Usmlestep

Front 1

history of developmental milestone loss is suggestive of an

Back 1

inborn error of metabolism that presents after 1 year of age when the specific enzymatic deficiency can no longer be compensated for normally. Examples of these disorders include mucopolysaccharidosis, mucolipidosis, oligosaccharidosis, and gangliosidosis

Front 2

Chromosomal abnormalities delay developmental milestone progression

Back 2

as opposed to loss of milestones as in this patient. These are usually apparent at or near birth because they are associated with birth defects and dysmorphisms, neither of which affects this patient.

Front 3

Perinatal asphyxia is usually associated with

Back 3

a hypoxic event at birth followed by some degree of mental retardation, not a regression of developmental milestones

Front 4

Post–streptococcal glomerulonephritis is best treated

Back 4

symptomatically. In patients with hypertension and mental status changes, diazoxide is the treatment of choice. Hypertension without encephalopathy may be treated with vasodilators such as nifedipine or hydralazine. Post–infectious glomerulonephritis develops several weeks after the acute streptococcal illness has cleared, and antibiotics are not helpful.

Front 5

Clearly outlined color change as infant lies on side; dependent lower half of body becomes pink and upper half is pale

Back 5

Harlequin color change:

Front 6

acute life-threatening event is defined

Back 6

An as an episode consisting of some combination of prolonged apnea, color change, change in muscle tone, and choking or gagging. The episode is usually very frightening to the observer. The differential is broad and depends on the combination of events.

Front 7

Apnea of prematurity

Back 7

is not common after 35 to 36 weeks’ conceptual age and is not associated with sudden loss of consciousness in an alert infant.

Front 8

Encephalopathy from poor or absent cerebral perfusion (thus both hypoxia and ischemia) often

Back 8

heralds a poorer outcome than that from trauma; the physician has little to offer to improve the outcome. Often there is diffuse, global brain injury. These children may have experienced a period of asystole and have required cardiopulmonary resuscitation (CPR) such as after near-drowning episodes, acute life-threatening events, smoke inhalation, upper or lower airway obstruction, shock, electrical injuries, and other life-threatening events. The lack of oxygen and perfusion produces primary neuronal damage. Secondary injury occurs from the response of the activation of mediators, which activate a cascade of events that result in cerebral edema and apoptosis and cell death.

Front 9

Encephalopathy from poor or absent cerebral perfusion (thus both hypoxia and ischemia) often heralds a

Back 9

poorer outcome than that from trauma; the physician has little to offer to improve the outcome. Often there is diffuse, global brain injury. These children may have experienced a period of asystole and have required cardiopulmonary resuscitation (CPR) such as after near-drowning episodes, acute life-threatening events, smoke inhalation, upper or lower airway obstruction, shock, electrical injuries, and other life-threatening events. The lack of oxygen and perfusion produces primary neuronal damage. Secondary injury occurs from the response of the activation of mediators, which activate a cascade of events that result in cerebral edema and apoptosis and cell death.

Front 10

kawasaki

Back 10

Presence of at least four of the following five signs:
Bilateral bulbar conjunctival injection, generally nonpurulent
Changes in the mucosa of the oropharynx, including injected pharynx, injected and/or dry fissured lips, strawberry tongue
Changes of the peripheral extremities, such as edema and/or erythema of the hands or feet in the acute phase; or periungual desquamation in the subacute phase
Rash, primarily truncal; polymorphous but nonvesicular
Cervical adenopathy, ≥1.5 cm, usually unilateral lymphadenopathy
Illness not explained by other known disease process

Front 11

What principles are involved in the choice of antibiotics for the treatment of PID?

Back 11

Regimens are designed to cover Neisseria and Chlamydia as well as anaerobes (such as Bacteroides fragilis), gram-negative aerobes, and streptococci (endogenous vaginal and lower gastrointestinal flora).

Front 12

What are the oral treatment options for PID?

Back 12

In 1998 the Centers for Disease Control and Prevention published recommendations for the treatment of PID. Although there are no data comparing different regimens, each regimen listed below has been shown to be effective in treatment.
Ofloxacin 400 mg plus metronidazole 500 mg orally twice a day for 14 days
Cefoxitin 2 g with probenecid 1 g × 1 dose orally concurrently or ceftriaxone 250 mg IM × 1 (or other third-generation cephalosporin such as ceftizoxime or cefotaxime)
plus
doxycycline 100 mg orally BID for 14 days

Front 13

In patients with traumatic brain injuries,....must be avoided.

Back 13

hypotension and hypoxia In a state where there is a traumatic elevation of intracranial pressure, avoidance of these two physiologic abnormalities will lead to a better outcome.

Front 14

Classically, projectile vomiting

Back 14

is seen with pyloric stenosis. This abnormality is seen more commonly in boys, and infants are hungry again after vomiting. At times there is a palpable “olive” in the abdomen that can be seen. Treatment is with surgical incision of the hypertrophied pylorus.

Front 15

Air contrast enema is diagnostic and therapeutic for

Back 15

intussusceptions. Typically, the vomiting in this is cyclic and not projectile.

Front 16

SURGICAL ISSUES

Back 16

Bilious (dark green) emesis in a newborn is a true gastrointestinal emergency; it is a sign of potential obstruction.
Malrotation is diagnosed on the basis of the malposition/malfixation of the ligament of Treitz, as seen on an upper gastrointestinal series. Malrotation can lead to acute volvulus and should always be repaired.
The classic triad of intussusception consists of the following: (1) colicky abdominal pain, (2) vomiting, and (3) bloody stools with mucous. However, it occurs in fewer than 20% of patients.
Pyloric stenosis typically appears with progressive, nonbilious, projectile vomiting and a hypochloremic, hypokalemic metabolic alkalosis in an infant between the ages of 3 and 6 weeks old.
The classic picture of appendicitis is anorexia followed by pain followed by nausea and vomiting, with subsequent localization of findings to the right lower quadrant. However, there is a large degree of variability, particularly among younger patients.

Front 17

of familial hypercholesterolemia

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Bile acid sequestrants combined with high potency 3-hydroxy-3-methylglutaryl coenzyme A (HMG CoA) reductase inhibitors often can reduce low-density lipoprotein–cholesterol complex (LDL-c) to recommended levels in homozygous familial hypercholesterolemia. The planar xanthomas seen on the knees in the figure are diagnostic for the homozygous state

Front 18

....is an approved pharmacotherapy for a patent ductus arteriosus (PDA).

Back 18

Indomethacin The patient has a continuous frequency murmur throughout systole and diastole; this describes the continuous machine-like murmur of a PDA. The primary treatment of a PDA is indomethacin, especially in pre-term infants. Indomethacin is an nonsteroidal anti-inflammatory drug (NSAID), and as such it inhibits the formation of prostaglandins. Prostaglandins are responsible for keeping the ductus open, so by blocking them, the ductus may close. If it does not resolve, then surgery may be performed to ligate the PDA.

Front 19

Increased levels of serum phosphate

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are typically seen after the glomerular filtration rate (GFR) falls to or below 30% of normal. Because of the reciprocal solubility of calcium and phosphate, hypocalcemia ensues. Secondary hyperparathyroidism develops; also contributing to bone resorption. This is called renal osteodystrophy, and is a common cause of growth delay in children with renal failure. Premature closure of epiphyses also may occur.

Front 20

free air seen on the x-ray. The patient also has a tight, tender

Back 20

A laparotomy is indicated in this situation because there is free air seen on the x-ray. The patient also has a tight, tender abdomen that is concerning for an intraabdominal injury

Front 21

What are the most common organisms responsible for bacterial meningitis in the United States?
0-1 month old

Back 21

What are the most common organisms responsible for bacterial meningitis in the United States?

Group B streptococci
Escherichia coli
Listeria monocytogenes
Streptococcus pneumoniae
Miscellaneous Enterobacteriaceae
Haemophilus influenzae (especially other than type b)
Coagulase-negative staphylococci (in hospitalized preterm infants

Front 22

What are the most common organisms responsible for bacterial meningitis in the United States?

Back 22

1-23 months old
Streptococcus pneumoniae
Neisseria meningitidis
Group B streptococci

Front 23

What are the most common organisms responsible for bacterial meningitis in the United States?

Back 23

2-18 years old
Neisseria meningitidis
Streptococcus pneumoniae

Front 24

rash, termed erythema migrans, is pathognomonic

Back 24

evidence of Lyme disease. The causative organism is a spirochete that is best treated with amoxicillin.

Front 25

Babesiosis

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Babesiosis is a tick-borne disease. Treatment is with clindamycin and quinine.

Front 26

Ehrlichiosis is

Back 26

Ehrlichiosis is a tick-borne disease caused by a rickettsial organism. Treatment is with doxycycline

Front 27

infant most likely has secondary hyperparathyroidism as a neonatal response to maternal hypocalcemia.

Back 27

The treatment of acute severe hypercalcemia (>14.0 mg/dL) includes hydration with isotonic sodium chloride solution to restore extracellular fluid volume and to induce calciuresis.

Front 28

More significant hypercalcemia often requires other therapies

Back 28

Glucocorticoids decrease intestinal absorption of calcium by blocking the action of 1,25-dihydroxyvitamin D. There is also a decrease in levels of 25-hydroxyvitamin D and 1,25-dihydroxyvitamin D. The usual dose of prednisone is 1 mg/kg/24 hr. Glucocorticoids are especially effective when vitamin D is central in the pathogenesis of hypercalcemia, such as subcutaneous fat necrosis and vitamin D intoxication

Front 29

This child is 9 months oldable to displace the left hip from the acetabulum while applying direct pressure on her adducted femur. He also feels a palpable “clunk” as the femoral head slides back and reduces into the acetabulum. The physician makes the diagnosis of developmental dysplasia of the hip.

Back 29

Children older than 6 months are usually too large to tolerate a brace, and closed reduction under general anesthesia is the initial treatment of choice. If reduction is successful, the hip is then placed in a spica cast for several months.

Front 30

CHILDHOOD (4-10 YR)

Back 30

Infection:
Septic arthritis:
Hip.
Knee.
Osteomyelitis.
Diskitis.
Transient synovitis, hip.
LCPD.
Tarsal coalition.
Rheumatologic disorder:
JRA.
Trauma.
Neoplasia.

Front 31

Primary atelectasis,

Back 31

in general, may present as respiratory distress alone with a very rapid respiratory rate. Evolution of symptoms progressing to cyanosis and distant heart sound indicate a diaphragmatic hernia.

Front 32

α1-antitrypsin

Back 32

child has symptoms and signs of emphysema, often caused by α1-antitrypsin deficiency. Without this key enzyme, elastase is unopposed in the lung tissues and destroys the alveolar architecture. Another manifestation of this illness is hepatic cirrhosis. Although most patients with α1-antitrypsin deficiency do not develop emphysema until their fourth decade, children with severe hepatic involvement are likely to develop emphysema earlier. Another early lung manifestation may be the development of pulmonary cysts with respiratory infection.

Front 33

Fanconi syndrome

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(dysfunction of proximal tubule transport, vitamin D–resistant bone disease and abnormalities in electrolyte and mineral balance)

Front 34

nephrotic syndrome.

Back 34

Edema is caused by the loss of massive amounts of albumin due to increased glomerular capillary permeability. Oncotic pressure causes fluid to disperse into the tissues, causing swelling. Fluid may also leak into the lungs, leading to respiratory distress, as well as into the intestinal mucosa, causing diarrhea. This patient’s respiratory distress calls for lowering of fluid volume, which is best accomplished by diuretics such as furosemide. Corticosteroids are the longer term treatment for the nephrotic syndrome.

Front 35

Post–streptococcal glomerulonephritis is best treated symptomatically

Back 35

. In patients with hypertension and mental status changes, diazoxide is the treatment of choice. Hypertension without encephalopathy may be treated with vasodilators such as nifedipine or hydralazine. Post–infectious glomerulonephritis develops several weeks after the acute streptococcal illness has cleared, and antibiotics are not helpful.

Front 36

Wiskott-Aldrich

Back 36

syndrome is an X-linked recessive syndrome characterized by atopic dermatitis and thrombocytopenic purpura with normal-appearing megakaryocytes. There appears to be an intrinsic platelet abnormality. Recurrent infections (pneumonia, otitis media) are caused by organisms with a polysaccharide capsule. Thrombotic thrombocytopenic purpura is the diffuse embolism and thrombosis of the smaller blood vessels of the brain characterized by changing neurologic signs such as speech deficits or blindness

Front 37

In patients with traumatic brain injuries

Back 37

, hypotension and hypoxia must be avoided. In a state where there is a traumatic elevation of intracranial pressure, avoidance of these two physiologic abnormalities will lead to a better outcome.

Front 38

In blunt trauma, CT scan of the abdomen and pelvis reveals free peritoneal fluid collections. When is observation appropriate instead of immediate surgical exploration?

Back 38

First and foremost, the patient must be hemodynamically stable, and the identified injury must be confined to the liver and spleen. Small lacerations to the liver and spleen may be managed by aggressive resuscitation. Immediate access to the operating room must be available at all times in case the patient's condition deteriorates. Evidence on CT scan that suggests biliary or bowel injury requires surgical exploration.

Front 39

Synoviocyte proliferation

Back 39

, with pannus expansion and joint destruction, is the mechanism for juvenile arthritis.

Front 40

What chest x-ray findings (Fig. 3-3) are considered characteristic for various congenital heart diseases

Back 40

Boot-shaped heart: Tetralogy of Fallot, tricuspid atresia
Egg-shaped heart: Transposition of great arteries
Snowman silhouette: Total anomalous pulmonary venous return (supracardiac)
Rib notching: Coarctation of the aorta (older children

Front 41

patient has total anomalous pulmonary venous return (TAPVR).

Back 41

The CXR shows cardiomegaly and increased pulmonary vasculature. The increased pulmonary vasculature is the result of severe obstruction to pulmonary venous return. It is a useful sign to differentiate between TAPVR and tetralogy of Fallot, where there are scant pulmonary markings because of decreased pulmonary blood flow.

Front 42

Hypoplastic left heart

Back 42

demonstrates very decreased or absent peripheral pulses and profound acidosis because of the inability of the left heart to effectively pump blood

Front 43

Supravalvular aortic stenosis

Back 43

typically presents with a loud systolic ejection murmur best heard at the upper right sternal border.

Front 44

Tetralogy is a cyanotic congential heart lesion,

Back 44

but it often presents with a loud ejection systolic murmur in the upper left sternal border. Also, the CXR shows scant pulmonary markings and a small, “boot-shaped” heart.

Front 45

Transposition is a cyanotic congenital heart lesion

Back 45

because in essence, there are two independent circulations. The aorta arises from the right ventricle, and the pulmonary artery arises from the left ventricle. Like total anomalous pulmonary venous return (TAPVR), transposition does not have a murmur, but the CXR shows a classic narrow upper mediastinum that is described as an “egg on a string” appearance.

Front 46

How is bronchiolitis distinguished from asthma in a wheezing infant?

Back 46

Asthma is a clinical diagnosis in all age groups that is characterized by reversible airway obstruction with hyperresponsiveness to various stimuli, including viral infections. However, the viral infections themselves-rather than underlying bronchial pathology-can be responsible for the wheezing in an infant with acute symptoms. Differentiating between the two diseases at the time of presentation can be virtually impossible. The infant wheezing from bronchiolitis generally has other symptoms (e.g., fever, rhinorrhea), and RSV antigen testing may be positive. If repeated episodes of reversible wheezing occur, especially if they are not during the RSV season, asthma is likely. Other diagnoses (e.g., cystic fibrosis, gastroesophageal reflux) must also be considered.

Front 47

primary enuresis ...What is the likely diagnosis?

Back 47

The bladder is easily palpable above the pubic symphysis. Laboratory data obtained from blood and urine are all within normal limits. There is no evidence of head trauma, spine trauma, or abuse. patient has signs of a bladder outlet obstruction, which most commonly involves the presence of posterior urethral valves in males. The obstruction causes a dilation of the prostatic urethra and detrusor as well as bladder neck hypertrophy. There may be associated vesicoureteral reflux with mild to severe hydronephrosis

Front 48

vesicoureteral reflux

Back 48

Reflux nephropathy occurs in patients who have vesicoureteral reflux. This child could have vesicoureteral reflux, but the bladder is not palpable in patients with this condition

Front 49

Sickle cell trait is associated with primary enuresis due to

Back 49

Sickle cell trait is associated with primary enuresis due to isosthenuria; this diagnosis is unlikely in the presence of normal blood work.

Front 50

rickets,

Back 50

the disruption of orderly development and mineralization of the growth plate, usually due to vitamin D deficiency. Other possible causes include malabsorption syndromes, liver disease, renal disease, and renal tubular abnormalities, which cause phosphate loss, and X-linked hypophosphatemia.

Front 51

Achondroplasia

Back 51

is characterized by abnormal bone growth that results in short stature with disproportionately short arms and legs, a large head, and characteristic facial features. Radiographic findings include narrowing of the interpediculate distance of the caudal spine, a notch-like sacroiliac groove, and a circumflex or chevron seat on the metaphysis

Front 52

Blount disease

Back 52

is a condition of disturbed growth of the medial growth plate of the upper tibia, causing severe bowing of the leg at the knee. It usually affects African–American children before the age of

Front 53

Campomelic dysplasia (CMD)

Back 53

is a congenital skeletal dysplasia characterized by prenatal bowing of the lower limbs and anomalies of the tracheobronchial tree or genitourinary tract. Perinatal and early neonatal death from respiratory failure commonly occurs.

Front 54

Osgood-Schlatter disease (OS).

Back 54

adolescent boy is presenting with signs and symptoms typical of Osgood-Schlatter disease (OS). It is one of the most common causes of knee pain in the adolescent. It is a self-limited condition that presents with localized swelling and tenderness of the tibial tubercle, although knee motion is usually normal.

Front 55

iliotibial band syndrome

Back 55

presents with aching or burning sensation on the lateral aspect of the knee during physical activity. Knee motion is normal, but swelling is often absent

Front 56

Osteochondritis dessicans (OCD)

Back 56

is an osteochondral fracture limited to the articular epiphysis and is usually due to trauma. Patients typically present during adolescence or early adulthood with a sensation of knee locking, catching or giving-way, and swelling that worsens with activity.

Front 57

Patellofemoral syndrome

Back 57

(chondromalacia patellae) is a softening of the cartilage in the posterior part of the patella due to abnormal compression of the posterior patella against the femur. Unlike OS, it is more common in young teenage females

Front 58

tibial fractures are often seen

Back 58

in older adolescent males and are most often the result of an isolated injury. A tibial tubercle fracture causes swelling and tenderness over the anterior tibia, and a palpable bone fragment and loss of active knee extension may be present.

Front 59

Osgood-Schlatter disease TX

Back 59

is a traction apophysitis that is a variant of PFPS, with the injury occurring at the insertion of the patellar tendon on the tibial tuberosity. Osgood-Schlatter disease is treated like patellofemoral syndrome, with the addition of a protective Osgood-Schlatter pad, which protects the tibial tubercle from direct trauma. Patients should miss little if any time from sports.

Front 60

Iliotibial band tendinitis

Back 60

is the most common cause of chronic lateral knee pain. Generally it is not associated with swelling and instability. Tenderness should be elicited along the iliotibial band as it courses over the lateral femoral condyle or at its insertion at the Gerdy tubercle, along the lateral tibial plateau; tightness of the iliotibial band is also noted using the Ober test.

Front 61

List some important points to keep in mind when making the diagnosis.

Back 61

If the pH is normal (< 4.5) the diagnosis is limited to candidiasis, atrophic vaginitis, or normal vaginal fluid.
Only Candida has the thick, white, "cottage cheese" appearance on gross exam.
The positive "whiff" test is specific for bacterial vaginosis.
In candidiasis and trichomoniasis, the organism is visible under the microscope.
In bacterial vaginosis, the diagnosis is inferred based on the alterations in the characteristics of the vaginal flora: elevated pH; abnormal odor; and the clumping of bacteria onto epithelial cells, leading to the microscopic appearance of "clue cells"

Front 62

Internal femoral torsion

Back 62

Sitting in the “W” position or sleeping prone with the legs extended and internally rotated may exacerbate the problem.

Front 63

Internal tibial torsion

Back 63

Internal tibial torsion is a common cause of intoeing that results in an internally rotated foot when the knee is straight. It usually resolves spontaneously by 7 or 8 years of age

Front 64

Internal femoral torsion

Back 64

is common in children. Marked femoral anteversion results in “kissing knees,” toeing-in, and clumsiness.

Front 65

Slipped capital femoral epiphysis

Back 65

is the most common adolescent hip disorder and always should be suspected in an adolescent with thigh, hip, knee, or groin pain. It results in pain, limping, and external rotation of the leg.

Front 66

Legg-Calvé-Perthes

Back 66

disease is the most common of the osteochondroses, characterized by a gradual onset of progressive gait abnormalities and hip joint pain, thigh muscle wasting, and limitation of joint movement.

Front 67

treatment of internal femoral torsion

Back 67

is predominantly by observation. Correction of abnormal sitting habits usually allows the torsion to resolve with normal growth and development. It takes 1-3 yr for complete correction to occur, depending on the age of the child when the sitting habits are corrected. The correction of sitting habits can be difficult in preschool-aged children and usually does not occur until they reach school age. The use of nighttime orthoses or daytime twister cables is of no value and may produce a compensatory external tibial torsion. The combination of internal femoral and compensatory external tibial torsion produces a pathologic genu valgum deformity. This can result in patellofemoral malalignment with patella subluxation or dislocation and pain.