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abp - peds boards > General > Flashcards

General Flashcards

1
Q

Small retinal hemorrhages with a whiteish-pale center are what and associated with what entity? They can also be edematous, exudative, hemorrhagic lesions of the eye.

A

Roth spots

Bacterial endocarditis

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2
Q

Non tender erythematous nodular lesions on the palms or soles

A

Janeway lesions

Bacterial endocarditis

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3
Q

Painful, raised lesions on palms and soles

A

Osler node

Bacterial endocarditis

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4
Q

Besides coronary artery aneurysm, what is another concerning finding in Kawasaki disease?

A

Gallbladder hydrops - RUQ mass that usually occurs in the first 2 weeks of illness and prevents as enlarged, balloon-shaped bladder on ultrasound.

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5
Q

How long after symptom onset is hepatitis A virus

A

7 days

Hep A booster are NOT recommended even if someone near you has infection.

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6
Q

Hypotension
Cyanosis
Are likely due to consumption of what substance?

What condition is induced?

How is it addressed?

A

Due to amyl nitrite (poppers)

Methemoglobinemia

Treatment: Methylene blue

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7
Q

How is methanol and ethylene glycol poisoning addressed?

A

IV alcohol or fomepizole

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8
Q

What are the characteristics of methemoglobinemia?

A

Cyanosis
Chocolate colored blood
Decreased O2sat but a normal PaO2 (partial pressure of oxygen)

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9
Q

What drugs can cause methemoglobinemia?

A

Aniline dyes
Nitrites
Nitrates

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10
Q

What are the critical labs?

A 
Cortisol
Glucose
Insulin
Growth factor
Ketones 
(lactate, pyruvate)
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11
Q

What is a laboratory marker of a testicular neoplasm?

A

Alpha feto protein

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12
Q

A port wine stain in the V1 distribution on a baby face means…

A

Sturge Weber syndrome. Risk of seizures and glaucoma due to capillary vascular malformations

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13
Q

What is a rare complication of adenovirus?

A

Hemorrhagic cystitis.

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14
Q

What time of ocular hemorrhages are associated with coughing?

A

Subconjunctival hemorrhages

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15
Q

What is the CBC like in pertussis?

A

Leukocytosis with lymphocytosis

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16
Q

What syndrome does a
Child with triangular shaped face, downslanting palpebral fissures, hypertelorism, short webbed neck have?

What other medical disease is associated with this syndrome?

A

Noonan syndrome

Bleeding diasthesis e.g. factor 11 deficiency

Heart: congenital heart defects — pulmonic stenosis

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17
Q

Increased melanin production should make me think of what endocrine disease?

A

Addison - primary adrenal insufficiency. Hypotension, hyponatremia, hyperkalemia, hypoglycemia. Metabolic acidosis.

Dehydration leads to postural changes in BP/HR.

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18
Q

What are some of the side effects of MDMA?

A

MDMA is ecstasy. It’s side effects are hyponatremia from excessive water drinking due to hyperthermia. Also can cause seizures due to hypoglycemia.

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19
Q

What is GHB?

A

Gamma hydroxy-butyrate. Date rate drug that produces euphoria and heightened sexuality. Also causes amnesia like flunitrazepam.

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20
Q

If a baby is born to a mom who is HepBsAg +ve but HepBeantigen negative, what is best next step?

A

Give HBIG and HepB vaccine at birth, within first 12 HOL and give Hep B vaccine at 1-2 and 6 months of age. Also, test for HepBsAg and anti-HepBsAg at 9 months of age.

This infants have a 5-20% risk of chronic Hep B infection whereas infants born to a mom who was also HepBeAg positive have a 40% chance of chronic Hep B infection.

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21
Q

Rib notching on X-ray is a sign of?

A

aortic coarctation

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22
Q

What is a pansystolic murmur associated with R ventricular lift a sign of?

A

R ventricular hypertrophy e.g. pulmonary HTN or VSD

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23
Q

What is a midsystolic click associated with a late systolic murmur associated with?

A

Mitral regurgitation with mitral valve prolapse

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24
Q

Prominent systolic ejection murmur at RUSB with systolic click best heard at apex

A

Aortic valve stenosis

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25
Q

What is the minimum time apart that LIVE vaccines (e.g. MMR and varicella) must be separated in administration if they are not given on the same day? Why?

A

28 days because the second LIVE vaccine may have decreased immune response.

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26
Q

How many kcal/g does fat have?

A

9kcal/g

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27
Q

How many kcal/g do carbs and protein have?

A

4kcal/g

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28
Q

A child born with deafness and prolonged QT interval has?

A

Jervell-Nielsen syndrome - autosomal recessive

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29
Q

A brownish-yellow discoloration in the posterior part of the cornea on slit-lamp exam is a …

A

Kayser-Fleischer ring associated with Wilson disease which can first have an insidious neurologic presentation.

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30
Q

What are the signs of LSD ingestion?

A

Hypertension, tachycardia, dilated pupils, hyperthermia.

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31
Q

A hypoplastic and high-riding scapula is often associated with?

A

Fused, cervical vertebrae in Sprengel deformity. There is also usually an ossified band tethered from the scapula to the cervical spine. This finding can occur in Klippel-Feil syndrome and be associated with heart, lung, or kidney abnormalities.

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32
Q

3 weeks of back pain in a child with tingling in their hands is a cause for what work-up?

A

Emergent MRI

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33
Q

In Patau syndrome (Trisomy 13), what is a derm finding?

A

Aplasia cutis congenita - sharply dermarcated ulcerated plaques.

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34
Q

An abdominal tumor high in alpha fetoprotein in a neonate is probably a …

A

Hepatoblastoma

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35
Q

What signs are associated with zinc deficiency?

A

Poor growth, rash (similar to acrodermatitis enteropathica), hypogeusia.

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36
Q

What signs are associated with copper deficiency?

A

neutropenia, osteopenia, hypercholesterolemia. If congenital then get Menkes dx with brittle/kinky hair, hypothermia, blood vessel tortuosity, decreased myelination, seizures, mental retardation

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37
Q

A full face, broad forehead, flattened nasal bridge and rounded full cheeks are seen in what syndrome?

What cardiac anomaly do they usually have, best heard at 1st R intercostal space and associated with a thrill in the suprasternal notch?

What is their personality like?

A

Williams syndrome. They also have a wide mouth.

Supravalvular aortic stenosis

Very social, strong expressive language skills, often perceived as charming.

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38
Q

Elevated serum levels of homogentisic acid is associated with?

A

Alkaptonuria

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39
Q

Elevated serum levels of alloisoleucine are associated with?

A

Maple syrup urine disease

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40
Q

What are the presenting symptoms of transverse myelitis?

A

Sudden onset localized lower back pain, paresthesias, painful burning, tingling, pricking, loss of sensation and even complete paralysis of legs. Sensitive to touch.

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41
Q

At what age or more should patients with sickle cell disease receive PPSV23 and MCV4?

A

2 years of age or more

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42
Q

1-5 days after blunt abdominal trauma, an individual presenting with vomiting and an upper abdominal mass may have a…

What are the X-ray findings?

A

duodenal hematoma (intramural accumulation of blood) - caused by compression of the duodenal wall against the vertebral column.

X-ray shows an air-fluid level in a dilated duodenal loop, a distended air-filled stomach and paucity of gas elsewhere in the GI tract.

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43
Q

Beyond 8-10 weeks of age, what sign is best to assess Developmental dysplasia of the hip?

A

Galeazzi sign

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44
Q

At what age is hip ultrasound best to assess for developmental dysplasia of the hip?

A

4 months or less

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45
Q

In an immunocompromised patient, a rapidly spreading itchy rash with papules and thick whitish crust-like scale is ? How is it diagnosed?
What is used as treatment and at what minimum age?

A

Crusted scabies/Norweigian scabies
Diagnosed by mineral oil to lesion and smear on slide
Treatment is 5% permethrin and minimum age is 2 or months.

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46
Q

What information about unborn child who died of a metabolic disorder is helpful to know about future siblings?

A

Sex of unborn child

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47
Q

A fetus affected by oligohydramnios, later born as a baby with flattened hands, widely separated hands, low set ears, and flattened facial features was likely exposed to what medication in utero?

A

Enalapril

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48
Q

What does the osmotic fragility test diagnose?

A

RBC membrane defects e.g. hereditary spherocytosis

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49
Q

A swollen, erythematous to purple cervical lymph node of 2 week duration, not responsive to beta-lactam antiobiotics is probably due to?

A

Mycobacterium avium intracellulare

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50
Q

Irregularity, metaphyseal fraying, and cupping are concerning for…

A

Rickets - can be associated with scoliosis

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51
Q

A firm, non-tender, palpable mass at the base of the L sternocleidomastoid but not anterior to it is consistent with…

A

Birth trauma

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52
Q

In a neonate who experienced any type of perinatal asphyxia or traumatic event, sudden onset gross hematuria and a new abdominal mass are c/w…

A

Renal vein thrombosis

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53
Q

Decreased quantities of what vitamin can prevent nephrolithiasis in a teen?

A

Decreased Vit C because high levels of vitamin C increase oxalate and cause calcium oxalate stones.

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54
Q

Omphalocoele is associated with what chromosomal defect?

A

Trisomy 13

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55
Q

Vaginal secretions normally have what pH and what type of bacteria are a normal finding?

A

pH <4.0-4.5 and lactobacilli are a normal finding.

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56
Q

In a patient with impaired fasting glucose, what is the next best test to assess if they have diabetes?

A

Glucose tolerance test. NOT random glucose.

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57
Q

In someone who has taken an SSRI, tachycardia, hypertension and diaphoresis could be suggestive of _____ (condition).

Treatment is ____

A

Serotonin syndrome

benzodiazepine and Cyproheptadine

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58
Q

What is Menkes disease?

A

X-linked dysfunction of copper metabolism with impaired absorption and transplant leading to low serum copper levels.

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59
Q

How does Neurofibromatosis 2 present?

A

BIlateral acoustic neuromas (tinnitus, unsteady gait, hearing loss).

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60
Q

A 15 yr old F with seizure disorder and partial anodontia and pegged teeth has numerous hypopigmented and atrophic streaks on arms, thighs, calves. Previously these lesions were bluish-brown. What is the condition and how did the lesions start out?

A

Incontinentia pigmenti (X-linked dominant)

Lesions started as inflammatory vesicles on trunk and extremities.

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61
Q

What is the likely CXR finding in Tetralogy of Fallot?

A

An uplifted cardiac apex, indicating RVH associated with concavity of the upper left heart border

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62
Q

Obesity, hypogonadism, (developmental delay, polydactyly) and retinitis pigmentosa is associated with….

A

Laurence-Moon-Biedl/Bardet-Biedl syndrome

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63
Q

In autoimmune polyglandular syndrome Type 2, what diseases do they get?

A

Type 1 DM, Addison, hypothyroidism

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64
Q

In autoimmune polyglandular syndrome Type 1, what diseases do they get?

A

hypoparathyroidism, mucocutaneous candidiasis, chronic active hepatitis, nail disease.

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65
Q

When distinguishing between Hepatitis B vs Hepatitis A, what findings are more associated with Hepatitis B?

A

Extra-hepatic findings like polyarthritis or arthralgias, urticarial rashes, thrombocytopenia. Papular rash like Gianotti-Crosti has also been linked.

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66
Q

A Cobb angle above x degrees in scoliosis is associated with what complications?

A

A cobb angle greater than 90 degrees is associated with restrictive lung disease and cor pulmonale

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67
Q

A patient who recently received amoxicillin and presents with an urticarial rash with central violaceous discoloration is concerning for…
what are the symptoms?

A

serum sickness

symptoms: headache, diarrhea, increased temp, LYMPHADENOPATHY, facial edema, arthralgias.

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68
Q

A patient who has been immobilized for a long time and has a shortened QT interval is concerning for…

A

Hypercalcemia

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69
Q

Diamond-Blackfan anemia is ____type of anemia and has ____ level of reticulocytes

A

Macrocytic anemia

Low reticulocytes

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70
Q

On X-ray, the steeple sign in crou[ is a result of …

A

Loss of the normal shoulders of the subglottic airway

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71
Q

A young child (less than 6 yrs) with recent varicella infection presents with horizontal nystagmus, ataxia, dysarthria, a few weeks after her viral infection. CSF shows mild pleocytosis (or is normal). Diagnosis is:

A

Acute cerebellar ataxia - symptomatic treatment and usually resolves.

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72
Q

EBV infection. Which studies are associated with acute infection vs remote infection.

EA: early antigen
anti-EBNA: EBV nuclear antigen

A

Early antigen (EA) - acute infection. Present when IgM is present and for a few weeks longer.

anti-EBNA - more than 6 months from infection and present for life.

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73
Q

What is the rule for the number of teeth expected at a certain # of months of age?

A 
7 months - 1st tooth
7+4 months (11 months) - 4 teeth
7+(4+4) months (15 months) - 8 teeth
7+4+4+4 months (19 months) - 12 teeth
etc.
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74
Q

What is the treatment for mild-to-moderate rosacea?

A

Topical metronidazole

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75
Q

What is the treatment for severe rosacea?

A

Oral tetracyclines with topical treatment (e.g. topical metronidazole)

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76
Q

In an 8 year old child who has never received DTaP, what is the appropriate catch-up schedule?

A

Tdap (note difference) at visit, followed by 1st Td in 4 weeks and 2nd Td 6 months after 1st Td.

DTap should only be given if less than 7 years old.

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77
Q

When do you consider bariatric surgery in an adolescent?

A

Mature adolescent with SMR 4 or 5, with BMI >120% of the 95%ile rating with a severe comorbidity or BMI > 140% of 95th %ile without a severe comorbidity

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78
Q

What condition is associated with pancytopenia and excessive chromosomal breakage to agents known to cause DNA damage and what are some of the findings?

A

Fanconi anemia – cutaneous hyper-pigmentation, abnormal thumbs/radial bones, microcephaly, small eyes, renal, cardiac or skeletal abnormalities. Incr risk of AML. BMT is curative.

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79
Q

Maculopapular and pustular lesions with central necrosis, fever, pain in wrists/hands/fingers is concerning for…

A

disseminated gonococcal infection

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80
Q

4-month old infant requires what length needle to reach the thigh muscle? The deltoid?

A

1 inch needle - thigh

deltoid - 5/8 to 1 inch

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81
Q

What EKG finding is asssociated with low K+?

A

Flattened T waves

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82
Q

What EKG finding is associated with hypermagnesemia?

A

Prolonged PR and QRS intervals

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83
Q

When treating an adolescent for gonococcal infection, empiric chlamydial treatment is recommended? Yes or No

A

Yes. If >45kg or an adolescent, treatment for both is recommended.

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84
Q

In children < 4years, what is considered a positive PPD even if they have had BCG?

A

Greater than 10mm induration unless they live near someone with known TB, or they are immunosuppressed, or have CXR evidence then greater than 5 mm induration.

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85
Q

What finding is common in RPA (retropharyngeal abscess)?

A

Torticollis

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86
Q

A painless genital pustule that evolves over days to a painful ulcer and is a gram negative is consistent with

A

Haemophilus ducreyi (chancroid disease)

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87
Q

What is tennis elbow?

What motions excerbate it?

A

It is lateral epicondylitis

It is exacerbated by wrist extension and supination

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88
Q

Anemia, thrombocytopenia, in the setting of worsening renal function with recent infection is concerning for?

A

HUS (hemolytic uremic syndrome due to EColi O157:H7)

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89
Q

Celiac disease can present with tooth enamel defects

A

yes

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90
Q

In Trisomy 18 and 21, what level is the AFP? (alpha fetoprotein)?

A

It is low

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91
Q

What is one potential cause of acute chest in a patient with sickle cell disease?

A

Fat embolism from the bone marrow

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92
Q

What level is elevated in Wilson’s disease?

Urine copper
or
Serum copper?

A

Urine copper levels are elevated. Serum copper levels are DEcreased due to decreased ceruloplasmin levels.

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93
Q

A child who appears jaundiced after minimal food intakes and has normal liver enzymes is concerning for…(disease)?

The best next step is?

A

Gilbert’s

Reassurance

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94
Q

What mental health co-morbidity is most common in ADHD?

A

Generalized anxiety disorder

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95
Q

Aniridia and hemihypertrophy are associated with what tumor of young childhood?

A

Wilms tumor - nephroblastoma. WAGR (wilms tumor, aniridia, genitourinary anomalies, reduced intellectual abilities)

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96
Q

White furlock, lateral displacement of the medial canthi, borad nasal bridge is associated with what syndrome?

A

Waardenburg syndrome

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97
Q

What bacteria, other agents the most common for CHRONIC rhinosinusitis?

A

Staph aureus, fungi, gram neg bacilli, anaerobes.

Chronic rhinosinusitis requires symptoms that persist for >12 weeks.

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98
Q

At what age are most kids (90%) able to sit without support?

A

7 months

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99
Q

How should perianal strep, which peaks at 4 years of age, be treated?

A

oral penicillin

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100
Q

Fusion of which suture is most common in craniosynostosis?

A

Midline saggital suture

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101
Q

Respiratory alkalosis with a metabolic acidosis is suggestive of what type of ingestion?

A

Aspirin

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102
Q

A between 5 and 15-year-old presents with a tender hard bump just below his knee. On X-ray, a broad-based mass originating from the surface of the bone.

A

Observation

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103
Q

A tumor in a young child that on CT has hemorrhagic and calcified parts, has elevated HVA/VMA, and on the skin has bluish-purplish subcutaneous nodules is?

A

Neuroblastoma

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104
Q

Children should be in a rear facing carseat until they reach the maximum weight/height for their seat

A

Yes

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105
Q

Children remain in booster seats and not transition to standard seat belts until…

A

They are 4’9” or until they are between ages 8-12

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106
Q

A 10 day old boy with a midline mass in the lower abdomen and a weak urinary stream is concerning for…

A

Posterior urethral valves

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107
Q

A teenager who appears in the ED with concern for a spontaneous pneumothorax in the setting of substance use, probably used what substance?
What symptoms are associated with that substances’s use?

A

Inhalant

Excessive lacrimation, rhinorrhea, salivation. Other symptoms: euphoria, diplopia, dysrhythmia

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108
Q

Myelomeningocele, large head circumference, and club feet are associated with…

A

Hydrocephalus. (and Chiari Type II malformation. ) This is very different than a Type I malformation.

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109
Q

What is pediculosis? It can appear as pale bluish 0.5-to-1cm macules on the lower abdomen, buttocks, and thighs

A

Lice. (Public lice in this case of the bluish macules)

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110
Q

What is the size of breasts in adolescent males consistent with pubertal gynecomastia?

A

less than 4cm

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111
Q

A Marfanoid-habitus child with history of developmental delay or learning difficulty is concerning for…

A

Homocystinuria. Marfan is NOT associated with delay/learning difficulty.

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112
Q

An intensely erythematous, facial rash with circumoral pallor, and a symmetric maculopapular lattice-like rash on extremities is concerning for

A

Parvovirus

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113
Q

Promienent mottling on the left abdominal and thoracic regions of a 4 week old male characterized by reddish-violaceous, reticulated patches with a sharp dermarcation at the mindline is _______ (diagnosis) and associated with _____ (other findings)?

A

Cutis marmorata telangiectatica congenita

Associated with limb overgrowth or undergrowth of the affected side.

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114
Q

What is the most common cause of acute fulminant liver failure in the pediatric population?

A

Drug hepatotoxicity - especially from acetaminophen

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115
Q

When a patient presents with an unplanned pregnancy, what is the best course of action in all states?

A

To perform an assessment of the patient’s safety and then involve the parent in the discussion.

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116
Q

10 yr old girl with hypopigmentation around the vulva and perianal skin with associated erythema, hemorrhagic erosions, several small tears, excoriations is consistent with ______ (diagnosis). What is the treatment?

A

Diagnosis: Lichen sclerosus
Treatment: Topical corticosteroid

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117
Q

What laboratory abnormality is common with anabolic steroids?

A

Elevated serum transaminases

also Decreased HDL and increases LDL

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118
Q

What is the most common cause of a simple, follicular ovarian cyst?

A

Cigarette smoking

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119
Q

Oral contraceptives decrease the risk of ovarian cyst formation

A

repeat

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120
Q

What is the treatment for PCP pneumonia in a newborn?

A

Bactrim and corticosteroids

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121
Q

3 yr old boy, small for his age, found to have temp T 105.6F, hypopigmented eczematous patches, flat nasal bridge, and numerous fine wrinkles around his eyes is concerning for _____ and likely to have what other findings?

A

Ectodermal dysplasia with the complete/partial absence of eccrine sweat glands and wide-spaced conical (peg-shaped) teeth.

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122
Q

A teenager who has consumed a substance that causes a non-gap metabolic acidosis and hypokalemia has probably consumed ____?

A

Toluene

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123
Q

What are treatments for hidradenitis suppuritiva?

A

Antibiotics for anti-inflammatory not anti-infectious purposes and spironolactone for anti-androgenic purposes.

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124
Q

When are coronary artery aneursyms seen in Kawasaki Disease?

A

After 10 days of illness

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125
Q

What percentage of kids with untreated KD will get coronary artery aneurysms?

A

25%

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126
Q

Getting IVIG and aspirin in the first 10 days of illness, reduces the risk of coronary artery aneurysm in KD

A

Yes

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127
Q

Which meningitis vaccine and schedule is appropriate for a 2 month old s/p splenectomy?

A

Menveo (ACYW-CRM) - 2, 4, 6, and 12 month doses. Can also get Hib-MenCY-TT (MenHibrix) at 2,4,6, and 12 months

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128
Q

What is the appropriate next step in a 4yr old child with microscopic hematuria without proteinuria who received UA due to complaint of stomach pain with a low calcium to creatinine ratio of less than 0.2?

A

Dip parents urine for blood because of familial hematuria (autosomal dominant). Confirm no hx of ESRD or deafness in family.

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129
Q

Which twins are at risk for twin-twin transfusion? what is the risk to both twins?

A

Monozygotic MONOCHORIONIC twins. The risk is hydrops fetalis

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130
Q

An adopted child with fair skin, musty odor, occasional seizures and head circumference at the 5th percentile is concerning for…
What should you test?

A

untreated PKU

Plasma amino acids

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131
Q

In patients with sickle cell disease, anemia with Hgb 7 is not an indication for transfusion but … TIA, ACS, acute splenic sequestration and surgery to Hgb 10 are indications

A

Yes

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132
Q

What is one side effect of anabolic steroids?

A

Gynecomastia from peripheral conversion of testosterone to estrogen

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133
Q

What is effective chemoprophylaxis against meningitis?

A

Rifampin 10mg/kg BID (max 600mg BID) x 2 days or single dose ciprofloxacin 500mg orally or ceftriaxone IM x 1.

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134
Q

Which amino acids can be substituted by each other?

A

Phenylalanine can be substituted by tyrosine.

Methionine can be substituted by cysteine

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135
Q

A newborn with evidence of meningitis and brain abscess formation is likely infected with what bacterium?

A

Citrobacter koseri

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136
Q

What bacterium has recently been associated with consuming uncooked hot dogs and goat cheese?

A

Listeria

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137
Q

Papular, symmetric lesion with no central umbilication located on the face, buttocks, and extensor surfaces are concerning for…

A

Giannoti-Crosti syndrome. Most associated with EBV infection.

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138
Q

In paraphimosis,…

A

A tight prepuce is able to be retracted to the level of the distal corona causing compression and possible ischemia to the distal glans.

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139
Q

A low-pitched sound heard in early diastole is…

A

S3 (blood hitting a VERY compliant, dilated left ventricle)

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140
Q

In what condition does MICROthrombocytopenia occur?

A

Wiscott-Aldrich. They have SMALL platelets and a decreased overall number of platelets

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141
Q

What is a cause of sudden death in patients with Rett syndrome?

A

Cardiac arrhythmia

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142
Q

What anomaly is thought to be due to a vascular accident involved the RIGHT umbilical vein?

A

Gastroschisis.

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143
Q

Which of the two, hordeolum or chalazion is characterized by pain and redness?

A

Hordeolum

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144
Q

What findings are associated with NF1?

A

Optic gliomas, iris hamartoma, axillary or inguinal freckling, greater than 6 cafe au lait macules >5mm (pre-pubertal) or 15mm (post-pubertal), SPHENOID DYSPLASIA or thinning of the long bone cortex, 1st deg relative with NF1

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145
Q

A newborn with a contrast enema consistent with small-diameter sigmoid and descending colon with dilated air-filled loops of small bowel and a collection of granuar-like material in the central abdomen is concerning for…

A

meconium ileus

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146
Q

Any child with varicella infection who is still running a fever on day 5 of illness should get further evaluation

A

Risk of secondary bacterial infection of their lesions - CBCd, BCx

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147
Q

A 7 month old with an enlarged liver presents to the ED after sleeping through the night for the first time, lethargic and with a glucose concentration of 10. What is the next best test?

A

Urine ketones to distinguish a Glycogen storage disease from a fatty acid oxidation defect. In a fatty acid oxidation defect, urine ketones would be low or absent whereas in a glycogen storage disease, they would be high.

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148
Q

A 4 month old with cleft palate, dysmorphic facial features of several facial bones, hypoplastic paranasal sinuses and non-pneumatized mastoid bones with malformed pinnae, colobomata along the eyelid, absence of eyelashes from the medial third of the eyelid, micrognathia is concerning for _______ syndrome with (what assistance device) _____ being a necessary component of their treatment?

A

Treacher-Collins syndrome (mandibulofacial dysostosis) - caused by failure of neural crest cells into the 1st/2nd branchial arch
Bone-conducting hearing aids

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149
Q

What is sufficient intrapartum antibiotic to GBS?

A

Penicillin, ampicillin, or cefazolin IV q4hr prior to delivery

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150
Q

In cutaneous and gastrointestinal angioedema, what are the labs?

A

Low C4 which is the precursor to C1 esterase. Also check C1 inhibitor protein and function which should be low.

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151
Q

What is a congenital heart abnormality that usually does not present until the 2nd decade of life?

A

ASD with fixed split 2nd heart sound

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152
Q

If a mother with a newborn has ACTIVE, symptomatic TB what is appropriate for her and her infant?

A

Mom should receive therapy as should the infant and the infant should be evaluated for congenital TB and be separated from mom until they are both receiving appropriate therapy. Mom must wear a mask and agree to infection control. After 2 weeks from initiating treatment for TB disease, mom is not considered to be infectious. Infant should receive isoniazid for 3-4 months if congenital TB is ruled out at which point the infant should again be assessed for active TB. If negative, the infant should complete 9 months of isoniazid.

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153
Q

If mom has LTBI (latent TB with positive PPD but neg CXR) what is appropriate for her and her infant?

A

Mom should get isoniazid therapy and she can be encouraged to breastfeed. All family members should be tested for TB.

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154
Q

Fever, regional lymphadenopathy with sudden-onset fever is concerning for…

A

Tularemia. Can also be associated with a tularemia related pneumonia, pharyngitis, or conjunctivitis. 30% mortality if delayed diagnosis. Rabbit exposure.

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155
Q

Vesicles in the external ear with onset of Bell’s palsy usually within first 7-10days is concerning for…_____. Treatment is ________.

A

Ramsay hunt syndrome due to VZV primary or latent infection. Treatment is acyclovir and corticosteroids. Only 50% recover full facial nerve function.

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156
Q

What is the treatment for a newborn with symptomatic hypoglycemia who is an infant of a diabetic mother is the glucose is less than 40?

A

Give D10W 2cc/kg

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157
Q

If a patient with NF1 has signs of precocious puberty, what is the likely cause?

A

Optic glioma NOT pituitary adenoma.

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158
Q

How do warfarin and diclofenac act synergistically?

A

Diclofenac (NSAID) displaces protein bound warfarin so more warfarin is biologically active.

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159
Q

What is another name for papular acrodermatitis?

A

Gianotti-Crosti syndrome

Flat-topped erythematous papules that involve the face, upper and lower extremities but spare the bottom.

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160
Q

Small, oval, reddish-brown macules and papules that become necrotic and/or crusted are concerning for ______
Treatment is __________

A

pityriasis lichenoides or pityriasis lichenoides et varioliformis acuta (PLEVA).
Treat with oral erythromycin or tetracyclines

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161
Q

Beckwith-Wiedemann is associated with what type of defects ____________

A

abdominal wall - omphalocoele, diastasis recti, etc.

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162
Q

A child eating paint chips with a smear has basophilic stiffling has whar disease? ________

A

lead poisoning

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163
Q

An unvaccinated 6 year old comes to primary care clinic. Which vaccines are indicated?

A

DTaP, MMR, Varicella, IPV, Hep A and Hep B.

Hib vaccine is not indicated in an immunocompetent host greater than 5 years of age.

In unvaccinated children over the age of 7, Tdap should be the first dose and then Td for subsequent doses.

If MMR and varicella ar not given at the same visit, they should be given 4 weeks apart.

MCV4 and PCV13 should be given 4 weeks apart

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164
Q

What bacteria is associated with an STI that has painful right inguinal lymphadenopathy and separate femoral lymphadenopathy?

A

Chlamydia trachomatis associated with lymphogranuloma venereum (L1, L2, L3). The primary papule is a painless papule.

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165
Q

painless, non-umbilicated, yellowish and skin-colored bumps near eyes, neck and trunk which are common in Asian, dark-skinned people, people with trisomy 21, marfan, or diabetes are known as …

A

Syringoma (benign tumors of eccrine gland)

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166
Q

A child with facial twitching and difficulty talking would be expected to have what type of EEG findings?

A

Centrotemporal sharp waves seen in benign rolandic epilepsy of childhood. Most ppl do not treat with AEDs as children outgrow these seizures.

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167
Q

What type of EEG wave is seen in GTCs?

A

4-6Hz polyspike and wave

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168
Q

When should an adolescent get work-up for short stature?

A

When their linear growth is less than 4-5cm/yr during early pubertal development or if linear growth does not start by age 16 in males (or age 14 in females).

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169
Q

Renal calyx and pelvis dilatation in the absence of ureteral dilatation is suggestive of….

A

ureteropelvic junction dysfunction

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170
Q

what drug is an effective prophylactic medication for cluster headaches?

A

verapamil

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171
Q

Which anti-seizure med can be used in conjunction with OCPs without interfering with their activity?

A

Levetiracetam

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172
Q

Osteomyelitis in the calcaneus bone as a result of a nail to the foot is usually due to…

A

Pseudomonas aeruginosa

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173
Q

As children age, what happens to TBW, ICW, ECW?

A

As children get older, TBW decreases, ICW increases, EBW decreases

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174
Q

14 yr old boy with worsening back pain over months. He has a moderate kyphosis and is unable to voluntarily correct the deformity. What will the X-ray show?

A

Vertebral endplate anomalies associated with anterior wedging.

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175
Q

What does CHARGE syndrome stand for?

A 
Coloboma of the retina
heart abnormalities
atresia of the choanae
retarded growth and mental development
genital abnormalities in males
ear abnormalities

Males are born with micropenis and cryptchordism

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176
Q

A child with gross hematuria, with UA consistent with proteinuria, found on ophthalmologic evaluation to have extrusion of the central portion of the lens into the anterior chamber, macular flecks, and corneal erosions is concerning for?

A

Alport syndrome (hereditary nephritis).

The hearing loss starts as high frequency hearing loss then progresses. The gross hematuria usually follows a URI.

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177
Q

What does the egg on a string CXR refer to?

A

Transposition of the great arteries.

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178
Q

Tricuspid regurgitation with a wall to wall appearance of the heart on CXR refers to….

A

Ebstein anomaly

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179
Q

A 3 yr old girl with developmental delay who was noted to have began to have delays in early infancy, who has no speech, communicates with gestures/vocalizations, has a head circumference less than the 3rd percentile, a wide based gait, and is a happy, easily excitable child is concerning for…

A

Angelman syndrome

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180
Q

What are the FDA indications for growth hormone?

A

Turner syndrome, CKD before transplantation, h/o SGA with lack of catch up growth by 2 years of age, SHOX haploinsufficiency, Noonan syndrome, severe idiopathic short stature, Prader-Willi syndrome.

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181
Q

A 28 month old child who is unvaccinated presents. Howa many doses of the HiB and PCV vaccines are required?

A

1 dose of Hib (once 15months - 59 months; only 1 dose requires)
1 dose of PCV 13 is required (between 24-59 months) only 1 dose is required.

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182
Q

How is iliotibial band friction syndrome characterized?

A

Pain during exercise, located over the lateral femoral condyle.

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183
Q

What size are the platelets in Wiscott-Aldrich syndrome?

A

Small platelets

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184
Q

A 2 year old with nasal discharge and fever presents again for evaluation after several days of URI symptoms. She has a fever, thick yellow nasal discharge, tender, anterior cervical lymphadenopathy, and her posterior pharynx is slightly erythematous.

A

Group A Strep - streptococcosis

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185
Q

A 4 month old male presents with a 2-3 day history of irritability and poor feeding. On exam, patient has a fever, Physical exam findings include induration and soft tissue swelling. An X-ray shows soft tissue swelling associated with calcification and cortical hyperostosis. What bone is most likely to be affected?

A

Caffey disease - infantile cortical hyperostosis.
Affected bone: mandible (95%) of the time

they have elevated alk phos and ESR

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186
Q

At what age are most kids able to rake a raisin without grasping it?

A

6 months.

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187
Q

At what age does the scissors grasp arise?

A

8 months.

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188
Q

If a patient has been seizure-free for 8 months but their drug level of carbamazepine reveals a low drug level, what are best next steps?

A

Keep them on the same dose of carbamazepine. Drug level ranges are guidelines but the required level to ensure a patient is seizure-free is different for everyone.

You do not consider weaning someone off AEDs unless they have been seizure-free for 2 years.

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189
Q

A gymnast with a 6 week history of lower back pain that worsens when asked to stand on one leg while extending backward, has normal flexion of the spine, and experiences hamstring spasm on straight leg test needs what imaging to confirm the diagnosis and what is the diagnosis?

A

Anteroposterior, lateral, and oblique radiographs of the lumbar region.

Assess for spondylolysis (fracture of pars interarticularis)

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190
Q

What disease worsens with giving antibiotics?

A

Botulism

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191
Q

A 3 year old boy with poor weight gain, irritability, frequent episodes of diarrhea, and right sided back pain is expected to have what findings on imaging.

A

A large right paraspinous mass associated with stippled calcifications on CT concerning for a neuroblastoma.

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192
Q

8 year old boy with loose stools, symmetric facial erythema, sharply dermacated, erythematous changes on dorsal surface of hands and wrists is concerning for deficiency of what vitamin?

A

Niacin. Vit B3. or pellagra (disease)

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193
Q

What behavior is unique to conduct disorder that is NOT seen in oppositional defiant disorder?

A

Starting physical altercations

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194
Q

A term infant develops respiratory distress requiring intubation and is unable to be weaned off mechanical ventilation for 4 weeks. Has a normal echo and CXR shows a diffuse interstitial pattern. What is the next best step?

A

Obtain a serum surfactant deficiency analysis.

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195
Q

What type of TE fistula is most common?

A

Esophageal atresia with a distal TE fistula

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196
Q

What skin findings are common in fanconi anemia?

A

Cafe au lait macules. In addition in fanconi anemia you see pancytopenias with GI atresias (e.g.anal atresia), and radial abnormalities (including absent thumbs)

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197
Q

A child has ingested large quantities of drain cleaner. What is the right next step?

A

NPO and refer for inpatient EGD. EGD should happen after 12-24 hours to see full extent of injury. Do NOT place a blind NGT for lavage due to risk of perforation.

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198
Q

A painless genital ulcer associated with inguinal painful lymphadenopathy is what disease cause by what bacterium?

A

Lymphogranuloma venereum (LGV) cause by chlamydia trachomatis (Serovar L2)

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199
Q

A painful genital lesion associated with painful lymphadenopathy is …

A

haemophilus ducreyi

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200
Q

An ASD associated with Left to right shunting with RV dilatation is heard as what type of murmur…

A

A 2/6 systolic ejection murmur, best heard at the L upper sternal border, with normal S1 and widely split S2 that does not vary with respiration. (think of it as a relative pulmonary stenosis)

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201
Q

What disease is associated with porphyria cutanea tarda (vesicles on sun exposed areas like the dorsum of the hands, the forearms, and face)?

A

Hepatitis C

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202
Q

Which vaccine has a minimum of 4 weeks between the 1st dose and 2nd dose?

A

Varicella

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203
Q

At what age, if testicles are not descended, should they be referred for surgical evaluation?

A

6 months of age

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204
Q

For a 4 year old unimmunized asplenic patient, at high risk of pneumococcal disease, what is the appropriate vaccination plan?

A

1 dose PCV 13, followed 8 weeks later by a 2nd dose of PCV 13
then, 1 dose of PPSV 23 8 weeks after the 2nd dose of PCV13, followed by another dose of PPSV 23 in 5 years.

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205
Q

In what type of sleep do night terrors occur?

A

Non-REM sleep in the first third of the night.

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206
Q

What type of DNA change is DiGeorge?

A

microdeletion

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207
Q

A 4 year old presents with history of eczema, intermittent thrombocytopenia, recurrent infections with over the last several weeks, weight loss, night sweats, nausea and vomiting with an abdominal mass seen at the ileocecal junction is concerning for…

A

Burkitt lymphoma (high grade, diffuse neoplasm) arising from the Peyer’s patches in the GI tract. This is a type of non-hogkins lymphoma

208
Q

What dietary deficiency is associated with the spponing of finger nails and toenails?

A

Iron deficiency - Koilonychia

209
Q

A 10 day old term infant with a CSF with 276 WBCs, 2 RBCs, Protein 440mg/dL, glu 14mg/dL with CBC with 18% monocytes is concerning for…

A

Neonatal listeriosis with a likely mild maternal flu like illness in the 2nd or 3rd trimester. Increased peripheral monocytes.

210
Q

What is the most common cause of cerebral palsy?

A

Prematurity (35%)

211
Q

For mild scoliosis with < 20degree curvature, what is the recommendation?

A

No restriction on all sports activities

212
Q

For moderate scoliosis between 25 deg and 45 degree

A

consider bracing

213
Q

What is the antibiotic choice for Bartonell/cat scratch disease?

A

Azithromycin

214
Q

What is the most likely complication of exogenous steroid use?

A

Early epiphyseal closure

215
Q

A newborn with partial sacral and lumbar agenesis, Absence of the acetabulum, bilateral hypoplasia of the femur, tibia and fibula is concerning for..

A

Caudal regression syndrome due to materal Type 1 DM

216
Q

what trait is associated with certain types of albinism?

A

nystagmus, photophobia, decr visual acuity.

217
Q

A patient who has knee pain with internally rotating the tibia with the knee flexed to 90degrees followed by careful extension (Wilson’s sign) and who has soft tissue swelling of the knee and pain at the joint line is concerning for..

A

Osteochondritis dissecans (a bony fragment in the medial condyle of the femur - disease occurs after an area of subchondral bone undergoes necrosis and the overlying cartilage loses its supporting structure.

218
Q

A patient with seizures, periventricular demyelination in the posterior regions of the cerebral white matter, and ADHD is concerning for..

A

X-linked adrenoleukodystrophy - accumulatio of Very long chain fatty acids. They are also at risk of adrenal insufficiency due to VLCFA accumulation there as well.

219
Q

Which progestin has the least androgenic effects?

A

Drospirenone

220
Q

What type of orthopedic problem is a tennis player at the greatest risk for?

A

Lateral epicondylitis - inflammation and pain around the lateral epicondyle of the humerus. Pain is reproducible on palpation.

221
Q

What type of orthopedic injury is a golfer most prone to?

A

Golfer’s elbow (medial epicondylitis) - of the humerus due to repeated flexion

222
Q

A cyanotic heart lesion with newborn EKG findings of Left axis deviation and LVH are concerning for…

A

tricuspid atresia

223
Q

What is good sleep hygiene for an infant?

A

Sleep on their back and always place in their crib/bassinet awake so they develop their own sleep routine.

224
Q

What does the rash of kwashiokor look like?

A

blanching erythema with overlying reddish-brown scaling and sharply demarcated raised edges

225
Q

When should an umbilical hernia be referred for surgical repair?

A

If it enlarges AFTER 1yr of age, if it persists at 5-6 years of age or if it is symptomatic.

226
Q

What facial defect is associated with Kallman syndrome?

A

Cleft lip/palate. Also associated with congenital heart disease and renal agenesis.

227
Q

What serogroup of N.meningitis is responsible for most infections?

A

Serogroup B Neisseria meningitidis

228
Q

How long does it take a drug to reach steady-state when no loading dose is administered?

A

5 half-lives

229
Q

For ethylene glycol, what is the treatment

A

IV fomepizole

230
Q

What does neonatal lupus rash appear like?

A

sharply demarcated, annular scaling plaques on cheeks and periocular areas

231
Q

Which metabolite of acetaminophen causes the hepatic damage?

A

NAPQI (N-acetyl-p-benzoquinone-imine)

232
Q

Charcoal absorbs NAC so separate their admin

A

See card.

233
Q

When after ingestion should the first acetaminophen levels be drawn?

A

4 hours after ingestion

234
Q

For burns, use the rule of 9s for each body surface area that has full or partial burns. Then calculate the aggreate percentage e.g. 27% (use the whole number not decimal) x 30kg (wt of child) x ___ml/kg for each 1% of burn

A

3 ml/kg. with half given in the first 8 hours and the other half in the 16 hours after.

235
Q

Treatment of what bacteria that causes diarrhea is associated with prolonged shedding?

A

salmonella

236
Q

What rash has a similar distribution as pityriasis rosea but is characterized by overlying silvery plaque and usually flares after an episode of strep or URI?

A

Guttate psoriasis

237
Q

Calcifications in the caudate nucleus and basal ganglia in a newborn that are associated with thrombocytopenia, hepatosplenomegaly and diffuse maculopapular rash are concerning for…

A

toxoplasmosis

238
Q

What is the distribution of calcifications in CMV?

A

CMV circumvents the ventricles (periventricular)

239
Q

A patient with a large hemangioma is at risk for what syndrome?

A

Kasabach-Merritt syndrome (hemangioma with thrombocytopenia) which is characterized by microangiopathic hemolytic anemia and DIC

240
Q

Is the X-ray abnormal in a nursemaid’s elbow, subluxation of the radial head? Forearm is held close to the body with elbow flexed and forearm pronated.

A

No X-ray abnormality

241
Q

What shape should a 3 yr old be able to draw?

A

Circle

242
Q

What is the right next step for children who are exposed to an index case of varicella who are unimmunized?

A

(If fully immunized, no prophylaxis is indicated.)
If unimmunized and immunocompetent, and above 12 months should get varicella vaccine ONLY within 3-5 days.

If immunocompetent and under 12 months, immunoglobulin and vaccine are not indicated.

If immunocompromised, or mom had a new, non-zoster varicella infection within 5 days prior to or 48 hours after delivery, or are a preterm infant less than 28 weeks or 1000g or are a preterm infant above 28 weeks whose mom had no immunity to varicella, and are within 10 days of expsoure, should get immunoglobulin.

243
Q

A radiolucent metaphyseal lesion surrounded by sclerotic bone that is extremely painful especially at night is a _____ and is treated with?

A

Osteoid osteoma, NSAIDs only. No bone biopsy.

244
Q

If worried about a malignant lesion, how should the tissue be biopsied?

A

open biopsy under general anesthesia

245
Q

A baby with long, thin eyelashes, thin confluent eyebrows, a low hairline, downward turned lip, a long philtrum, micrognathia, a hypoplastic proximally shaped thumb, short 5th finger, and mild clinodactyly is concerning for…

A

Will also have Hirsutism due to Cornelia de Lange syndrome

246
Q

What is the risk of an in utero parvovirus infection and how does it cause it?

A

hydrops fetalis with ascites, pleural effusions, pericardial effusions.

247
Q

A patient who lives in the mid-Atlantic/southeast US/central US who has fever, chills, cough, and pleuritic chest pain who recently went camping is concerning for

A

Blastomycosis

248
Q

What infection should multi-nucleated giant cells make me think of?

A

Herpes infection

249
Q

A 2yr old with grouped vesicles on her right lower extremity also associated with bullous lesions and wart-like lesions with stain revealing eosinophils associated with inflammatory changes is concerning for…
What other findings are associated?

A

Incontinentia pigmenti

Dental, hair, nail changes.

250
Q

A patient with purulent and possibly blood-tinged vaginal discharge as well as watery diarrhea, abdominal pain is concerning for infection by …

A

SHIGELLA flexneri or SHIGELLA sonnei. Treat with ceftriaxone or azithromycin.

251
Q

Multiple, reddish, urticarial rashes on lower extremities and abdomen with epidermal and subdermal edema associated with eosinophils is concerning for…

A

papular urticaria

252
Q

What is spondylolisthesis?

A

Spondylolisthesis is when bilateral pars interarticularis defects permit the vertebral body to subluxate over another usually anteriorly and most commonly around L5 and S1

253
Q

A 4 week old noted to be jaundiced with an indirect bilirubin of 10.7mg/dL and total bilirubin of 12mg/dL is concerning for likely…

A

breastmilk jaundice which starts from 3-5 days of life and can last from 3-12 wks.

254
Q

A 26 day old female infant, born at 36 weeks with sudden onset irritability, poor feeding and facial swelling is concerning for infection due to?

A

Strep agalactaie (GBS) with cellulitis-adenitis syndrome that happens in the 3-4 week of age stage and manifests as bacteremia with focal infection such as meningitis, septic arthritis, osteomyelitis, adenitis, cellulitis.

255
Q

After successfully treating scabies, how long do symptoms last?

A

weeks

256
Q

What murmur is most common to hear in anorexia nervosa?

A

An apical systolic murmur with a mid-systolic click concerning for similar murmur to mitral valve prolapse but the etiology is myofibrillar atrophy.

257
Q

What is the best next step of work-up/treatment for suspected reactive arthritis?

A

Work-up: CBC, ESR, RF, consider joint aspiration. If consistent with reactive arthritis, give NSAIDs a try. Other symptoms seen in reactive arthritis are enthesitis and dactylitis.

258
Q

Patient with a genital ulcer. Wright stain with rod-shaped, oval organisms within the cytoplasm of mononuclear phagocytes. What is the bacterium noted?

A

Klebsiella granulomatous. Disease: Donovanosis

259
Q

A 6 yr old patient presents with the 3rd episode of bleeding from a bump on index finger with a bright-red vascular papule with hemorrhagic crust consistent with…

A

pyogenic granuloma

260
Q

What disease is associated with facial dysmorphism, corpus callosum agenesis, narrow prominent chin, and hypertelorism. What other disease is associated with it?

A

Mowat-Wilson syndrome associated with Hirscprun’s dx

261
Q

An 11 yr old boy is found to have a widely fixed and split 2nd heart sound with a medium pitched systolic murmur and first-degree heart block. What other findings are associated with these?

A

Bifid thumbs or radial abnormalities. Holt-Oram syndrome - autosomal dominant inheritance.

262
Q

An 18 month old child with swollen cervical lymph node of 3 weeks duration with a PPD induration of 4 mm, is best managed how?

A

Surgical excision and biopsy. Likely non TB mycobacterium (e.g. mycobacterium avium).

263
Q

13 y.o. M with blurred vision, dysarthria, trouble swallowing, and vomiting with rapidly progressing onset over the past 24 hours is concerning for?

A

Food ingestion related botulism

264
Q

What part of the bone is the metaphysis?

A

The part near the growth plate

265
Q

A lytic bone lesion with “onion skin” appearance is concerning for what disease and what are its characteristics?

A

Ewing sarcoma. More common in Caucasians and in males under the age of 10. Occurs in the diaphysis of bones.

266
Q

An 8 yr old boy with redness and peeling fo the soles of his feet with toes and the more distal portions of his feet with a pink hue, glazed appearance, has this condition due to?

A

Hyperhidrosis and frictional irritation. Disease: Juvenile plantar dermatosis. Emollients and medium potency corticosteroids are required.

267
Q

A leptomeningeal angioma (large tortuous venous structures) is likely associated with what other finding?

A

Cutaneous port-wine stain. Sturge weber disease.

268
Q

Pap smears should begin at age 21 with the exception of what group who should begin screening earlier?

A

HIV infected women

269
Q

What live vaccine should be avoided in the siblings of immunocompromised patients?

A

Oral polio vaccine

270
Q

Hypopigmented macular lesions on the face covered by fine adherent scales are likely…

A

pityriasis alba, it is associated with atopic dermatitis.

271
Q

Hypopigmented macules on the trunk are likely

A

tinea versicolor

272
Q

a baby born with congenital growth hormone deficiency might present with…

A

hypoglycemia, micropenis, DIRECT hyperbilirubinemia

273
Q

To calculate the age level equivalent of a child developmentally based on their drawing, assign 1/4 to each body part with paired parts only getting a 1/4. Then add to base of 3.

A

A child who draws 2 eyes, a nose, a mouth, hair, 2 legs is approx 4.25yrs equivalent.

274
Q

A newborn baby with myelomenigocele and VSD is concerning for exposure to what class of anti-hypertensives in utero?

A

ACE-inhibitors, lisinopril

275
Q

A 4 yr old girl who had mild edema of her hands and feet as a newborn, convex nails, and is short stature compared to her family is concerning for…

A

Turner syndrome. Assess with chromosomal analysis.

276
Q

a near term male newborn with pre and post ductal split and right to left shunting across the foramen ovale and PDA likely was exposed to what substance in utero?

A

SSRI

277
Q

Patients with Rett syndrome live at least until their mid 20s and can live up to the 4th or 5th decade

A

Yes

278
Q

Expressive language and expressive social skill dysfunction is a critical component of Rett syndrome; seizures occur in up to 80% of people

A

Yes

279
Q

A few weeks to months after an illness, a 14 year old girl notices her hair is thinning and clumps are coming out everytime she washes her hair.

A

Telogen effluvium - reassurance needed.

280
Q

By the time the child with parvovirus has the rash, they are no longer infectious so they are okay to go to school including with pregnant teachers nearby.

A

yes

281
Q

What is first-line therapy in long QT syndrome?

A

propanolol

282
Q

a 3 yr old boy who returns from vacation with new spots on his chest and lower extremities that were initially red and have hyperpigmented in somewhat linear and irregular patterns is concerning for

A

phytophotodermatitis from the juice of a mango

283
Q

What is cercarial dermatitis?

A

Swimmer’s itch - larvae infested waters

284
Q

10 day old hypotonic newborn is found to have a partial chromosome 15q deletion, diminished cry, poor suck, almond-shaped eyes, thin upper lip, dolichocephaly concerning for…

A

Prader-Willi, with associated obesity after age 1yr

285
Q

what is Russell sign?

A

sign seen in bulimia/purging where hands hit the teeth in self-induced vomiting

286
Q

What is the biggest risk with gasoline/hydrocarbon ingestion?

A

chemical pneumonitis

287
Q

In an adolescent diagnosed with Type 2 DM, what is the HgbA1c cutoff that drives management?

A

Below HgbA1c of 8.5%, use metformin and diet/exercise,

Above HgbA1c of 8.5%, consider adding insulin to the above regimen.

288
Q

A study that agrees with its null hypothesis could be making what type of error?

A

Type 2 error

289
Q

Small testicular volume/pre-pubertal testes can occur in Kallmann syndrome

A

Yes

290
Q

In an orbital floor fracture, inferior rectus entrapment leads to a limitation of…

A

Limitation of UPWARD gaze

291
Q

A 6 week old boy has a micropenis and nystagmus, is only 1 lb above birthweight, and has a direct bilirubin of 5, what is his diagnosis?

A

Septo-optic dysplasia (SOD) with agenesis of the corpus callosum, midline defects - hypoplasia of the optic nerve, pituitary deficiencies (isolated growth hormone deficiency or panhypopituitarism). Etiology of the direct hyperbilirubinemia is unclear.

292
Q

In ITP, if any kind of bleeding is present including gum bleeding, the right next step is to…

A

give IVIG

293
Q

Which inherited disorder of clotting is associated with activated protein C resistance?

A

Factor V Leiden - a mutation that prevents the binding of activated protein C.

294
Q

How is priapism in sickle cell disease treated?

A

First, IV hydration and analgesics. If no improvement after 4-5hrs, then intracavernous phenylephrine.

295
Q

A patient with hypertension due to renal artery stenosis and supravalvular aortic stenosis is likely to have what syndrome and what facies?

A

Williams syndrome, elfin facies.

296
Q

An 8 week newborn with seizures in the setting of bilateral subdural bleeds with bleeding from his umbilical stump on separation with normal PT, PTT, platelet count, and platelet function assay, normal skeletal survey and no retinal hemorrhages is concerning for…

A

Factor 13 deficiency

297
Q

Recent treatment with IVIG should delay which vaccines and for how long?

A

MMR and varicella vaccines should be delayed for 8-10 months after.

298
Q

Penetrating eye injury with a ring abscess is concerning for…

A

Bacillus cereus, pseudomonas or proteus

299
Q

A patient with short stature, high PTH, low calcium and high phosphorus is concerning for…

A

pseudohypoparathyroidism

300
Q

What guides return to play after a concussion?

A

6-step graduated return to play - first non sports activities,

301
Q

a patient with significant neutropenia or myelosuppression while taking 6-MP (6 mercaptopurine) is concerning for…

A

TPMT deficiency

302
Q

A cherry red spot on a fundoscopic exam, developmental regression, startle myoclonus, and muscle weakness in a Cajun/French Canadaian or Jewish patient are concerning for…

A

Tay-Sachs disease

303
Q

In kallmann syndrome, mirror movements (involuntary movements of the other extremities) may occur.

A

Yes

304
Q

Pancytopenia, high ferritin, high triglycerides and low fibrinogen is concerning for…

A

HLH

305
Q

How do you manage a corneal abrasion

A

Topical anesthetic drops, antibiotic ophthalmic drops, recheck in 1 day.

306
Q

Fanconi anemia (poor DNA repair mechanisms) is associated with what malignancies?

A

AML

307
Q

What type of hairloss is associated with hashimoto’s thyroiditis?

A

alopecia areata - smooth, non-erythematous areas of complete hairloss

308
Q

Brushfield spots

A

form a ring in the mid-zone of the iris. Can occur in up to 25% of normal, blue-eyed children.

309
Q

At what age can a child draw a circle?

A

3 years

310
Q

At what age can a child draw a cross?

A

3-4 years

311
Q

At what age can a child draw a triangle?

A

5 years

312
Q

What is usually the preferred treatment in functional fecal incontinence?
Can urinary symptoms be associated.

A

Disimpaction with oral stool softener.

Yes, urinary symptoms can often be associated.

313
Q

A patient with mid facial pain and green discharge 5 days after a URI should be treated with

A

nasal decongestants and irrigation

314
Q

For Type 2 DM, when are you recommended to screen for microalbuminuria?

A

Time of diagnosis

315
Q

For Type 1 DM, when are you recommended to screen for microalbuminuria?

A

5 yrs after diagnosis

316
Q

A patient with fatigue, gingival swelling, PERIFOLLICULAR hemorrhages, hyperkeratosis and painful, swollen right knee is concerning for…

A

Vitamin C deficiency/scurvy

317
Q

What bacteria are associated with reactive arthritis?

A

Shigella, Salmonella, Mycoplasma, Chamydia, strep viridans, yersinia.

318
Q

Sotos syndrome characterized by height and weight above 97%ile and large skull is associated with…

A

decreased IQ. It has NO endocrine associations an bone age is normal for age.

319
Q

What is a contraindication for T21 patients to participate in Special Olympics? VSD or frequent bowel/bladder control loss with gait abnormality on exam?

A

Frequent bowel and bladder loss with gait abnormality on exam is concerning for atlanto-axial instability. That is a contraindication.

320
Q

A newborn term neonate has erythematous bullae nd erosions on the knees, ankles, and wrists. What is the best test for the diagnosis?

A

skin biopsy for epidermolysis bullosa

321
Q

After what age is rotavirus vaccine NOT indicated?

A

After 8 months of age

322
Q

Telogen effluvium, painless, non-inflammtory hair loss can be associated with…

A

propanolol, amphetamines, ACE-i, oral contraceptives, retinoids, and lithium.

323
Q

Unilateral conjunctival erythema and preauricular lymphadenopathy is concerning for what infection?

A

Bartonella henselae - conjunctival granuloma, minimal pain. (parinaud occuloglandular syndrome)

324
Q

What organism at people with Hyper IgE syndrome/ Job’s syndrome prone to?

A

Staph aureus, haemophilus and strep pneumoniae. They also get sinopulmonary infections, intensely pruritic, distributed eczematous rash.

325
Q

in an Adolescent boy with a varicocele, what is the next step in management?

A

observation only. a pre-adolscent boy with a varicocele requires an abdominal ultrasound due to the association with a Wilms tumor.

326
Q

Guttate psoriasis is usually preceded by what?

A

Exudative pharyngitis

327
Q

Bilateral triphalangeal thumbs, snub nose, and wide set eyes at birth in a newborn are concerning for…

A

Diamond-Blackman anemia

328
Q

A child who is jaw-winking Marcus Gunn phenomenon), meaning has ptosis when she sucks demonstrates…

A

abnormal innervation of the trigeminal nerve (muscles of mastication) and oculomotor nerve (levator palpabrae).

329
Q

What is the first sign of puberty in a male?

A

testicular development followed by phallic growth and pubic hair development. Only after does axillary hair come

330
Q

What antibiotic is treatment for tularemia?

A

Gentamicin. Fransicella tularensis is a gram negative.

331
Q

Someone with erythema multiorme and a history of travel to the SW is concerning for…

A

Coccidiodomycosis

332
Q

What street drug is associated with rhabdomyolysis?

A

PCP, its metabolites can be found in urine

333
Q

What sign distinguishes Tinea pedis from juvenile plantar dermatosis?

A

Tinea involves the intertriginous area.

334
Q

A spermatocele transilluminates and there is nothing to do.

A

Yes

335
Q

What is the CXR appearance of a chlamydial pneumonia in a neonate?

A

HYPERinflation with minimal interstitial and alveolar infiltrates

336
Q

A newborn term neonate has erythematous bullae nd erosions on the knees, ankles, and wrists. What is the best test for the diagnosis?

A

skin biopsy

337
Q

An infant with a metabolic disorder with hepatomegaly and urine ketones but no urine reducing substances is concerning for.. and should be treated with…

A

glycogen storage disorder and be treated with a nocturnal glucose infusion by NG tube feeding

338
Q

An infant with a metabolic disorder with no urine ketones or positive urine reducing substances has

A

defect in fatty acid metabolism

339
Q

An infant with a metabolic disorder with positive reducing substances and no ketones is concerning for…

A

Galactoesemia or fructose intolerance

340
Q

A newborn born to a mom with inadequate treatment for GBS has stable vital signs and completes a 48 hr antibiotic course. what is the next best step?

A

discharge home with NO 24 hour observation off antibiotics because 24hrs later there are still detectable levels of antibiotics.

341
Q

in an Adolescent boy with a varicocele, what is the next step in management?

A

observation only. a pre-adolscent boy with a varicocele requires an abdominal ultrasound ue to the association with a Wilms tumor.

342
Q

What sedating agent is contraindicated in soy or egg allergy?

A

propofol

343
Q

Mesangial deposits of IgA and what associated clinical symptom in the setting of URI or GI illness is unique to IgA nephropathy (Berger’s disease)?

A

gross hematuria in the setting of URI or GI illness

344
Q

A child who is jaw-winking, meaning has ptosis when she sucks demonstrates…

A

abnormal innervation of the trigeminal nerve (muscles of mastication) and oculomotor nerve (levator palpabrae).

345
Q

What is the first sign of puberty in a male?

A

testicular development followed by phallic growtn and pubic hair development. Only after does axillary hair come

346
Q

What antibiotic is treatment for tularemia?

A

Gentamicin. Tularemia is a gram negative.

347
Q

What imaging modality is the gold standard for osteomyelitis?

A

MRI

348
Q

What are absolute contraindications to estrogen containing birth control pills?

A

Migraine with aura, past VTE/PE with known clotting abnormality, HTN with

349
Q

What sign distinguishes Tinea pedis from juvenile plantar dermatosis?

A

Tinea involves the intertiginous area.

350
Q

What virus has been associated with DRESS?

A

HHV 6

351
Q

An 8 year old child with cleft lip/palate, short stature, dysmorphic facial features, long/narrowed face, small mouth, prominent nose, short stature, and cryptorchidism is concerning for…

A

velocardiofacial syndrome (11q.2 band of chromosome 22) so they can have hypocalcemia

352
Q

What conditions require antibiotic prophylaxis for dental procedures?

A

Prosthethic cardiac valve, prior history of endocarditis, UNREPAIRED cyanotic heart disease, completely repaired heart disease for 6 months after procedure, cardiac transplant patients who develop cardiac valvulopathy

353
Q

To start statins, a child has to be 10 years or older with and LDL-C > 130mg/dL with 2 high risk factors (BMI >97%ile and Type 1 DM, HTN on pharmacotherapy, Chronic renal dx, post renal or heart transplant, Kawasaki with coronary aneursyms) or 1 high risk and 2 moderate risk factors (Kawasaki w/regressed coronary aneursyms, HTN w/o pharmacotherapy, BMI 85-97%ile, HDL < 40, JIA, HIV, nephrotic syndrome.

A

yes

354
Q

An infant with a metabolic disorder with hepatomegaly and urine ketones but no urine reducing substanc.es is concerning for.. and should be treated with…

A

glycogen storage disorder and be treated with a nocturnal glucose infusion by NG tube

355
Q

An infant with a metabolic disorder with no urine ketones or reducing substances has

A

defect in fatty acid metabolism

356
Q

In a patient undergoing treatment for tinea capitis with griseofulvin, you note small, flesh-colored papules and erythematous scaly patches on the face, neck, trunk and extremities with excoriation. What is best step in management?

A

Add corticosteroids to griseofulvin due to concern for id reaction

357
Q

What lymph node is most likely to be infected with non tuberculous mycobacterium?

A

Submandibular

358
Q

A teenage athlete presentes with hypertension, fasting blood of 220 and elevated HgbA1c 8.2% concening for which performance enhancing drug?

A

growth hormone

359
Q

13 month old child with lymphopenia, thrombocytopenia, failure to thrive and h/o otitis media x 2 and foul smelling diarrhea as well as pneumonia is concerning for…. and likely has what skeletal findings?

A

Schwachman Diamond syndrome and has bilateral irregularities of the proximal and distal femoral metaphyses.

360
Q

What disease is granuloma annulare (annular plaques, raise borders and depressed center) possibly associated with…

A

Type 1 DM

361
Q

What immunosuppresant medication causes a phenotype similar to Trisomy 13 or 18?

A

Methotrexate

362
Q

What bacteria does a double disk diffusion test, test for?

A

MRSA

363
Q

How do you test for HCV in a neonate?

A

HCV RNA

364
Q

What is the best way to help an older child adjust to a new sibling?

A

Have the older child have special one-on-one time with another adult.

365
Q

What two heart defects present with extreme left axis deviation?

A

Tricuspid atresia and AV canal defect

366
Q

An 8 year old child with cleft lip/palate, short stature, dysmorphic facial features, long/narrowed face, small mouth, prominent nose, short stature, and cryptorchidism is concerning for…

A

velocardiofacial syndrome

367
Q

Bartholin glands are at 4 o’clock and 8 o’clock. Skene glands are near the opening of the urethra.

A

yes

368
Q

At what point do 90% of children have a social smile

A

2 months of age

369
Q

A child with eczema presents with a tense, fluid-filled blister surrounded by erythema over the volar fat pad on the 3rd and 4th fingers of his R hand. What is the infectious agent responsible?

A

Group A strep causing blistering distal dactylitis.

370
Q

What is the test to evaluate for Cushing syndrome or hypercortisolism?

A

24 hour urinary free cortisol

371
Q

What are the risk factors for IV radiocontrast reactions

A

past reaction, female gender, atopy, and cardiovascular disease.

372
Q

What primitive reflex appears between 8 and 9 months of age?

A

parachute reflex

373
Q

When does the crossed adductor reflex disappear?

A

7 months

374
Q

What precautions must be taken by an adult with varicella zoster lesions who is around children who is starting acyclovir?

A

Ok to return immediately as long as lesions are covered with clothing.

375
Q

A TORCH infection with cataracts and cardiac disease is most likely due to?

A

Rubella

376
Q

12 hours after birth, a 35 week infant dies with exam notable for low set rotated ears, severe micrognathia, compressed, broadened nose and wide-spaced eyes, bilateral clubfeet concerning for…

A

Potter sequence

377
Q

A cauliflower like growth from cortical and medullary bone in the long bones is concerning for… and is best managed by…

A

osteochondroma, best managed by clinical monitoring with repeat X-ray in 6 months

378
Q

Are there interactions between OCPs and valproic acid, retinol, fluoxetine, or doxycycline?

A

No. Not CYP3A4.

379
Q

What is an antihelminthic agent e.g. for cutaneous larva migrans?

A

albendazole. Not itra….

380
Q

A 19 month old male who refuses to bear weight on his leg has an X-ray with anterior tibial bowing and a fracture in the middle third of the tibia with diagnosis due to…

A

NF 1

381
Q

A child with herpetic whitlow will likely have numerous shallow, rounded, discrete, gingival ulcers

A

yes

382
Q

Bartonella henselae can be treated with azithromycin or immunocompetent hosts can receive supportive care.

A

yes

383
Q

What virus is associated with rosella?

A

HHV 6

384
Q

Compared to adult migraines, pediatric migraines are more likely to have what feature?

A

Bilateral pain

385
Q

A girl with high arched palate, wide spaced nipples, prominent webbing of the neck, cubitus valgus is concerning for _________ syndrome and likely has what cardiac lesion?

A

Turner syndrome with bicuspid aortic valve

386
Q

The silk glove sign or thickened processus vaginalis occurs in what condition?

A

Inguinal hernia

387
Q

Hyponatremia is associated with which AED?

A

Oxcarbazepine

388
Q

An adolescent has fever and sore throat with a maculopapular rash that began on the distal extremities and spread to the trunk. A gram positive rod bacterium that grows in blood rich media is to blame… What do you treat with?

A

Arachnobacyerium haemolyticum

Treat with erythromycin

389
Q

What is SBE prophylaxis dose?

A

Amoxicillin 50mg/kg or max 2g, 1 hour prior to procedure.

390
Q

The parents of a child are distraught because she is viewed as overly friendly and entered the care of an unknown family at school?

A

Williams syndrome

391
Q

What is a bacterium that causes bloody diarrhea that is a spiral-shaped, gram negative, non-spore forming bacilli?

A

Camplyobacter jejuni

392
Q

A 2 yr old boy with recurrent sinusitis and pneumonia with prolonged fever/cough is found to have PCP pneumonia. What is his underlying deficiency?

A

Hyper IgM syndrome due to abscess of CD40 ligand on T-helper cells.

393
Q

In what conditions are C3 levels low?

A

SLE, post-strep GN, and MPGN

394
Q

In pyloric stenosis, what kind of IV resuscitation should be done in a dehydrated child?

A

IVF with 20-40mEq/L KCl to replete hypochloremic, hypokalemic metabolic alkalosis.

395
Q

What are the absolute contraindications to breastfeeding?

A

HIV, HTLV-1, HTLV-2, and untreated brucellosis. Ok to breastfeed with Hep B and Hep C.

396
Q

What vaccine must be given subcutaneously?

A

MMR

397
Q

A baby with no abdominal tone, undescended testes, and wrinkled belly has _____ syndrome and is likely to have ______

A

prune belly syndrome and lung hypoplasia. Renal dysplasia can occur leading to oligohydramnios.

398
Q

PGE can help palliate aortic coarctation

A

yes

399
Q

A 4 yr old unvaccinated boy with fever rash and prominent posterior cervical, retroauricular, and posterior occipital lymphadenopathy is concerning for…

A

Rubella (erythematous macular rash with reddish spots on soft palate).

400
Q

What is a part of the DSM 5 criteria for bulimia?

A

Recurrent inappropriate compensatory behavior to prevent weight gain.

401
Q

Children with thrombocytopenia–absent radii syndrome have COMPLETE thumbs.

A

Their marrow will lack megakaryocytes.

402
Q

Children who were born IUGR are at more risk of having….

A

metabolic syndrome

403
Q

Genes HAX1 and ELA2 are associated with congenital neutropenia.

A

yes

404
Q

A cardiac rhabdomyoma is likely associated with …

A

tuberous sclerosis. infantile spasms also associated.

405
Q

What is the mortality of DKA associated cerebral edema and who is most at risk?

A

Mortality is 25%. Incidence is 1-5% and most at risk are new onset diabetes and age less than 5 years.

406
Q

In serum sickness, you can get deposits in the joints, renaal glomeruli, blood vessel walls leading to arthralgias, proteinuria, lymphadenopathy, skin rashes, muscle aches.

A

Yes

407
Q

What’d darier’s sign?

A

An urticarial eruption that occurs in cutaneous mastocytosis after stroking.

408
Q

What is the best management of a bronchogenic cys that is symptomatic with expiratory stridor shortly after URI?

A

surgical excision

409
Q

Activated charcoal for GI decontamination does not work on which substances?

A

Potassium, lithium, sodium, iron, lead, alcohols, caustics, cyanides.

410
Q

DNAase B is used to diagnose a recent ____ infection

A

Group A Strep skin infection

Anti-streptolysin O is for recent throat infection

411
Q

A 2 hour old term baby with hypotonia, decreased muscle mass and weakness, diminished tendon reflexes, undescended testicles with subsequent need for resp support is concerning for….

A

myotubular myopathy and usually has associated polyhydramnios because they are unable to swallow.

412
Q

How do you evaluate for hearing loss?

A

Auditory brainstem responses

413
Q

Prominent prevertebral swelling refers to… cause by what bacterium?

A

retropharyngeal abscess…Group A strep.

414
Q

Jerky movements with difficult to write handwriting and a positive Romberg sign are concerning for…

A

Syndeham’s chorea. Can use diazepam.

415
Q

A child who was playing outside who develops severe abdominal or chest pain within 2 hours with a bull eye rash on thigh/arm, hypertension and tachycardia, elevated glucose and LFTs is concerning for ____ and must be treated with _____

A

black widow spider bite and must be treated with a benzodiazepine.

416
Q

A hockey player with a painful, distal 1/3rd of the femur calcified soft tissue mass on X-ray is concerning for

A

traumatic myositis ossificans. PT and immobilization first. Excision only 6-12 months after injury.

417
Q

A 13 yr old F who has “talked out of her head”, vomited 5 times, has conjunctival injection, and erythematous strawberry tongue, diffuse intensely erythematous rash with elevated transaminases and elevated BUN44 and Cr 3.6, fever, tachycardia, and hypotension is concerning for.

A

Toxic shock syndrome

418
Q

What maternal exposure is associated with symmetric IUGR?

A

chronic alcohol abuse

419
Q

A child with cleft lip with normal TSH and low fT4 has …

A

central hypothyroidism

420
Q

Sickle cell is characterized by a blood smear with

A

sickled cells and polychromasia

421
Q

A 3 yr old boy with asthma presents with fever, CXR with bilateral peribronchial infiltrates and CBC with eosinophilia, and hepatomegaly on exam, after recently getting a new dog. What is the diagnosis?

A

Toxocara canis

422
Q

Most infants who present with SVT do not have an underlying congenital heart disease.

A

Yes

423
Q

Is receiving the inactivated flu vaccine contraindicated in egg allergy?

A

No. Inactivated flu vaccine is contraindicated in individuals with egg allergy who have had a prior reaction to the flu vaccine. There is no special precaution to take for children with egg allergy and you do not need to ask about it to give the flu vaccine.

424
Q

What vaccines is contraindicated in egg allergy?

A

Yellow fever vaccine

425
Q

A pre-pubertal child with a L ankle injury associated with lateral malleolus tenderness with minimal swelling and limping with a normal X-ray should receive what care next?

A

Should be splinted, placed on ibuprofen, ice, elevation and rest and should be referred to orthopedics for evaluation.

426
Q

Enlarged Basophilic lymphocytes with foamy cytoplasm in a patient concerning can be seen in a patient with EBV infection. What other clinical signs might one see?

A

Periorbital and eyelid edema

427
Q

What commonly prescribed medicine for duodenal ulcer can cause gynecomastia?

A

Cimetidine, estrogens, TCAs, atyical anti-psychotics, ACE-inhibitors, ketoconazole, isoniazid.

428
Q

In a ‘healthy’ child found to have neutropenia with bone marrow aspirate showing granulocytopenia with normal trilineage precursors, what is the diagnosis?

A

chronic benign neutropenia. these children have autoantibodies against granulocytes and have ANCs 0-500 with improvement by 2 years of age.

429
Q

What is Kostmann syndrome?

A

Severe congenital neutropenia. Most never have an ANC > 200 and have promyelocytic arrest in the precursor cells.

430
Q

In what population, independent of family history is DDH highest?

A

Girls in breech position - 12% whereas a female with a 1st degree relative affected has a 4.5% chance.

431
Q

By 36-37 weeks, 2/3rd of the anterior sole is covered by creases.

A

Yes.

432
Q

By 34-35 weeks, the areola is raised but breast tissue remains absent

A

Yes

433
Q

A 5-week old male, not moving his R leg with X-ray findings of transverse bands of increased density across the femoral metaphyses with patchy bony destruction in the diaphyses with solid new periosteal formation around the femur is concerning for…

A

Congenital syphilis with osteochondritis and periostitis with need for obtaining VDRL including form CSF (expect pleocytosis and elevated protein). Can also see complete white out on CXR (pneumonia alba).

434
Q

KOH prep of a skin scraping with short, blunt-ended hyphae and clusters of spores is concerning for …

A

Malassezia globosa - tinea versicolor. Spaghetti and meatballs.

435
Q

What is the most common cardiac defect in rubella?

A

PDA

436
Q

What are facial anomalies like in Fragile X?

A

Prominent forehead, protuberant ears, narrow face, prominent jaw.

437
Q

What is the morphology of gonorrhea?

A

Gram-negative intracellular diplococcus

438
Q

Preterm infants less than 2 kg with HepsAg negative moms should receive Hep B vaccine when?

A

At hospital discharge or 1 month of chronological age, whichever comes sooner.

439
Q

If born to a HepsAg positive mom, besides receiving Hep B vaccine and HBIG within the first 12 hours, what other testing is suggested in the 1st year of life?

A

1-2 months after the last Hep B vaccine and no earlier than 9 months, the infant should get tested for HepBsAg and anti-HBs

440
Q

How many cubes can an 18 month old tower?

A

4 cubes

441
Q

How many cubes can a 24 month old tower and how do they run?

A

6 cubes; and they run really well!

442
Q

An adolescent with an enlarging, hairy nevus on the chest is concerning for…_____ and treatment is ____

A

Becker nevus. (hair growth occurs 12-24 months after pigmentation starts). Treatment is cosmetic intervention if desired by the patient.

443
Q

If you received a Td vaccine yesterday, is a Tdap today contraindicated?

A

No, I can receive a Tdap today if I received a Td yesterday. You only need 1 lifetime Tdap for now, unless pregnant to help boost your immunity.

444
Q

Even fully-immunized healthcare workers who are working in the setting of a child found to have pertussis should received chemoprophylaxis

A

Yes

445
Q

What does ingestion of goatmilk as an infant put you at risk for…

A

Folate deficiency

446
Q

A newborn with flattening of the L upper chest, hypoplastic, caudally displaced L nipple, and hypoplasia of the upper L shoulder girdle and arm is concerning for…

A

Poland syndrome with unilateral absence or hypoplasia of the medial (sternal) portion of the pectoralis muscle.

Expect syndactyly and shortening of the arm on the affected side.

447
Q

What is a cause of unconjugated hyperbilirubinemia besides Gilbert’s?

A

Criggler-Najar syndrome

448
Q

What is the best way to diagnose a fatty acid oxidation defect?

A

Plasma acylcarnitine profile

449
Q

What is the treatment for E.Coli O157:H7 infection? and why?

A

Supportive care only. Because antibiotics have been shown to increase risk of HUS

450
Q

What treatment is indicated for hot tub folliculitis due to pseudomonas?

A

None. Supportive care.

451
Q

What is pilocarpine iontophoresis?

A

The substance used to induce the sweat test.

452
Q

A Giemsa stain with scrapings with multi-nucleated giant cells refers to?

A

Herpesviridae infection

453
Q

A skin rash with each rash surrounded by a halo of vasoconstriction refers to ….

A

Roseola rash

454
Q

A 17 yr old adolescent presenting with a contaminated wound who needs tetanus prophylaxis, received 5 doses of DTaP before the age of 6 and a Td booster at 11 years, requires what vaccination for tetanus prophylaxis?

A

Tdap because everyone needs at least one Tdap from age 11-12 years onwards as booster for pertussis immunity.

455
Q

When do you get tetanus immunoglobulin?

A

When you have had less than 3 doses of a tetanus containing vaccine and have a contaminated wound with dirt, feces or saliva or a puncture wound, burn, avulsion or frostbite.

456
Q

A chronically ill, adolescent patient with a tender papulopustule on his anterior tibial surface that became necrotic and ulcerated is concerning for…

A

pyoderma gangrenosum in the setting of ulcerative colitis.

457
Q

A 17 yr old female complains of menorrhagia, irregular menses, lower abdominal pain, and heaviness. Calcifications are seen in her L ovary on plain X-ray. What is the diagnosis?

A

Mature cystic teratoma/dermoid cyst

458
Q

An 18-month-old concerning for a nutritional deficiency with an X-ray with long with “ground glass appearance”, thin bony cortex and sharply outlined epiphyseal ends is concerning for…

A

Vit C deficiency/scurvy. The sharply outlined epiphyseal end is the white line of Frankel.

459
Q

How does an ACL tear occur?

A

A sudden deceleration and pivoting of the knee

460
Q

If a boy on a Boys scout trip gets a scratch from a bat and the bat is unavailable for testing, what is the best next step?

A

Cleanse wound, Give rabies immunoglobulin and 4-dose series of human diploid cell vaccine or purified chick embryo cell vaccine (day 1, then 3, 7 and 14 days) for a bite, scratch or mucous membrane contact with a bat.

If the bat is found and is not rabid, no intervention would have been needed.

461
Q

Rectal prolapse is high which population of patients?

A

CF patients. get a sweat chloride.

462
Q

What is the risk of spina bifida in the normal population?

A

0.1%

463
Q

What is the risk of spina bifida in a sibling of an affected child?

A

2-3%

464
Q

In scarlet fever, what is usually spared?

A

Circumoral area, palms and soles

465
Q

In what form of rickets is PTH normal?

A

X-linked hypophosphatemic rickets where increased renal losses of phosphorus occur. low phosphorus does not trigger PTH.

466
Q

A 4 yr old boy with fever, back pain over the last several days with restricted ability to walk, T3-T4 tenderness with paraspinal muscle spasm, WBC 9K and ESR 95mm/hr, will have X-ray findings concerning for

A

Discitis with narrowing of the intervertebral space. Blood cultures are negative in over 50% of cases.

467
Q

What serum lab test is elevated in JDM?

A

Creatine kinase, aldolase, LFTs

468
Q

What are some characteristics of Diamond-Blackfan anemia in a child with macrocytic anemia and horseshoe kidney?

A

Short, webbed neck. Macrocytic anemia, cardiac, craniofacial, ophthalmologic, urogenital anomalies. Skeletal anomalies with bifid, triphalangeal, or hypoplastic or absent thumbs, light colored hair, thick upper lip.

Treatment in 1st year of life are RBC transfusions, then steroids after

469
Q

At what age does peak height velocity in puberty occur for girls and for boys?

A

For girls - 11.5 yrs it is 8.3cm/yr

For boys at 13.5 yrs - it is 9.5cm/yr

470
Q

In boys, the lean muscle mass increases from 80 to 90% in puberty whereas it decreases in girls in puberty.

A

yes

471
Q

What percentage of your adult height does pubertal growth account for?

A

17-18% in both sexes

472
Q

Varicella vaccine must be stored frozen at 5F

A

yes. Other vaccines should be stored at 35 to 46 F

473
Q

an IDM is holding the L upper extremity supinated with elbow bent and wrist extended. The child is unable to grasp the examiner’s finger? What else may they be at risk for?

A

Horner’s syndrome - ptosis, miosis, anhidrosis. Injury to T1 causes horner’s by interrupting sympathetic outflow to the orbit.

474
Q

In HIV positive children, what are the parameters to receive MMR and Varicella vaccines and which vaccine is contraindicated?

A

MMR and varicella vaccines may be given if T-cell percentage is greater than 15% under the age of 13 or if above 200 Tcells/mm3 for aged 14 yrs and above. MMRv is contraindicated. MMR doses should be given 28 days apart and Varicella doses 3 months apart. If a child is above 13, the 2nd dose of Varicella vaccine can be administered 28 days apart.

475
Q

If a tick, even in a Lyme endemic area, has been present on the skin of a patient for less 36 hours, and was removed in the office, what is best next step in management?

A

Observation. No prophylaxis.

476
Q

When is Lyme prophylaxis indicated and what is it?

A

All these criteria are satisfied: When a tick has been present for 36hours, when the tick can be identified as the adult/nymph I. scapularis, when prophylaxis is within 72 hours of tick removal, when Lyme is common in the location where the patient lives.
Dose: Doxycycline 4mg/kg x 1 with a max dose of 200mg

477
Q

At what age is hepatoblastoma more common than HCC?

A

less than 3 years. Children who are premature are at increased risk of hepatoblastoma.

478
Q

What EKG changes are associated with hyperkalemia?

A

Peaked T waves and prolonged PR interval

479
Q

What EKG changes are associated with hypercalcemia?

A

Shortened Qtc and abrupt upstroke in T wave from decreased proximal portion of T wave

480
Q

What are the EKG with hypocalcemia?

A

Prolonged QTc

481
Q

What are the EKG changes in hypothermia?

A

Prolonged RR, QT, PR and QRS intervals

482
Q

What are the causes of pseudohyperkalemia?

A

Hemolysis, thrombocytosis (>450K), and leukocytosis (WBC > 100K)

483
Q

An 18 yr old with pruritis 2 to 10mm shiny, flat-topped, thin violaceous papules on the penile shaft, with similar, discrete, polygonal violaceous papules on his lower legs and flexural surfaces of wrist is also likely to have…

A

fine, white papules forming linear, lace-like pattern on buccal mucosa - lichen planus.

484
Q

In which urea cycle defect is orotic acid high?

A

Ornithine transcarbamylase deficiency

485
Q

A 17 yr old with recent travel outside the US months before who presents with 2 non tender, firm, enlarged cervical nodes and one fluctuant node is concerning for….
Diagnostic eval is ___
Treatment is ___

A

TUBERCULOUS adenitis (scrofula), diagnosis is made by fine needle aspiration.

Treatment is usually medical management as opposed to NTM. 6-9 of RIPE therapy, can narrow during treatment if susceptibilities return.

486
Q

On EKG, inverted T waves in V1 and V4R are normal up to what age?

A

Up to early adolescence. They can continue to be inverted even after that.

487
Q

What characterizes anovulatory bleeding?

A

Absence of mid-surge LH and absence of a corpus luteum

488
Q

Peripheral neuropathy in TB therapy is due to…

A

Isoniazid

489
Q

How long do newborns have upright T waves?

A

Until day 7

490
Q

A homogenous, chronic ground-glass opacities on CXR of an HIV infected patient with tachypnea, mild retractions, and crackles are concerning for…

A

lymphocytic interstitial pneumonitis

491
Q

What is the treatment for PFAPA?

A

Prednisone

492
Q

What treatment is used in familial Mediterranean fever?

A

Colchicine to prevent amyloidosis.

493
Q

What type of cardiac disease is associated with fetal alcohol syndrome?

A

VSD/septal defects

494
Q

The ability to roll back to front occurs when?

A

5-6 months

495
Q

What is a Type 2 error?

A

Concluding there is no difference in outcomes when there actually is. i.e. failing to reject the null hypothesis when it is false. You reveal this by looking at the “power” of the study.

496
Q

When should an infant be able to lift their head off the table surface in anticipation of being lifted?

A

6 months

497
Q

When starting isotretinoin, what lab tests should be obtained?

A

CBC, LFTs, fasting triglycerides, and cholesterol before initiating therapy, then 4 weeks and then 8 weeks thereafter.

498
Q

When do the majority of events occur in benign rolandic epilepsy of childhood?

A

During sleep or on awakening. Mono therapy is optional but usually effective at decreasing generalized not partial seizures. Seizures usually last 2 years. And spontaneously remit before age 12-13.

499
Q

What is the management of a new hydrocele in a child over one year or enlargement of a pre-existing one in a child over one year in age?

A

Surgical evaluation. Spontaneous resolution is unlikely over one year of age.

500
Q

In a puncture wound bite from a dog, which bacteria are you concerned about? And what should you treat with?

A

Pasteurella as well as anaerobes and staph aureus.

You can use augmentin (Amox-clav) or doxy/Bactrim + clindamycin.

501
Q

What is the definition of microbe is in a newborn?

A

Less than 2-2.5cm

502
Q

When in relation to menarche does ovulation begin?

A

Ovulation usually begins 12-24 months AFTER menarche. Not before.

503
Q

What is the most common cause of osteomyelitis in a US born sickle cell patient?

A

Salmonella

504
Q

At what age should head lag completely be absent in a newborn?

A

4 months

505
Q

What dose of steroid is considered high dose in determining whether to hold vaccination for 4 weeks?

A

Prednisone equal to or greater than 2mg/kg/day or 20mg/day for 2 or more weeks duration. If receiving this, wait 4 weeks after completion of therapy to vaccinate.

506
Q

What infectious illness can have perceptual distortions (seeing people as small vs large) as a presenting feature?

A

EBV (infectious mononucleosis)

507
Q

A 4yr old girl with a large segmental cafe au lait macule that stops at the midline with findings of precocious puberty is concerning for… and will have what other findings?

A

McCune Albright syndrome.
Other findings: multiple areas of fibrous dysplasia in the long bones and pelvis. Lytic lesions with scalloped borders and ground glass appearance.

508
Q

Wormian bones and generalized cortical thinning is concerning for…

A

Osteogenesis imperfecta

509
Q

Absence of the radius, dislocation of the carpus, rudimentary thumb is concerning for…

A

Holt Oram syndrome

510
Q

A 16 yr old presents with hypotension, decreased resp rate, pinpoint pupils, bradycardia after ingesting his sibling’s prescription. What is the drug and what is it used to treat?

A

Clonidine - ADHD

511
Q

What is the appropriate evaluation for premature thelarche?

A

FSH/LH, estradiol and bone age.

For thelarche, there is no need for thyroid tests.

512
Q

What substance is absorbed in the terminal ileum and what type of anemia is someone at risk for when it is respected?

A

Vitamin B12; megaloblastic anemia

513
Q

What type of cardiac disease is associated with fetal alcohol syndrome?

A

VSD

514
Q

What is a Type 1 error?

A

Concluding there is a difference in outcomes when there is not (rejecting the null hypothesis when it is true). You can reveal this by look at p values of the result

515
Q

When do children with growth hormone deficiency fall off the growth curve?

A

Usually 18-24 months of age

abp - peds boards (1 decks)

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