Peds Shelf 5

Front 1

Storage disease: infant w/ FTT, hepatospenomegaly, neurodegeneration

Back 1

Niemann-Pick disease (deficient sphingomyelinase)

Front 2

Storage disease: infant w/ hepatospenomegaly and skeletal abnormalities

Back 2

GM1 gangliosidosis (deficient beta-galactosidase)

Front 3

Storage disease: child with cherry red macula, neuro symptoms, hepatosplenomegaly

Back 3

GM2 gangliosidoses, incl. Tay-Sachs' and Sandhoff's (deficiency of hexosaminidase)

Front 4

Storage disease: bone pain and joint swelling

Back 4

Farber disease (deficiency of ceramidase --> accumulation of ceramide esp in joints)

Front 5

Formula mixing for powder and concentrate

Back 5

Formula: 2 scoops to 4oz water
Concentrate: 1 part concentrate to 1 part water

Front 6

Caloric requirement from birth to 2mo per day and for pre-emies

Back 6

100-200cal/kg/day
More (up to 150) for small pre-emies

Front 7

Pharyngitis, fever, dysphagia, neck stiffness, muffled voice

Back 7

Retropharyngeal abscess (look for increased space btwn trachea and vertebrae on XR)

Front 8

Pts with Turner Syndrome are at increased risk for

Back 8

Osteoporosis

Front 9

Rx for nephrotic syndrome in kids

Back 9

Prednisone: 60% is minimal change disease, which is very steroid responsive (add cyclophosphamide if needed)

Front 10

Differentiate bacterial and viral lymphadenitis

Back 10

Bacterial is usually unilateral (+ tender, warm, and erythematous); S. aureus is most common; viral may have more systemic symptoms (bilateral, fever, pharyngitis, hepatosplenomegaly)

Front 11

Non-TB mycobacteria lymphadenopathy

Back 11

Firm, nontender, small lymphadenopathy
Skin becomes thin and violaceous

Front 12

What is the only congenital heart disorder that does not develop endocarditis

Back 12

ASD

Front 13

Side effect of hydroxyurea

Back 13

Bone marrow suppression --> leukopenia, anemia, thrombocytopenia

Front 14

Kids with bronchiolitis (RSV infection) are at increased risk for what later in life?

Back 14

Asthma

Front 15

Incidence of cyclic vomiting is high in children whose parents have a history of

Back 15

Migraine headaches

Front 16

What is the reticulocyte count in spherocytosis?

Back 16

High (up to 9%)

Front 17

Adverse reactions to the DTaP vaccine are usually attributed to which component?

Back 17

Pertussis

Front 18

Recurrent infections with staph and aspergillus

Back 18

Chronic granulomatous disease (impaired oxidative metabolism, predisposing to abscesses from catalase-producing organisms)

Front 19

Most common congenital obstructive urethral lesion in males (--> oliguria in newborns)

Back 19

Posterior urethral valves (can lead to a distended bladder, palpable as abdominal mass)

Front 20

Diagnostic test of choice for suspected posterior urethral valves

Back 20

VCUG

Front 21

Worrisome xray finding in NEC

Back 21

Pneumoperitoneum (indicating intestinal perforation)

Front 22

Neonate with hypocalcemia seizures

Back 22

Think about DiGeorge syndrome

Front 23

Neonate with vomiting, bloody diarhea, stool with RBCs and eosinophils

Back 23

Milk protein intolerance (may have a FH of atopy)

Front 24

Most common cause of congenital hypothyroidim

Back 24

Thyroid dysgenesis

Front 25

Most common complication of supracondylar fracture

Back 25

Entrapment of brachial artery (so check the radial pulse)

Front 26

Newborn with evanescent rash with red halos and eosinophils in the skin lesions

Back 26

Erythema toxicum

Front 27

Strongest risk factor for intraventricular hemorrhage

Back 27

Prematurity/ low birth weight (also exposure to vascular perfusion injuries)

Front 28

Rx for severe asthma exacerbation that is unresponsive to meds

Back 28

Mechanical ventilation (intubation)

Front 29

How to distinguish preseptal and orbital cellulitis

Back 29

Eyelid symptoms (e.g. discoloration) indicate preseptal

Decreased eye movements or visual acuity, double vision, or proptosis indicate orbital cellulitis (as well as systemic symptoms)

Front 30

Rx for preseptal vs. orbital cellulitis

Back 30

Antibiotics for both, but orbital cellulitis requires hospitalization (and possibly surgical decompression)

Front 31

Neonatal hypoglycemia, macroglossia, visceromegaly, omphalocele, earlobe crease

Back 31

Beckwith-Widemann syndrome

Front 32

Self-mutilation, mental retardation, hyperuricemia, dystonia, choreoathetosis, cerebral palsy

Back 32

Lesch-Nyhan syndrome (deficiency in hypoxanthine-guanine phosphoribosyl transferase)

Front 33

Diagnostic test for pyloric stenosis

Back 33

Ultrasound of the abdomen

Front 34

Penicillin and amoxicillin have no activity against this common skin flora bug

Back 34

S. aureus

Front 35

Rx for bacterial lymphadenitis

Back 35

Dicloxacillin (covers both staph and strep, 2 most common agents)

Front 36

Microcephaly and IUGR most likely indicate which class of problems

Back 36

TORCH infection

Front 37

Pathogenesis of Meckel's diverticulus

Back 37

Failure of vitelline duct to obliterate during fetal development

Front 38

Murmur of large VSDs is generally ____ compared to that of small VSDs

Back 38

Soft

Front 39

Newborn with hypoplastic nails, cleft palate, vita K deficiency

Back 39

Fetal hydantoin syndrome (phentyoin use in pregnancy)

Front 40

5 common GI complications of Down's

Back 40

Duodenal atresia
Hirschsprung's
Esophageal atresia
Pyloric stenosis
Malro

Front 41

Type of anemia seen in sickle cell anemia

Back 41

Hemolytic anemia (hemolysis of sickled cells --> reticulocytosis, hyperbili, elevated LDH with low haptoglobin)

Front 42

Rx for SCFE

Back 42

Surgical fixation at current degree of slippage to avoid risk of avascular necrosis

Front 43

Is an LP necessary with a febrile seizure?

Back 43

No, unless signs of meningitis present, child already on Abx, or not immunized

Front 44

How to distinguish a microcytic anemia from iron deficiency vs. thalassemia?

Back 44

RDW: high (>20%) indicates iron deficiency, normal in thalassemia or anemia of chronic disease

Front 45

Peripheral blood smear findings in sickle cell

Back 45

Sickled cells and reticulocytosis

Front 46

Nocturnal vaginal itching: presumed diagnosis, diagnostic test, and treatment

Back 46

Vulvovaginitis from pinworm infection
Scotch tape test
Mebendazole

Front 47

Most common causes of acute bacterial sinusitis

Back 47

Same as ear infections:
Strep pneumo
Non-typable H flu
Moraxella

Front 48

Most common cause of viral meningitis

Back 48

Enteroviruses (PERCH, though not polio: usually echo or coxsackie)

Front 49

Physical exam manifestation of endocardial cushion defect

Back 49

Endocardial cushion defect --> pulmonary HTN --> Loud P2 (Rx is early surgery)

Front 50

Inspiratory noises w/o other complaints; "epiglottis rolling in from side to side" on direct laryngoscopy

Back 50

Laryngomalacia (aka congenital flaccid larynx)

Front 51

Rx for laryngomalacia

Back 51

Reflux precautions
Should resolve by 2yo

Front 52

Organ with highest M&M after HUS

Back 52

Kidneys

Front 53

Why is their jaundice in HUS?

Back 53

Hemolysis (NOT b/c of liver involvement)

Front 54

Down's patient w/ torticollis, urinary incontinence, dizzines, UMN signs (leg spasticity, hyperreflexia, positive Babinski)

Back 54

Alantoaxial instability (due to excessive laxity in posterior transverse ligament, causing increased mobility btwn atlas and axis)

Front 55

Rx for atlantoaxial instability

Back 55

Surgical fusion of C1 to C2

Front 56

How long must disruptive behavior last for conduct disorder diagnosis

Back 56

1yr

Front 57

Differentiate btwn conduct disorder and antisocial personality disorder

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<18yo for conduct, >18 for antisocial

Front 58

Differentiate btwn conduct disorder and oppositional defiant disorder

Back 58

Conduct: older teens, violation of social norms
ODD: late childhood, negativity, hostility, defiance

Front 59

Definition of infantile colic

Back 59

Starts before 3wks, crying >3hrs/day, >3 days/wk, >3wks/mo

Front 60

Rx for infantile colic

Back 60

Nothing universal: soothing, simethicone, probiotics

Usually resolves by 4mo

Front 61

Drug that opens PDA

Back 61

Prostaglandin E1

Front 62

Drug that closes PDA

Back 62

Indomethacin

Front 63

Rx for labial adhesions (benign fusion of labia minora)

Back 63

Nightly application of estrogen cream + daily petrolatam for 1mo

Front 64

Lax, wrinkled abdominal wall, dilated urinary tract, intra-abdominal testes, renal dysfunction

Back 64

Prune belly syndrome

Front 65

Prune belly syndrome is associated w/ these extra-abdominal effects

Back 65

Oligohydramnios, clubfeet, hip dislocation, malro

Front 66

Anorexia, V, polydipsia, polyuria, fevers, glucosuria w/ normal serum glucose, high urine pH, hyperchloremic metabolic acidosis, albuminuria w/ normal serum protein and albumin

Back 66

Fanconi Syndrome

Front 67

How to distinguish Type 1 from Type 2 RTA

Back 67

Type 2 has a normal urine anior gap, where Type 1 (and type 4) have positive urine anion gaps

Front 68

What is phimosis

Back 68

Inability to retract the foreskin

Front 69

When is phimosis normal?

Back 69

Before 3yo

Front 70

What is acute lobar nephronia

Back 70

In between pyelonephritis and renal abscess

Front 71

Rx for nephronia

Back 71

Prolonged IV and then PO antibiotics

Front 72

Orchiopexy (moving an undescended testicle into the scrotum) reduces the risk of?

Back 72

Testicular torsion (b/c decreases mobility)
Doesn't reduce the risk of testicular malignancy, though does make accessible for periodic exams

Front 73

What counts as the most sterile urine collection in a child?

Back 73

Suprapubic tap

Front 74

PSGN can occur after which two types of strep infections

Back 74

Pharyngitis or impetigo

Front 75

Microangiopathic hemolytic anemia, thrombocytopenia from increased platelet utilization, and renal insufficiency from vascular endothelial injury and local fibrin deposition + bloody diarrhea

Back 75

HUS

Front 76

Pain relief upon elevation of the testicle

Back 76

Prehn sign: indicates epididymitis

Front 77

What is HSP

Back 77

Systemic IgA-mediated vasculitis

Front 78

Bag of worms

Back 78

Varicocele

Front 79

Rx for varicocele?

Back 79

Reassurance
May require surgery for infertility later on

Front 80

Fluid-filled area surrounding testicle that transilluminates

Back 80

Hydrocele

Front 81

Rx for hydrocele

Back 81

Observe, usually resolves by 1yo; larger ones that persist may need surgery

Front 82

Genetics of Alport syndrome

Back 82

X-linked dominant

Front 83

Recurrent gross hematuria + dysuria/abdominal pain

Back 83

Idiopathic hypercalciuria (stones may form over time)

Front 84

Rx for acute glomerulonephritis with oliguria

Back 84

Restrict fluid intake to avoid hypervolemia

Front 85

Elevated cholesterol and triglycerides + edema

Back 85

Nephrotic syndrome

Front 86

Is HTN more likely associate with glomerulonephritis or nephrotic syndrome

Back 86

Glomerulonephritis, apparently

Front 87

FTT with constipation, weakness, vomiting, polyuria/polydipsia, dehydration + hypokalemia, hypercalciuria, alkalosis

Back 87

Bartter Syndrome (juxtaglomerular hyperplasia): also have hyperaldosteronism and hyperreninemia

Front 88

Is mumps usually unilateral or bilateral?

Back 88

Unilateral and spreads to the opposite side

Front 89

Viral infection + sleepiness, emesis, abnormal liver fxn, seizures, coma, respiratory distress, loss of DTRs, posturing

Back 89

Reye's syndrome

Front 90

Reye's is a ____ caused by ___ and ____

Back 90

Mitochondrial hepatopathy
Influenza or varicella infection + aspirin use

Front 91

Cause of death in Reye's

Back 91

Cerebral edema and subsequent herniation

Front 92

What is Ekiri syndrome?

Back 92

Rare and fatal toxic encephalopathy seen with Shigella infection

Front 93

Rx for shigellosis

Back 93

Supportive care and third generation cephalosporin

Front 94

Major cause of tinea capitis

Back 94

Tricophyton tonsurans

Front 95

Rx for trichophyton tonsurans

Back 95

Oral griseofulvin

Front 96

Linear hair loss + regional adenopathy

Back 96

Traction alopecia

Front 97

Hairs near area of hair loss that can be extracted w/ gentle traction --> attenuated or catagen bulb at termination of hair shaft (exclamation hair)

Back 97

Alopecia areata

Front 98

Is mumps usually unilateral or bilateral?

Back 98

Unilateral and spreads to the opposite side

Front 99

Viral infection + sleepiness, emesis, abnormal liver fxn, seizures, coma, respiratory distress, loss of DTRs, posturing

Back 99

Reye's syndrome

Front 100

Reye's is a ____ caused by ___ and ____

Back 100

Mitochondrial hepatopathy
Influenza or varicella infection + aspirin use