitine levels.

Front 209

By what age do patients with sickle cell disease develop functional asplenia?

Back 209

2-3 years of age.

Front 210

What is the most common cause of sepsis in functionally asplenic patients?

Back 210

S.pneumoniae.

Front 211

Patients with this congenital infection are usually born asymptomatic. Those with early symptoms present with hepatosplenomegaly, jaundice, anemia, and rhinorrhea. Radiographs demonstrate metaphyseal dystrophy and periostitis. Late congenital manifestations (presenting after two years of age) include frontal bossing, high arched palate, interstitial keratitis, perioral fissures, saddle nose, and notched teeth. Diagnosis?

Back 211

Congenital Syphilis.

Front 212

What are the initial screening tests used for congenital syphilis? What are the confirmatory tests?

Back 212

The initial screen is with nontreponemal tests such as the venereal disease research laboratory (VDRL) test, rapid plasma reagin (RPR) test, or enzyme immunoassay (EIA) test; confirmatory testing is with treponemal tests such as the fluorescent treponemal antibody absorption (FTA-ABS) test, or the treponema pallidum particle agglutination assay (TPPA).

Front 213

What is the treatment of choice for syphilis?

Back 213

Parenteral penicillin G.

Front 214

Hepatosplenomagaly, hydrocephalus, chorioretinits, and intracranial calcifications are major clinical findings in what congenital infection?

Back 214

Toxoplasmosis.

Front 215

Sensorinerual hearing loss, cataracts, heart defects, hepatosplenomegaly, microcephaly, and thrrombocytopenic purpura (commonly described as a "blueberry muffin" rash are all symptomatic of what congenital infection?

Back 215

Congenital Rubella.

Front 216

Intrauterine growth retardation, hepatosplenomegaly, petechiae or purpura, microcephaly, chorioretinitis, sensorinueral hearing loss, and periventricular calcifications describes the constellation of symptoms associated with what congenital infection?

Back 216

Congenital CMV!!!

Front 217

How long after birth does it commonly take for Tetralogy of Fallot to present with Cyanosis?

Back 217

Tetralogy of Fallot commonly presents with cyanosis a few years after birth.

Front 218

What is the most common cyanotic heart disease which presents with cyanosis in the first 24hrs of life? Infants of mothers with what condition have a higher incidence of this?

Back 218

Transposition of the great vessels is the most common cyanotic heart disease that presents with cyanosis in the first 24 hours of life. It is commonly seen in infants of diabetic mothers and in males.

Front 219

This disease is associated with joint hypermobility, hearing loss, defects in tooth formation, and blue sclera. Diagnosis?

Back 219

Osteogenesis imperfecta.

Front 220

Congenital heart disease, head trauma, infections of the jaw or mouth, infections of the face or scalp, meiningitis, and cranial instrumentation put pediatric patients at increased risk for what infectious CNS complication?

Back 220

BRAIN ABSCESS!!!

Front 221

This condition is characterized by fever, toxicity, pharyngitis, SANDPAPER-LIKE rash, circumoral pallor and strawberry tongue; Penicillin V is the drug of choice to treat. Diagnosis?

Back 221

Scarlet fever.

Front 222

Hirschprung's disease, esophageal atresia, pyloric stenosis, and malrotation of the bowel are all congenital GI malformations that can be seen in the setting of what genetic syndrome?

Back 222

Down Syndrome!!!

Front 223

What is the most appropriate way to limit the risk of infection of Bordetella pertussis in household contacts?

Back 223

Prescribe Erythromycin for 14 days to all household contacts. In addition to this prophylaxis, close contacts should receive the pertussis vaccination according to the recommended immunization schedule if they are not already fully immunized. Only treatment during the catarral stage of transmission will help shorten the disease duration. Treatment after this stage will only help decrease transmission.

Front 224

What is the initial step in management of children with speech delay?

Back 224

Audiology evaluation.

Front 225

At what age do infants regard faces, follow items to the midline, begin to vocalize, respond to a bell and lift their head from te examining table?

Back 225

1 month.

Front 226

At what age should a child be able to smile and coo when smiled at or talked to?

Back 226

2 months.

Front 227

At what age would an infant be expected to follow a moving toy from side to side and also in the vertical plane?

Back 227

3 months

Front 228

By what age should a child's language be fully understandable and the child be able to name four colors, define five words, and know three adjectives?

Back 228

4 years.

Front 229

By what age should a child begin to be able to copy a square?

Back 229

4-5 years.

Front 230

How long should a 4 and 5 year old be able to balance on one leg respectively?

Back 230

4 year old; 2 seconds
5 year old; 5 seconds

Front 231

By what age should a child be able to independently dress themselves?

Back 231

5 years old.

Front 232

Chronic uveitis resulting in blindness, is a major morbidity associated with what type of juvenile rheumatoid arthritis? What type of examination screens for this condition?

Back 232

Pauciarticular rheumatoid arthritis; because chronic uveitis can require treatment with local or systemic steroids and develop without signs or symptoms, it is recommended that all children with this form of arthritis have frequent SLIT-LAMP eye examinations.

Front 233

This rash is preceeded by fever, irritability, erythema, extraordinary tenderness of the skin, and is most commonly seen in children under 5 years of age. Circumoral erythema; crusting of the eyes, nose and mouth; blisters on the skin can develop; intraoral mucosal surfaces are not affected; peeling of the epidermis in response to mild sheer forces leaves the patient susceptible to problems similar to those of a burn injury, including infection and fluid and electrolyte imbalance; cultures of bullae are negative, but the source site or blood may often be positive. Diagnosis?

Back 233

Staph scalded skin syndrome.

Front 234

Most older children with and adults present with intensely pruritic and threadlike burrows in the interdigital areas, groin, elbows, and ankles; the palms, soles, face and head are spared in adults and are often involved in infants; the clinical manifestations closely resemble those of atopic dermatitis. Diagnosis?

Back 234

Scabies.

Front 235

What is the main treatment for scabies? Which treatment is not recommended for children under 2 months of age?

Back 235

Gamma benzene hexachloride (Lindane) can cause neurotoxicity through percutaneous absorption, especially in small infants and those with abnormal skin (impetigo, etc.) and is therefore, not recommended in children as first-line therapy for scabies. An excellent alternative is 5% Permethrin cream (Elimite), which is safer and is more often recommended.

Front 236

Persistence of a transient or fixed deviation of an eye beyond what age, requires referral to an opthalmologist?

Back 236

Pesistence beyond 4 months of age. Prognosis for normal vision if diagnosis is delayed is guarded. Routine vision and strabismus screening are essential at ages 3-4 years.

Front 237

At what temperature should the hot water heater be set in order to avoid accidental scald burns in children?

Back 237

49C, 120F

Front 238

What are the recommendations for putting a child in a rear facing car-seat, interms of age/weight?

Back 238

Rear facing infant seats are recommended for a child less than 20 lbs and 1 year of age with the switch to a forward-facing seat to occur between 20-40 lbs and older than 1 year

Front 239

What type of HIV lab test is ordered for a newborn infant in order to screen that child for HIV?

Back 239

An HIV ELISA may still reflect maternal antibody at this age; thus, an antigen test like the HIV PCR is preferred.

Front 240

This rash presents initially as an erythematous, papulovesicular, weeping eruption, which progresses over time to a scaly lichenified dermatitis. From about 3 months to 2 years of age, the rash is prominent on the cheeks, wrists, scalp, posauricular areas, and arms and legs; in a young child 2 to 12 years of age, mainly the extensor surfaces of arms, legs, neck are involved; pruritus is a predominant feature and the rash as a chronic and relapsing course. Diagnosis?

Back 240

Eczema.

Front 241

This presents as a discrete whitish polyp that extends through the tympanic membrane in a child with a history of Otitis Media; this lesion can be congenital or acquired, and is a small sac lined with epithelium-containing debris; these masses can grow aggressively, leading to CNS complications like facial nerve damage, hearing loss, and intracranial extension; a CT scan of the temporal bones can define the extent of the disease and a referral to and otolaryngologist is required. Most likely diagnosis?

Back 241

Cholesteatoma.

Front 242

How long after birth does the umbilical cord typically separate from the infant?

Back 242

10-14 days after birth, although some will remain for 3 weeks; an intact cord after 1 month of age is considered "delayed separation".

Front 243

How is the diagnosis of Leukocyte Adhesion Deficiency made?

Back 243

Diagnosis is made by measuring surface CD11b using flow cytometry.

Front 244

How often are booster vaccines given to prevent diphtheria, tentanus, and pertussis?

Back 244

It used to be just a Td vaccine which was given every 10 years to maintain immunity against both diphtheria and tentanus, however, recognition of an increased incidence of pertussis in adolescents and young adults (and this group's serving as a reservoir for disease in infants) has lead to a vaccine that incorporates immunity also against pertussis. Current recommendations are for the new Tdap to replace to Td to help eliminate pertussis. This is given in a booster every 10 years.

Front 245

What is the most common organism seen in infected Cat and Dog bites?

Back 245

Pasturella multocida.

Front 246

What are the most common organisms seen in infected human bites?

Back 246

S.viridans, S.aureus, and Bacteroides spp.

Front 247

A child shows signs of medication intoxication; the three year old boy is lethargic, has miosis, bradycardia, and hypotension; he develops apnea, respiratory depression, and has to be intubated. Diagnosis? Tx?

Back 247

This medication could be Clonidine; treatment includes aggressive PICU support and Naloxone (which has variable effect)

Front 248

What is the difference in classical findings of heat stroke in elderly, or average adult patients, and those findings of a young athlete with heat stroke? Tx?

Back 248

Hyperthermia with dry, hot skin and mental status changes characterize classic heat stroke, typically seen in the elderly with gradual onset. Young athletes exerting themselves in a hot environment may develop acute heat stroke with signs and symptoms of hyperthermia and mental status changes; THE DIFFERENCE, however, is that the ATHLETES WILL CONTINUE TO SWEAT PROFUSELY; Heat stroke is a medical emergency and patients should be treated with IV fluids, agressive cooling, and perhaps O2, lab evaluation and ICU admission.

Front 249

How does inappropriate dilution of an infant's formula over time lead seizure activity? How is this treated?

Back 249

Over time, inappropriate dilution can cause hyponatremia and water intoxication, leading to seizure activity. The technique used to corect the patient's hyponatremia depends on the etiology. Chronic hyponatremia may be treated rapidly with 3% NaCl to rapidly increase the serum sodium and theoretically to decrease cerebral edema. Otherwise, starting the child on a regular formula and eliminating excess water will correct the deficiency over time.

Front 250

1 week old child; transient rash with splotchy areas of erythema with a central clear pustule; benign self-limited condition found in about 50% of newborns; lesions are yellow-white and 1 to 2mm in size with surrounding edge of erythema; this rash waxes and wanes over the first few days to weeks of life; microscopic examination of pustules reveals eosinophils; Diagnosis?

Back 250

Erythema toxicum.

Front 251

Adolescent boy complains of splotchy red rash on the nape of his neck discovered when he had his head shaved for football season; rash becomes more prominent with exercise or emotion; mother notes he's had it since infancy, but hair grew over it; it was also on his face and eyelids. Diagnosis?

Back 251

Salmon patch (aka Nevus simplex or Nevus flamus)

Front 252

African American newborn is thought to have a bacterial skin infection; areas in question have many scattered pustules full of milky fluid, upon examining pustules, they easily wipe away, revealing a small hyperpigmented macule. Diagnosis?

Back 252

Pustular melanosis.

Front 253

An area of a newborn's scalp seems to have no hair and is scaly can yellowish; this is a sharply demarcated lesion that occurs most commonly on the head and neck of infants. Diagnosis?

Back 253

Sebaceous nevi (Nevus of Jadassohn).

Front 254

These are fine yellowish whit 1-2 mm lesions scattered over the face and gingivae of the neonate (when found on the mucosa they are called Epstein pearls); they are cysts that contain keratinized material; commonly these lesions resolve spontaneously without therapy. Diagnosis?

Back 254

Milia.

Front 255

This rash commonly presents as cradle cap in the newborn; the rash is greasy, scaly, and erythematous and in smaller children involves the face, neck, axilla, and diaper area; in older children the rash can be localized to the scalp, and intertriginous areas; pruritus can be marked. Diagnosis?

Back 255

Seborrheic dermatitis.

Front 256

This occurs from cold exposure and is manifest by small, firm, white, cold patches of skin in exposed areas; there can be blistering and peeling of exposed areas ; the lesions generally start as firm cold white areas that begin with stinging, but then become more sensitive than the surrounding skin; treatment is rewarming the areas before they become numb. Diagnosis?

Back 256

Frostnip.

Front 257

These are small, ulcerated lesions on exposed areas of skin such as the ears and fingertips, to a very cold environment; lesions may last 1-2 weeks and are painful and itchy. Diagnosis?

Back 257

Chilblains

Front 258

This can occur from extended cold exposure to something as benign as a popsicle; there may be areas of erythematous, firm, and slightly swollen skin at the corner of the mouth, without tenderness. Diagnosis?

Back 258

Cold panniculitis.

Front 259

Prolonged cold/moisture exposure can cause the foot to become cold, numb, pale, edematous, and clammy; prolonged autonomic disturbance after this condition can persist for years. Diagnosis?

Back 259

Trenchfoot.

Front 260

In this condition of prolonged cold exposure tissue initially begins stinging, followed by aching , culminating in numb areas; once rewarmed the areas become blotchy and painful; tissue has been frozen and destroyed and gangrene develops. Diagnosis?

Back 260

Frostbite.

Front 261

Photophobia, blurred vision, burning and itching of eyes, poor growth and cheilosis are all symptoms associated with what vitamin deficiency?

Back 261

Riboflavin deficiency.

Front 262

Irritability, convulsions, and hypochromic, microcytic anemia are all symptoms of what vitamin deficiency?

Back 262

Vitamin B6 (pyridoxine) deficiency.

Front 263

Megaloblastic anemia, glossitis, pharyngeal ulcers, and impaired immunity are all symptoms of what vitamin deficiency?

Back 263

Folate deficiency.

Front 264

What medications can reduce Vitamin B6 levels?

Back 264

Isoniazid, Penicillamine, corticosteroids, and anticonvulsants.

Front 265

Which trisomy syndrome is associated with microcephaly, a sloping forehead, cutis aplasia on the scalp, micropthalmia, and cleft lip and palate; echocardiogram may demonstrate a complex heart lesionin including ASD, VSD, and/or dextrocardia.

Back 265

Trisomy 13.

Front 266

This syndrome is known as "happy puppet syndrome" because of the unusual gait and the unprovoked outbursts of laughter; in most cases it is caused by an interstitial deletion of chromosome 15q1-q13; the deletion always comes from the maternal side; the child may also have a large mouth with tongue protrusion, hypopigmentation with blonde hair and pale blue eyes. Diagnosis?

Back 266

Angelman syndrome.

Front 267

This syndrome occurs with profound mental retardation, self-injury behavior, hypersensitivity to sound, and repetitive behaviors are commonly seen; deletion of the short arm of chromosome 5 is the etiology; about 85% of cases are of paternal origin; there is a distinctive cry associated. Diagnosis?

Back 267

Cri du chat syndrome.

Front 268

This is an association of commonly seen findings of unknown etiology: (V)ertebral defects, (A)nal atresia, (C)ardiac defects, (T)racheoesophageal fistula, (R)enal/(R)adial defects (or both), and (L)imb abnormalities. Intelligence is normal. Diagnosis?

Back 268

VACTERL syndrome.

Front 269

Features of this syndrome include bushy eyebrows, hirsutism, limb defects, VSD and mental retardation. Diagnosis?

Back 269

Conelia de Lange.

Front 270

This disorder has an unknown etiology and is most common among obese boys with delayed skeletal maturation or in thin, tall adolescents having recently had a growth spurt; onset of this disorder is frequently gradual; PAIN REFERRED TO THE KNEE in 20% of cases can mask the hip pathology. Diagnosis?

Back 270

Sipped capital femoral epiphysis.

Front 271

Presenting symptoms include a limp and pain in the anterior thigh, groin, or knee, although classic symptoms include a painless limp; boys between the ages of 2 and 12 years are most frequently affected. Diagnosis?

Back 271

Legg-Calve-Perthes disease (idiopathic avascular necrosis of the femoral head).

Front 272

This condition affects children usually from 2-6 years of age; these children usually present with a painful limp; this is a diagnosis of exclusion as septic hip arthritis and osteomyelitis must be ruled out; WBC and ESR may be normal, but may also be slightly eleveated; early aspiration of the joint space may aid in diagnosis; this can be common after a URI. Diagnosis?

Back 272

Transient synovitis, which is a self-limiting disorder.

Front 273

A 2 year old refuses to walk, has fever, has significant pain with EXTERNAL ROTATION of the right leg, and has an elevated WBC count; this disease usually presents with focal bone tenderness and fever. Diagnosis?

Back 273

Osteomyelitis.

Front 274

This drug can cause an idiosyncratic reaction causeing extrapyramidal symptoms such as oculogyric crisis, tremors, torticolis, opisthotonus, and dysphagia. What is the toxic compound that is found in various antipsychotics that is responsible for this? Tx?

Back 274

These are a constellation of dystonic symptoms that look like Phenalthiazine overdose; this responds surprisingly quickly to intravenous, or intramuscular administration of diphenhydramine (Benadryl).

Front 275

Symptoms of overdose with this compound occurs in two phases: GI symptoms such as bloody vomiting or diarrhea and abdominal pain, followed by a latent period of up to 12 hours or more and terminating with vascular collapse. Diagnosis? Tx?

Back 275

Iron poisoning; deferoxamine given IV or IM, is used to complex with the iron and excrete it in the urine.

Front 276

Hypervitaminosis of this vitamin slows normal growth and causes hyperostosis (excess bone growth), hepatomegaly, increased CSF pressure, and drying of skin. Diagnosis?

Back 276

Hypervitaminosis A.

Front 277

Hypervitaminosis of this vitamin can cause skin flushing and pruritis; long-term use can cause tachycardia, liver damage, hyperglycemia, and hyperuricemia. Diagnosis?

Back 277

Hypervitaminosis B3 (Niacin/Nicotinic acid).

Front 278

Excessive doses of this vitamin can cause renal stones, diarrhea and cramps, and also an increase in the normal requirements for this vitamin once large doses are discontinued. Diagnosis?

Back 278

Hypervitaminosis C.

Front 279

Prolonged and excessive intake of this vitamin causes nausea, diarrhea, weight loss, polyuria, and soft-tissue calcification of the heart, kidney, blood vessel, bronchi, and stomach. Diagnosis?

Back 279

Hypervitaminosis D.

Front 280

Excessive intake of this vitamin can result in sensory neuropathy with altered sensation of touch, pain, and fever. Diagnosis?

Back 280

Hypervitaminosis B6 (pyridoxine).

Front 281

Taking this medication during pregnancy can result in fetal death, hydrocephalus, CNS defects, micronotia/anotia, small or missing thymus, conotruncal heart defects, and micrognathia. Diagnosis?

Back 281

Vitamin A (or retanoic acid) during pregnancy.

Front 282

The use of this mood stabilizing drug is associated with Ebstein anomaly at birth. Diagnosis?

Back 282

Lithium.

Front 283

Renal dysgenesis, oligohydramnios, and skull ossification defect are all teratogenic effects of what antihypertensive drug?

Back 283

ACE inhibitors.

Front 284

Infants with this congenital infection can be born with deafness, CATARACTS, mental retardation, and heart defects. Diagnosis?

Back 284

Congenital Rubella.

Front 285

What is the main cause of cutaneous larva migrans? Where does it typically happen (location given in the question stem)? Tx?

Back 285

Cutaneous larvae migrans is most commonly due to dog or cat hookworms; after exposure, such as walking barefoot on the BEACH in Cental/South America), The larvae penetrate the skin at the dermal/epidermal junction and create and intensely pruritic, serpiginous lesion. Left untreated the larvae will die, but treatment with an antihelminthic hastens the resolution.

Front 286

Chronic seborrheic dermatitis in an infant associated with FTT could potentially be signs of what deadly disease?

Back 286

Histiocytosis X.

Front 287

Rashes, thrombocytopenia, and congenital heart block are among the more common problems found in newborn infants whose mothers had this condition. Whereas the other symptoms usually resolve in the first months of life, the congenital heart block can be irreversible and can result in heart failure, need for early pacemakers and an increased incidence of early death. Diagnosis of the mother?

Back 287

The mother has SLE; more common with mothers that have antibodies to Ro/SSA and some with antibodies to La/SSB.

Front 288

Vitamin A supplementation has been found to be effective in preventing morbidity and mortality associated with what viral infection?

Back 288

Vitamin A.

Front 289

What condition must be considered in an infant with atopic dermatitis, thrombocytopenia, and unusual infections? What test is done to confirm?

Back 289

This history raises suspicion for Wiskott-Aldrich syndrome; the syndrome is unlikely if platelet counts of the infant are normal, so a platelet count is a good initial test to do

Front 290

What immunologic testing should be done in patient with suspected DiGeorge syndrome?

Back 290

Candida skin testing is unlikely to be positive in a patient with T-cell deficiencies such as a child with DiGeorge.

Front 291

In a pediatric patient thought to have B-cell defects, which result in low antibody production, what is the first test that should be done to evaluate the patient?

Back 291

Antiglobulin class serum levels!!!

Front 292

Why can neonatal sepsis cause increased neurologic problems in a newborn with jaundice?

Back 292

This is because sepsis interrupts the blood brain barrier, and allows more bilirubin to cross.

Front 293

When attempted to feed a newborn's oxygen saturation levels are found to be in the 60s. When he is stimulated and cries, his oxygen levels increase to the 90s. Infants with this condition has trouble breathing while they are feeding and sleeping. Diagnosis? and what test is most important to establish the diagnosis?

Back 293

This is likely Choanal atresia; passage of a catheter into the nose will quickly establish whether the nasal passages are block or not.

Front 294

When should a newborn infant receive Varicella-zoster immunoglobulin given the mother's prior exposure?

Back 294

VZIG should be administered to the infant immediately after delivery if the mother had the onset of varicella within 5 DAYS PRIOR to delivery, and immediately upon diagnosis if her chicken pox started within 2 DAYS AFTER delivery. If untreated, about 50% of these infants will develop serious varicella as early as 1 day of age.

Front 295

If a normal full term infant is exposed to varicella 2 days or more postnatally, what steps should be taken?

Back 295

VZIG and isolation are not necessary because these babies appear at no greater risk for complications than older children. Normal newborn care should be given. Acyclovir may be used in infants at risk for severe varicella such as those exposed perinatally.

Front 296

What is the first test that should be done if a newborn infant appearing to be in good health, passes a bloody meconium stool, or has hematemesis?

Back 296

Apt test!!!; this test differentiates maternal hemoglobin from fetal hemoglobin based on the reaction of the hemoglobin to alkali exposure. Hematemesis and melena are not uncommon in the neonatal period, especially if gross placental bleeding occurred during the time of delivery.

Front 297

What minerals is breast milk lacking in sufficient quantities in order to provide the catch-up growth necessary for a low-birth weight infant?

Back 297

Calcium and phosphorus.

Front 298

A neonate born preterm that had been feeding well, but suddenly started vomiting feedings, seems less active, and has grossly bloody stools with abdominal distention, is suspicious for possible NEC. What are the next steps that should be taken in evaluation?

Back 298

If an abdominal radiograph show distended loops of bowel with air in the bowel wall (pneumotosis intestinalis) and no perforation is suspected, the infant should be put on bowel rest with nasogastric decompression, and systemic antibiotics should be initiated. Electrolytes and vital signs are monitored closely, and serial abdominal films should be ordered to evaluate abdominal perforation. Only if free air is identified on plain radiographs or if the infant clinically worsens with medical management will surgical consultation be required.

Front 299

Why would a newborn's respiratory rate go up if it's body temperature drops?

Back 299

This occurs because newborn infants do a very poor job of regulating their body temperatures; they are wet, have a large surface area to weight ratio, and have very little subcutaneous fat. Thus in order to raise their body temperature they must increase their metabolic rate, and if the metabolic rate increases they must increase their RR in order to provide adequate O2 tissues with increased demands. Because a preterm infant is likely to have respiratory difficulty and be unable to oxygenate adequately, lactate can accumulate and lead to metabolic acidosis.

Front 300

What are the particular set of complications that a small-for-date infant faces as a opposed to a preterm infant?

Back 300

A small-for-date infant has a higher incidence of major congenital anomalies and is at increased risk for future growth retardation, especially if length, head circumference as well as weight are small for gestational age. Also more common is neonatal aspyxia and the meconium aspiration syndrome, which can lead to pneumothorax, pneumomediastinum or pulmonary hemorrhage. Neonatal symptomatic hypoglycemia is also more commonly found in small-for-date infants.

Front 301

What is thought to be the main cause of apneic episodes (absence of respirations for longer than 20 sec) in a newborn (especially one that is premature)? How is this differentiated from Periodic Breathing?

Back 301

Apneic episodes (20 seconds or greater) are thought to be due to immaturity of the respiratory center in the brain; Periodic breathing, a common respiratory pattern in low birth weight babies, is characterized by recurrent breathing pauses of 3 to 10 seconds.

Front 302

In the newborn, respiratory failure in the first hours of life, a scaphoid abdomen, and the presence of bowel sounds in the chest are all indicative of what condition?

Back 302

Congenital diaphragmatic hernia.

Front 303

Why can the administration of Propranolol to a pregnant woman during her pregnancy affect the health of a newborn at the time of delivery?

Back 303

This can happen because Propranolol may cause growth retardation when given throughout pregnancy; this diminishes the ability of an asphyxiated infant to increase heart rate and cardiac output during birth. It has also been associated with hypoglycemia and apnea.

Front 304

This type of neonatal jaundice becomes apparent on the second or third day of life, peaks to levels no higher than about 12mg/dL on the fourth or fifth day, and disappears by the end of the week. The rate of rise is LESS THAN 5 mg/dL per 24 hours and levels of conjugated bilirubin do not exceed about 1 mg/dL. Diagnosis?

Back 304

Physiologic jaundice of the newborn.

Front 305

Jaundice appearing in the FIRST 24 HOURS of life in a neonate is usually the result of what type of process?

Back 305

A hemolytic process!!!

Front 306

How often does bilateral cleft lip and palate occur in the siblings of affected infants?

Back 306

4%; the incidence in the general population is 1 in 1000

Front 307

When are cleft lips repaired? Cleft palates? What are the most likely complications to occur despite the closure of the palates?

Back 307

Repair of the cleft lip usually occurs at 2-3 months; repair of the cleft palate is repaired later usually between the ages of 6 months-5 years. Complications include recurrent otitis media and hearing loss, as well as speech defects, which may be present despite good anatomic closure.

Front 308

2 week old infant sleeps most of the day and has to be awakened for feeding; will only take ~1 oz of formula at a time; infant has persistently hard, pellet-like stools; infant has large head, low HR, and low temp; child is still jaundiced; large fontanelles, distended abdomen, umbilical hernia. Diagnosis?

Back 308

Congenital Hypothyroidism.

Front 309

A black child with fifth-finger polydactyly should raise no immediate concern especially if there is a family history, as this can be a common finding. If the finding is made in a white child, what organ system should be examined for anomaly?

Back 309

The Cardiac system should be examined for congenital defects; thumb-sided polydactyly also warrants examination for congenital defects.

Front 310

Neonatal withdrawal from this drug is characterized by onset in the first 2 days of life, hyperirritability and coarse tremors, along with vomiting, diarrhea, fever, high-pitched cry, and hyperventilation; seizures, and respiratory depression are less common. The production of surfactant can actually be elevated in this newborn. Diagnosis?

Back 310

Heroin withdrawal.

Front 311

In utero exposure to this drug leads to growth retardation, microcephaly, flat philtrum, thin upper lip, cardiac defects, and hypoplastic fifth fingernails. Diagnosis?

Back 311

Fetal Alchohol Syndrome.

Front 312

What is a differential of apnea in a full-term newborn?

Back 312

Sepsis, GERD, Congenital heart disease, seizure, hypoglycemia, airway obstruction...

Front 313

What "signs" are not frequently seen in neonates with meningitis, that are seen in adults?

Back 313

Nuchal rigidity, Kernig, and Brudzinski signs.

Front 314

Temperature instability in a newborn is frequently a sign of what type of process?

Back 314

INFECTION!!!

Front 315

Longitudinal striations in the metaphyses of long bones are characteristic of what congenital infection?

Back 315

RUBELLA!!!

Front 316

Osteochondritis or periostitis found on radiograph is indicative of what congenital infection?

Back 316

SYPHILIS!!!

Front 317

A newborn has bilateral cataracts and micropthalmia, intrauterine growth retardation, hemorrhagic skin lesions scattered throughout the body (blueberry muffin rash) and a harsh systolic murmur heard at the left sternal border and radiating to the lung fields. Laboratory anomalies may include a hemolytic anemia with thrombocytopenia, elevated liver function tests and pleocytosis in the spinal fluid. Diagnosis?

Back 317

Congenital Rubella.

Front 318

Newborns with this infection can show isolated CNS involvement, isolated cutaneous infection, or systemic generalized infection. Often times the newborn will just present with a fever and a focal seizure. Diagnosis?

Back 318

HSV infection

Front 319

The classic triad of this congenital infection is hydrocephalus, chorioretinitis, and intracranial calcifications; these infants may also display symptoms similar to other congenital infections, such as anemia, a petechial rash, organomegaly, jaundice, and seizures. Diagnosis?

Back 319

Congenital Toxoplasmosis.

Front 320

Infection estimates of this congenital infection range from 0.4-2.4% of all live births. Many cases are asymptomatic, while others may manifest multiorgan disease including intrauterine growth restriction, hepatosplenomegaly, jaundice, petechiae, or purpura, microcephaly, chorioretinitis, and intracranial calcifications. More than half the infants with this congenital infection develop sensorineural hearing loss. Diagnosis?

Back 320

Congenital CMV.

Front 321

A child presents with lateral displacement of the medial canthi, broad nasal bridge, medial hyperplasia of the eyebrows, partial albinism commonly expressed by a white forelock or heterochromia (or both) and deafness in 20% of cases. Diagnosis?

Back 321

Waardenburg syndrome, which is an autosomal dominant trait.

Front 322

At how many WGA is the Moro reflex present? At what age does it disappear?

Back 322

The Moro reflex is present starting at 32 weeks gestation, well established by 37 weeks gestation and disappears by 3-6 months of age.

Front 323

At how many WGA is the Stepping reflex present? At what age does it disappear?

Back 323

The stepping reflex is present starting at 32 weeks of gestation and disappears by 1-2 months of age.

Front 324

At how many WGA are the Grasp reflexes present? At what age do they disappear?

Back 324

Palmar and Plantar grasp reflexes are well established by 32 weeks of gestational age and disappear by 3 months of age.

Front 325

At how many WGA is the Asymmetrical Tonic Neck reflex present? At what age does it disappear?

Back 325

ATNR appears at 35 weeks gestation, is well established by 1 month postnatal age and disappears by 3-6 months of age; ATNR that is seen as a resting posture rather than being elicited is never normal.

Front 326

At how many months does the Rooting reflex disappear?

Back 326

4-6 months.

Front 327

What cranial nerves does the sucking reflex assess?

Back 327

CNs V, VII, IX, X, and XII.

Front 328

The absence of the plantar grasp reflex in term newborn infants is associated with an increased risk of what CNS condition?

Back 328

Cerebral palsy.

Front 329

What are the MAJOR JONES CRITERIA in diagnosing Rheumatic heart disease?

Back 329

Carditis, arthritis, erythema marginatum, chorea, and subcutaneous nodules; to make the diagnosis two major and one minor criteria must be met along with strong evidence that there has been a Group A Strep infection.

Front 330

What are the MINOR CRITERIA in diagnosing Rheumatic heart disease?

Back 330

Arthraligia (the most common finding overall), fever or history of rheumatic fever, increased ESR, positive C reactive protein, increased WBC and anemia, and prolonged PR and QT intervals on ECG.

Front 331

Harsh, blowing holosystolic murmur best heard along the left lower sternum, with radiation throughout the precordium. Diagnosis?

Back 331

VSD.

Front 332

A middiastolic rumble at the lower left sternal border. A history of asphyxia, or findings of other cardiac lesions is also present. Diagnosis?

Back 332

Tricuspid regurgitation.

Front 333

A pulse rate greater than 250 beats/min suggests the presence of a tachyarrythmia. What are some of the common causes for superventricular tachycardia in a newborn (or in general)?

Back 333

Wolff-Parkinson-White syndrome, congenital heart disease, and sympathomimetic drugs.

Front 334

A quadruple rhythm associated with the murmur of tricuspid regurgitation and a middiastolic murmur at the lower left sternal border, suggests a diagnosis of what? (hint: the presence of right atrial hypertrophy and right ventricular conduction defect confirms this diagnosis)

Back 334

Ebstein's anomaly (downward displacement of the tricuspid valve).

Front 335

Patients with this congenital heart defect usually have a hypoplastic right ventricle, and therefore the ECG shows left-axis deviation and left ventricular hypertrophy; this translates to a left ventricluar impulse on physical examination. Diagnosis?

Back 335

This is the picture of Tricuspid aresia; almost all other forms of cyanotic congenital heart disease are associated with elevated pressures in the right ventricle and increased right ventricular impulse. In those conditions therefore, the ECG will show right-axis deviation and right ventricular hypertrophy.

Front 336

This congenital heart defect shows an intact ventricular septum and presents with early cyanosis, a normal sized heart (classic "egg on a string" radiograph pattern in 1/3 of cases), normal or slightly decreased pulmonary vascular markings, and an ECG showing right-axis deviation and right ventricular hypertrophy. Diagnosis?

Back 336

Transposition of the Great Vessels.

Front 337

This congenital heart disease causes early cyanosis, diminished pulmonary arterial blood flow; the pulmonary fields on x-ray demonstrate dimunition of pulmonary vascularity, and left axis and left ventricular hypertrophy are shown on ECG. Diagnosis?

Back 337

Tricuspid atresia.

Front 338

This syndrome is diagnosed in a newborn who demonstrates profound thrombocytopenia, bilateral absence of radius and abnormally shaped thumbs; CARDIAC LESIONS include TOF and ASD; about 40% of patients die in newborn period as a result of low-platelet-induced bleeding. Diagnosis?

Back 338

Thrombocytopenia absent radius syndrome (TAR).

Front 339

This syndrome occurs in both sexes and the most common features include short stature, downslanting palpebral fissures, ptosis, low-set and malformed ears, webbed-neck, shield-like chest, PULMONIC STENOSIS, and cryptorchidism. Mental retardation is seen in 1/4 of affected individuals; it is associated with advanced paternal age and is termed "male Turner syndrome". Diagnosis?

Back 339

Noonan syndrome.

Front 340

Newborn infants of mother's with this condition can have thrombocytopenia, neutropenia, rash, liver dysfunction, and congenital heart block. Diagnosis?

Back 340

Maternal Lupus.

Front 341

What would the initial treatment be for a patient that is suspected to have asthma with an acute exacerbation due to a respiratory illness?

Back 341

Bronchodilators and a short course of of steroids are a treatment for asthma.

Front 342

What is the most appropriate form of treatment in a patient that is suspected to have bronchiolitis?

Back 342

Monitoring hydration and oxygenation status.

Front 343

Administration of what medication may be the appropriate next step after diagnosing a patient with viral croup?

Back 343

A single dose of steroids.

Front 344

While fever, respiratory distress, and localized chest radiograph findings should point initially toward an acute pneumonia, the rapid clearing of radiographic findings, hemoptysis, digital clubbing found in this child point toward a diagnosis of what? What is the next best step?

Back 344

Idiopathic Pulmonary Hemosiderosis; Broncoalveolar lavage will be the best next step and would reveal hemosiderin laden macrophages, and would be most likely to make the diagnosis.

Front 345

There is a distinct subset of patients with pulmonary hemosiderosis that have a hypersensitivity to cow's milk, that may improve with a diet free of cow's milk. What is the name of this syndrome (cow's milk allergy linked to IPH)?

Back 345

Heiner syndrome.

Front 346

What is a complication that can occur suddenly in a child patient (or otherwise) that has been diagnosed with a Staphylococcal, or other bacterial, pneumonia?

Back 346

Tension pneumothorax is a recognized complication of staphylococcal (and other) pneumonia. It is caused by toxin production by the bacteria leading to rupture of the alveoli into the pleural space.

Front 347

What is a feared, severe, but uncommon, life-threatening sequelae of viral laryngotracheobronchitis (croup)? Treatment?

Back 347

Bacterial tracheitis; Intubation and preservation of the airway, and IV antibiotics are indicated

Front 348

The typical story of this illness is one of several days of viral upper respiratory symptoms, followed by an acute elevation of temperature and an increased in respiratory distress (e.g. biphasic stridor). Diagnosis? Treatment?

Back 348

Bacterial tracheitis; Intubation and preservation of the airway, and IV antibiotics are indicated.

Front 349

What are the three most common bacterial causes of Epiglottitis now that the HiB vaccine has come into existence?

Back 349

Due to the widespread use of the HiB vaccine, this condition is now more commonly caused by Group A streptococcus, Moraxella, or S.pneumoniae.

Front 350

Infection with this parasite can present with a visceral larva migrans; dirt-eating children ingest infectious ova that have been excreted by dogs, and the larva penetrate the intestine and migrate to visceral sites, such as the liver, lung and brain, but do not return to the intestine, so the stools do not contain ova or parasites. Thus, the diagnosis can be made by a specific ELISA for this organism?

Back 350

Toxocara canis!!!

Front 351

Examination of the nose that reveals pus draining from the middle meatus can indicate infection in which sinuses?

Back 351

Maxillary, frontal, or anterior ethmoid sinusitis.

Front 352

Examination of the nose that reveals pus draining from the superior meatus can indicate infection in which sinuses?

Back 352

Sphenoid or Posterior ethmoid sinuses.

Front 353

What are the viral agents usually suspected to be responsible for causing croup? Treatment?

Back 353

Parainfluenze types 1 and 3, Influenza A and B, RSV, and occasionally other viruses. Treatment is usually supportive, but racemic epinephrine and corticosteroids reduce the length of time of emergency room visits and hospitalizations.

Front 354

What is the antibiotic of choice for a suspected atypical pneumonia due to mycoplasma pneumoniae?

Back 354

A Macrolide antibiotic

Front 355

This is a congenital malformation that is thought to arise from an embryonic disruption before the thirty-fifth day that causes improper development of bronchioles. The cystic mass is usually identified on prenatal ultrasound around the 20th week of gestation; large lesions may compress the affected lung and cause pulmonary hypoplasia, which may cause a midline shift away from the lesion. Diagnosis? Treatment?

Back 355

Congenital Cystic Adenomatoid Malformation; treatment is typically surgical excision of the affected lobe; some patients may be at risk for primary pulmonary malignancy.

Front 356

Presenting signs of this condition include a history of pharyngitis, abrupt onset of fever with sore throat, refusal of food, drooling, and muffled or noisy breathing. The most common organisms to cause this are Staph and GAS. Diagnosis? Diagnostic tests?

Back 356

Retropharyngeal abscess; A bulge in the posterior pharyngeal wall is diagnostic as are radiographs of the lateral neck that reveal the retropharyngeal mass.

Front 357

Finding a dilated, stool-filled anal canal with poor tone on the physical exam of a well-grown child supports a diagnosis of what?

Back 357

Functional constipation, although Hirschsprung disease is usually suspected in the chronically constipated child despite 98% of such children having FUNCTIONAL CONSTIPATION!!!; Hirschsprung usually presents in infancy with increasingly difficult defecation in the first weeks of life; typically NO STOOL is found in the rectum and anal sphincter tone is abnormal.

Front 358

In this condition males are more affected than females and the history usually includes atopy or food allergy; symptoms are similar to those seen in GERD (intermittent vomiting dysphagia, epigastric pain food getting "stuck" in the throat), but are not relieved with acid blockade; some have elevated IgE levels or peripheral eosinophilia; endoscopy reveals mucosal furrowing; strictures can develop as well; biopsy reveals many eosinophils; treatment involves avoidance of specific food allergens and inhaled or systemic steroids have been helpful. Diagnosis?

Back 358

Eosinophilic Esophagitis!!!

Front 359

1 day old baby presents with abdominal distension, bilious emesis, echogenic bowel seen on prenatal ultrasound; radiographs show loops of distended bowel and a focal bubbly or granular patten; a postpartum barium enema study showing ribbon-like microcolon. Diagnosis?

Back 359

Meconium ileus; the visual of the colon on radiograph is seen due to the disuse of the colon and focal obstruction.

Front 360

Are perianal disease and mouth ulcerations more likely to present in a patient with Crohn disease or Ulcerative Colitis?

Back 360

Crohn disease; these are not seen in Ulcerative colitis.

Front 361

ASCA and p-ANCA are associated with Ulcerative colitits and Crohn disease. Which goes with which?

Back 361

ASCA is positive in about 55% of those with Crohn disease, but is uncommon in Ulcerative colitis; conversely p-ANCA is positive in about 70% of patients with Ulcerative Colitis, but less than 20% of patients with Crohn disease.

Front 362

This is the most serious complication in a patient with Ulcerative colitis that presents with fever, tachycardia, dehydration, leukocytosis, and electrolyte abnormalities associated with markedly dilated colon; this complication comes with high risk of intestinal perforation. Diagnosis?

Back 362

TOXIC MEGACOLON!!!

Front 363

What are the main diagnostic tools used to establish a possible diagnosis of Hirschprung disease before taking a rectal biopsy or performing histochemical staining for acetylcholinesterase?

Back 363

Radiologic study by barium enema and rectal manonmetry.

Front 364

What is the diagnostic test of choice for a suspected Cholecystitis?

Back 364

Ultrasound is the diagnostic test of choice for a suspected Cholecystitis.

Front 365

2 year old child presents with reddish-colored stools 2 days prior to arrival and has since had several diapers with just blood. The child is tachycardic, but appears happy and playful. Diagnosis? What study will confirm this diagnosis?

Back 365

Meckel's diverticulum; diagnosis can ve made by Technetium scan (radionuclide scan) that labels gastric mucosa, and treatment is surgical excision. Barium studies DO NOT readily reveal the diverticulum!!!

Front 366

What two tests might you use in a child with direct hyperbilirubinemia to rule out the presence of a Choledocal cysts and assess the patency of the biliary tree?

Back 366

Ultrasound examination to rule out the Choledocal cysts may be included with a Technetium Hepatic Iminodiacetic Acid (HIDA) scan to assess the patency of the biliary tree.

Front 367

An alert awake infant witha a 2-day history of diarrhea presents with depresed fontanelle, tachycardia, sunken eyes, and the loss of skin elasticity. The child is also restless, has absent or reduced tears, weak radial pulses, and orthostatic hypotension. What percent dehydration would you say that this child is? What if they were in a moribund state?

Back 367

This child is 5-9% dehydrated; if the child appeared to be in a moribund state they would likely be greater than 10% dehydrated.

Front 368

What type of saline infusion is used only in the event of seizures caused by rapid rehydration, or in patients with hyponatremic dehydration and associated central nervous system symptoms, along with other emergent measures typically used to reduce cerebral edema?

Back 368

Hypertonic (3%) Saline.

Front 369

What are the teeth that are the first to erupt and when do they show up?

Back 369

Mandibular incisors at 5 to 7 months.

Front 370

What are the teeth that are the second to erupt and when do they show up?

Back 370

Maxillary incisors 6 to 8 months.

Front 371

What are the teeth that are the third to erupt and when do they show up?

Back 371

Lateral incisors (mandibular then maxillary) erupt at 7 to 11 months.

Front 372

What are the teeth that are the fourth, fifth, and sixth to erupt and when do they show up?

Back 372

These are the first molars (10-16 months), the cuspids (16-20 months), and the second molars (20-30 months).

Front 373

What is the diagnostic method used to evaluate a suspected Tracheoesophageal Fistula?

Back 373

A MODIFIED barium swallow with fluoroscopy allows direct visualization of the swallow reflex; normal barium swallow does not rule out fistula!!!

Front 374

What is the preferred test to diagnose GERD in an infant?

Back 374

Esophageal 24-pH probe.

Front 375

What is the proper treatment of Labial adhesions (usually a benign condition in which the labia minora are fused) in a young female (most common in young girls who are in the low estrogen state of preadolesence)?

Back 375

Treatment can merely be observation, as the condition should resolve with the estrogenization that occurs with puberty, however, nightly application of an estrogen cream for a week resolves this condition in the majority of patients. Reoccurrence is common though, and patients must be instructed to apply daily petrolatum for a month or two after separation.

Front 376

This syndrome is a malformation that occurs mostly in males and is characterized by a dilated urinary tract, intra-abdominal testes, and a wrinkled abdominal wall; additional urinary tract abnormalities include significant renal dysfunction or dysplasia; oligohhydramnios and commonly associated pulmonary complications, such as pulmonary hypoplasia and pneumothorax are seen; congenital hip dislocation, club feet, and intestinal malrotation with possible secondary volvulus can occur. Diagnosis?

Back 376

Prune Belly Syndrome

Front 377

A patient has the nonspecific findings of anorexia, polydipsia, and polyuria, vomiting, and unexplained fevers; laboratory findings include glucosuria, but normal blood sugar*; abnormally high urine pH in the face of mild or moderate serum hyperchloremic metabolic acidosis*; mild albuminuria in the presence of normal serum protein and albumin. Diagnosis?

Back 377

FANCONI SYNDROME!!! (a.k.a. Global Proximal Tubular Dysfunction).

Front 378

At what age should a child be able to fully retract the foreskin from around his penis? If he cannot do it at this age, what is it called?

Back 378

This should be able to happen after 3 years of age. If it cannot, the diagnosis of Phimosis may be made.

Front 379

What is the appropriate management of Grade I-II Vesicoureteral reflux in terms of antibiotic prophylaxis and urine cultures? How do you manage Grade V VUR?

Back 379

Grade I-II VUR can be managed with conservatively with close observation, daily low-doses antibiotics, and urinalyses and culture every 3-4 months. Grade V lesions (and some Grade IV) require surgical reimplantation of a ureter if the findings persist.

Front 380

Orchipexy of an undescended testicle, eliminates the risk of development of what condition with that testicle?

Back 380

TORSION!!!; risk of malignancy is not eliminated.

Front 381

Unilateral lymphadenopathy and non-purulent conjunctivitis are diagnostic criteria necessary for what pediatric vasculitis?

Back 381

Kawasaki disease.

Front 382

Significant symptoms of this pediatric vasculitis include edema, arthralgia or arthritis, colicky abdominal pain with GI bleeding, acute scrotal pain, and renal abnormalities ranging from microscopic hematuria to acute renal failure; patients can have headache and may have behavioral changes. With this vasculitis any organ system can be affected. Diagnosis?

Back 382

Henoch-Shonlein Purpura.

Front 383

What is the appropriate treatment for a teenager presenting with Varicocele that is not painful and feels like a "bag of worms"?

Back 383

Varicocele is a common condition seen after 10 years of age and occurs in about 15% of adult males. It results from the dilatation of the pampinifiorm plexus (usually on the left side) due to valvular incompetence of the spermatic vein. Reassurance and education are appropriate as this condition is not harmful in and of itself.

Front 384

What is the appropriate treatment for a small Hydrocele (an accumulation of fluid in the tunica vaginalis in the scrotal sac)?

Back 384

Small Hydroceles usually resolve spontaneously in the first year of life; larger ones or those that have a variable fluid level with time, will likely need surgical repair.

Front 385

What are the pain and urinalysis findings that Idiopathic Hypercalciuria can cause?

Back 385

Idiopathic hypercalciuria causes recurrent gross hematuria, persistent microscopic hematuria, and complaints of dysuria or abdominal pain without initial stone formation. Overtime stones will form in 15% of cases.

Front 386

This syndrome is an autosomal recessive condition that causes hypokalemia, hypercalciuria, alkalosis, hyperaldosteronism, and hyperreninemia; blood pressure is normal; clinical presentations occurring frequently between 6 and 12 months of age include failure to thrive with constipation, weakness, vomiting, polyuria, and polydypsia. Treatment is aimed at preventing dehydration, providing nutritional support, and returning potassium level to normal. Diagnosis?

Back 386

Bartter syndrome (also known as Juxtaglomerular Hyperplasia).

Front 387

Is the gene for predisposition to malignant hyperthermia autosomal dominant or recessive? What is te gene and what chromosome is it on?

Back 387

Autosomal Dominant; the gene is for the ryanodine receptor (calcium release channel) and it is found on Chromosome 19.

Front 388

What is the test used to test someone for the possibility for developing malignant hyperthermia?

Back 388

This is the caffeine contracture test is which a muscle biopsy tissue specimen is attached toa a strain gauge and then exposed to caffeine. Patients at risk for malignant hyperthermia have a diagnostic muscle spasm

Front 389

This is a subperiosteal collection of blood on the newborn's scalp that does not cross the suture lines. Diagnosis?

Back 389

Cephalhematoma.

Front 390

Homocystinuria is an autosomal recessive metabolic disease caused by deficiencies of what enzymes?

Back 390

Cystathione B-synthase, Methylenetetrahydrofolate reductase, or the coenzyme for N-methyltetrahydrofolate methyltransferase.

Front 391

What is Craniosynostosis? Explain the difference between primary and secondary Craniosynostosis.

Back 391

Craniosynostosis is when the skull forms small and narrow either to to premature fusion of cranial sutures or lack of brain growth. Primary is when there is premature closure of sutures with normal brain growth and no neurological impairment. Secondary is when there is a smaller, more narrow head due to lack of brain growth.

Front 392

The infant in question, who is completely healthy otherwise with a normal neurological exam, appears to have one suture of the cranium prematurely fused (most commonly the sagittal suture). This has resulted in scaphocephaly (long narrow skull). Diagnosis? Treatment?

Back 392

This is Simple Primary Craniosynostosis (Simple because one suture, Primary because it is due to bone growth and not brain growth). This condition is usually sporadic and more commonly affects the sagittal suture, which results in scaphocephaly. In general, premature fusion of a single suture does not cause increased intracranial pressure or hydrocephalus; these features are more common with premature fusion of two or more sutures. The therapy for this condition is controversial, but usually involves surgery; consultation with a neurosurgeon would be indicated.

Front 393

If Myasthenia gravis was suspected in a young infant what would be the test of choice to use in order to diagnose?

Back 393

In older children this test is accomplished with edrophonium chloride, but it should be avoided in young infants, as cardia arrhythmias may result. In young infants Neostigmine would be used instead.

Front 394

A patient presents with seizure-like activity with no epileptiform activity on EEG. The episodes may be very convincing for seizure and may include unusual posturing and sounds, but typically do not involve loss of bowel or bladder control. Patients will not cause self-injury and pupillary response to light is normal. Diagnosis? What is the best way to start management of this condition?

Back 394

This describes Pseudoseizure. If a patient had been thought to be having true seizures and is on anti-epileptic medication, it would be inappropriate to discontinue medication abruptly because many patients with pseudoseizures also have true epileptic seizures. The next best step to manage this patient is to start with a psychiatric or psychological evaluation.

Front 395

There is abrupt onset of hemiparalysis in a previously healthy 7 year old; his eyes deviate away from the paralyzed side. Diagnosis? Etiology?

Back 395

This is most likely Acute Infantile Hemipelgia.; most frequently this is due to a thromboembolic occlusion of the middle cerebral artery or one of its major branches. THE DIAGNOSIS HAS ALSO BEEN USED TO DESCRIBE AN ACUTE SYNDROME OF FEVER AND PARTIAL SEIZURE WITH RESULTING HEMIPARESIS!!!

Front 396

What are some of the causes of childhood stroke?

Back 396

Childhood stroke can result from trauma, infection, a hypercoagulable state, arteritis, and congenital structural or metabolic disorders.

Front 397

This type of paralysis can occur in children with a HISTORY OF MIGRAINE and during a headache episode. Diagnosis?

Back 397

Hemipelgic migraine paralysis.

Front 398

This type of paralysis follows AFTER A JACKSONIAN OR FOCAL SEIZURE and generally does not last more than 24 to 48 hours. Diagnosis?

Back 398

TODD PARALYSIS!!!

Front 399

In this condition an elevated creatinine kinase (especially in the preclinical phase) often is found, and psychomotor retardation can be the presenting complaint (but may only be identified in retrospect); ptosis, baldness, hypogonadism, facial immobility with distal muscle wasting (in older children), and neonatal respiratory distress ( in the newborn period) are major features of this disorder; cataracts are commonly seen, presenting either congenitally or at any point during childhood; the prominence of distal muscle weakness seen in this disease is in contrast to the proximal muscle weakness seen in most other forms of myopathies. Diagnosis???

Back 399

MYOTONIC MUSCULAR ATROPHY!!!

Front 400

Children with this type of headache most often have bifrontal headache without an aura or diffuse throbbing headache of only a few hours; the headaches can be terminated with vomiting or sleep; FAMIlLY HISTORY IS FREQUENTLY POSITIVE!!! Diagnosis?

Back 400

MIGRAINE!!! Children present slightly differently than adults; severity of the headache and throbbing nature should suggest migraine; and look for a positive family history.

Front 401

A child that has a subacute disorder of the CNS that produces cranial nerve abnormalities (especially of the CN VII and lower bulbar nerves!!!), long tract signs, unsteady gait secondary to spasticity, and some behavioral changes, is likely to have what type of CNS tumor?

Back 401

PONTINE GLIOMA!!!; this is a diffuse tumor that is difficult to treat.

Front 402

What CNS lesion related to infection is more commonly seen with patients who have cardiac defects that have right-to-left shunts associated with them?

Back 402

Brain abscess.

Front 403

What is Cushing's triad of increased intracranial pressure?

Back 403

Rising blood pressure, irregular respiration rate, and falling heart rate.

Front 404

The findings of poor growth, diabetes insipidus, and papilledema in a 6 year old child can be explained by what diagnosis?

Back 404

Craniopharyngioma; this tumor is one of the most common supratentorial tumors in children, often causing growth failure through disruption of pituitary excretions such as growth hormone; upward growth of a craniopharyngioma results in compression of the optic chiasm causing bilateral temporal hemianopsia; early in the growth of a CP though, a unilateral superior quadrantanopsic defect can develop.

Front 405

What are the most common solid tumors of childhood?

Back 405

Brain tumors; they account for 25-30% of all pediatric malignancies.

Front 406

Are supratentoral or subtentorial tumors more common in children?

Back 406

While suprtentorial tumors predominate in the first year of life (including choroid plexus tumors and teratomas), brain tumors in children 1 to 10 years old are more frequently infratentorial/subtentorial (posterior fossa) and include cerebellar brain tumors such as meduloblastoma or cerebellar astrocytoma!!!

Front 407

What is the diagnostic test for Spinal Muscular Atrophy in an infant?

Back 407

Muscle biopsy.

Front 408

What is often the first presenting symptom in an infant with infantile botulism?

Back 408

Constipation.

Front 409

How is the diagnosis of myotonic dystrophy in an infant made?

Back 409

This diagnosis is confirmed by examination of both parents for percussion and grip myotonia, and by EMG depiction of myotonic discharges.

Front 410

In this disease an excess of motor neuroblasts is noted, and through apoptosis only about half survive in the normal newborn; the SMN (survivor motor neuron) gene regulates this natural destruction and normally limits the apoptosis that takes place; a defect in this gene results in a continuation of apaptosis, resulting in progressive destruction of motor neurons in the brain stem and spinal cord. Diagnosis?

Back 410

Spinal muscular atrophy; (Werdnig-Hoffman disease, or progressive spinal muscular atrophy).

Front 411

A 15 year old girl has acute, recurrent, pulsatile headaches localized behind the eyes that tends to occur more frequently around menses; she has no symptoms that occur prior to headache; her neuro exam is normal. Diagnosis?

Back 411

MIGRAINE!!! (vascular headache)

Front 412

Worsening headaches with nausea, and emesis (particularly morning emesis) are concerning for what process?

Back 412

Increased intracranial pressure from a mass lesion.

Front 413

A child with a unilateral face lesion and blindness suggests Sturge-Weber syndrome, or what other syndrome?

Back 413

PHACE syndrome (like on your face)
P- posterior fossa malformation
H- hemangioma
A- arterial anomalies
C- coarctation or other cardiac defects
E- Eye abnormalities

These patients can have seizures, hemiparesis, intracranial calcifications, and mental retardation.

Front 414

A young girl shows large, irregular, unilateral cafe au lait spots and fibrous displasia of bones in association with precocious puberty in girls. Diagnosis?

Back 414

McCune-Albright syndrome.

Front 415

This virus has a vaccine to prevent mild disease; symptoms include a diffuse maculaopapular rash that lasts for 3 days, marked enlargement of the posterior cervical and occipital lymphnodes, low grade fever, mild soar throat, and occasionally conjuctivitis, arthralgia or arthritis. Diagnosis?

Back 415

Rubella.

Front 416

What is the normal cause of a death associated with Reye's syndrome?

Back 416

Death is usually from cerebral edema and subsequent herniation.

Front 417

What is Ekiri syndrome?

Back 417

This is a rare and fatal toxic encephalopathy associated with Shigella infection.

Front 418

This fungus occurs in children 6 months-Puberty an produces an infection within the hair follicle that is unresponsive to topical treatment alone and requires long-term oral therapy with griseofulvin or another antifungal for eradication; fluorescence is absent on Wood lamp; enlarged occipital lymphnodes are common. Diagnosis?

Back 418

Trichophyton tonsurans causing tinea capitis.

Front 419

This infection tends to begin abruptly, with fever and marked, localized tenderness that usually occurs ar the metaphysis; redess and swelling frequently follow; although this is usually the result of hematogenous spread, the actue presentation can follow an episode of deep cellulitis or septic joint and should be suspected whenever these occur. Diagnosis? Test?

Back 419

Osteomyelitis; radionuclide bone scans can be helpful in the diagnosis of osteomyelitis within 24 to 48 hours of symptoms and in ints differentiation from cellulitis and septic arthritis, but MRI has become more widely used because it is mor sensitive and specific.

Front 420

In an infant who appears otherwise normal, the sudden onset of high fever, together with a marked elevation and shift to the left of the WBC count, suggests what type of infection?

Back 420

Pneumococcal bacteremia!!!

Front 421

The symptoms of this disease are usually a mild disease, including a diffuse maculopapular rash that lasts for 3 days, marked enlargement of the posterior cervical and occipital lymph nodes, low grade fever, mild soar throat and occasionally a conjunctivitis, arthralgia, or arthritis. Diagnosis?

Back 421

Rubella

Front 422

A 4 year old child has swelling in the front of and in back of the ear on the affected side, as well as altered taste sensation. Diagnosis?

Back 422

Mumps

Front 423

What is the recommendation of the CDC for anyone considered to have a close encounter with a bat, even without an obvious bite? What does this treatment consist of?

Back 423

The CDC recommends that postexposure prophylaxis (PEP) be considered. PEP consists of wound cleansing (if there is a wound), rabies immune globulin (RIG), and the five injection rabies vaccine series.

Front 424

This immunodeficiency will usually present after 3 months of age (after maternal antibodies wane) with recurrent and often simultaneous bouts of otitis media, pneumonia, diarrhea, and sinusitis, but usually without fungal and viral infection. Diagnosis?

Back 424

X-linked agammaglobulinemia

Front 425

Mild T-cell dysfunction, diminished serum IgM, marked elevation of IgA and IgE, eczema, recurrent middle-ear infections, lymphopenia, and thrombocytopenia. Diagnosis?

Back 425

Wiskott-Aldrich

Front 426

This is a disorder of phagocytic chemotaxis associated with hypergammaglobulin E, eczema-like rash, and recurrent severe staphylococcal infections. Diagnosis?

Back 426

Joe-Buckley

Front 427

What is the most commonly acquired aplasia in childhood?

Back 427

Transient Erythroblastopenia of childhood (TEC).

Front 428

This condition is commonly diagnosed between the ages of 1 to 3 years and some affected children have a history of a recent viral infection; physical findings are minimal and include pallor and tachycardia; lab studies reveal a profound anemia and reticulocytopenia; the condition lasts for several months, and may require 1 or 2 transfusions, but ultimately is self limited and steroids are not useful. Diagnosis?

Back 428

Transient Erythroblastopenia of Childhood.

Front 429

This phenomenon, is seen to present with large vascular anomalies (i.e. kaposiform hemangioendothelioma and tufted angioma), platelet and RBC sequestration within the vascular tumor which causes peripheral thrombocytopenia, coagulopathy, and microangiopathic hemolytic anemia; treatment options include corticosteroids, alpha interferon, and vincristine. Diagnosis?

Back 429

Kasabach-Merritt phenomenon.

Front 430

Which chromosome is associated with Wilm's Tumor and the abnormalities that may be found?

Back 430

Chromosome 11.

Front 431

What is the main therapy initiated to treat acute bleeding episodes in a patient with mild Von Willebrand Disease?

Back 431

DESMOPRESSIN alone can transiently increase the levels of vWF three- to fivefold, so it is used frequently for acute bleeding episodes.

Front 432

Symptoms of this neonatal condition usually disappear in 2 to 4 months and include tachycardia, tachypnea, irritability and hyperactivity, low birth weight with microcephaly, severe vomiting and diarrhea, thrombocytopenia, jaundice, hepatosplenomegaly and heart failure. Diagnosis?

Back 432

Neonatal Thyrotoxicosis from maternally acquired thyrotropin receptor-stimulating antibody (TRSAb).

Front 433

At what age is puberty said to be delayed in a male, if physical changes are not apparent?

Back 433

By the age of 14.

Front 434

This syndrome is the most common of several syndromes that are characterized by both deafness and pigmentary changes; features of this syndrome, which is inherited as an autosomal dominant disorder, include a distinctive white forelock, heterochromia irides, unilateral or bilateral congenital deafness, and lateral displacement of the inner canthi. Diagnosis?

Back 434

Waardenburg syndrome.

Front 435

What is the most common cause of Cushing's syndrome? What is should be suspected if this first reason is not the cause?

Back 435

The most common cause of Cushing syndrome is exogenous administration of adrenocorticotropic hormone or of glucocorticoid. If this is not happening and a pediatric patient is developing Cushing syndrome suspect bilateral adrenal hyperplasia.

Front 436

A child is short for his age, has downslanting palpebral fissures, ptosis, low-set and malformed ears, a broad and webbed neck, shield chest, and cryptorchidism; this condition can also show cubitus valgus, pulmonary stenosis, edema of the dorsum of the hands and feet, hearing loss, pectus excavatum, bleeding diathesis, and mental retardation in about 1/4 of cases. Diagnosis?

Back 436

Noonan syndrome; "male Turner syndrome"

Front 437

What type of sepsis are patients with Galactosemia particular susceptible to?

Back 437

E.coli sepsis.

Front 438

1 month old presents with dehydration, listlessness, and irritability; there is significant jaundice and hepatosplenomegaly; liver function tests are elevated and glucose is 38 mg/dL. Diagnosis?

Back 438

Galactosemia

Front 439

Signs of this congenital condition include constipation, prolonged jaundice, sluggishness, poor feeding, apnea, choking, macroglossia, and excessive sleepiness; the physical exam is usually normal early on except for mild jaundice and a distended abdomen in a sleepy infant. Diagnosis?

Back 439

Congenital hypothyroidism.

Front 440

By the time a child starts kindergarten what are the vaccinations that the child should have had?

Back 440

Hepatitis B- 3 doses
Rotavirus- 3 doses
DTaP- 5 doses
Hib- 4 doses
PCV-4 doses
IPV- 4 doses
MMR- 2 doses
Varicella- 2 doses
HepA- 2 doses

Front 441

At the 4-5 year old visit what are the vaccines that a child should be receiving?

Back 441

At the 4-5 year old visit, a child should receive his DTap #5, IPV#4, MMR#2, and Varicella #2.

Front 442

A child presents with short palpebral fissures, epicanthal folds, long philtrum, thin upper lip, cardiac defects (ASD, VSD, persistence of arterial canal), multiple joint anomalies, prematurity, growth retardation and mental retardation. Normal karyotype with no Robertsonian translocation? Diagnosis?

Back 442

Fetal alcohol syndrome.

Front 443

What is the most common cause of mental retardation in children?

Back 443

Fetal Alcohol Syndrome.

Front 444

What is the treatment required for an infant with a fracture of the clavicle?

Back 444

Generally, no treatment is required.

Front 445

Precocious pubarche with signs of severe androgen excess that occur all at once instead of sequentially is suggestive of Precious Puberty or Pseudopuberty?

Back 445

This is suggestive of Precocious Pseudopuberty that is caused by a gonadotropin-independent process (typically an excess of sex steroids, such as that that would be caused by 21-hydroxylase deficiency).

Front 446

Patients with this disease typically expire in utero due to multiple intrauterine and/or perinatal fractures, as well as restrictive lung disease; clinical findings include limb deformities, growth retardation, multiple fractures and blue sclerae. Diagnosis?

Back 446

Osteogenesis Imperfecta Type II

Front 447

This syndrome is due to 5p deletion and presents as microcephaly, hypotonia, short stature and a cat-like cry. Diagnosis?

Back 447

Cri-du-chat.

Front 448

What is the emergent treatment that is recommended for an infant that is in respiratory distress due to a suspected Congenital Diaphragmatic Hernia?

Back 448

The first step here is immediate placement of an orogastric tube and connecting it to a continuous suction, in order to prevent bowel distension and further lung compression. Endotracheal intubation and mechanical ventilation are also priorities for all infants with severe CDH who present in the first hours of life.

Front 449

All children with complaints of anal/genital itching that is worse at night, should be examined for what? Treatment?

Back 449

PIN WORN INFECTION!!!; scotch tape test. Treatment is Mebendazole.

Front 450

This is one of the most common elbow injuries in children; it occurs when infants or children are lifted or pulled by the hand or arm; physical examination reveals no significant swelling or angular deformity, and the child typically keeps the affected arm in a pronated position and refuses (cries out in pain) attempted forearm supination. Diagnosis?

Back 450

This is Nursemaind's elbow, also called SUBLUXED RADIAL HEAD. This pulling injury occurs when the radial head slips through parts of the annular ligament. Rotating the hand and forearm to a supinated position with pressure over the radial head usually reduces the annular ligament with an audible click, and restore full, normal use of the extremity. The response to reduction of the displaced ligament is dagnostic.

Front 451

This type of jaundice in the newborn is an exaggeration of physiologic jaundice commonly seen in exclusively seen in exclusively breastfed infants who are not receiving enough breastmilk; when effective breastfeeding is not established in the first few days of life, the inadequate enteral intake prolongs the intestinal transit time, resulting in an increased enterohepatic circulation; in addition the infant becomes relatively dehydrated from the limited fluid intake. The increased enterohepatic circulation and relative dehydration together cause an elevated unconjugated bilirubin level. Diagnosis?

Back 451

BREASTFEEDING JAUNDICE!!!

Front 452

A child presents with a conjugated hyperbilirubinemia 1-6 weeks after birth; e has clay colored stools, dark urine, and an enlarged liver. Diagnosis?

Back 452

Biliary Atresia.

Front 453

The most likely diagnosis of painless melena in a 2 to 3 year-old child is what?

Back 453

Meckel's diverticulum with heterotopic gastric mucosa.

Front 454

What are the main features that can differentiate the behavior of a child with hearing impairment from a child with autism?

Back 454

Hearing impairment does not present with repetitive behaviors that are characteristic of autism. Also, features of autism usually become evident prior to age three, and social isolation is more prominent than in the hearing impaired.

Front 455

What is the most common cause of subarachnoid hemorrhages in children?

Back 455

Arteriovenous malformations (AVM) with rupture into the subarachnoid space. Besides that, the local effect of AVM typically produces seizures and migraine-like headaches. Therefore, a history of seizures helps in making the correct diagnosis.

Front 456

From what embryonic tissue does a WIlm's tumor arise from?

Back 456

Mesonephros.

Front 457

What is the feature about palpable kidneys that differentiates Wilm's Tumor from Neuroblastoma?

Back 457

A unilateral mass in a child LESS THAN 3 years of age is highly suspicious for neuroblastoma. Neuroblastoma displaces the kidneys inferiolaterally, thereby making the kidneys non-palpable. In Wilm's tumor the unilateral flank mass is more commonly found in patients greater than 3 years of age and the kidneys are still palpable.

Front 458

With supra condylar fractures what artery can become compromised?

Back 458

The brachial artery can become compromised, resulting in the loss of the radial artery pulse; therefore the radial artery pulse must be assessed when the fracture is reduced.

Front 459

A 24-hour old child presents with bilious vomiting, failure to pass meconium at birth, history of polyhydramnios. Diagnosis?

Back 459

Likely to be Cystic Fibrosis.

Front 460

One of the possible complications of meconium ileus is intestinal perforation. What are the differences found on X-ray if the perforation occurs before vs. after birth?

Back 460

If the intestinal perforation occurs before birth, intraabdominal calcifications will be seen. If it occurs after birth, pneumoperitoneum will be seen.

Front 461

Children with a parental history of elevated cholesterol levels (>240 mg/dL), or risk factors for coronary artery disease should get what screening test?

Back 461

A screening test for total cholesterol level.

Front 462

A sudden onset of respiratory distress in a child may be caused by a foreign body aspiration. What procedure of choice would be both diagnostic and therapeutic?

Back 462

Direct laryngoscopy and rigid bronchoscopy would be diagnostic and therapeutic. Flexible bronchoscopy is a useful diagnostic tool; however, it is nor helpful in the retrieval of the foreign body.

Front 463

What are the most common causes of acquired Torticolis? What is the first step in treatment?

Back 463

The most common causes of acquired Torticolis are upper respiratory infections, minor trauma, and cervical lymphadenitis. More serious causes include retropharyngeal abscess and atlantoaxial subluxation. Cervical spine radiographs should be obtained in children presenting with torticolis to ensure there is no cervical spine fracture or dislocation.

Front 464

Like Osteomyelitis, what is the usual origin of Septic Arthritis in a child? What is the most common organism causing septic arthritis in children? How do you treat it?

Back 464

Hematogenous origin and frequently following a URI are the most common origin of Septic Arthritis in children. The most common organisms are Staph aureus and Streptococcus. Septic joint in a child is a true surgical emergency and needs immediate drainage. A delay of even 4-6 hours can lead to avascular necrosis of the femoral head. Intravenous antibiotics are required along with surgical drainage.

Front 465

What is the general treatment for Septic Arthritis of the hip joint?

Back 465

Ultrasound guided aspiration of the synovial fluid should be performed immediately. The synovial fluid white cell count of more than 100,000/uL indicates a definite septic arthritis. An X-ray is often normal and empiric antibiotics based on the gram stain results and child's age should be administered immediately.

Front 466

This syndrome is characterized by high levels of IgM with deficiency of IgG and IgA and poor specific antibody responses to immunizations; the patient will present with recurrent sinopulmonary infections and Pneumocystis jiroveci pneumonia.; the unique susceptibility to opportunistic infections and neutropenia, with high IgM levels, distinguishes this condition from XLA or other hypogammaglobulinemias. Diagnosis?

Back 466

Hyper-IgM syndrome (HIM).

Front 467

Symptoms of this vitamin deficiency are typically mild and non-specific in presentation, but symptoms may include sore throat, hyperemic and edematous oropharyngeal mucous membranes, chelitis, stomatitis, glossitis, normocytic-normochromic anemia, seborrheic dermatitis, and photophobia. This uncommon in developed countries. Diagnosis?

Back 467

Riboflavin deficiency.

Front 468

What are 4 other major risk factors associated with the development of RDS other than prematurity?

Back 468

Male sex, cesarean section without labor, perinatal asphyxia, and maternal diabetes!!!

Front 469

What is the pathophysiology of how maternal diabetes increases the incidence of RDS?

Back 469

The proposed mechanism is that fetal hyperinsulinism antagonizes the action of cortisol and may delay the lung maturation process.

Front 470

The EEG of a 6 month old infant presenting with clusters of brief symmetrical contractions of the neck, trunk and extremities (aka infantile spasms) demonstrates a unique pattern of EEG, which consists of chaotic, high-voltage, bilateral, asynchronous, slow-wave activity (hypsarrhythima). Diagnosis? Treatment?

Back 470

This is a characteristic finding of Tuberous Sclerosis. The best drug available for infantile spasms in the United States is intramuscular ACTH.

Front 471

The characteristic appearance of a patient with this condition includes doll-like face (fat cheek), thin extremities, short stature, and a protuberant abdomen (due to the enlarged liver and kidneys); the spleen and heart are normal. The typical patient is 3-4 months of age with hypoglycemia, lactic acidosis, hyperuricemia, and hyperlipidemia. Hypoglycemic seizures may occur. Diagnosis?

Back 471

Glucose-6-phosphatase deficiency.

Front 472

Floopy baby with feeding difficulties, macroglossia, heart failure (due to progressive hypertrophic cardiomyopathy), and hepatomegaly. Diagnosis?

Back 472

Pompe's disease.

Front 473

Diagnose this combination of signs and symptoms: Neurologic (ataxia dysarthria), Skeletal (scoliosis, feet deformities) and Cardiac (concentric hypertrophic cardiomyopathy). What is the most common cause of death?

Back 473

This describes Friedreich ataxia; the most common causes of death are cardiomyopathy and respiratory complications!!!

Front 474

What is the recommended treatment for a child under the age of 9 with stage 1 lyme disease? Over the age of 9?

Back 474

Under the age of 9 yo: Amoxicillin

Over the age of 9 yo: Doxycycline

For those who cannot take amoxicillin or doxycyclin: Cefuroxime axetil or Erythromycin for 21 days

Front 475

What is the treatment of choice for Stage 3 Lyme disease, which is a disseminated disease with neurological and /or cardiac manifestations?

Back 475

Cetriaxone or Penicillin G for 14- 21 days.

Front 476

At what point is surgery necessary for a clubbed foot?

Back 476

SUrgical treatment is indicated if conservative management gives unsatisfactory results, and is preferably performed between 3 and 6 months of age.

Front 477

Slowly developing back pain and neurologic dysfunction (e.g. urinary incontinence) in combination with a palpable "step-off" at the lumbosacral area are typical for what condition in a preadolescent child?

Back 477

High-grade Spondylolisthesis; this is a developmental disorder characterized by a forward slip of vertebrae (usually L5 over S1). Such a displacement results in chronic back pain and in neurological dysfunction, if significant. The area of vertebral displacemnt (a palpable "step-off") is detected on physical examination if the problem is severe.

Front 478

What is the best test to confirm diagnosis of a patient with Duchenne muscular dystrophy? What about in atypical cases? How do you screen for this?

Back 478

Muscle biopsy can confirm the diagnosis in the patient with Duchenne muscular dystrophy. although performing genetic studies are currently the most appropriate way of confirming the diagnosis in atypical cases. Serum CK levels are used for screening the muscular dystrophies.

Front 479

What is the definition of Bronchiolitis caused by RSV? What is the treatment?

Back 479

Bronchiolitis is defined as the first episode of wheezing associated with an upper respiratory tract infection. The infection is usually caused by respiratory syncytial virus and is common in the winter months. It affects 50% of children in the first two years of life. Treatment is supportive care with humidified oxygen and bronchodilators. Ribavirin may be administered in high-risk children.

Front 480

What is the syndrome that you should suspect in an infant with macrosomia, macroglossia, visceromegaly, omphalocele, hypoglycemia, and hyperinsulinemia; additional features include prominent eyes, prominent occiput, ear creases and hyperplasia of the pancreas? What chromosomal/genetic abnormality can be associated? What malignancies do patients with this syndrome have an increased risk for?

Back 480

This is Beckwith-Wiedemann syndrome; it is sometimes associated with duplication of Chromosome 11p. This region contains the gene encoding for IGF-2, which may explain the macrosomia. Sometimes the hypoglycemia may be severe and intractable, and subtotal pancreatectoy may be needed. Patients have an increased risk of neoplasms such as Wilm's tumor, hepatoblastoma, and gonadoblastoma.

Front 481

Caudal regression syndrome, transposition of the great vessels, duodenal atresia and hypoplastic left colon, and anencephaly and neural tube defects are common congenital problems associated with what maternal condition during pregnancy?

Back 481

Gestational diabetes.

Front 482

What are the abnormal genes associated with the WAGR syndrome (Willm's tumor, Anridia, Genitourinary anomaly, Retardation)

Back 482

A deletion in Chromosome 11 involving the gene WT1 (Wilm's tumor 1 gene) and Aniridia gene PAX6.

Front 483

A few days or weeks after a child starts taking formula or breast milk, he shows signs of liver failure (hepatomegaly, direct hyperbilirubinemia, disorders of coagulation), abnormal renal function, emesis, anorexia, acidosis and glycosuria. Diagnosis?

Back 483

Galactosemia!!!

Front 484

This syndrome is characterized by macrosomia, macroglossia, visceromegaly (liver and kidneys), omphalocele, hypoglycemia and hyperinsulinemia. Diagnosis?

Back 484

Beckwith-Wiedemann syndrome.

Front 485

A patient with recurrent bacterial infections and necrotic periodontal infection is most likely suffering from what congenital immunodeficiency?

Back 485

Leukocyte adhesion defect; normal lymphocyte count and gamma globulin concentration help to differentiate this condition from a variety of cell and/or humoral immune defects.

Front 486

What is the most common cause of congenital hypothyroidism in the U.S.?

Back 486

Thyroid dysgenesis.

Front 487

What should you suspect in a young child of 2-5 years of age witha a localized abdominal mass that does not cross the midline and hematuria? What about a child under 1 year of age presents with an abdominal mass that crosses the midline, and has fever, weight loss, hepatomegaly and hypertension?

Back 487

WIlm's Tumor; Neuroblastoma.

Front 488

What congenital heart defect should you suspect in an infant with left axis deviation on EKG? (*VSD or PDA are necessary for survival; sometimes there is an associated pumonary stenosis, and the blood passes through the PDA to reach the pulmonary circulation)

Back 488

Suspect TRICUSPID ATRESIA!!!

Front 489

What is the most important factor leading up to a neonate that develops Tetanus?

Back 489

An UN-IMMUNIZED MOTHER!!!

Front 490

Neonatal withdrawal form this drug can include jitteriness, excessive sucking, and a hyperactive moro reflex; long-term effects of behavior, attention level and intelligence may be seen. Diagnosis

Back 490

Cocaine withdrawal.

Front 491

Neonatal withdrawal from this drug may cause irritability, a high-pitched vry, poor sleeping, tremors, seizures, sweating, sneezing, tachypnea, poor feeding, vomiting, and diarrhea. Diagnosis?

Back 491

Neonata abstinence syndrome caused by withdrawal to opiates.

Front 492

Neonate presents with periods of apnea, pallor or cyanosis, poor suckling, abnormal eye signs, high-pitched cry, muscular twitching, convulsions, decreased muscle tone or paralysis, decreased hematocrit, metabolic acidosis, and shock; fontanels may be tense and bulging. Diagnosis?

Back 492

Intracranial hemorrhage in a neonate.

Front 493

What is the antibiotic of choice when treating an inflamed, fluctuant cervical lymph node that is typically caused by streptococcal or staphylococcal infection?

Back 493

Dicloxacillin; other antibiotics include Cephalexin or Clindamycin.

Front 494

Common early manifestations of this condition are craniotabes (ping-ping ball sensation over the occiput or posterior parietal bones), rachitic rosary (enlargement of the costochondral junctions), and thickening of the wrists and ankles; other findings include Harrison groove (horizontal depression on lower border of chest) and a large anterior fontanelle. Diagnosis? Test for diagnosis?

Back 494

Vitamin D deficiency rickets; diagnosis can be confirmed by obtaining the serum calcifediol level (decreased), alkaline phosphatase level (increased), and observing characteristic radiologic changes (cupping and fraying of the distal ends of long bones, and double contour along the lateral outline of the radius)

Front 495

At what age is stranger anxiety expected in an infant? When does it peak?

Back 495

Stranger anxiety usually starts anywhere between 6-8 months, and peaks at 12-15 months.

Front 496

In a low birth weight infant of 32 weeks gestation, the following are seen (he comes to you at 2 months of age):

Peripheral smear showing normocytic and normochromic anemia, with no abnormal forms; low reticulocyte count and red cell precursors in the bone marrow are decreased; normal WBC and platelet counts; normal total bilirubin level. Symptoms include poor weight gain, poor feeding, lethargy, tachypnea, tachycardia, and pallor; Hb is usually around 7-10 g/dL. Diagnosis? Pathophysiology?

Back 496

Anemia of prematurity; this occurs due to transitions in the erythropoesis sites of the neonate; sorter life span of the red blood cells in neonates, and diminished fetoplacental transfusion (occurs when the baby is held above the level of the placenta after delivery).

Front 497

A child presents with macrocytic anemia, low reticulocyte count, and congenital anomalies. The macrocytic anemia is distinct from that of megaloblastic anemia because there is no hypersegmentation of the nucleus in neutrophils; electrophoresis shows elevated HbF; congenital anomalies can include webbed neck, cleft lip, sheilded chest, triphalangeal thumbs!!! Chromosomal studies are normal and therapy is mainly corticosteroids. What are you suspicious for?

Back 497

Diamond-Blackfan syndrome; the primary pathology is an intrinsic defect of erythroid progenitor cell defect, which results in increased apoptosis.

Front 498

This is an autosomal recessive disorder characterized by progressive pancytopenia and macrocytosis; the average diagnosis is at 8 years; associated deformities include cafe-au-lait spots, microcephaly, micropthalmia, short stature, horseshoe kidneys and ABSENT thumbs!!!

Back 498

Fanconi's anemia!!!

Front 499

This is the most common cause of chronic inspiratory noise in infants; the noises usually begin during the first two months of life; the baby is usually happy and vital signs and oxygen sat is usually normal; the noise is usually inspiratory; it is a self-limiting condition in most cases and generally subsides by 18 months of age; finding of an epiglotis rolling from side to side is diagnostic. Diagnosis?

Back 499

Laryngomalacia, or congenital flaccid larynx; laryngoscopy shows flaccidity of the larynx, and collapses during inspiration

Front 500

Sensorineural deafness, cardiac malformations (e.g. ASD and PDA), and cataracts is the classic triad of what congenital syndrome?

Back 500

Congenital RUBELLA SYNDROME!!!

Front 501

Which antipsychotic drug has been found to cause weight gain and increase prolactin levels due to dopamine antagonism, to a greater extent than many of the other antipsychotics, thus resulting in breast tenderness, amenorrhea, and galactorrhea?

Back 501

Risperidone.

Front 502

Meckel's diverticulum is diagnosed by using what test?

Back 502

Technetium-99m pertechnate scan.

Front 503

A Barium enema is diagnostic and therapeutic in children with what acute condition?

Back 503

Intussusception.

Front 504

Jaundice that appears in a newborn AFTER the 3rd day and within the first week of life usually suggests what condition?

Back 504

Bacterial sepsis or UTI, and this requires prompt and aggressive evaluation; the usual clues of sepsis are a history of poor feeding, lethargy, vomiting, or alteration of usual activity; a patient with suspected diagnosis of sepsis should be investigated with blood cultures and lumbar puncture.

Front 505

Cervical lymphadenopathy; morbiliform truncal exanthem; indurative edema of the the extremities and desquamation of the finger and toe tips; erythema, fissuring and crusting of the lips, strawberry tongue, and diffuse mucosal injection; bulbar conjunctival injection; fever for greater than 5 days. Diagnosis?

Back 505

Kawasaki disease.

Front 506

What are the main differences in presentation between Kawasaki disease and Scarlet fever?

Back 506

Scarlet fever will have a positive streptococcal throat test and NORMAL APPEARING LIPS!, as opposed to negative streptococcal throat test and inflamed lips in Kawasaki disease.

Front 507

What is the main treatment in a child diagnosed with Scarlet fever?

Back 507

The treatment of choice for Scarlet Fever is a ten-day course of Penicillin C; in penicillin-allergic patients Erythromycin or Clindamycin can be used.

Front 508

Which is the only congenital heart disorder that does not predispose to endocarditis?

Back 508

ASD.

Front 509

This congenital heart disorder presents with both LVH and RVH is the defect is long-standing; there may be a systolic murmur in the left axilla or back; with long-standing occurrence there may also be notching of the ribs. Diagnosis?

Back 509

Coarctation of the aorta.

Front 510

This syndrome is characterized by hypoplastic nails, cleft palate, and Vitamin K deficiency, which may result in bleeding. Diagnosis?

Back 510

Fetal Hydantoin syndrome.

Front 511

Hypotonia, hypogonadism, obesity, short stature, narrow bifrontal diameter of face, diamond-shaped eyes, and a small, down-turned mouth. Diagnosis?

Back 511

Prader-Willi syndrome.

Front 512

This condition occurs in young adults and teenagers and is characterized by thrombocytopeina, microangiopathic hemolytic anemia (schistocytes), neurological disturbances, renal dysfunction and fever. Diagnosis?

Back 512

Thrombotic Thrombocytopenic Purpura.

Front 513

What should be suspected in patient who present with pharyngitis, moderate fever, dysphagia, neck stiffness, a muffled voice, and a posterior pharyngeal bulge?

Back 513

Retropharyngeal abscess.

Front 514

Hand-foot syndrome or dactylitis is the earliest manifestation of what condition in African-American patients; patients usually present at the age of 6 months to 2 years with an acute onset of pain and symmetric swelling of the hands an feet; fever is sometimes present?

Back 514

Vaso-occlusion in sickle cell anemia; therefore, any child presenting with this condition warrants a complete workup for sickle cell even if they are completely asymptomatic. The pathophysiology of dactylitis involves vascular necrosis of the metacarpals and metatarsals, which may be seen on plain radiographs as osteolytic lesions.

Front 515

What are the drugs of choice used for empiric treatment of Septic Arthritis after arthrocentesis?

Back 515

The most common cause of septic arthritis is Staph aureus. Therefore, the commonly used empirical antibiotics are nafcillin or cefazolin. Vancomycin should be used if the suspicion of MRSA is high.

Front 516

What condition should you suspect in an infant who presents with cyanosis that is aggravated by feeding and relieved by crying? How do you diagnose this condition? What is the first step in management?

Back 516

Choanal atresia!!! Failure to pass a catheter through the nose 3-4 cm into the oropharynx is suggestive of the diagnosis; the diagnosis is confirmed by CT scan with intranasal contrast, which shows a narrowing at the level of the pterygoid plate. The first step in management consists of placing an oral airway and lavage feeding, while definitive treatment involves repairing the obstruction with surgery or endoscopy.

Front 517

What is the usual cause of the holosystolic murmur associated with acute viral myocarditis?

Back 517

A holosystolic murmur may be identified secondary to dilated cardiomyopathy and the resulting functional mitral regurgitation.

Front 518

The workup for this pediatric illness include CBC with differential, sedimentation rate and C-reactive protein, cardiac enzymes, and blood and viral cultures; chest radiograph demonstartes cardiomegaly and pulmonary edema; EKG most commonly shows low voltage QRS and sinus tachycardia; ECG shows global hypokinesis, LVH, left ventricular dysfunction, and pericardial effusion. What is the gold standard for diagnosis of this disease?

Back 518

Myocardial biopsy, which allows for disease stage classification.