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Pediatric Boards - YOU CAN DO IT! > MedStudy Question Bank > Flashcards

MedStudy Question Bank Flashcards

1
Q

When does adrenal crisis occur in congenital adrenal hyperplasia (CAH)?

A

Adrenal crisis at 1-2 weeks of life (normal newborn period)

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2
Q

What is the presentation of Sotos syndrome?

A

Intellectual impairment, overgrowth, and developmental delay without endocrinologic issues

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3
Q

What is the first step in evaluation of a patient with tall stature and signs of precocious puberty?

A

Bone age

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4
Q

What are the cancer screening guidelines in patients with Beckwith-Wiedemann syndrome?

A

Complete abdominal US and alpha-fetoprotein every 3 months until 4 years of age and then renal US every 3 months from 4-7 years of age

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5
Q

What is the growth pattern in Sotos syndrome?

A

Accelerated growth for first 5 years of life then return to normal thereafter

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6
Q

Which test can differentiate between constitutional growth delay and genetic short stature?

A

Bone age

Constitutional growth delay: delayed bone age
Genetic short stature: normal bone age

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7
Q

What is the presentation of Wolfram syndrome?

A

Central DI, DM, optic atrophy, deafness

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8
Q

What are the characteristic lab findings in primary polydipsia?

A

Low sodium and low serum osmolality

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9
Q

Which cardiac lesion is commonly associated with Williams syndrome?

A

Supravalvular aortic stenosis

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10
Q

What is the upper limit of normal of peak pubertal growth in males and females?

A

Males: by 16 years of age
Females: by 14 years of age

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11
Q

Which risk factor is associated with development of follicular ovarian cysts?

A

Cigarette smoking

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12
Q

What is the cause of familial male-limited gonadotropin-independent precocious puberty?

A

Activating mutation in LH receptor

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13
Q

What is the definition of precocious puberty?

A

Puberty < 9 years of age in boys and < 8 years of age in girls

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14
Q

When is peak height velocity in females?

A

Between thelarche (10-11 years of age) and menarche (11-12 years of age)

SMR 2-3

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15
Q

Which 4 common AEDs induce CYP450?

A

Carbamazepine, phenytoin, phenobarbital, topiramate

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16
Q

What is the average time between thelarche and onset of menarche?

A

2-2.5 years

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17
Q

What is the most common cause of toileting refusal in young children?

A

Strong will

Will often void in the toilet but not stool

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18
Q

What is the classic XR finding in rickets?

A

Cupping and fraying of metaphysis

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19
Q

What is the appropriate management of a baby born to a mother who is HBsAg-positive?

A

HBIG and hep B vaccine at birth followed by hep B vaccine at normal intervals (2 months, 6 months) and lab evaluation (HBsAg and anti-HBsAg) at 9-12 months

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20
Q

What is the Pelger-Huet nuclear anomaly?

A

Hyposegmented neutrophil in setting of malignancy or genetic anomaly

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21
Q

What is guttate psoriasis?

A

Erythematous scaly papules associated with concurrent Strep infection

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22
Q

What is the first-line treatment of alopecia areata?

A

Intralesional corticosteroids

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23
Q

What is the difference in appearance of tinea capitis and alopecia areata?

A

Both cause localized area of hair loss but tinea capitis has scaling

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24
Q

Which triptan is FDA-approved for young children?

A

Rizatriptan

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25
Q

What is the difference in presentation of alpha-1-antitrypsin deficiency in children and adults?

A

Children: liver disease
Adults: liver AND lung disease

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26
Q

What is the most common lab abnormality associated with anabolic steroid misuse?

A

Increased LFTs

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27
Q

Which lab can distinguish between hypoparathyroidism and pseudohypoparathyroidism?

A

Hypoparathyroidism: normal PTH
Pseudohypoparathyroidism: high PTH

Both present with low calcium and high phosphorous

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28
Q

What is the presentation of mucopolysaccharidosis type 2 (Hunter syndrome)?

A

Coarse facial features, hypertelorism, joint stiffness, myocardial hypertrophy, pulmonary HTN

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29
Q

What is the management of epidural hematoma?

`

A

Urgent NSGY evaluation due to lucid interval followed by herniation

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30
Q

What is the management of children with parvovirus B19 infections who are around pregnant women?

A

Can return to those settings once slapped-cheek rash appears because that indicates end of contagious period

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31
Q

When do night terrors occur during the sleep cycle?

A

Non-REM sleep during first 1/3 of the night

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32
Q

What is the presentation of chancroid?

A

Painful unilateral inguinal LAD associated with painful genital ulcer

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33
Q

What is the cause of chancroid?

A

H. ducreyi

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34
Q

What is the most common bacteria that causes blistering distal dactylitis?

A

Group A Strep

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35
Q

At what age can children sit without support?

A

9 months

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36
Q

What is the presentation of Peutz-Jeghers syndrome?

A

Intestinal hamartomas (causing colicky abdominal pain secondary to intussusception) and mucocutaneous nodules in the mouth and around the lips

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37
Q

What bone abnormality is seen in lead poisoning?

A

Linear hyperdensities in the metaphyses of bone (lead lines)

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38
Q

What capillary lead level warrants confirmation with a venous sample?

A

> 5

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39
Q

What lead level necessitates chelation?

A

45+

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40
Q

What is the management of urethral prolapse in females?

A

Topical estrogen cream

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41
Q

What is the treatment of dermatitis herpetiformis?

A

Dapsone

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42
Q

What is the presentation of pityriasis lichenoides et varioliformis acuta (PLEVA)?

A

Papules with hemorrhagic crusting followed by hypopigmented or hyperpigmented macules

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43
Q

What is the treatment for pityriasis lichenoides et varioliformis acuta (PLEVA)?

A

Oral tetracyclines

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44
Q

What is the inheritance pattern of G6PD deficiency?

A

X-linked recessive

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45
Q

What is the presentation of Mayer-Rokitansky-Kuster-Hauser syndrome?

A

Underdeveloped upper vagina and uterus

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46
Q

Which nerve roots are affected in Erb and Klumpke palsies?

A

Erb: C5, C6
Klumpke: C8, T1

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47
Q

What is the appropriate post-exposure prophylaxis after exposure to hep A in unimmunized individuals?

A

Hep A vaccine (preferred) or immunoglobulin

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48
Q

Which STI is associated with punctate hemorrhages on the vagina and cervix?

A

Trichomoniasis

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49
Q

What is the treatment for bacterial vaginosis?

A

Metronidazole for 7 days

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50
Q

What is a highly specific antibody for lupus?

A

Anti-smith antibody

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51
Q

Which lab tests are typically positive in rheumatoid arthritis?

A

RF and anti-CCP antibody

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52
Q

What is the management of nevus sebaceous?

A

Elective excision due to cosmetic concerns and risk of malignant transformation

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53
Q

What is the presentation of Alport syndrome?

A

Glomerulonephritis and hearing loss

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54
Q

What is the diagnosis of a child who recently underwent cardiac surgery who presents several weeks later with cough, fever, dyspnea, and chest pain?

A

Post-pericardiotomy syndrome

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55
Q

Which cardiac anomalies are associated with a biphasic pulse?

A

Aortic stenosis and hypertrophic obstructive cardiomyopathy

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56
Q

What is the management of juvenile plantar dermatosis?

A

Avoid impermeable shoes, absorbent powders, and topical emollients

“Sweaty sock syndrome”

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57
Q

Which age group is at the highest risk of child abuse?

A

Birth-1 year

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58
Q

At what ages do Wilms tumors typically present?

A

3-4

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59
Q

Which genetic mutation is commonly associated with hereditary pancreatitis?

A

PRSS1

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60
Q

Which genetic mutation is strongly associated with Crohn’s disease?

A

NOD2

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61
Q

Which genetic mutation is associated with Alagille syndrome?

A

JAG1

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62
Q

What is the management of children < 12 months of age who are traveling to measles-endemic areas?

A

1 MMR vaccine in children 6-12 months

Children < 6 months still have protection from transplacental antibodies

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63
Q

What are the most significant risk factors for later development of asthma?

A

Parent with asthma and/or personal history of concurrent eczema

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64
Q

When should children develop a pincer grasp?

A

12 months

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65
Q

How does selenium deficiency present?

A

Hypothyroidism and cardiomyopathy

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66
Q

At what ages is separation anxiety developmentally appropriate?

A

6 months-3 years

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67
Q

Which cardiac anomaly is associated with a fixed split S2?

A

ASD

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68
Q

What is the presentation of phenylketonuria?

A

Light-colored hair and skin, eczema, seizures, developmental delay

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69
Q

What is the cause of homocystinuria?

A

Deficiency of cystathionine beta-synthase

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70
Q

How does homocystinuria present?

A

Marfanoid body habitus, downward dislocation of the lens, thromboembolism

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71
Q

What are the tetanus immunoglobulin and vaccine guidelines after a wound?

A

Tdap preferred over Td in children > 7

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72
Q

What is the first-line treatment for idiopathic intracranial hypertension?

A

Acetazolamide

Carbonic anhydrase inhibitor

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73
Q

What is the presentation of Denys-Drash syndrome?

A

Wilms tumor, nephropathy, ambiguous genitalia

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74
Q

Which disease is associated with anti-smooth muscle antibody?

A

Autoimmune hepatitis

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75
Q

What neutrophil count is expected in cyclic neutropenia?

A

< 200

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76
Q

Where is the pain in tennis elbow versus golfer’s elbow?

A

Tennis elbow: lateral epicondyle
Golfer’s elbow: medial epicondyle

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77
Q

Which limb abnormality is associated with trisomy 13?

A

Polydactyly

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78
Q

What is the main presentation of Klippel-Feil syndrome?

A

Fusion of cervical vertebrae

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79
Q

What is the first step in management of eosinophilic esophagitis?

A

Elimination of cow’s milk

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80
Q

What are important adverse effects of IUDs?

A

Uterine perforation and menstrual irregularities

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81
Q

When is the peak incidence of ALL?

A

2-5 years

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82
Q

What ages are poor prognostic indicators in ALL?

A

< 1 or > 10 years

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83
Q

What WBC count at diagnosis is a poor prognostic indicator in ALL?

A

> 50,000

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84
Q

What is the presentation of tyrosinemia type I?

A

Hepatic and renal dysfunction

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85
Q

How is tyrosinemia type I diagnosed?

A

Urine succinylacetone level

Presents with hepatic and renal dysfunction

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86
Q

What is the treatment of tyrosinemia type I?

A

NTBC

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87
Q

What lab findings are associated with fatty acid oxidation disorders?

A

Hypoglycemia with absence of urine reducing substances and ketones

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88
Q

How are fatty acid oxidation disorders diagnosed?

A

Plasma acylcarnitine profile

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89
Q

What is the presentation of nail-patella syndrome?

A

Nail hypoplasia or dysplasia, orthopedic anomalies, and kidney disease

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90
Q

What are the primary physical exam findings in Fanconi anemia?

A

Hypoplastic thumbs, hyperpigmented skin and/or cafe-au-lait spots, short stature, abnormal radii

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91
Q

Which type of cancer are patients with Fanconi anemia most at risk of developing?

A

AML

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92
Q

Which infection is associated with rash that develops after defervescence?

A

Roseola caused by HHV-6

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93
Q

What are the guidelines for screening children for hyperlipidemia?

A

All children 9-11 and 17-21

High-risk patients such as those with obesity or strong family history of hyperlipidemia/metabolic syndrome/MI should be screened between 2-8

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94
Q

What is the pathognomonic EEG finding with benign childhood epilepsy?

A

Centrotemporal spikes

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95
Q

What type of seizure is typical of benign childhood epilepsy?

A

Focal aware seizure

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96
Q

What is the management of Klinefelter syndrome?

A

Testosterone replacement

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97
Q

Which condition is associated with cardiac rhabdomyomas early in life?

A

Tuberous sclerosis

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98
Q

What is the hallmark pulmonary finding in Alagille syndrome?

A

Peripheral pulmonary stenosis

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99
Q

What is the hallmark orthopedic finding in Alagille syndrome?

A

Butterfly vertebrae

100
Q

Which malignancy is associated with opsomyoclonus?

A

Neuroblastoma

101
Q

Which lab findings are associated with neuroblastoma?

A

Elevated urinary HVA and VMA

102
Q

What is the first-line treatment for head lice?

A

Topical permethrin

103
Q

What is the cause of myotonic dystrophy?

A

CTG repeats in DMPK gene

104
Q

How does myotonic dystrophy present?

A

Weakness of distal muscles with relative sparing of proximal strength, facial muscle atrophy, cardiac abnormalities

105
Q

What is the recommended maximum of juice intake for children under the age of 5?

A

No more than 6 oz per day

106
Q

What is a possible neurologic consequence of chronic cocaine use?

A

Ischemic or hemorrhagic stroke

107
Q

What is the most common complication of sickle cell disease in children less than 2?

A

Dactylitis

108
Q

What are the relative dystrophin levels in Becker muscular dystrophy and Duchenne muscular dystrophy?

A

Becker: reduced
Duchenne: absent

109
Q

Which muscle groups are typically affected in myotonic dystrophy?

A

Face and jaw

110
Q

Which groups need antibiotic prophylaxis against infective endocarditis?

A

Patients with a prosthetic heart valve, unrepaired cyanotic heart disease, repaired congenital heart defect (for first 6 months after repair)

111
Q

What is the next diagnostic step if a RUQ US is equivocal for cholecystitis but clinical suspicion remains high?

112
Q

Which antibodies are seen in glomerulonephritis after Strep pharyngitis and impetigo?

A

Strep pharyngitis: ASO
Impetigo: anti-DNAse B

113
Q

What are the most common adverse effects of long-acting progestin contraception?

A

Irregular menses and delayed return of fertility

114
Q

When can children with Strep throat return to school?

A

Afebrile and on antibiotics for at least 12 hours

115
Q

Which lab findings are associated with autoimmune hepatitis?

A

Hypergammaglobulinemia (high IgG) and anti-smooth muscle antibiodies

116
Q

What is the presentation of essential fatty acid deficiency?

A

Delayed neurodevelopment, eczematous dermatitis, reduced growth velocity

117
Q

What is the vaccine schedule for PCV-13?

A

2, 4, 6 months followed by 12-5 months

118
Q

What are the typical symptoms of hyperimmunoglobulin D syndrome?

A

Periodic fever with tender LAD, abdominal pain, arthralgias/arthritis, splenomegaly

119
Q

What is the preferred treatment of infantile spasms secondary to tuberous sclerosis?

A

Vigabatrin

120
Q

Which nutritional deficiency is associated with development of hypothyroidism?

A

Selenium deficiency

121
Q

What is the presentation of adrenoleukodystrophy?

A

Behvioral issues, hyperpigmentation, gait issues, slurred speech

122
Q

What lab finding is associated with adrenoleukodystrophy?

A

Elevated levels of very long-chain fatty acids

123
Q

Which mutation is associated with Prader-Willi syndrome?

A

Chromosome 15q

124
Q

How is Fanconi anemia diagnosed?

A

Chromosomal breakage analysis followed by genetic sequencing

125
Q

What are the adverse effects of creatine?

A

Muscle cramps and weight gain

126
Q

What is the management of juvenile fibroadenomas of the breast?

A

Observation and reevaluation every 1-2 months if < 3 cm

127
Q

What is the refrigerator shelf life of breast milk?

128
Q

How does early- and late-onset Listeria infection present in neonates?

A

Early-onset: PNA, sepsis
Late-onset: meningitis

Early-onset = < 7 days

129
Q

What is the silk-glove sign?

A

Suggests inguinal hernia rather than hydrocele

Secondary to persistent processus vaginalis

130
Q

Which age group should receive antibiotic treatment for Salmonella gastroenteritis?

A

< 3 months

131
Q

What is the typical cause of infectious mono if EBV is negative?

132
Q

Which lab finding is characteristic of alkaptonuria?

A

Elevated homogentisic acid

133
Q

What is the difference in presentation of nightmares and night terrors?

A

Nightmares: vivid remembrance of the dream
Night terrors: distress with no recollection of the dream

134
Q

What is the most common pathogen that causes chronic rhinosinusitis?

135
Q

What is the most common pathogen that causes acute otitis media?

136
Q

When do patients with X-linked agammaglobulinemia start to develop infections?

A

~4-6 months of age when transplacentally acquired antibodies decline

137
Q

Which disease is associated with anti-histone antibodies?

A

Drug-induced lupus

138
Q

What are the most common bacteria that cause osteomyelitis in patients with sickle cell disease?

A

S. aureus and Salmonella

139
Q

Which cardiac anomaly is associated with Noonan syndrome?

A

Pulmonic stenosis

140
Q

What is the expected testicular volume at the end of puberty?

141
Q

What is the management of acute adrenal crisis?

A

Hydrocortisone 50-100 mg/m2

Fludrocortisone not needed because high-dose hydrocortisone has mineralocorticoid effects

142
Q

Which lab findings can be expected in the window period for acute hepatitis B infection?

A

Negative HBsAg and positive anti-HBc IgM

143
Q

How does spasmus nutans present?

A

Nystagmus, torticollis, and head nodding

144
Q

How does Parinaud syndrome present?

A

Impaired upward gaze, nystagmus, and dilated pupils

145
Q

Which tetanus vaccine preparation is indicated for previously unvaccinated children > 7 years?

146
Q

When would an IPV booster be indicated prior to international travel?

A

Adults 18+ years of age, even those with 4 previous IPV vaccines

4 doses of IPV confers lifelong immunity but should receive another dose as a precaution

147
Q

What is the Russell sign?

A

Calluses on knuckles secondary to repeated induced vomiting

Often seen with bulimia

148
Q

What is the required minimum interval between administration of live vaccines?

149
Q

Which biomarkers are associated with Langerhans cell histiocytosis?

A

CD1a, CD207, S100

150
Q

Which cardiac manifestation is associated with congenital rubella?

151
Q

Which medical conditions are associated with increased risk of SCFE?

A

Hypothyroidism and GH deficiency

152
Q

What is the preferred treatment of tularemia?

A

Gentamicin or streptomycin

153
Q

What are the components of tetralogy of Fallot?

A

Large VSD, right ventricular outflow obstruction, RVH, and overriding aorta

154
Q

How does partial androgen insensitivity present?

A

Ambiguous genitalia at birth

155
Q

How do NF1 and NF2 present?

A

NF1: cafe-au-lait spots, neurofibromas, Lisch nodules, optic glioma
NF2: bilateral acoustic neuromas (schwannoma)

156
Q

Which congenital anomalies are associated with warfarin use during pregnancy?

A

Nasal hypoplasia and stippled epiphyses

157
Q

Which congenital anomalies are associated with anti-epileptic use during pregnancy?

A

Neural tube defects

158
Q

Which cardiac anomalies are associated with fetal alcohol syndrome?

A

VSD and ASD

159
Q

What is a common adverse effect of inhalant abuse?

A

Pneumothorax and pneumomediastinum

160
Q

How can Noonan syndrome be easily differentiated from Turner syndrome?

A

Pulmonic murmurs and hypertelorism

161
Q

What type of intussusception is associated with IgA vasculitis (HSP)?

A

Ileoileal

In contrast to ileocolic with idiopathic intussusception

162
Q

What is the treatment of cutaneous larva migrans?

A

Albendazole or ivermectin

163
Q

What is the treatment of neonatal chlamydia conjunctivitis?

A

Oral erythromycin

164
Q

What is the empiric treatment of gonorrhea and chlamydia?

A

Ceftriaxone x1 for gonorrhea and doxycycline x10 days for chlamydia

165
Q

What is appropriate post-exposure prophylaxis for pertussis?

A

Azithromycin

Can use erythromycin or clarithromycin but avoided in children < 1 month of age due to risk of pyloric stenosis (especially with erythromycin)

166
Q

What is a main contraindication to ketamine?

A

Age < 3 months of age

167
Q

Which condition is associated with benign paroxysmal vertigo of childhood?

A

Increased risk of later development of migraines

168
Q

What is typically the first sign of Rett syndrome?

A

Deceleration of head circumference growth at ~3 months of age

169
Q

What is the presentation of incontinentia pigmenti?

A

Vesicles and bullae in a swirling pattern, dental abnormalities, and peripheral eosinophilia

170
Q

What is the mutation in Kallmann syndrome?

A

Mutation in ANOS1 at Xp22.3

171
Q

Which mutation leads to Noonan syndrome?

A

Deletion of chromosome 12q

172
Q

What EKG findings are seen in Wolff-Parkinson-White syndrome?

A

Shortened PR and prolonged QRS intervals

173
Q

When is the typical onset of gonorrhea and chlamydia conjunctivitis in neonates?

A

Gonorrhea: early at 2-5 days of age
Chlamydia: later at 5-12 days of age

174
Q

What are the causes of pseudohyperkalemia?

A

Thrombocytosis and leukocytosis

175
Q

What is the presentation of Waardenburg syndrome?

A

Premature graying, hearing loss, and iris heterochromia

176
Q

What is the most common presentation of Meckel diverticulum in children?

A

SBO

Can also present with painless bright red bleeding per rectum

177
Q

Where is the tumor often located in neuroblastoma?

A

Adrenal glands

178
Q

What is Legg-Calve-Perthes disease?

A

Avascular necrosis of the hip

179
Q

Where is the pain localized in Osgood-Schlatter disease?

A

Tibial tuberosity

180
Q

What is failure modes and effects analysis (FMEA)?

A

Proactively identifying and addressing potential sources of failure before sentinel events occur

181
Q

When can most children walk up and down the stairs with one foot on each step?

A

4 years of age

182
Q

When can most children hop on one foot?

A

5 years of age

183
Q

What is the treatment of linear IgA bullous dermatosis?

184
Q

When can a tuberculin skin test be performed after MMR administration?

A

4-6 weeks after MMR administration because it temporarily suppresses tuberculin skin test activity

Okay to get TB test on same day as MMR administration

185
Q

What is the pathophysiology of factor 5 Leiden mutation?

A

Protein C resistance

186
Q

What is the difference between Apert and Crouzon syndromes?

A

Both cause craniosynostosis but Apert syndrome also causes syndactyly of the 2nd-4th digits

187
Q

What is found on a CBC in pertussis?

A

Leukocytosis with lymphocytic predominance

188
Q

Which interstitial lung disease is associated with HIV in children?

A

Lymphocytic interstitial pneumonitis

189
Q

What are the criteria for psoriatic arthritis?

A

Arthritis and psoriasis OR arthritis and 2/3 of the following:
* dactylitis
* nail findings
* first-degree relative with psoriasis

190
Q

How long should individuals with mono and splenomegaly refrain from strenuous exercise and contact sports?

A

At least 4 weeks and until the spleen recedes

191
Q

What is the appropriate post-exposure prophylaxis against measles in children < 1 year of age?

A

< 6 months of age: IMIG or IVIG
6-12 months of age: MMR vaccine or IMIG or IVIG

192
Q

What is the presentation of roseola?

A

Rash following defervescence, palpebral/periorbital edema, papules on soft palate and uvula

193
Q

What is the presentation of Chediak-Higashi syndrome?

A

Oculocutaneous albinism and immunodeficiency (skin and mucosal infections)

Large granules in leukocytes

194
Q

Which congenital cardiac anomaly is associated with a snowman appearance on CXR?

195
Q

Which nutritional deficiency is associated with corn-heavy diets?

A

Nicotinic acid (vitamin B3) leading to pellagra

Diarrhea, dementia, dermatitis

196
Q

```

~~~

What are the symptoms of riboflavin (vitamin B2) deficiency?

A

Cheilosis and sore tongue

197
Q

What are the symptoms of thiamine deficiency?

A

Wernicke’s encephalopathy (ophthalmoplegia, ataxia, and confusion) and cardiac dysfunction

198
Q

What is the recommended vaccine schedule to prevent against Neisseria infection in asplenic infants?

A

MenACWY-CRM (Menveo) at 2, 4, 6, and 12 months of age

199
Q

What do eosinophils look like on peripheral smear?

A

Bilobed cells with granules

201
Q

What are the FSH and LH levels in PCOS?

A

Increased LH:FSH but absolute LH and FSH are usually within normal range

202
Q

When should antihistamines be stopped prior to skin prick testing?

A

Short-acting antihistamines: stop 48 hours before testing
Long-acting antihistamines: stop 5 days before testing

203
Q

What is Smith-Lemli-Opitz syndrome?

A

Disorder of cholesterol metabolism leading to elevated 7-dehydrocholesterol levels and decreased plasma cholesterol levels along with microcephaly, micrognathia, and hypertelorism

204
Q

What is Menkes disease?

A

Disorder of copper metabolism leading to decreased copper and ceruloplasmin levels

205
Q

What is the mutation in Tay-Sachs disease?

A

Beta-hexosaminidase

206
Q

Which drugs are most commonly implicated in toxic epidermal necrolysis (TEN)?

A

Anti-seizure medications (i.e. carbamazepine and phenobarbital), penicillins, and cephalosporins

207
Q

What is the treatment for cholinergic toxicity in the setting of organophosphate exposure/ingestion?

A

Atropine (blocks ACh receptor) and pralidoxime (prevents irreversible binding to cholinesterase)

208
Q

How is Becker muscular dystrophy different than Duchenne muscular dystrophy?

A

Later onset (late childhood into adolescence) and milder course

209
Q

Which potential adverse effect is associated with MMRV vaccine as opposed to separate MMR and varicella vaccines?

A

Febrile seizures

210
Q

What is the presentation of congenital varicella infection?

A

Abnormalities of the eyes (i.e. cataracts), limbs, skin (i.e. scarring), and CNS

211
Q

Which condition is most associated with aplasia cutis congenita?

A

Trisomy 13

212
Q

What is the treatment of metabolic crisis in medium-chain acyl-CoA dehydrogenase (MCAD) deficiency?

A

Carnitine supplementation

213
Q

What are the symptoms of hypervitaminosis A?

A

Hepatomegaly, bone pain secondary to hyperostosis, dry skin and mucous membranes, pseudotumor cerebri

214
Q

What is the appearance of tinea versicolor under the microscope?

A

Spaghetti and meatball appearance

215
Q

Which condition is associated with rash just during febrile episodes?

A

Systemic JIA

216
Q

Which lab finding is associated with chlamydia PNA?

A

Peripheral eosinophilia

217
Q

What is the treatment of complicated Lyme disease (i.e. with meningitis and/or carditis)?

A

Ceftriaxone

218
Q

What are the screening and confirmatory tests for Lyme disease?

A

Screening: ELISA
Confirmatory: Western blot

219
Q

What are the symptoms of Behcet’s disease?

A

Recurrent oral aphthous ulcers, neurologic complications, and arterial aneurysms

220
Q

What affect does splenic sequestration have on platelets?

A

Thrombocytopenia due to sequestration of platelets in the spleen

221
Q

Which condition can occur concomittantly with lupus and increase risk of pyogenic infections?

A

C2 deficiency

222
Q

What is the presentation of properdin deficiency?

A

Recurrent meningococcal infection

223
Q

What is considered a normal sweat chloride test result?

224
Q

What is the presentation of Shwachman-Diamond syndrome?

A

Cytopenias with neutropenia, bifid thumbs, growth failure, pancreatic insufficiency

Often compared to CF because both present with pancreatic insufficiency

225
Q

What is the treatment of lymphogranuloma venereum?

A

Doxycycline for 3 weeks

226
Q

Which bacteria causes lymphogranuloma venereum?

A

Chlamydia trachomatis

227
Q

What is the most common cardiac abnormality in Turner syndrome?

A

Bicuspid aortic valve

Coarctation of aorta is second most common anomaly

228
Q

What is the appropriate post-exposure prophylaxis against varicella?

A

Unimmunized close contacts > 1 year of age should receive varicella vaccine

229
Q

What are the expected lab values in central hypothyroidism?

A

Low free T4 and inappropriately low TSH

TSH should elevate in response to low free T4 in cases of primary (and not central) hypothyroidism

230
Q

Which benign neonatal rash is associated with neutrophils under the microscope?

A

Transient neonatal pustular melanosis

231
Q

When should patients with cryptorchidism be referred to surgery?

A

6 months of age

232
Q

After patients with trisomy 21 develop transient myeloproliferative disorder, which cancer are they at higher risk of developing in the future?

233
Q

What is the most common cause of erythema multiforme?

234
Q

What are the criteria for simple febrile seizure?

A
  • < 15 minutes
  • Only 1 episode in 24 hours
  • Generalized seizure
235
Q

What is the most common adverse effect of L-asparaginase?

A

Pancreatitis

236
Q

What is the cause of Bernard-Soulier syndrome?

A

Deficiency of glycoprotein 1b

237
Q

Which seizure/mood medication is associated with hyponatremia?

A

Oxcarbazepine

238
Q

If manual detorsion of a testicle is attempted, which direction of rotation is appropriate?

239
Q

Which finding can differentiate between PCP and cocaine use?

A

PCP causes nystagmus whereas cocaine does not

240
Q

Which lab finding is seen in Lesch-Nyhan syndrome?

A

Elevated uric acid levels in blood and urine

Caused by mutation in enzyme responsible for hypoxanthine metabolism

241
Q

Which cardiac anomaly is associated with an “egg on a string” appearance on CXR?

242
Q

What differentiates SJS and TEN?

A

SJS: < 10% epidermal detachment

TEN: > 30% epidermal detachment

Patients with 10-30% epidermal detachment are considered to have a mixed picture

243
Q

What is the treatment for serotonin syndrome?

A

Cyproheptadine

244
Q

What birth weight threshold is associated with neonatal hypoglycemia?

245
Q

When can children draw a circle?

A

3 years of age

Pediatric Boards - YOU CAN DO IT! (1 decks)

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