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420 Cards in this Set

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Organism that causes afebrile pneumonia in infancy
C trachomatis (also consider TB)
Innocent heart murmur that is vibratory, musical, systolic, increases with fever, and most commonly heard in children 2-7 yo
Still’s murmur
Innocent heart murmur usually heard in the newborn, systolic, heard best in axilla or back and disappears by several months of age
Peripheral Pulmonic Stenosis (PPS)
Antidote for phenothiazine toxicity
Diphenhydramine IV
Idiopathic adolescent scoliosis normally has a thoracic curvature to which side
Right
Mottling of the skin with venous prominence seen commonly in the healthy newborn
Cutis marmorata
Squatting, oxygen, morphine, and propranolol help with what type of spell
“Tet spell” before surgical repair of TOF: all these things help to decrease the right to left shunting across the VSD
Permanent deflection of 1 or more fingers
Clinodactyly
Embryonal tumor of renal origin associated w/ sporadic aniridia, hemihypertrophy, neurofibromatosis, Beckwith-Wiedemann syndrome, and GU tract anomalies
Wilms Tumor
Drug given for symptomatic bradycardia
Atropine
Mean of transmission for RSV
Direct contact
Increase urine output, hypernatremia, and dehydration are a result of?
Diabetes insipidus
Why are patients with renal failure anemic
Failing kidneys stop producing erythropoietin
Norwood, Fontan, and heart transplants are surgical procedures associated with what Congenital Heart Disease
Hypoplastic left heart syndrome
Possible complications to a newborn infant of a diabetic mother
Hypoglycemia, hypocalcemia, malformations (congenital heart disease or variants of caudal regression syndrome), polycythemia, renal vein thrombosis, or LGA
Bacterial cause of diarrhea can be associated with seizures secondary to neurotoxin production
Shigella
Microangiopathic hemolytic uremia, thrombocytopenia, and acute renal failure
HUS
Causes limp usually in very obese or very tall adolescents with a peak age range of 10-13 years. On PE, internal rotation of hip is limited, urgent situation
Slipped Capital Femoral Epiphysis
Slipped Capital Femoral Epiphysis
Disorder associated with hair loss due to compulsive pulling, twisting or breaking of the hair
Trichotillomania
Cephalosporin with the longest half life
Ceftriaxone
Causes of leukocoria in an infant
Congenital cataract, retinoblastoma, retinopathy of prematurity, congenital glaucoma, ocular Toxocariasis
Term for an increased distance between the orbits
Hypertelorism
Only CHD that does not require endocarditis prophylaxis
Secundum ASD
Self-limited condition that causes hip pain and limp in 3-10 year old, may follow a viral illness and more commonly in boys. Lab values are normal except ESR may be mildly elevated
Transient synovitis or toxic synovitis of the hip
Proximal tubular transport defects causing glycosuria, aminoaciduria, and phosphaturia; often associated with bicarbonate wasting, proteinuria and hyperkaluria
Fanconi Syndrome
Syndrome associated with LGA,, Hypoglycemia, hepatosplenomegaly macro-glossia, and omphalocele
Beckwith-Weidemann Syndrome
Most common renal malformation in newborns
Horseshoe kidney
Metabolic abnormalities seen in pyloric stenosis
Hypokalemic hypochloremic metaboic alkalosis
Cause of roseola
HHV-6
Name the syndrome in which as the result of congenital heart disease a right to left shunt is created in response to elevation of the pulmonary vascular resistance
Eisenmenger syndrome
Most common cause of sporadic acute encephalitis in the USA and its treatment
Herpes simplex
Acyclovir
Other conditions in the ifferential diagnosis of testicular torsion
Epididymitis
Torsion of the Appendix epididymitis
Torsion of the Appendix testes
Orchitis
Acute hydrocele
Name given for avascular necrosis of the femoral head that usually appears b/w ages 3-8
Legg-Calve-Perthes Disease
Excess of what vitamin can cause pseudotumor cerebri
Vitamin A
Rapidly fatigable weakness and double vision are seen in which disorder
Myasthenia Gravis
Reasoning to not usually treat Samonella gastroenteritis
It may prolong the "carrier state" and may not significantly alter the clinical course
In Guillian-Barre syndrome what happens to the CSF protein level
Usually becomes elevated within a few days of onset
Two most common organisms that cause bacterial endocarditis
Streptococcus viridans
Staphylcoccus auerus
A fixed split S2 is associated with which acyanotic congenital heart defect
ASD
At which age is an umbilical hernia likely to close
Usually close by age 3 to 5 years, but if defect is larger than 1.5 cm it is unlikely to close

Are 9x more common in African-American children
Treatment of choice in Wilson's disease
Copper Chelation with D-penicillamine
What tumor has increased incidence in Klinefelter's
Breast cancer
Patients with HSP are most likely to have a recurrence if they had what other manifestation
Nephritis
What is the normal renal calcium excretion
< 4 mg/kg/day or urinary calcium/creatinine ratio < 0.2
Most common cause of neonatal seizures
Hypoxic Ischemic Encephalopathy (HIE)
Condition in which infants are born with profound thrombocytopenia secondary to having different platelet antigens from their mother, with subsequent platelet destruction by maternal antiplatelet antibodies
Neonatal Alloimmune Thrombocytopenia
The usually benign, self-limited reddening of one side of the infant with a sharp line of demarcation at the midline possibly related to autonomic factors
Harlequin color change
Bounding pulses and a continuous murmur is associated with which CHD
PDA
What are the cardiac manifestations of hyperkalemia
First seen are peaked T waves, then prolonged PR intervals, flattened P waves, widened QRS complexes, and terminally seen is ventricular tachycardia and fibrillation
Sarcoma that develops in the bone marrow and is described as "small blue round cells" it occurs most often in male adolescents and usually affects the femur, humerus, ribs, and flat bones such as the scapula
Ewing sarcoma
Contraindications to breast feeding
Toxic medications, Maternal infection (HIV or active TB), abnormal swallowing in the infant, or maternal substance abuse
Treatment of choice for pinworms
Mebendazole (Vermox)
How long is a child infectious with chickenpox after the lesions erupt
Contagious for 1-4 days before the lesions erupt and usualy 7-10 days afterward
Drug of choice to close a PDA
Indomethacin
At what age can one be sure of permanent eye color in infants?
Between 6-12 months
Toxocara canis and catis cause which disorder
Viceral larva migrans
Name this disoder characterized by inflammation of the skin and muscle. Gottron papules, an eruption over the extensor surfaces of the knuckles, elbows, and knees and the heliotrop rash, which is purplish discoloration of the eyelids, are common findings
Dermatomyositis
Name of early morning hyperglycemia associated with rebound from severe nocturnal hypoglycemia
Somogyi
What is the primary symptom of short gut or short bowel syndrome
Diarrhea
- Short gut is usually caused by marotation with a midgut volvuus, small intestinal atresia, or necrotizing enterocolitis (NEC)
Name used to describe short digits or fingers
Brachydactyly
4 classic characterizations of Tetralogy of Fallot
VSD
Pulmonary Stenosis
Aortic override
Right ventricular hypertrophy (RVH)
An embryonal tumor of neural creast origin that is the second most common solid tumor of childhood is
Neuroblastoma
What is generally the length of time considered an apneic period of clinical importance
Greater than 20 seconds or are symptomatic
Rare renal complication of Impetigo
Post-streptococcal glomerulonephritis
What are the complications of an untreated undescended testicle
Infetility and increased incidence of cancer (Seminoma)
Syndrome that occurs in girls which is a progessive degenerative disorder marked by loss of purposful hand movement
Rett syndrome
Elongation and downward displacement of the medulla and cerebellum into the spinal canal with obstruction of the fourth ventricle, resulting in hydrocephalus is called?
Arnold-Chiari Malformation
4 year-old boy presents for a routine year physical exam. His PMH is significant for hypospadias. On PE he has a mildly elevated blood pressure and a painless abdominal mass. It is smooth, hard, and only on the left side of the abdomen. The U/A reveals sma of blood.
Wilm's tumor
What deficiency causes Christmas disease or Hemophilia B
Factor IX deficiency
"Ash leaf" hypopigmented macules and shagreen patches are seen in which AD disorder
Tuberous sclerosis
What condition may exist if a hematoma-like or bluish collection underneath the nail occurs without a history of trauma
Melanoma
This disorder can be classified by seizures, a facial hemangioma (port-wine stain), hemiparesis, intracranial calcifications, and even mental retardation
Sturge-Weber disease
Pastia's lines or dark lines that occur in skinfold areas are associated with which disease
Scarlet fever
Disorders associated with Hischsprung disease
Down syndrome
Waardenburg syndrome
Cartilage-hair hypoplasia
Neonatal appendicitis
What other tumor may be seen in patients with hereditary retinobastoma
Osteosarcomas
Corticosteroids are beneficial in HSP if it is associated with which manifestations?
Joint and abdominal pain

no benefit in treating nephritis
What syndrome is associated with exocrine pancreatic insufficiency, bone marrow hypoplasia, soft tissue infections, and growth retardation
Shwachman-Diamond syndrome
What does VACTERL stand for
Vertebral
Anorectal (imperforate anus)
Cardiac
Tracheal
Esophageal (TE fistula)
Renal/GU
Limb/Lumbar

Infnats need to be screened for all these is one is present
Name the bubbles of subserosal air in the bowel wall that is considered pathognomonic of necrotizing enterocolitis
Pneumatosis intestinalis
Presenting signs and symptoms of Biliary Atresia
Jaundice
Hepatomegaly
Acholic stools
Dark urine
Direct (conjugated) hyperbilirubinemia
Increased Alkaline phosphatase levels
Largest protozoan infecting man
Balantidium Coli
Incubation period for varicella or chickenpox
10-21 days with a mean of 14-16 days
Ameba that causes CNS infection via invasion through the cribiform plate to the subarachnoid space that lives in fresh water
Naegleria fowleri
After what age is pertussis vaccine no longer given
7 years
3 Stages of Pertussis
Catarrhal
Paroxysmal
Convalescent
Late complication of measles with an average incubation period of over 10 years
Subacute Sclerosing Panencephalitis (SSPE)
Viral cause of acute hemorrhagic cystitis in children
Adenovirus 11 and 21
What is the Nitro-Blue tetrazolium dye rxn test used to identify
Chronic Granuomatous Disease (Myeloperoxidase deficiency)
What mediates a Type IV Hypersensitivity reaction? Give example
Cell Mediated
- Graft Versus Host Disease
- Sarcoidosis
- Contact Dermatitis
- Tuberculin Skin Test
What mediates a Type I Hypersensitivity reaction? Give example
IgE Mediation
- Anaphalaxis to insect bite, food, or drug
Iron binding protein in human milk
Lactoferrin
Most common acquired thyroid disorder in children and adolescents
Autoimmune Thyroiditis (Hashimoto's)
5 Major Jones criteria of Acute Rheumatic Fever
Carditis
Polyarthritis
Sydenham chorea
Erythema marginatum
Subcutaneous nodules
Most common cause of third degree heart block in the newborn
Neonatal SLE
A baby is SGA. On exam she has midline facial hypoplasia, murmur, smooth philtrum with thin smooth upper lip, altered palmar crease patterns and small fifth fingernails. Dx?
Fetal Alcohol Syndrome
A progressive disease of the anterior horn cells usually inherited as an autosomal recessive trait
Spinal muscular atrophy (Type 1 is called Werdnig-Hoffman disease)
In looking for Fragile X, culture media should be deficient in what substance
Folate
What is the characteristic lesion seen on cardiac biopsy in Acute Rheumatic Fever
Aschoff bodies
A child is born with hypoplastic kidneys, bilaterial cryptorchidism, and absent abdominal wall musculature. What is this condition?
Prine Belly Syndrome
Eagle-Barrett Triad
What is considered neonatal oliguria in the first 2 days of life
Urine output of <15-20 cc/kg/day in the first 24-48 hours of life
Most common cause of delayed puberty in boys
Constitutional delay of puberty
A girl is discovered at puberty to have no menses or axillary hair. She is found to have a Y chromosome. What disorder is this?
Testicular feminization syndrome or Male pseudohermaphroditism
3 most common causes of intoeing
Metatarsus adductus
Tibial torsion
Femoral anteversion (inward femoral torsion)
Most common type of testicular tumor
Germ cell tumor
Boys of less than what Tanner stage should not participate in collision sports such as football or hockey with fully mature boys
4
What mediates a Type III Hypersensitivity reaction? Give examples
Immune Complex
- Autoimmune disease
- Serum sickness
- Arthus rxn
Enzyme lacking in hereditary angioedema
C1 esterase
Name 4 clotting factors that require vitamin K as a co-factor
II, VII, IX, X
Name laboratory findings of Iron Deficiency Anemia other than a low hemoglobin or hematocrit
Hypochromic/microcytic RBCs
Decrease reticulocytes, MCV, MCH, Iron, Ferritin, Transferrin saturation
Increased RDW, total iron binding capacity (TIBC), Free erythrocyte protoporphyrins (FEP)
What temp does the core body temp have to be below for hypothermia
36 C
96.8 F
Age that sperm production usually begins
Tanner stage III or 13-14 yo
A % body fat less than what number is considered unhealthy in Boys and Girls
Males: 7-20%
Females: 10-25%
- below these levels raises concern for eating disorder
Milk from which animal can lead to megaloblastic anemia secondary to folate deficienc
Goat
After what age is puberty considered delays in females and in males
If there has been no development of secondary sexual characteristics by age 13 in girls and 14 in males
Overdose with what substance can result in fever, hypoglycemia, respiratory alkalosis, tinnitus, and metabolic acidosis
Aspirin
Name the substance taken in an overdose that may be seen on abdominal radiograph
CHIPS:
- Chloral hydrate
- Heavy metals
- Iodides
- Phenothiazines
- Slow release capsules
Which renal tubular acidosis usually presents with hyperkalemia
Type 4
What % of neonates void in the first day of life
92% in the first day, 99% by 48 hours
At what preferred vertebral level should the tip of the umbilical artery catheter lie
Just above the bifurcation of aorta at the level of L3-5 or above the celiac axis at the level of T6-10
Name physical exam tests used in the evaluation for congenital hip dysplasia or developmental dysplasia of the hip
Galiazzi test
Ortolani test
Barlow maneuver
What does a suprasternal thrill indicate
Vascular aortic stenosis
Hepatomegaly, wheezing, and marked eosinophilia describe infection with what organism
Toxocara canis (viceral larva migrans)
Name the disorder caused by eating raw, infected pork
Trichinosis
Deafness, heterochromia of iris, and white forelock is seen in what syndrome
Waardenburg Syndrome
A 1-year old child has high spiking fevers for 6 days and you are worried about Kawasaki disease. You know the child needs 4 out of 5 clinical signs to make this diagnosis. What are the 5 signs?
Conjunctival injection
Oral mucosal changes
Peripheral extremity changes
Rash
Lymphadenopathy
Diarrhea and malabsorption secondary to zinc deficiency is called
Acrodermatitis enteropathica
This skin disorder is self-limited. It presents at birth with 1 to 2 mm pustules that disappear in 1 to 2 days leaving pigmented macules with a scale. The hyperpigmentation fades in 3 to 12 weeks. What is this disorder
Transient Neonatal Pustuar Melanosis (TNPM)
What are the most typical infectious manifestations of children infected with atypical mycobacteria
Cervical lymphadenitis
- M. avium is most common but also caused by M. scrofulaceum or M. kansasii
Name the congenital heart disease that can present with a "snowman" shape of the heart on CXR
Total Anomalous Pulmonary Venous Return (TAPVR) has this shape secondary to a vertical vein, large SVC, and an enlarged Right Atrium
At what preferred vertebral level should the tip of the umbilical vein catheter lie
At the junction of the Right atrium and IVC at the level of T10-T11
What the two common treatments for sickle cell vaso-occlusive crisis
Hydration
Pain control usually with narcotis
Name the chemotherapeutic agent used in the treatment of TB that can cause red-green color blindness or optic neuritis
Ethambutol
What condition is described by bullous skin lesions on palms/sole, osteochondritis, "snuffles", CN VIII deafness, and hepatosplenomegaly
Congenital syphilis
Name the disorder with a chronic ulcer with satellite lesion caused by a sandfly bite
Leishmaniasis
Most common polyps in children
Juvenile Inflammatory polyps
In infants, what is the normal number of stools per day
95% have b/w 1 stool every other day to 4 stools/day
Most common cause (not organism) of perianal abscess in an infant
Infectious diaper rash
Which Hepatitis B antigen correlates best with infectivity
Hepatitis Be Antigen (HBeAg)
In patients with celiac sprue that continue to ingest gluten the risk of developing which disorder is increased
Small bowel lymphoma
Most common subtentorial CNS tumor in children
Medulloblastoma
What neoplasm has the highest rate of metastasis from mother to fetus
Malignant Melanoma
Possible presentations of this tumor are abdominal mass (seen in >50%), respiratory distress, Horner syndrome, proptosis, bilateral orbital ecchymosis ("panda eyes"), paraplegia, opsoclonus, and nystagmus or "dancing-eye" syndrome
Neuroblastoma
Name the most common malignant bone tumor in children. It most commonly affects the distal femur, proxima tibia, and proximal humerus. It is more common in males and may present as persistant pain after a minor trauma
Osteogenic sarcoma
Useful tumor marks for this tumor are urine VMA, HVA, and metanephrine levels
Neuroblastoma
What bone in the body is most commonly effected by Ewing sarcoma
Femur - 22%
Ileum/Pelvis - 19%
Tibia - 11%
Humerus - 10%
How can you clinically distinguish yellowing of the skin b/w carotenemia and jaundice
Absence of yellowing in sclera and oral cavity with carotenemia
Molecular abnormality is Duchenne muscuar dystrophy
Abnormality (usually a partial gene deletion) of the dystrophin gene on the short arm of the X chromosome
Type of cardiac problem seen in pts with Lyme disease
Conduction defects or heart blocks
Primary medical treatment for transposition of the great arteries
Prostaglandin infusion to maintain patency of ductus arteriosus until septostomy or surgery
Most common lipid storage disease in humans and what defect is its cause
Gaucher Disease (Glucocerebrosidosis), decreased activity of beta-glucocerebrosidase
Prenatal onset of joint contactures is called
Arthrogryposis
Three most common CNS malignancies of children
Astrocytomas
Medulloblastoma
Ependymomas
2 chemotherapeutic agents that can cause pulmonary fibrosis
Bleomycin
Methotrexate
What tumor in children has the quickest doubling time
Burkitt's lymphoma
Disorders that predispose a child to intussusception
CF
HSP
Meckel diverticulum
Juvenile inflammatory polyps
Ascaris lumbricoides infestation
In patients with celiac sprue what is substituted in the diet for wheat, oats, rye, and barley
Corn and rice
Herniation of the bowel through the abdominal wall 2-4 cm lateral to the umbilicus is called
Gastroschisis
What substance is deficient in SCID
Adenosine deaminase
What cyanotic congenital heart lesion presents with tachypnea, tachycardia, holosystolic murmur and a oud single S2
Truncus arterosus
Enzyme deficient in Lesch-Nyhan Syndrome
Hypoxanthine Guanine Phosphoribosyl Transferase (HGPRT)
What antibiotic and for what duration is given to individuals exposed to Pertussis
Erythromycin for 14 days
What is the most effective treatment for Sporotrichosis
KI (potassium iodide)
Name this Disorder. It is a process in which a small segment of subchondral bone becomes avascular of unclear etiology. It may produce locking of the knee. Radiographs of the knee typically shows involvment of the lateral surface of the media femoral condyle
Osteochondritis Dissecans
Surgical procedure to treat Biliary Atresia
Kasai portoenterostomy
Hypercalcemia, mental retardation, and supravalvular aortic stenosis are all part of which syndrome
Williams syndrome
Term, 5-lb, 2-day-old infant has irritability, nasal stuffiness, and coarse tremors. He feeds poorly and has diarrhea
Cocaine or Heroin withdrawal
Three-week-old infant presents with paroxysmal cough and tachypnea, but no fever; bilateral diffuse crackles, hyperinflation, and patchy infiltrates on x-ray; had conjunctivitis at 10 days of age
Think: Chlamydia trachomatis
Post-mature, 41-week gestational age newborn on first day of life has grunting respirations, signs of air trapping, and RR 100/min
Think: Meconium aspiration
6-day-old, 2 lb. neonate develops episodes of apnea, abdominal distention, and bloody diarrhea
Think: Necrotizing Enterocolitis
A child smiles spontaneously, babbles, sits without support, reaches, feeds herself a cookie but has no pincer grasp. What is her approximate age?
Think: 8-9 months (pincer grasp at 10 months)
An 18-month-old infant brought in for temper tantrums has normal gross & fine motor skills but lacks language development and is cooperative and alert on exam
Think: Hearing loss
A 14-month-old infant presents with anorexia, pruritus, and failure to gain weight; has a bulging anterior fontanelles and tender swelling over both tibias. Mother buys all foods at a natural foods store.
Think: Hypervitaminosis A
A female infant has slanted palpebral fissures, epicanthal folds, and some delayed development
Think: Down’s syndrome
A newborn infant has lymphedema of the hands and feet, extra skin folds at a short neck, widely spaced nipples, and decreased femoral pulses
Think: Gonadal dysgenesis (45, X) Turner’s syndrome, and do a chromosomal analysis to confirm the diagnosis
Male adolescent presents with hematuria, proteinuria, and decreased hearing
Think: Alport’s syndrome
A 3-month-old, breast-fed infant has FTT severe hepatomegaly, thin extremities, fasting hypoglycemia lipemia and metabolic acidosis
von Gierke’s
A 2-week-old neonate has jaundice, hepatomegaly and positive urinary-reducing substance. Odor of urine is normal
Galactosemia
For the past 2 weeks, a 6-year-old boy has had aggressive edema at various sites – puffy cheeks and eyes on awakening and swelling of the feet and abdomen as the day progresses. His history includes upper respiratory illness and a sting by a yellow jacket
Serum sickness
A 10-year-old girl has persistent, unresponsive infection of the oral cavity and nails
Chronic mucocutaneous candidiasis
Triad of thrombocytopenia, eczema, and recurrent bacterial infections in males
Wiskott-Aldrich syndrome
A 2-month-old infant with congenital heart disease is hospitalized with cough and tachypnea. X-ray films of the chest show diffuse infiltrates and no thymic shadow. Serum calcium is 6.5 mg/dl (low)
DiGeorge’s syndrome
A 14-year-old boy has had lifelong skin infections. His leukocytes are unable to reduce nitroblue tetrazolium
Chronic Granulomatous disease
A 5-year-old boy present with sudden onset of high fever and reddish-purple spots. He is rapidly progressing to shock
Meningococcemia
A 2-year-old boy is brought in by his mother for evaluation of a rash. She is worried because for the last 3 days the boy has had a high fever. Today his fever has completely resolved, but he broke out into a diffuse rash
Roseola infantum
A 10-year-old boy who originally presented with fever and swollen parotid gland presents 8 days later with a swollen tender testis
mumps orchitis
A 10-year-old boy steps on a dirty nail that punctures his foot through his sneaker
Wound likely to become infected with Pseudomonas
Signs of FTT: SMALL KID
Subcutaneous fat loss
Muscle atrophy
Alopecia
Lagging behind norms
Lethargy
Kwashiorkor/marasmus
Infection
Dermatitis
Developmental Milestones at 1, 2, 3, & 4 years old
- At age 1, a child uses 1 word, follows a 1-step command
- At age 2, a child uses 2 to 3 word phrases, & follows 2-step commands, & others can understand 1/2 of the child’s language
- At age 3, a child uses 3 word sentences, & others can understand 3/4 of the child’s language
- At age 4, a child should be 40 lbs & 40 inches tall and should be able to draw a 4-sided figure
Breast Feeding Jaundice vs Breast Milk Jaundice
breast Feeding jaundice occurs in the First week;
- breast Milk jaundice occurs Many weeks later
You know a patient is dehydrated when he or she is PARCHED
PARCHED
Pee, Pressure (blood)
Anterior fontanelle
Refill, capillary
Crying
Heart rate
Elasticity of skin
Dryness of mucus membranes
Skeletal injuries are suspicious of abuse:
“Some Parents Are Maliciously Mean”
“Some Parents Are Maliciously Mean”
- Spinal fractures of lower extremities in nonambulatory children
- Posterior rib fractures (usually caused by squeezing the chest)
- Age (fractures of different Age)
- Metaphyseal “chip” fractures (usually caused by wrenching)
- Multiple fractures
CNS injuries suspicious of abuse:
“Mothers, Refuse Shaking!”
“Mothers, Refuse Shaking!”
- Metaphyseal fractures
- Retinal hemorrhages,
- Subdural hematoma
Sturge-Weber syndrome: hallmark features
Sturge-Weber:
- Seizures
- portWine stain
Guthrie card: diseases identified with it
"Guthrie Cards Can Help Predict Bad Metabolism":
- Galactosaemia
- Cystic fibrosis
- Congenital adrenal hyperplasia
- Hypothyroidism
- Phenylketonuria
- Biotidinase deficiency
- Maple syrup urine disease
Croup: Symptoms
"3 S's"
- Stridor
- Subglottic swelling
- Seal-bark cough
Ataxia-Telangiectasia (AT): common sign
AT
- Absent
- Thymus
Duodenal atresia vs. Pyloric stenosis: site of obstruction
- Duodenal Atresia: Distal to Ampulla of vater.
- Pyloric stenosis: Proximal to it
Bilirubin: phototherapy
BiLirUbin absorbs light maximally in the BLUe range.
Williams syndrome: features
"WILLIAMS"
WILLIAMS:
- Weight (low at birth, slow to gain)
- Iris (stellate iris)
- Long philtrum
- Large mouth
- Increased Ca++
- Aortic stenosis (and other stenoses)
- Mental retardation
- Swelling around eyes (periorbital puffiness)
Russell Silver syndrome: features
"ABCDEF"
ABCDEF:
- Asymmetric limb (hemihypertrophy)
- Bossing (frontal)
- Clinodactyly/ Cafe au lait spots
- Dwarf (short stature)
- Excretion (GU malformation)
- Face (triangular face, micrognathia)
Dentition: eruption times of permanent dentition
"Mama Is In Pain, Papa Can Make Medicine":
"Mama Is In Pain, Papa Can Make Medicine":
- 1st Molar: 6 years
- 1st Incisor: 7 years
- 2nd Incisor: 8 years
- 1st Premolar: 9 years
- 2nd Premolar: 10 years
- Canine: 11 years
- 2nd Molar: 12 years
- 3rd Molar: 18-25 years
Cyanotic heart diseases: 5 types
"Use your five fingers"
Use your five fingers:
- 1 finger up: Truncus Arteriosus (1 vessel)
- 2 fingers up: Dextroposition of the Great Arteries (2 vessels transposed)
- 3 fingers up: Tricuspid Atresia (3=Tri)
- 4 fingers up: Tetralogy of Fallot (4=Tetra)
- 5 fingers up: Total Anomalous Pulmonary Venous Return (5=5 words)
Head circumference with age
"Remember 3, 9, and multiples of 5"
Remember 3, 9, and multiples of 5:
- Newborn 35 cm
- 3 mos 40 cm
- 9 mos 45 cm
- 3 yrs 50 cm
- 9 yrs 55 cm
Weights of children with age
- Newborn 3 kg
- 6 mos 6 kg (2x birth wt at 6 mos)
- 1 yr 10 kg (3x birth wt at 1 yr)
- 3 yrs 15 kg (odd yrs, add 5 kg until 11 yrs)
- 5 yrs 20 kg
- 7 yrs 25 kg
- 9 yrs 30 kg
- 11 yrs 35 kg (add 10 kg thereafter)
- 13 yrs 45 kg
- 15 yrs 55 kg
- 17 yrs 65 kg
Hemolytic-Uremic Syndrome (HUS): components
"remember to decrease the RATE of IV fluids in these patients":
"remember to decrease the RATE of IV fluids in these patients":
- Renal failure
- Anemia (microangiopathic, hemolytic)
- Thrombocytopenia
- Encephalopathy (TTP)
Cough (chronic): differential
When cough in nursery, rock the "CRADLE":
When cough in nursery, rock the "CRADLE":
- Cystic fibrosis
- Rings, slings, and airway things (tracheal rings)/ Respiratory infections
- Aspiration (swallowing dysfunction, TE fistula, gastroesphageal reflux)
- Dyskinetic cilia
- Lung, airway, and vascular malformations (tracheomalacia, vocal cord dysfunction)
- Edema (heart failure)
Cystic fibrosis: presenting signs
"CF PANCREAS"
CF PANCREAS:
- Chronic cough and wheezing
- Failure to thrive
- Pancreatic insufficiency (symptoms of malabsorption like steatorrhea)
- Alkalosis and hypotonic dehydration
- Neonatal intestinal obstruction (meconium ileus)/ Nasal polyps
- Clubbing of fingers/ Chest radiograph with characteristic changes
- Rectal prolapse
- Electrolyte elevation in sweat, salty skin
- Absence or congenital atresia of vas deferens
- Sputum with Staph or Pseudomonas (mucoid)
Cystic fibrosis: exacerbation of pulmonary infection
"CF PANCREAS"
"CF PANCREAS"
- Cough (increase in intensity and frequent spells)
- Fever (usually low grade, unless severe bronchopneumonia is present)
- Pulmonary function deterioration
- Appetite decrease
- Nutrition, weight loss
- CBC (leukocytosis with left shift)
- Radiograph (increase overaeration, peribronchial thickening, mucus plugging)
- Exam (rales or wheezing in previously clear areas, tachypnea, retractions)
- Activity (decreased, impaired exercise intolerance, increased absenteeism)
- Sputum (becomes darker, thicker, and more abundant, forming plugs)
Pyloric stenosis (congential): presentation
"Pyloric stenosis is 3 P's"
"Pyloric stenosis is 3 P's"
- Palpable mass
- Peristalsis visible
- Projectile vomiting (2-4 weeks after birth)
Breast feeding: benefits
"ABCDEFGH"
"ABCDEFGH"
Infant:
- Allergic condition reduced
- Best food for infant
- Close relationship with mother
- Development of IQ, jaws, mouth
Mother:
- Econmical
- Fitness: quick return to pre-pregnancy body shape
- Guards against cancer: breast, ovary, uterus
- Hemorrhage (postpartum) reduced
Perez reflex
Eliciting the PErEz reflex will make the baby PEE.
WAGR syndrome: components
"WAGR"
"WAGR"
- Wilm's tumor
- Aniridia
- Gential abnormalities
- Mental retardation
Hematuria: differential in children
"ABCDEFGHIJK"
"ABCDEFGHIJK"
- Anatomy (cysts, etc)
- Bladder (cystitis)
- Cancer (Wilm's tumour)
- Drug related (cyclophosphamide)
- Exercise induced
- Factitious (Munchausen by proxy)
- Glomerulonephritis
- Hematology (bleeding disorder, sickle cell)
- Infection (UTI)
- In Jury (trauma)
- Kidney stones (hypercalciuria)
Vitamin toxicities: neonatal
"excess vitamin A, E, K"
excess vitamin A
- Anomalies (teratogenic)
excess vitamin E:
- Enterocolitis (necrotizing enterocolitis)
excess vitamin K
- Kernicterus (hemolysis)
Rubella: congenital signs
"Rubber Ducky, I'm so blue!"
"Rubber Ducky, I'm so blue!"
- Rubber: Rubella
- Ducky: Patent Ductus Arteriosus, VSD and pulmonary artery stenosis.
- I'm: Eyes (cataracts, retinopathy, micropthalmia, glaucoma).
- Blue: "Blueberry Muffin" rash (extramedullary hematopoesis in skin +purpura)
--> Also, deafness, growth retardation, and some more.
Pediatric milestones in development
"1, 2, 3, 4"
1 year:
- 1 (single) words
2 years:
- 2 word sentences
- understands 2 step commands
3 years:
- 3 word combos
- repeats 3 digits
- rides TRIcycle
4 years:
- draws square (4 sides)
- counts 4 objects
Gastroschisis: usual location
"GasTRoscHIsis "
GasTRoscHIsis usually occurs on the RIGHT side of the umbilicus.
(Unscramble the letters).
Milk protein: women vs. cows
Woman: Whey (mostly)
Cow: Casein (mostly)
Short stature: differential
"ABCDEFG"
"ABCDEFG"
- Alone (neglected infant)
- Bone dysplasias (rickets, scoliosis, mucopolysaccharidoses)
- Chromosomal (Turner's, Down's)
- Delayed growth
- Endocrine (low growth hormone, Cushing's, hypothyroid)
- Familial
- GI malabsorption (celiac, Crohn's)
Breast feeding: contraindicated drugs
"BREAST"
"BREAST"
- Bromocriptine/ Benzodiazepines
- Radioactive isotopes/ Rizatriptan
- Ergotamine/ Ethosuximide
- Amiodarone/ Amphetamines
- Stimulant laxatives/ Sex hormones
- Tetracycline/ Tretinoin
APGAR score components
"APGAR"
"APGAR"
- Appearance: cyanosis--peripheral, central, none
- Pulse: pulse rate
- Grimace: response to stimulation
- Activity: movement of the baby (muscle tone)
- Respiration: respiratory rate
Beckwith-Widemann syndrome: features
"HOMO"
"HOMO"
- Hypoglycemia
- Omphalocele
- Macroglossia/ Macrosomia
- Organomegaly
Neonatal resuscitation: successive steps
"Do What Pediatricians Say To, Or Be Inviting Costly Malpractice"
"Do What Pediatricians Say To, Or Be Inviting Costly Malpractice"
- Drying
- Warming
- Positioning
- Suctioning
- Tactile stimulation
- Oxygen
- Bagging
- Intubate endotracheally
- Chest compressions
- Medications
AR disorder resulting in panctyopenia; common signs include
hyperpigmentation, Cafe au lait spots, Microcephaly, Microophthalmia, short stature, horseshoe or absent kidney, and absent thumbs
Fanconi anemia
Physical exam findings of Hyperpigmentation, blocked lacrimal gland, and dystrophic nails
Dyskeratosis congenita
Abnormal neutrophil fcn resulting in increased susceptibility to infection of skin & respiratory tract (s. aureus, beta-hemolytic strep), mild bleeding diathesis, Partial oculocutaneous albinism, progressive peripheral neuropathy, lymphoma-like syndrome, pancytopenia
Chediak-Higashi syndrome (AR)
Frontal bossing occurs with what types of anemias
Thalassemia minor
Severe iron deficiency
Chronic subdural hematoma
AD & AR, present with mild macrocytosis & reticulocytopenia, on electrophoresis there is elevated HbF and fetal i antigen is present on red cells; Associated congenital anomalies include short stature, web neck, cleft lip, Shield chest or widespread nipples, and Triphalangeal thumb; increased risk of leukemia later in life
Dianomd-Blackfan syndrome
- Congenital macrocytic pure red cell aplasia
Most common causes of Hypochromic Microcytic anemias
Iron deficiency anemias
Thalassemia syndromes
Anemia of Chronic disease
Physical exam findings of Skin and mucus membrane pallor, glossitis, angular stomatitis, koilonychia (spoon nails), tachycardia and a systolic ejection murmur at the left upper sternal border
Moderate Iron deficiency anemia (Hb 6-8/dL)
Signs of Congestive Heart failure, which include tachycardia, S3 gallop, cardiomegaly, hepatomegaly, distended neck veins, and pulmonary rales in what type of anemia
Severe Iron deficiency anemia (Hb < 3/dL)
These patients suffer from pancytopenia, and bone marrow aspirate reveals hypocellular marrow
Severe Aplastic Anemia
Profound defect in B lymphocytes causing increased susceptibility to infections with Streptococcus, H. fu, and echovirus meningoencephalitis, hypoplasia of tonsils, adenoids, and peripheral lymph nodes
Bruton's Congenital Agammaglobulinemia (X-linked)
Disorder of phagocytosis resulting in a defect in generation of microbial oxygen metabolites resulting in severe recurrent infections of skin and lymph nodes, Pneumonitis --> Pneumatocele, osteomyelitis, and hepatosplenomegaly
Chronic Granulomatous disease
Condition diagnosed by decreased IgM antibodies, alternative complement pathway, and tuftsin, Increased requirements for opsonic Abbs, Howell-Jolly bodies in erythrocytes
Asplenia
Most likely causes of sepsis in patients with asplenia
0-6 months: gram-negative enteric (Klebsiella, E. coli)
After 6 months: S. pneumoniae, H. flu type B, Neisseria meningitdis
Neutrophil chemotactic defect resulting in recurrent staph infections, resistance to therapy, pruritic eczematoid dermatitis, and coarse facial features
Job Syndrome (Hyper IgE)
Rash consisting of 2-6 mm pink initially blanchable macules that first appear peripherally on wrists, forearms, ankles, palms, and soles. Within 6-18 hrs the exanthem spreads centrally to the trunk, proximal extremities, and faces. Within 1-3 days the macules evolve to deep red papules & w/in 2-4 d exanthem is hemorrhagic & no longer blanchable
RMSF
Reflux, back arching, stiffness, and torticollis are components of what syndrome
Sandifer's syndrome
An 8-month-old infant has been hospitalized for 4 months in the ICU. In the last 2 weeks, the nurses have noted that he is regurgitating several times an hour. He makes chewing movements preceding these episode of regurgitation
Rumination
What is the most common cause of of acute intestinal obstruction under 2 years and age and where does it occur
Intussusception (most common cause of bowel obstruction in children age 2 month - 5 years)
Most common site is ileocolic (90%)
A 4-year-old girl has not had a bowel movement for a week and this has been a recurrent problem. Various laxatives and enemas have been tried in the past. Prior to toilet training, the girl had one BM/day. PE is normal except for the presence of stool in the sigmoid colon and hard stool on the rectal exam. After removing the impaction, what is the appropriate next step
Administer mineral oil or stool softners
Absence of Meissner's and Auerbach's plexus in distal colon leading to large bowel obstruction
Hirschsprung's
A well-nourished 3 month-old infant is brought to the emergency department because of constipation, blood-streaked stools, and excessive crying on defication
Anal fissure
A 14-year-old girl has a 2-month history of crampy and diffuse abdominal pain with anorexia and a 4.5-kg weight loss. The pain is unrelated to meals, and there is no diarrhea or constipation. Appropriate initial management would include?
- Rectal Exam
- Stool exam for ova, cysts, and parasites
- CBC and ESR;
- Review of family emotional stress
- Do not refer to an eating disorder clinic
Diarrhea + Emesis =

Diarrhea + Fever =

Diarrhea + Tenesmus =
Noninflammatory (emesis, afebrile, crampy ab pain, water)

Inflammatory process (fever, severe ab pain, tenesmus, blood/mucus stool)

Large colon involvement
A 9-month-old infant who attends day care has a temp of 104 F (40 C) rectally and diarrhea x 2 days. The stools are blood-streaked and contain mucus. WBC count is 23,000 with 40% segmented neutrophils, and 20% band forms. 60 minutes earlier, pt had a breif generalized seizure. PE & neuro exam are normal
Shigella sonnei
Why is E. coli 0157:H7 not treated with antibiotics?
There is a higher incidence of hemolytic uremic syndrome with treatment
A 15-year-old girl with spots on her lips has some crampy abdominal pain associated with bleeing
Peutz-Jeghers syndrome
A 5-year-old girl present with a protuberant abdomen and wasted extremities
Gluten-induced enteropathy
Signs and symptoms include fever, malaise, and watery diarrhea acutely; After 1 week, chronic malabsorption and signs of malnutrition including night blindness, glossitis, stomatitis, cheilosis, muscle wasting
Tropical sprue
Indirect hyperbilirubinemia, reticulosis, and red cell destruction suggest
Hemolysis
What can Direct hyperbilirubinemia indicate
Hepatitis
Cholestasis
Inborn errors of metabolism
CF
Sepsis
Stools pale yellow, persistance of increased levels of indirect bilirubin after 1st week of life in absence of hemolysis
Crigler-Najjar I Syndrome (AR, secondary to mutations in glucuronyl transferase)
Fatal within 6-12 months, severe generalized hypotonia, impaired neurologic fcn w/ psychomotor retardation, abnml head & unusual facies, hepatomegaly, renal cortical cysts, ocular abnormalitis, congenital diaphragmatic hernia, absence of eroxisomes in hepatic cells
Zellweger syndrome (rare AR, progressive degeneration of liver & kidneys)
A 10-year-old boy is diagnosed with acute hepatitis A. How would you treat the parents and siblings who are doing fine?
IV immunoglobulin
What is the most likely clinical manifestation of Alpha1-Antitrysin deficiency in the newborn
Jaundice (neonatal cholestasi)
What should you consider ordering in any patient with an unexplained elevation of LFTs?
Serum Ceruloplasmin
(Low in Wilson's disease, High serum copper level)
A 7-year-old girl is well when she leaves for school but arrives home afterwards with a sore throat and runny nose
Rhinovirus
A 17-year-old sexually active adolescent has acute onset of fever, cough, conjunctivitis, and pharyngitis
Adenovirus
What is the best treatment for the common cold?
Increase oral fluids, not pharmacologic treatment
Normal Respiratory rate at
Birth-6 weeks:
6 weeks-2 years:
2-6 years:
6-10 years:
Over 10 years:
Birth-6 weeks: 45-60/min
6 weeks-2 years: 40/min
2-6 years: 30/min
6-10 years: 25/min
Over 10 years: 20/min
Respiratory infection with Barking cough, steeple sign
Croup (parainfluenza virus)
Respiratory infection with tripod position (hyperextended neck, leaning forward, mouth open), thumb sign (swollen epiglottis on x-ray)
Epiglottitis (s. pneumoniae, h. flu type B)
Small, firm, white, cold patches of skin in exposed areas in cold weather
Frostnip
Condition in which tissue is frozen and distroyed. There is initial stinging, followed by aching, culminating in numb areas. Once rewarmed the area becomes red, blotchy, and painful
Frostbite
Small, ulcerated lesions on areas exposed to the cold such as the eats and fingers
Chilblains
Destruction of fat cells caused by exposure to cold weather or a cold object
Cold panniculitis
What type of vitamen deficiency can present with cheilosis, glossitis, a variety of ocular problems (keratitis, conjunctivitis, and corneal vascularization) and seborrheic dermatitis?
Riboflavin deficiency
What type of vitamen deficiency can present with seizures, periheral neuritis, dermatitis, and microcytic anemia?
Vitamin B6 (Pyridoxine)
What type of vitamen deficiency can present with pellagra
Niacin
What type of vitamen deficiency can present with dermatitis/sebirrhea
Biotin
Syndrome that causes catlike cry caused by abnormal laryngeal development, profound MR, self-injury behavior, hypersensitivity to sound, & repetitive behaviors
Cri du chat syndrome (deletion of short arm on chromosome 5)
Features include bushy eyebrows, hirsutism, limb defects, VSD, and MR
Corneilia de Lange
Presents with focal bone tenderness and fever
Osteomyelitis
Present with a painful limp, affecting children from 2-6 years old, self-limited disorder
Transient synovitis
May present as a painless limp, or a limp and pain in the anterior thight, groin, or knee, boys b/w 2-12 are most frequent affected (mean of 6-7 yo)
Legg-Calve-Perthes disease (avascular necrosis or idiopathic osteonecrosis of the femoral head)
poisoning causes metabolic acidosis and respiratory alkalosis, hyperglycemia or hypoglycemia, paradoxical aciduria, dehydration, and lethargy
Ingestion of? Antidote?
Salicylate poisoning

Antidote: Acetazolamide and IV Sodium bicarbonate; Hemodialysis can also be used
Ingestion can cause an idiosyncratic reaction causing extrapyramidal symptoms such as oculogyric crisis, tremors, torticollis, opisthotonus, and dysphagia
Ingestion of? Antidote?
Phenathiazine

Antidote: IV or IM Diphenhydramine
Antidote for heavy metal ingestion
Dimercaprol (BAL)
Excessive intake of this vitamin can cause hyperostosis (excess bone growth), hepatomegaly, increased CSF pressure, and drying of skin
Vitamin A
Excessive intake of this vitamin can cause skin flushing and pruritus, long-term can cause tachycardia, liver damage, hyperglycemia, and hyperuricemia
Nicotinic acid (a vasodilator)
Excessive intake of this vitamin can cause kidney stones, diarrhea, and cramps
Vitamin C
Prolonged excessive intake of this vitamin can cause nausea, diarrhea, weight loss, polyuria, and soft-tissue calcification of heart, kidney, blood vessels, bronchii, and stomach
Vitamin D
Excessive intake of this vitamin can cause sensory neuropathy with altered sensation of touch, pain, and fever
Pyridoxine (vitamin B6)
Teratogen associated with Ebstein anomaly
Lithium early in pregnancy
Teratogen associated with renal dysgenesis, oligohydramnios, and skull ossification defect
ACE inhibitors
Teratogen associated with fetal death, hydrocephalus, CNS defecrs, microtia/anotia, small or missing thymus, conotruncal heart defect, and micrognathia
Isoretinoin
Maternal illness associated with infants borns with deafness, cataracts, MR, and heart defects
Rubella in first trimester --> Congenital Rubella syndrome
Treatment for seborrheic dermatitis
Mild cleansing cream (anti-seborrheic shampoo), topical moisturizers, and topical steroids
Most common congenital cardiac malformation, result in left-to-right shunt, loud systolic murmur at LLSB can result from turbulent blood flow
VSD
What vitamin supplementation is need in patients with hemolytic anemias (i.e. sickle cell) because of ongoing compensatory erythropoiesis
Folate, to supply the need of raiidly dividing RBC precursors
A child with frequent abscess liver abscesses should have what diagnostic test
Nitroblue Tetrazolium to help ID phagocytic-cell defects (i.e. Chronic Granulomatous disease)
What should be considered in a child with severe eczema, thrombocytoenia, and unusual infections
Wiskott-Aldrich syndrome
What diagnostic test is used that will show no response in a patient with T-cell deficiencies (i.e. DiGeorge)
Intradermal skin test using Candida albicans
Condition that results in Howell-Jolly bodies and increased risk for encapsulated organism infections (i.e. Neisseria meningitidis, Streptococcus pneumoniae)
Asplenia
An 18-month-old boy with inspiratory stridor and a barking cough and agitation when lying down is brought at night to the ED by parents. He has steeple sign on x-ray
Croup
Most common infectious cause of acute upper airway obstruction
Croup
MCC of stridor in a febrile child
Croup
Most common pathogen causing Bronchiolitis with paroxysmal wheezing
RSV (Parainfluenza type 3)
Most common pathogen causing pharyngitis with sore throat and tonsillar involvement
Viral and Group A strep
Most common pathogen causing bacterial pneumonia with productive cough and lobar consolidation
S. pneumoniae
Most common pathogen causing pulmonary abscess (cavity with air-fluid level)
S. auerus
Corticosteroids are given to febrile child with stridor for what reasons
Croup (Acute Laryngotracheobronchitis)
Epiglottitis
Retropharyngeal abscess
Bacterial Trachieitis

- NOT indicated in spasmodic croup (Laryngismus Stridulus, Midnight Croup)
A 4-year-old boy is brought to the ED is flushed, making high-pitched noises on forced inspiration, leaning forward in his mother's lap, and drooling; x-ray shows thumb sign
Epiglottitis, and get him to an OR to intubate and treat
A previously healthy 4-month-old who had rhinorrhea, cough, and a low-grade fever develops tachypnea, mild hypoxemia, and hyperinflation of the lungs
RSV bronchiolitis
A 7-year-old boy with URI occasionally has black, tarry, foul-smelling stools, but is otherwise healthy. X-ray shows two discrete densities located in the right uper lobe of the lungs
Bronchiectasis (abnormal & permanent dilatation of bronchi)
Etiologies:
- Viruses (adeno-, influenza)
- Bacteria (S. aureus, Klebsiella, anaerobes)
- Primary ciliary dyskinesia
- Kartagener's
- CF (p. aeruginose)
- Alpha1-antitrypsin deficincy
What is the drug of choice for Group A Beta Hemolytic Strep
Penicillin
What is the most reliable sign of pneumonia
Tachypnea
In pharyngitis, the more mucous membranes, the more likely the infection is?
Viral
What should be considered in children with neck stiffness or acute abdominal pain
Pneumonia
Pneumonia with hilar adenopathy on CXR
Adenovirus
CXR in viral pneumonia: Hyperinflation, perihilar infiltrate, & atelectasis
Round pulmonary infiltrate on CXR
S. pneumoniae pneumonia
CXR in bacterial pneumonia: Alveolar consolidation
Type of Pneumonia based on CXR
- Hyperinflation, perihilar infiltrate, hilar adenopathy & atelectasis
- Alveolar consolidation
- Interstitial infiltrates
- Hilar adenopathy
- Reticulonodular infiltrates
- Hyperinflation, perihilar infiltrate, hilar adenopathy & atelectasis --> Viral
- Alveolar consolidation --> Bacterial
- Interstitial infiltrates --> Mycoplasma
- Hilar adenopathy --> TB
- Reticulonodular infiltrates --> Pneumocystis
A 2-month-old wuth fever, tachypnea, and mottled skin has a CXR showing infiltrate in the right upper lung lobe, a pneumatocele, and a pleural infusion
S. aureus pneumonia
A previously healthy 9-year-old boy has a 7-day history of increasing cough, low-grade fever, and fatigue on exertion. CXR shows widespread diffuse perihilar infiltrates
Mycoplasma pneumonia
A 3-year-old has had six episodes of pneumonia with Pseudomonas being isolated from sputum; loose stools; and is at the 20th percentile for growth
CF
Causes of false-positive sweat test (not CF)
Nephrogenic diabetes insipidus
Myxedema
Mucopolysaccharidosis
Adrenal Insufficiency
Ectodermal dysplasia
Features of CF:
CF PANCREAS
CF PANCREAS
- Chronic cough
- Failure to thrive
- Pancreatic insufficiency
- Alkalosis
- Nasal polyps
- Clubbing
- Rectal prolapse
- Electrolytes increased in sweat
- Absence of vas
- Sputum mucoid
Fat-soluble vitamin deficiencies
A - night blindness
D - decreased bone density
E - neurologic dysfunction
K - bleeding
A 5-year-old boy with a history of sleeping problems presents with a nonproductive nocturnal cough and shortness of breath and cough during exercise
Asthma, and start on a trial of a bronchodilatory
Percussion of lung fields:
- Hyperresonant:
- Dull:
- Hyperresonant: overinflation
- Dull: atelectasis
a 2-year-old boy is brought to the ED with the acute onset of audible wheezing. His RR is 24, and he has mild intercostal retractions. His babysitter found him playing in his room
Foreign body aspiration
A previously healthy 12-year-old boy develops pneumonia with consolidation of the right lower lobe on three different occasions in 6 months
Aspiration of a foreign body
Low Apgar scores, meconium staining, and ensuing respiratory distress suggest asphyxia occured, what happen during a period of asphyxia in the neonate?
The resulting hypoxemia, acidosis, and poor perfusion can damage the neonate's:
- Brain: cerebral edema, irritability, seizures
- Heart: cardiomegaly, heart failure
- Kidney: renal failure
- Liver: poor liver fcn, disseminated intravascular coagulopathy
- Lungs: respiratory distress syndrome, there can be excessively high pulmonary arterial pressure at the same time systemic BP begins to fall resulting in a persistant R --> L shunt across a PDA or FO
What is likely to be insufficient in breast milk for catch-up growth in a premature infant
Calcium and Phosphorus
Neonate presenting with feeding intolerance & a distended abdomen, 25% have grossly bloody stool, pneumatosis intestinalis on abdominal x-ray
Necrotizing Enterocolitis
(pneumatosis intestinalis = distended loops of bowel with air in bowel wall on x-ray)
Pattern of respiration in low-birth-weight babies, characterized by recurrent breathing pauses of 3-10 sec
Periodic breathing
This condition, if not ID'd by newborn state screen can present with jaundice, hepatomegaly, vomiting, hypoglycemia, convulsions, lethargy, irritability, feeding problems, poor weight gain, aminoaciduria, cataracts, liver cirrhosis/failure, and MR
Galactosemia
Manifestations include tremulousness or jitteriness than can progress to seizure activity
Hyperviscosity syndrome
(seizure b/c of sludging of blood in the cerebral microcirculation)
Condition in which there is a bony or membranous septum b/w the nose and pharynx
Choanal atresia
What maternal drug causes diminished ability of the asphyxiated infant to increase heart rate and cardiac output, and has been associated w/ hypoglycemia and apnea
Propranolol
Usually presents on 3-4 day of life with acute onset of bilious vomitus
Midgut volvulus, which may obstruct the bowel in area of the duodenojejunal jcn
Neonate presents with tachypnea, retractions, and sometimes cyanosis. CXR demonstrates prominent pulmonary vascular markings with fluid in the fissures, & hyperexpansion (flat diaphragms)
Transient Tacypnea of the Newborn
- Result of retained fetal lung fluid
Infiltrative maculopapular peeling rash prominent on the face, palms, and soles, "snuffles", hepatosplenomegaly, lymphadenopathy, early jaundice, saddle nose
Congenital syphilis
Syndromes associated w/ polydactyly
Trisomy 13
Rubenstein-Taybi
Meckel-Gruber
Ellis-van Creveld
In Twin-to-Twin transfusions, which twin is likely to have oligohydramnios, anemia, and hypovolemia
Donor twin

- recipient twin is likely to have hydramnios and plethora, hct > 65% can develop hyperviscosity, respiratory distress, hyperbilirubinemia, hypocalcemia, renal vein thrombosis, CHF, & convulsions
In what conditions are INCREASED pulmonary vasculature markings seen on CXR (in lateral 1/3 of lung field)
Acyanotic child:
- ASD
- VSD
- PDA
- Endocardial cushion defect
- Partial APVR
Cyanotic Child:
- Transposition of great arteries
- TAPVR
- Hypoplastic left heart syndrome
- Persistent truncus arteriosis
- Single ventricle
In what conditions are DECREASED pulmonary vasculature markings seen on CXR (lung fields dark w/ small vessels)
- Pulmonary Stenosis
- Pulmonary Atresia
- Tricuspid Stenosis
- Tricuspid Atresia
- TOF
In what conditions is pulmonary venous congestion seen on CXR (hazy lung fields)
- LV failure
- Obstruction of pulmonary veins
- Mitral stenosis
- TAPVR
- Cor Triatriatum
- Hypoplastic left heart syndrome
- Any left-sided obstructive lesion w/ heart failure
Structural heart defects (PDA. Pulm artery stenosis, and septal defects) only one type of TORCH defect
Congenital Rubella
"Classic Triad" consists of Hydrocephalus, Chorioretinitis, and Intracranial calcifications
Congenital Toxoplasmosis
Condition associated with features of widely spaced eyes, low set ears, broad nose, receding chin, limb abnormalities, bilateral renel agenesis, immediate respiratory distress
Primary Pulmonary Hypoplasia (Potter sequence)
AD trait with variable penetrance and can include 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
Waardenburg syndrome
A flat capillary vascular malformation in the distribution of the trigeminal nerve is the basic lesion of what syndrome
Sturge-Weber syndrome
Syndrome thay involves a vascular malformation that involves the meninges and results in atrophy of the underlying cerebral cortex, manifested by grand mal seizures, mental deficiency, hemiparesis or hemianopsia on CL side
Sturge-Weber syndrome
AD disease with hypopigmented oval or irregularly shaped macules (ash leaf), cerebral sclerotic tubers, myoclonic seizures, adenoma sebaceum
Tuberous sclerosis
Major Jones criteria for rheumatic fever
JONES
JONES
- Joints (polyarthritis)
- O (heart shape - carditis)
- Nodules, subcutaneous
- Erythema marginatum
- Sydenham's chorea
A 6-year-old girl with PDA develops fever and anorexia. Hgb is 9, she has hematuria, increased ESR, (+) Rheumatoid factor, and immune complexes are present
Bacterial endocarditis
A pear or water bottle-shaped heart on CXR indicates
Pericardial effusion
A 2-year-old boy with fever for 7 days, often reaching 104 F (40 C) develops nonexudative conjunctival injection bilaterally; intensely erythematous lips, palms, and soles; generalized erythema multiforme; and an enlarged, tend anterior cervical lymph node. Blood cultures are sterile, and platelets are increased
Kawasaki disease, and beware of his risk for coronary aneurysms and MI
Organisms not covered by cephalosporins are LAME
LAME
- Listeria (amoxicillin, ampicillin)
- Atypicals: Chlamydia, Mycoplasma (macrolides, tetracyclines)
- Mrsa (vancomycin)
- Enterococci (amox, amp & gent)
Clinical use of Aztreoname
Gram (-) Rods (Klebsiella, Pseudomonas, Serratia)
- no activity against gram (+) or anaerobes
Ampicillin/Amoxicillin Coverage:
"ampicillin/amoxicillin HELPS kill enterococci"
"ampicillin/amoxicillin HELPS kill enterococci"
- Haemophilus influenza
- E. coli
- Listeria
- Proteus
- Salmonella
Tetracycline clinical use:
"VACUUM THe BedRoom"
"VACUUM THe BedRoom"
- Vibrio cholerae
- Acne
- Chlamydia
- Ureaplasma
- Urealyticum
- Mycoplasma pneumoniae
- Tuleremia
- H. pylori
- Borrelia burgdorferi
- Rickettsia
Metronidazole clinical use:
"GET GAP on the Metro"
"GET GAP on the Metro"
- Giardia
- Entamoeba
- Trichomonas
- Gardnerella vaginakus
- Anaerobes (bacteroides, clostriudium)
- h. Pylori
Wiskott-Aldrich Syndrome
"WIPE"
WIPE
- Wiskott-(Aldrick = inc igA)
- pyogenic Infections (b cell def)
- Purpura (thrombocytic)
- Eczema
Job's Syndrome
"FATED"
FATED (neutrophil def)
- Facies coarse
- Abscesses
- retained primary Teeth
- inc igE
- Dermatologic problems (eczema)
Tetralogy of Fallot: "PROVe"
PROVe
- Pulmonary stenosis
- Right ventricular hypertropy
- Overriding aorta
- Vsd
Patent Ductus Arteriosis: drugs
- ENDomethacin (indomethacin) ENDs patency
- pgEE kEEps it open
Bacterial endocarditis:
"Bacteria FROM JANE"
"Bacteria FROM JANE"
- Fever
- Roth's spots
- Osler's nodes
- Janeway lesions
- Anemia
- Nail-bed hemorrhage
- Emboli
Rheumatic heart disease:
"FEVERSS"
"FEVERSS"
- Fever
- Erythema marginatum
- Valvular damage (vegitation & fibrosis)
- Esr inc
- Red-hot joint (migratory polyartheitis)
- Subcutaneous nodules (aschoff bodies)
- St. vitus' dance (chorea)
Common triad of Henoch-Schonlein Purpura
Skin
Joints
GI
Cerebellar tonsillar herniation associated w/
Arnold-Chiari malformation
Aschoff bodies
Rheumatic Fever
Calf pseudohypertrophy
Duchenne's muscular dystrophy
Charcot-Leyden crystals
Bronchial asthma (eosinophil membranes)
Deficiency in Chediak-Higashi disease
Phagocyte
Cherry-red spots on macula
Tay-Sachs and Niemann-Pick
Continuous machinery murmur
PDA
Cough, Conjunctivitis, Coryxa + Fever
Measles
Congenital unconjugated bilirubin
Crigler-Najjar syndrome
Curschmann's spirals
Bronchial asthma (whirling mucus plugs)
Congenital Conjugated hyperbilirubinemia (black liver)
Dubin-Johnson syndrome
Late cyanosis shunt (uncorrected L --> R shunt becomes R --> L shunt)
Eisenmenger's complex
Superior trunk (C5-C6) brachial plexus injury, "waiter's tip"
Erb-Duchenne palsy
Benign congenital unconjugated hyperbilirubinemia
Gilbert's syndrome
Heinz bodies
G6PD deficiency
Hypersensitivity vasculitis associated with hemorrhagic utricaria and URIs
Henoch-Scholen purpura
Homer Wright rosettes
Neuroblastoma
Palpable purpura on legs and buttocks
HSP
Reed-Sternberg cells
Hodgkin's lymphoma
Left-to-right shunt (VSD, PDA, ASD), mitral regurgitation, LV failure all cause what sound
S3
"Strawberry tongue"
Scarlet fever
Suboccipital lymphadenopathy
Rubella
Most common brain tumor
Medulloblastoma (cerebellum)
Most common supratentorial brain tumor
Craniopharyngioma
Most common cardiac primary tumor
Rhabdomyoma
Most common location of brain tumors
Infratentorial
Adolescent ingestion causes tachycardia, initial hypertension followed by hypotension, widening of the QRS complex and BBB
- Toxicity? Antidote?
TCA's
- close monitoring in ICU & TCA Fab antibody fragments
CHD that has a fixed split-second heart sound and a pulmonic stenosis murmur
ASD
CHD that commonly presents as a harsh or blowing holosystolic murmur along the LLSB w/ radiation throughout the pericordium
VSD
CHD with a middiastolic rumble at the LLB
Tricuspid regurgitation
CHD that on clinical examination has an apical murmur late in systole, that can be preceded by a midsystolic click
Mitral valve prolapse
A pulse greater than 250 beats per minute suggests the presence of?
Tachyarrhythmia --> Supraventricular tachycardia
What can prlonged SVT lead too and what is the first-line treatment
Heart failure, hepatomegaly, and respiratory compromise

Rx: Stimulate the vagus nerve using carotid massage immersion of face in cold water, or voluntary straining --> if unsuccessful IV adenosine
Quadruple rhythm associated with the murmur of tricuspid regurgitation & a middiastolic murmur at LLSB, presence of RAH & RV conduction defects suggest
Ebstein anomaly (downward displacement of the tricuspid valve
Condition that frequently causes spindle-shaped swelling of finger joints and can involve unusual joints (i.e. Sternoclavicular joint), can be associated w/ spiking high fevers and diffuse rash
JRA (or Still disease)
CHD where pts typically have a hypoplastic RV, ECG shows left-axis deviation & LVH --> LV impulse on PE
Tricuspid atresia
CHD that presents w/ early cyanosis, normal-sized heart, normal or slightly inc pulm vascular markings, ECG showing right-axis deviation and RVH
Transposition of the great vessels
Cyanotic infants who do not improve their saturation with supplement O2 should be evaluated for?
Ductal-dependent cyanotic congenital heart lesion
- Pulmonary atresia w/o corresponding VSD
- Transposition of the great vessels w/o a septal defect
Syndrome diagnosed as a newborn w/ profound thrombocytopenia, bilateral absence of radius, and abnormally shaped thumbs. Cardiac lesions include TOF and ASD
Thrombocytopenia Absent Radius syndrome (TAR)
Cardiac defect in children, usually first causing syncoal episodes in late childhood or adolescence
Prolonged QT syndrome
Life-threatening sequeale of viral laryngotrachceobronchitis, w/ Hx of several days of viral URI Sx followed by acute elevation of temp & inc in respiratory distress.
Dx and managment
Dx: Bacterial Tracheitis
Rx: Intubation (secretions can be thick & copious enough to occlude the airway) and IV ABX
In general, a "cold" lasting longer than 10-14 days with fever and facial pain is indicative of?
Sinusitis
Treatment for Croup
Usually a single dose of steroids
- Racemic epinephrine and corticosteroids
Treatment for Bronchiolitis
Supportive, observation for hypoxia and dehydration alone
Treatment for mycoplasma pneumoniae
MAcrolide
Syndrome with traid of situs inversus, chronic sinusitus & otitis media, and airway disease
Kartagener syndrome
A child presenting who has a subacute disorder of the CNS that produces cranial nerve abnormalities (esp of CN VII & lower bulbar nervers), long tract signs, unsteady gait secondary to spasticity, & some behavioral changes is most likely to have?
Pontine Glioma
Type of tumor that can produce upper motor neuron signs, with ataxia as the gait disturbance, Dysmetria and nystagmus
Tumors of the cerebellar hemisphere
Presenting signs HA, vomiting, and on PE finding of papilledema
Supratentorial tumors
These pts have insidous behavior change, deterioration of schoolwork and finally demention. No other findings are usually seen
SSPE
Findings of poor growth, diabetes insipidus, and papilledema, upward growth of tuman results in compression of the optic chiasm, suprasellar calcifications sometimes seen
Craniopharyngioma (supratentoria tumor)
Type of brain tumors predominating in the 1st year of life
Supratentorial
- Choroid plexus tumors
- Teratomas
Type of brain tumors predominating in children 1 to 10 years old
Intratentorial (posterior fossa) and include cerebellar and brainstem tumors
- Medulloblastoma
-- Cerebella astrocytoma
Type of brain tumors predominating in children n over 10 years old
Supratentorial tumors
- Diffuse astrocytoma
Disorder presenting in older children as acroparesthesia (mumbness or tingling in one or more extremities), intermittent painful crises of the abdomen, low-grade fevers, and sometimes cataracts
Fabry disease (reduce alpha-galactosidase)
Child with unilateral facial lesion & blindness suggests
Sturge-Weber or PHACE syndrome
- Posterior fossa malformations
- Hemangiomas
- Arterial anomalies
- Coarctations of the aorta and other cardiac defects
- Eye abnormalities
These pts can have seizures, hemiparesis, intracranial calcifications and MR
Syndrome with large, irregular, usually unilateral cafe au lait spots and fibrous dysplasia of bones in association with precocious puberty in girls
McCune-Albright syndrome