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Shelf Exam Study Materials > Pediatrics: UWorld > Flashcards

Pediatrics: UWorld Flashcards

1
Q

Most common cause of sepsis in sickle cell patient

A

-pneumococcus (encapsulated organisms)

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2
Q

Preventative management in sickle cell patients

A

-vaccination -penicillin (until age 5) -folic acid supplementation -hydroxyurea (pts w/recurrent pain events)

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3
Q

Maternal estrogen effects in newborns

A

-breast hypertrophy -swollen labia -physiologic leukorrhea -uterine w/drawal bleed

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4
Q

Indications for renal & bladder US in children

A

-infants/children with first febrile UTI @ 2-24mo.

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5
Q

Complications of prematurity

A

-RDS -Patent ductus arteriosus -Bronchopulmonary dysplasia -Intraventricular hemorrhage -necrotizing enterocolitis -retinopathy of prematurity

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6
Q

Causes of precocious puberty

A

-central ==> high FSH & LH -peripheral ==> low FSH & LH (e.g. excess peripheral conversion, estrogen-producing ovarian cyst, CAH)

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7
Q

Contraindications to DTaP vaccine

A

w/prev. vaccine: -anaphylaxis -unstable neuro d/p -encephalopathy

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8
Q

Clinical features of pineal gland mass

A

**parinaud syndrome: -limited upward gaze -ptosis -upper eyelid retraction -pupillary abnormalities **obstructive hydrocephalus -papilledema -HA, vomiting -ataxia **central precocious puberty

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9
Q

Work-up for suspicion of pineal gland mass

A

-brain mri -serum/CSF alpha-fetoprotein & B-hcg

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10
Q

Prevention/tx of neonatal chlamydial conjuctivitis

A

-routine maternal screening during pregnancy -tx = oral erythmromycin

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11
Q

Erythromycin opthalmic ointment ==>

A

prevention of gonoccocal conjunctivitis

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12
Q

Risk factors for cryptorchidism

A

-prematurity -SGA/low birth weight -in utero DES/pesticide exposure -genetic d/o -NTDs

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13
Q

Cryptorchidism tx

A

-orchiopexy @

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14
Q

Risks after successful orchiopexy

A

-No risk of testicular torsion -highest risk of subfertility -some testicular cancer risk

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15
Q

III/IV harsh holosytolic murmur @ LLSB in infant ==> ?

A

VSD

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16
Q

OCD criteria/characteristics and tx

A

-recurrent intrusive thoughts with repeptitive mental/physical rituals -tx = high-dose SSRIs

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17
Q

Neonate w/failure to thrive, bilateral cataracts, jaundice, and hypoglycemia

A

-galactosemia -galactose-1-phosphate uridyl transferase defieciency - + hepatomegaly, convulsions -pt. at risk for E.coli neonatal sepssis

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18
Q

Galactokinase deficiency ==>

A

bilateral cataracts only; otherwise asymptomatic

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19
Q

Uridyl diphosphate galactose-4-epimerase deficiency ==>

A

-rare -same sx as transferase deficiency (failure to thrive, bilateral cataracts, jaundice, hypoglycemia, hepatomegaly) & hypotonia and nerve deafness

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20
Q

Minimal change disease characteristics

A

-most common cause of nephrotic syndrome -usually @ 2-3yo;

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21
Q

AOM causes/tx/complications

A

-risks: smoke exposure, URI, day care, formula -organisms: s. pneumo, H. infl, moraxella -tx: oral amox x 10 days -complications: mastoiditis, TM rupture, conductive hearing loss

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22
Q

Well-apearing neonate w/painless, bloody stools ==>

A

milk- or soy-protein allergy

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23
Q

Factor deficiencies in cystic fibrosis

A

-vitamin K related factors -II, VII, IX, X and protein C,S

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24
Q

Legg-Calve-Perthes disease characteristics

A

-idiopathic osteonecrosis of femoral head -boys age 4-10yo -p/w hip or knee pain of insidious onset + antalgic gait (decreased time weight bearing on affected side)

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25
Q

SCFE characteristics

A

-slipped capital femoral epiphysis -p/w limp and insidious hip pain -obese adolescents

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26
Q

Tx for ringworm

A

-topical antifungals (e.g. terinafine)

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27
Q

Osteosarcoma vs. Osteoid osteoma vs. Ewings

A

-Osteosarcoma = most common primary bone tumor; large, tender mass @ metaphyses of long bones; “sunburst pattern” XR -Ewing’s = second most common; “onion skin” XR -Osteoid osteoma = sclerotic, cortical lesion w/central lucency; p/w pain @ night, not worse w/activity, helped w/ NSAIDs

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28
Q

Common bacteria in CF pneumonia

A

-gram neg rods: pseudomonas, b. dolosa, stenotropomonas -gram neg coccobac: H. influenza -gram pos cocci chains: s. pneumo -gram pos cocci clusters: s. aureus

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29
Q

Cholesteatoma characteristics

A

-congenital or acquired from chronic middle ear dz -new-onset hearing loss or chronic ear drainage w/abx therapy -granulation tissue and skin debris w.in pockets of TM

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30
Q

Colic definition

A

-crying in otherwise healthy infant for >3hrs daily (usually evening) - >3x/week for >3weeks -tx = soothing techniques

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31
Q

Risk factors for neonatal RDS

A
  1. prematurity 2. male sex 3. perinatal asphyxia 4. maternal DM 5. C-section w/out labor
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32
Q

Presentation of RDS

A

-tachypnea, retractions, grunting, nasal flairing, cyanosis @ birth -CXR: diffuse reticulogranular pattern (ground-glass opacities) & air bronchograms

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33
Q

Tx of RDS

A

antenatal: corticosteroids postnatal: exogenous surfactant and respiratory support

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34
Q

Bilious emesis w/u

A
  1. abdominal xray; pneumoperitoneum ==> surgery 2. water-soluble contrast enema (if stable) ==> find level of obstruction
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35
Q

Meconium ileus presentation

A

-bilious emesis -microcolon on contrast enema -pathognomonic for CF

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36
Q

Tx of Meconium ileus

A
  1. hyperosmolar (gastrografin) enema to try to break up meconium 2. Surgery if not resolved 3. all pt.s receive sweat chloride testing after stabilized
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37
Q

Characteristics of Reye syndrome

A

-children who receive aspirin for virus-induced fever -hyperramonemia -transaminitis (w/microvesicular steatosis) -coagulopathy -emesis -AMS

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38
Q

Diarrheal illness + renal failure, hemolytic anemia, thrombocytopenia ==> dx?

A

HUS

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39
Q

HUS causes

A

-E.Coli 0157:J& -Shigella -Salmonella -Yersinia -Campylobacter

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40
Q

Hallmark of HUS

A

microangiopathic hemolytic anemia

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41
Q

Peripheral smear in HUS

A

schistocytes and giant platelets

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42
Q

Transmission of lyme disease

A

Ixodes transmits Borrelia burgdorfer via bite

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43
Q

Presentation of eczema herpeticum

A

-HSV infection associated w/atopic dermatitis -umbilicated vesicles over area of healing atopic derm - + fever + adenopathy -can be life-threatening ==> rapidly initiate tx

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44
Q

Impetigo causes

A

non-bullous: staph or strep pyogenes bullous: staph aureus

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45
Q

Appearance of bullous impetigo

A

-rapidly enlarging flaccid bullae w/yellow fluid -“collarette” of scale at periphery of ruptured lesions

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46
Q

Tx of non-bullous and bullous impetigo

A

-non-bullous: topical abx (mupirocin) -bullous: oral abx (cephalexin, dicloxacillin, clindamycin)

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47
Q

Management of pertussis cases

A

-macrolide abx for pt. -macrolide abx for all close contacts regardless of age, immunizations, sx

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48
Q

Pertussis prophylaxis abx

A
  • 1 mo. old: Azithro x 5D vs. Clarithro x 5D vs. Erythro x 5D
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49
Q

Epidemiology of Meckel’s diverticulum

A

Rule of 2s: -2% prevalence -2:1 male-to-female ratio -2% are sx at age 2 -Located w/in 2 ft. of the ileocecal valve

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50
Q

Clinical presentation of Meckel’s diverticulum

A

-asymptomatic incidental finding -painless hematochezia -intussusception -intestinal obstruction -volvulus

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51
Q

Dx & Tx of Meckel’s diverticulum

A

Dx: technium-99 scan Tx: surgery for sx patients

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52
Q

Characteristics of myotonic muscular dystrophy

A

-genetics: AD -onset: age 12-30 -presentation: facial weakness, hand grip myotonia, dysphagia -comorbidities: arrhythmias, cataracts, balding, testicular atrophy -prognosis: death from respiratory or heart failure

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53
Q

Duchenne M. dystrophy comorbidities

A

-scoliosis -cardiomyopathy

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54
Q

Becker m. dystrophy comorbidities

A

cardiomyopathy

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55
Q

CD3 = CD19 =

A

CD3=T lymph CD19 = B lymph

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56
Q

Tx for X-linked agammaglobulinemia

A

IVIG

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57
Q

Indications for neuroimaging in child w/HA

A
  1. hx of coordination difficulties 2. numbness, tingling, focal neuro signs 3. H/A that awakens from sleep 4. increasing H/A frequency
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58
Q

Rheumatic fever features (major)

A

Joints O(carditis) Nodules Erythema marginatum Sydenham chorea

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59
Q

Rheumatic fever minor features

A

-fever -arthralgias -elevated ESR -prolonged PR

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60
Q

Red flag sx for intracranial pathology

A

-nocturnal headaches -morning vomiting

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61
Q

Dx features of acute bacterial rhinosinusitis

A
  1. persistent sx > 10 days OR 2. Severe sx, fever >=39, purulent nasal drainage, face pain x 3days OR 3. Worsening sx >=5 days after initial improvement from viral URI
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62
Q

Tx of acute bacterial rhinosinusitis

A

amox-clav PO

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63
Q

Characteristics of Waterhouse-Friderichsen syndrome

A

-infant w/meningococcemia -skin rash (large purpuric lesions on flanks) -sudden vasomotor collapse 2/2 adrenal hemorrhage

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64
Q

Unique CXR feature in children

A

thymic silhouette visible at superior right heart border

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65
Q

Characteristics of early disseminated lyme disease

A

-carditis: AV block, cardiomyopathy -neurologic: unilateral or bilateral CN n. defects (e.g. VII), meningitis, encephalitis -muscular: migratory arthralgias -conjunctivitis -skin: multiple erythema migrans

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66
Q

Complex partial seizures vs. partial seizure with generalization

A

-both: aura, LOC -complex partial ==> motor automatisms (picking, chewing, etc.) during LOC -partial w/generalization ==> tonic-clonic activity

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67
Q

Characteristics of simple partial seizure

A

-no LOC -deja-vu -can have aura -pt. may remember well

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68
Q

Firm, smooth unilateral abdominal mass that is asymptomatic w/hematuria ==>

A

-Wilms tumor -most common childhood cancer -peak age 2-5yo

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69
Q

Dx & Tx of Wilms tumor

A
  1. US to differentiate from other masses 2. CT w/contrast ==> look for pulm mets 3. Tx = surgery + chemo; radiation for end-stage *Good prognosis in early stages
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70
Q

Presentation of neuroblastoma

A

-3rd most common peds cancer -first year of life -typically: abdominal mass that crosses midline

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71
Q

Presentation of duodenal atresia

A

-prenatal US w/polyhydramnios -bilious vomiting w/in first 2 days of life -“double bubble” sign on abdXR -strongly associated w/Down’s & assoc. heart defects

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72
Q

CV defects in Turner’s

A

-bicuspid aortic valve -coarctation of the aorta -aortic root dilation

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73
Q

Renal defects in Turner’s

A

horshoe kidney

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74
Q

Ataxia + scoliosis/foot deform + cardiomyopathy ==> dx?

A

Friedrich ataxia

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75
Q

Presentation/characteristics of Friedrich ataxia

A

-most common spinocerebellar ataxia -AR; presents

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76
Q

Causes of neuro sx in Friedrich ataxia`

A

-degeneration of spinal tracts -spinocerebellar ==> ataxia -posterior columns ==> falling -pyramidal ==> dysarthria

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77
Q

Complication of trauma to soft palate

A

==> internal carotid artery disection ==> stroke

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78
Q

Breast milk jaundice vs. BF failure: timing

A

-failure: w/in first week -BM: starts @ 3-5 days, peaks @ 2 weeks

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79
Q

Breast milk jaundice vs. BF failure: clinical features

A

-failure: suboptimal BF, signs of dehydration -BM: adequate BF, normal exam

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80
Q

Pathophysiology of BF failure jaundice

A

-decreased bilirubin elimination -increased enterohepatic circulation

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81
Q

Pathophysiology of BM jaundice

A

high levels of b-glucoronidase in BM deconjugate intestinal bilirubin and increase enterohepatic circulation

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82
Q

Threshold for phototherapy or exchange transfusion in neonatal jaundice

A

-phototherapy = ~w/in first week = 20 mg/dL+ -exchange transfusion = 25+

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83
Q

Normal/recommended newborn feeding patterns

A

-~8-12x/day for 10-20 min/breast -approx. every 2-3 hours

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84
Q

DDx of stridor

A

-croup (laryngotracheobronchitis) -laryngomalacia -FB aspiration -vascular ring

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85
Q

Distinguishing features of laryngomalacia

A

-most severe @ 4-8 mo. -worse in supine; improves in prone position

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86
Q

Distinguishing features of vascular ring (==>stridor)

A

-presents

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87
Q

Drug of choice in PNA in young CF pt.

A

IV vancomycin

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88
Q

Tx of long-QT syndrome

A

-avoid vigorous exercise -avoid QT prolonging meds -maintain normal Ca, K, Mg -Beta-blockers -sx (e.g. lightheadedness, palpitations) or history of syncope ==> pacemaker

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89
Q

NEC on abd. XR

A

-air visible in bowel wall (“double-line” or “train-track”) ==> pneumotasosis intestinalis -portal venous air

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90
Q

Common neuro complication in sickle cell

A

stroke 2/2 sludging and occlusion of vasculature

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91
Q

Tx of stroke in sickle cell pt.

A

exchange transfusion

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92
Q

Indication for intubation of asthmatic

A

-severe asthma unresponsive to meds w/: -fatigue -AMS -CO2 retention, worsening hypoxemia -poor air movement

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93
Q

Most common causes of viral meningitis

A

non-polio enteroviruses: echoviruses or coxsackieviruses

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94
Q

Signs/sx of intussusception

A

-colicky, severe abdominal pain -“currant jelly” stools (2/2 mesenteric ischemia) -“target sign” on US

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95
Q

Palpable purpura + arthritis/athralgia + abdominal pain + renal disease ==> dx?

A

Henoch-Schonlein purpura = IgA mediated vasculitis

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96
Q

Lab findings in Henoch-Schonlein purpura

A

-normal platelets/coags -normal to increased Cr -hematuria +/- RBC casts +/- proteinuria

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97
Q

Tx of Henoch-Schonlein purpura

A

-most patients: hydration + NSAIds -severe: admission w/systemic steroids

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98
Q

Aplastic anemia causes

A

-drugs -toxins -viral infections ==> pancytopenia

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99
Q

Presentation of Fanconi’s anemia

A

-pancytopenia starting gradually at 4-12yrs. old -congenital anomalies: hyperpigmentation of trunk, neck, intertriginous areas; cafe-au-lait spots, short stature, upper limb abnormalities, hypogonadism, skeletal anomalies, eye/eyelid changes, or renal malformations-

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100
Q

Clinical presentation of rubella

A

-low-grade fever -conjunctivitis, coryza, cervical lymphadenopathy, Forscheimer spots (patchy erythema) on soft palate -cephalocaudal and centrifugal “pink” maculopapular rash from face to body

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101
Q

Clinical presentation of immune thrombocytopenia

A

-antecedant viral infection -asx petechiae & ecchymoses (msot commonly) -mucocutaneous bleeding

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102
Q

Tx of immune thrombocytopenia (children)

A

-skin manifestations only: observe -bleeding: IVIG or glucocorticoids

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103
Q

Riboflavin (B2) deficiency presentation

A

-angular cheilitis (fissures @ corners of lips) -glossitis -stomatitis (hyperemic/edematous oral mucosa) -normocytic-normochromic anemia -seborrheic dermatitis

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104
Q

Niacin deficiency presentation

A

-symmetric reddish rash on exposed skin, glossitis, diarrhea/vomiting -neuro disturbances

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105
Q

Thiamine (B1) deficiency presentaion

A

-dry beriberi: peripheral neuropathy -wet beriberi: cardiomyopathy -wernicke-korsakoff

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106
Q

Major advantages of breastfeeding

A

-absorbs better and improves gastric emptying -IgA and other proteins

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107
Q

Tx of pinworms

A

albendazole

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108
Q

Risk factors for intraventricular hemorrhage in newborn

A
  1. preme 2. low birth weight 3. exposure to vascular perfusion injuries: hypoxia/ischemia, hypotension, reperfusion of damaged vessels, increased venous pressure, abrupt changes in cerebral flow
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109
Q

PKU pathophysiology

A

-autosomal recessive mutation in phenylalanine hydroxylase -failure to convert phenyl ==> tyrosine results in hyperphenylalaninemia (elevated aa) and neuro injury

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110
Q

PKU Clinical features

A

-severe intellectual disability -seizures -musty body odor -hypopigment of skin, hair, eyes

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111
Q

Dx of PKU

A

a. newborn screen b. quantitiative amino acid analysis ==> elevated phenylalanine

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112
Q

NF1: gene, chromosome, clinical features

A

-NF1 tumor suppressor ==> neurofibromin -@ chromosome 17 -cafe-au-lait spots -multiple neurofibromas -lisch nodules

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113
Q

NF2: gene, chromosome, clinical features

A

-NF2 tumor suppressor ==> merlin -@ chromosome 22 -bilateral acoustic neuromas

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114
Q

Pediatric viral myocarditis: etiology, clinical presentation

A

-coxsackie B vs. adenovirus -viral prodrome ==> -heart failure: dyspnea, tachycardia, syncope, N/V, hepatomegaly

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115
Q

Dx of viral myocarditis

A

-CXR: cardiomegaly, pulmonary edema -ECG: sinus tachycardia -Echo: decreased EF, diffuse hypokinesis

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116
Q

Congenital diaphragmatic hernia presentation

A

-usually on left -polyhydramnios -concave abdomen + barrel shaped chest -absent lung sounds on affected side -can displace heart/heart sounds ==> pulmonary hypoplasia nad pulm HTN ==> respiratory distress as neonate

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117
Q

Management of congenital diaphragmatic hernia

A
  1. intubate (NO bag-valve mask) 2. NG/OG tube 3. umbilical a. line for monitoring 4. umbilical v. line for fluids/meds
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118
Q

Tumor that produces estrogen

A

Granulosa-cell tumor

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119
Q

Sertoli-leydig cell tumor ==>

A

androgen production ==> masculinization

120
Q

Glucose-6-phosphatase deficiency physical presentation

A

-doll-like face -thin extremities -short stature -protuberant abdomen

121
Q

Glucose-6-phosphatase deficiency metabolic disturbances

A

-hypoglycemia ==> seizures -lactic acidosis -hyperuricemia -hyperlipidemia

122
Q

Hep B associated w/renal disease?

A

membranous nephropathy

123
Q

Nutritional intervention for preterm infants

A

-iron supplimentation in exclusively breast-fed infants ==> 1yo -all exclusively breastfed infants ==> vit D supplement

124
Q

Electrolyte abnormalities in refeeding syndrome

A

-decreased serum phosphorous, K, Mg -decreased serum thiamine -increased sodium and water retention

125
Q

Hip pain but able to bear weight and w/out significant lab/vital abnormalities ==> dx? tx?

A

-transient synovitis = most common hip pain in children (usually 3-10yo) -NSAIDs and rest, f/u in 1 wk.

126
Q

Transposition of great vessels: exam/findings

A

-single S2 -+/- VSD murmur -CXR: “egg-on-a-string” heart (narrow mediastinum)

127
Q

Tetrology of Falloy: exam/findings

A

-harsh pulmonic stenosis murmur -VSD murmur -CXR: boot-shaped heart (RVH)

128
Q

Tricuspid atresia: exam/findings

A

-Single S2 -VSD murmur -minimal pulm. blood flow

129
Q

Truncus arteriosus: exam/findings

A

-single S2 -systolic ejection murmur -increased pulm. flow, edema

130
Q

Total anomalous pulm. venous return: exam/findings

A

-severe cyanosis -respiratory distress -pulm. edema; snowman sign (enlarged supracardiac veins and SVC)

131
Q

Most common complication of sickle cell trait

A

painless hematuria

132
Q

Evaluation of primary amenorrhea

A
  1. pelvic US; if no uterus ==> karyotype 2. FSH if no breast development 3. FSH low ==> pituitary MRI 4. FSH high ==> karyotyping
133
Q

Jejunal atresia presentation and risk factors

A

-presentation: bilious vomiting and abdominal distention -abd. xr: “triple bubble” sign and gasless colon -risk factors: prenatal exposure to cocaine, tobacco, or vasoconstricive drugs

134
Q

Risks for rickets

A

-exclusive BF -dark skin (?) -lack of sun exposure

135
Q

Exam findings in rickets

A

-craniotabes (“ping-pong ball” skull) -delayed fontanel closure -enlarged: skull (frontal bossing), costochondral joints (rachitic rosary), long bone joints (wrist widening) -genu varum

136
Q

Lab abnormalities in rickets

A

-Ca: normal to low -Ph: normal to low -alk phos: very elevated -PTH: elevated -vit D: low

137
Q

Fragile X gene mutation

A

expanded CGG trinucleotide repeats and abberrant methylation @ FMR1

138
Q

Risks if born SGA

A

-SGA =

139
Q

Risks if born LGA

A

-hip sublux -talipes calcaneovalgus

140
Q

Dx of kawasaki dz.

A

-fever 5+ days and 4+ of: -bilateral nonex. conjunctivitis -mucositis (including strawberry tongue) -cervical lymphadenopathy -erythematous polymorph rash -extremity changes (edema, erythema)

141
Q

Tx of Kawasaki dz.

A

ASA + IVIG

142
Q

Complications of Kawasaki dz.

A

-coronary a. aneurysms -MI or ischemia

143
Q

Characteritics of osgood-schlatter disease

A

-common cause of knee pain, esp. @ adolescent athletes -traction apophysitis of the tibial tubercle @ the insertion of the paterllar tendon -PE: tenderness/edema @ tibial tubercle + pain with resisted extension -XR: anterior swelling, lifting of tubercle from shart, irregularity/fragmentation of tubercle

144
Q

Tx of osgood schlatter

A

-activity restriction -stretching -NSAIDs

145
Q

Presentation of homocytinuria

A

-AR -marfinoid body habitus -fair har/eyes -downward lens dislocation -developmental delay -hypercoaguability

146
Q

Homocystinuria pathophys + tx

A

-cystathione synthase deficiency -tx: -vitamin supplementation -antiplatelet/anticoag

147
Q

Tay-Sachs characteristics

A

-AR -decificency in B-hexosaminidase A -presents w/ID, weakness, seizures, cherry-red macula

148
Q

Lab results in Vitamin K deficiency

A

-prolonged PT -prolonged PTT

149
Q

Lab results in factor VIII deficiency

A

-prolonged PTT -normal PT

150
Q

Risks of vitamin K defiency/prevention

A

-low vitamin k ==> bruising, bloody stools, intracranial hemorrhage -prevention with vitamin K injection after delivery

151
Q

Metatarsus adductus management

A

-Type I = correction to abduction w/movement ==> reassurance -Type II = correct to neutral ==> orthosis/corrective shoes (2nd line = casts) -Type III = rigid, no correction ==> serial casting

152
Q

Lab findings in measles

A

-leukopenia (t-cell cytopenia) -thrombocytopenia

153
Q

Tx that reduces mortality in measles

A

-vitamin A

154
Q

Management of caustic/alkali ingestion

A
  1. secure airway, breathing, circulation 2. decontaminate: remove contaminated clothing and irrigate exposed skin 3. DO NOT attempt to neutralize w/acid and DO NOT pass blind NG tube 4. CXR if respiratory sx 5. endoscopy in 24 hours
155
Q

Duration of PCN prophylaxis in rheumatic fever

A

-RH w/out carditis: until 21yo (or 5 years if >16yo) -RH w/card but w/out valvular: until 21yo (or 10y if >11yo) -RH w/card + valv: until 40yo (or 10y if >30yo) *PCN injection IM qmonth

156
Q

Selective mutism criteria

A

-fail to speak in certain situations but not others - > 1 mo. -interferes w/education or social communication -not 2/2 lang. barrier

157
Q

self mutalation + neuro findings + elevated uric acid ==> dx?

A

-Lesch-Nyan syndrome -HPRT (hypoxanthine-guanine phosphoribosyl transferase) deficiency

158
Q

Sx of L-N syndrome

A

-self-mutilation -neuro: mental disability, dystonia, spasticity, choreoathetosis -gouty arthritis/tophi

159
Q

Measles prodrome and exanthem

A

-prodrome: -fever, malaise, anorexia -coryza, conjunctivitis, koplik spots (clustered white spots on buccal mucosa) -exanthem: -blanching, reddish-brown maculopapular rash -cephalocaudal & centrifugal spread -spares palms/soles

160
Q

GI Risk in H-S Purpura

A

ileo-ileal itussusception

161
Q

Presentation/management of androgen insensitivity syndrome

A

-XY w/female phenotype -bilateral gonadectomy after completion of puberty

162
Q

Contraindications to rotavirus vaccine

A

-anaphylaxis to ingredients -hx of intussusception -hx of uncorrected congenital malform. of GI tract (e.g. Meckel’s) -SCID

163
Q

Kallmann sydnrome presentation

A

-delayed/absent puberty -karytype matches phenotype -FSH/LH low

164
Q

presentation of heriditary angioedema

A

-swelling/edema episodes after trauma, dental procedure, infections -non-inflammatory edema of face, limbs, genitalia -laryngeal edema can be life-threatening -edema of intestines ==> abdominal pain -no urticaria

165
Q

Pathophysiology of heriditary angioedema

A

-C1 inhibitor deficiency or dysfxn - ==> elevated levels of C2b and bradykinin ==> edema

166
Q

Precocious puberty w/elevated LH ==>

A

brain MRI to rule out intracranial pathology

167
Q

Reproductive consequences of CF

A

-congenital bilateral absence of vas deferens in male ==> infertility @ 95%+ -20% of women infertile

168
Q

MSK consequences of CF

A

-osteopenia ==> fx -kyphoscoliosis -digital clubbing

169
Q

Omphalocele associated with…

A

-NTDs -cardiac malformations -trisomy

170
Q

DDx of regurgition/vomiting in infants

A

-physiologic reflux -milk protein allergy -GERD -pyloric stenosis

171
Q

Infant GERD presentation

A

-FTT -significant irratibility -sandifer syndrome (intermittent opisthotonic posturing = arched back, rigid neck)

172
Q

Management of infant GERD

A

-thickened feeds -antacid therapy -esophageal monitoring with pH probe -upper endoscopy

173
Q

Lab findings in hereditary spherocytosis

A

-increased MCHC -spherocytes on peripheral smear -negative coombs -increased osmotic fragility of acidified glycerol lysis test -abnormal eosin-5-maleimide binding test

174
Q

parvovirus B19

A

xx

175
Q

Acute lymphadenitis cause/presentation

A

-acute, unilateral cervical lymphadenitis = red, warm, swollen -usually 2/2 staph aureus

176
Q

Pathogen for acute, unilateral cervical lymphadenopathy w/: -rabbit/animal contact -periodontal disease -violaceous color

A

-rabbit = tularemia -periodontal = peptostreptococcus -color = nonTB myco (e.g. MAC)

177
Q

Infant botulism tx

A

human-derived botulism IG

178
Q

foodborne botulism tx

A

equine-derived botulism antitoxin

179
Q

Labs in thalassemis

A

-MCV: very low -Iron: high -TIBC: low -Ferritin: high -Transferrin sat: very high

180
Q

Dx test for CF

A

sweat chloride testing via quantitative pilocarpine iontophoresis

181
Q

infant w/macrocytic anemia, low retic count, congenital anomalies ==> dx?

A

diamond-blackfan syndrome

182
Q

avg. age of dx of fanconi’s anemia?

A

8 yo

183
Q

congenital anomalies in diamond-blackfan syndrome

A

-short stature -webbed neck -cleft lip -shielded chest -triphalangeal thumbs

184
Q

congenital anomalies in fanconi’s anemia

A

-hyperpigmentation of trunk, neck, intertriginous areas -cafe-au-lait spots -short stature -upper limb abnormalities/absent thumbs -hypogonadism -skeletal anomalies -eye/eyelid changes -renal malformations

185
Q

Transient erythroblastoma presentation

A
  • >1yo -pure red cell aplasia w/out macrocytosis -no congenital anomalies
186
Q

Congenital rubella presentation

A

-sensorineural hearing loss -intellectual disability -cardiac anomalies -cataracts, glaucoma

187
Q

Wolff-Parkinson-White syndrome characteristics

A

-shortened PR -delta waves (slurred initial portion of QRS) -widened QRS -2/2 accessory pathway conducts from atria to ventricles faster than through AV node ==> supraventricular tachycardia

188
Q

Characteristics of serum sickness-like rxn

A

1-2 wks after B-lactam or TMP-SMX therapy -Type III hypersensitivity rxn -fever, urticaria, polyarthralgia -labs: nonspecific hypocomplementemia, elevated inflammatory markers (ESR, CRP)

189
Q

Tx of serum sickness rxn

A
  1. remove offending agent 2. if severe ==> steroids *avoid in future (even though not true allergy)
190
Q

Chronic granulomatous disease presentation

A

-recurrent/unusual lymphadenitis, abscesses, osteo -infections w/catalase-positive organisms: s. aureus, aspergiluus, serratia marcescens, burkholderia cepacia -positive nitroblue tetrazolium test

191
Q

Wiskott-Aldrich syndrome presentation

A

-X-linked recessive -eczema, thrombocytopenia, recurrent infections w/encapsulated organisms -@ birth: petechaie, bruises, bleeding w/circumcision, bloody stools -immune: low IgM, high IgA and IgE

192
Q

Chediak-Higashi syndrome characteristics

A

-decreased degranulation, chemotaxis/fxn of PMNs -coagulopathy, neuropathology, hepatosplenomegaly, pancytopenia -frequent bacterial infections (usually staph) -==> neutropenia + giant lysosomes in PMNs

193
Q

Tx of chediak-higashi

A

-daily TMP-SMX -daily ascorbic acid

194
Q

Leukocyte adhesion deficieny presentation

A

-neutrophilia w/out polymorph in infected pus -delayed separation of umbilical cord -recurrent bacterial infections -necrotic skin lesions -dental/oral decay

195
Q

CSF in guillan-barre

A

-elevated protein -normal cell count

196
Q

Tx of guillan-barre

A

-pooled immunoglobulin -plasmaphersis

197
Q

Anterior horn diseases

A

-polio -spinal muscular atrophy -ALS

198
Q

NMJ diseases

A

-botulism -myasthenia gravis -lambert-eaton (proximal m. weakness) -organophosphate poisoning

199
Q

Vitamin B12 deficiency neuro sx

A

-subactue degeneration of dorsal and lateral columbs (upper motor neurons) - ataxia, bilateral paresthesia, loss of vibration/position sense

200
Q

Most common leukemia in 2-10yo

A

ALL

201
Q

ALL presentation

A

-children 2-10yo -fever, infection vs. lymphadenopathy vs. splenomegaly -PAS+ -TdT +

202
Q

Chlamydial vs. Gonoccocal conjunctivitis

A

-gonoccocal: @ 2-5 days old w/purulent discharge -chlamydial: @ 5-14 days w/watery discharge

203
Q

Dx of hereditary spherocytosis

A

-presents w/coombs-neg. hemolytic anemia, jaundice, splenomegaly -positive Eosin-5-maleimide binding & acidified glycerol test

204
Q

Spondylolisthesis definition

A

-developmental d/o w/forward slip of vertebra -usually L5 over S1

205
Q

Trachoma presentation

A

-follicular conjunctivitis + neovascularization @ cornea -concurrent infection of nasopharynx nasal d/x -dx: giemsa stain of conjuctival scrapics

206
Q

Tx of trachoma

A

-topical tetracycline or oral azithromycin

207
Q

Causes of secondary enuresis

A

-psych stress -UTI -DM -Diabetes insipidus -OSA

208
Q

Dx of cyclic vomiting syndrome

A

->3 episodes in 6 mo. Period -family recognition -lasts 1-10 days -vomiting >4x/hour @ peak -no sx in between episodes -no underlying condiditon -often hx of migraines in parents

209
Q

Method of removal of small vaginal foreign body in young child?

A

-calcium alganate swab -warm irrigation w/topical anesthetic -large FB ==> may need to remove under anesthesia

210
Q

sleep stage for night terrors

A

non-rem

211
Q

head trauma w/LOC ==> ?

A

CT w/out contrast

212
Q

Pathophys/renal complications in sickle cell trait + painless hematuria?

A

-renal papillary necrosis -inability to concentrate urine -distal renal tubular acidosis

213
Q

Thrombosis-related complications in sickle cell trait

A

-splenic infarct (esp. @ high altitude) -VTE -priapism

214
Q

Most common cardiac malformation in Down’s + presentation

A

-complete AV septal defect ==> HF @ 6wks old -HF ==> poor feeds, diaphoresis, dyspnea -loud S2 + systolic ejection murmur +/- VSD

215
Q

One of earliest manifestations of sickle cell anemia

A

dactylitis (hand-foot syndrome) = vaso-occlusion/crisis @ hands and feet ==> lytic lesions on xr

216
Q

Common neonatal rashes

A

-erythema toxicum neonatorum = asx, scattered red macules, papules, pustules = benign -neonatal HSV = vesicles -neonatal varicella = fever + clustered vesicles -staph scalded skin = fever, irritable + erythema => blister => exfoliation

217
Q

McCune-Albright characteristics

A

-3 P’s: -Precocious puberty -Pigmentation (cafe-au-lait spots) -Polyostotic fibrous dysplasia = multiple bone defects -assoc. w/cushing syndrome

218
Q

Common predisposing factor for orbital cellulitis

A

bacterial sinusitis

219
Q

Bilious vomiting + abdominal distension + gasless on abdominal xray ==> ?

A

upper GI series

220
Q

bilious vomiting + dilated loops of bowel on abd xray ==>

A

-contrast enema -microcolon on contrast enema ==> mec ileus -rectosigmoid transition zone ==> hirschsprung

221
Q

Fetal Alcohol Syndrome dysmorphic features

A
  1. small palpebral fissures 2. smooth philtrum 3. thin vermilion border (thin lip)
222
Q

Maternal benefits of breast feeding

A

-decreased PP bleeding -weight loss -child spacing -bonding -reduced breast & ovarian cancer

223
Q

Contraindications to breastfeeding

A

-MOC w/TB -Maternal HIV -herpetic breast lesions -varicella infections

224
Q

Lab findings in thallasemias

A

-MCV: low -RDW: normal (vs. high in iron def.) -RBCs: normal (vs. low in iron def.) -normal/increased iron and ferritin -smear: target cells (vs. microcytosis, hypochromia in iron def.)

225
Q

Hb electrophoresis in iron def. vs. alpha thal minor vs. b-thal minor

A

-iron = normal -a-thal minor = normal -b-thal minor = increased hb A2

226
Q

Congenital hypothyroid presentation/timeline

A

-normal @ birth; ==> apathy, weakness, hypotonia -sluggish, large tongue, abdominal bloating, umbilical hernia **usually screened for @ birth in US

227
Q

Condition in which pt.s may have anaphylactic rxn to blood transfusion?

A

IgA deficiency 2/2 abs formed against IgA

228
Q

Timing for meningococcal vaccine

A

-initial dose @ 11-12yo -booster @ 16yo

229
Q

Edward’s syndrome characteristics

A

-Trisomy 18 -micrognathia, microcephaly -rocker bottom feet, overlapping fingers, absent palmar creases -VSD = most common defect

230
Q

Cause of croup

A

parainfluenza virus

231
Q

UMN findings in Down’s syndrome child ==> dx?

A

atlantoaxial instability

232
Q

Nikolsky sign (positive) ==> ?

A

-Nikolsky sign = gentle lateral pressure @ skin surface adjacent to blister leads to slip/detachment of superficial layer of skin -positive ==> staph scalded skin syndrome

233
Q

Toxin-mediated skin rashes

A

-Staph scalded skin syndrome -Scarlet fever

234
Q

Characteristics of scarlet fever

A

-group A beta-hemolytic strep toxin-mediated -viral prodrome: fever, H/A, vomiting, sore throat -12-48h ==> fine pink blanching papules @ neck, trunk ==> flexor surfaces

235
Q

Pathophysiology of physiologic jaundice of newborn

A

-unconjugated hyperbili 1. high hb turnover in first few days of life 2. UGT is slow in newborn (asians have decreased activity compared to other ethnicities) 3. enterohepatic recycling is increased in newborn gut

236
Q

Phototherapy to prevent …?

A

kernicterus

237
Q

Adenosine deaminase deficiency = characteristic of which dz?

A

SCID

238
Q

Dysmorphogenesis of third/fourth pharyngeal pouch = ?

A

digeorge ==> t cell dysfxn

239
Q

CGD 2/2 to ?

A

inability to oxidize pathogens w/in phagocytes

240
Q

Elevated screening lead ==> ?

A
  1. draw venous lead level (2/2 false + on fingerstick) 2. mild elev. (5-44) ==> no meds, repeat w/in 1 mo. 3. mod elev. (45-69) ==> meso-2,3-dimercapto-succinic acid (DMSA) 4. severe (70+) ==> dimercaprol + ca disodium edetate (EDTA)
241
Q

Most common brain tumors in peds

A
  1. benign astrocytoma 2. medulloblastoma
242
Q

Most common causes of bacterial sinusitis

A
  1. strep pneumo 2. h. influenza
243
Q

Sx/risk factors/definition of polycythemia in neonate

A

-Hct > 65% @ term neonate -delayed cord clamp, maternal HTN, maternal DM -respiratory distress, hypoglycemia, neuro problems

244
Q

Examination findings in endometriosis

A

-uterosacral nodularity -adnexal tenderness

245
Q

Examination findings in adenomyosis

A

-uterine tenderness/enlargement

246
Q

Common causes of acquired torticollis + inititial evaluation

A

-URI -minor trauma -cervical lymphadenitis -retropharyngeal abscess -C-spine Xray to rule our c-spine fx/dislocation

247
Q

NF1 features

A

-cafe-au-lait -macrocephaly -feeding problems -short stature -learning disabilities ==> fibromas, neurofibromas, other tumors

248
Q

Congenital syphillis presentation

A

-HSM -cutaneous lesions -jaundice -anemia -rhinorrhea -On xray: metaphyseal dystrophy and periostitis

249
Q

Sturge-Weber features

A

-unilateral hemangioma along trigeminal distribution -intracranial calcifications that resemble a tramline

250
Q

Absence seizure tx

A

-ethosuximide -valproic acid

251
Q

Tx of battery ingestion distal vs. proximal to esophagus

A

-proximal ==> immediate endoscopic removal -distal ==> observe w/stool exam/f/u radiographs

252
Q

Bug/Tx of typical acute unilateral cervical adenitis

A

-usually 2/2 staph aureus vs. strep pyogens -tx = clindamycin

253
Q

Bug/Tx of acute unilateral cervical adenitis in child w/dental disease

A

-anaerobic -tx = clinda vs. amox/clav

254
Q

Tx of acute unilateral cervical adenitis in child w/non-TB myco

A

-excision -marcolide +/- rifampin

255
Q

Neuroblastoma characteristics

A

-3rd most common cancer in peds -NC cells ==> sympathetic chains/adrenal medulla -common site is abdomen -calcification/hemorrhage on xray or CT -elevated homovanillic acid or vanillylmandelic acid

256
Q

Recurrent episodes of hematuria + sensorineuronal deafness + fhx of renal failure ==> dx?

A

Alport syndrome

257
Q

EM in Alport syndrome

A

-thinned and thickened cap loops -splitting of glomerular basement membrane

258
Q

Tx of lyme disease in children

A
  • amox - >9yo ==> doxy
259
Q

Lower intestinal pathology assoc. w/Down’s

A

Hirschsprung

260
Q

Fluid for correction of hypernatremia in infants

A

NS

261
Q

Source of delayed/absent puberty in turner’s

A

-streak ovaries ==> decreased estrogen - ==> elevated FSH

262
Q

Initial management of epiglottitis

A
  1. to OR for intubation vs. tracheostomy 2. admission + IV antibiotics NO racemic epi or steroids
263
Q

Late-onset CAH presentation/cause

A

-21-hydroxylase deficiency -presents in late childhood -leads to signs of androgen excess ==> premature adrenarche/pubarche, severe cystic acne, accel linear growth, advanced bone age -NORMAL ELECTROLYTES

264
Q

Management of abnormal hip exam in child

A
  • (+) ortolani/barlow ==> ortho -asymmtery w/ (-) o/b @ 2wks - 6mo. ==> hip US -asymmtery w/ (-) o/b @ 4 - 6mo. ==> hip xray
265
Q

Gold standard for Duchenne MD dx?

A

genetic testing

266
Q

Iron poisoning presentation

A

-abdominal pain -hematemsis -shock -metabolic acidosis -xray w/radioopaque pills

267
Q

Iron poisoning tx

A

-deferoxamine -IV fluids if needed

268
Q

Management of neonatal weight loss

A

-may lose up to 7% BW w/in first 5 days; should regain BW by 10-14 days - exclusive BF can continue, f/u for weight recheck - >7% ==> assess for oromotor dysfxn, assess for lactation failure, daily weights, consider supplementing w/formula

269
Q

Breath-holding spell work-up

A

CBC to eval for anemia

270
Q

Cause of hemophilic arthropathy

A

-2/2 recurrent hemarthrosis - ==> hemosiderin deposition ==> synovitis and fibrosis in joint

271
Q

Cat bite tx/ppx

A

amox-clav

272
Q

Cause/metabolic consequence of RTA

A

-defect in H excretion or HCO3 resorption in kidney - ==> FTT 2/2 chronic, normal anion-gap metabolic acidosis -tx = oral bicarb replacement

273
Q

Niemmann Pick vs. Tay Sachs

A

-Both: cherry-red macula, loss of motor milestones, feeding difficulties -Tay-Sachs: hyperreflexia, b-hexos deficiency -NP: HSM, areflexia, sphingomyelinase

274
Q

Complication of neonatal heart surgery

A

-pericardial effusion -aka “postpericardiotomy” ==> fluid accumulation ==> distant heart sounds, hypotension, distended jugular veins

275
Q

Scalp swelling limited to one cranial bone in newborn ==> dx?

A

-cephalohematoma = subperiosteal hemorrhage -w/in a few hours after birth -most do not require tx -resorb spontaneously w/in a few months

276
Q

Cranial menigocele presentation

A

-scalp swelling w/pulsations -increased pressure w/crying -evidence of bony defects

277
Q

Caput succedaneum presentation

A

-diffuse, ecchymotic, swelling of the scalp -can extend across suture lines

278
Q

infant w/intermittent cyanosis during feeding and relief w/crying ==> dx?

A

-choanal atresia ==> inability to pass catheter through the nose -CT: narrowing @ pterygoid plate in post. nasal cavity

279
Q

Herpangina: etiology, age, seasonality, clinical features, tx

A

-Coxsackie A virus -3-10y -summer/fall -fever, pharyngitis, gray vesicles/ulcers on post. oropharynx -supportive tx

280
Q

Herpetic: etiology, age, seasonality, clinical features, tx

A

-HSV-1 -6 mo - 5yrs -no season -fever, pharyngitis, erythematous gingiva, clusters of small vesicles on anterior oropharynx -tx: oral acyclovir

281
Q

Strawberry hemangioma characteristics/tx

A

-composed of capillaries separated by connective tissue -generally observation/reassurance because most regress spontaneously -beta-blockers if disfiguring or dangerous

282
Q

Fixed split S2 ==> ?

A

-ASD -fixed split is 2/2 to increased flow in R. heart leading to delay - + SEM 2/2 to increased flow across pulmonic valve

283
Q

Diastolic rumble = ?

A

increased flow across mitral valve (e.g. eisenmenger syndrome) vs. mitral stenosis

284
Q

Fanconi anemia

A

-AR -congenital marrow failure -poor growth -morphologic abnormalities (microcephaly, abnormal thumbs, hypogonadism) -macrocytic anemia

285
Q

Acquired causes of aplastic anemia

A

-drugs (NSAIDs, sulfonamides) -toxic chemicals -idiopathic -viral infections (HIV, EBV) -immune disorders -thymoma

286
Q

fetal macrosomia, umbilical hernia, macroglossia, hemihyperplasia ==> Dx?

A

-Beckwith-Wiedemann syndrome -deregulated imprinted gene on 11p -can lead to Wilms tumor/heaptoblastoma -surveillance alpha-feto and abdominal/renal US

287
Q

Diseases that present w/fever, pharyngitis, and oral lesions in young children

A

-Herpangina (coxsackie A virus) -Herpetic gingivostomatitis (HSV-1)

288
Q

Indications for imaging in headache

A
  1. Ataxia/ coordination difficulties 2. Numbness/tingling 3. Focal Nero signs 4. History of headache awakening from sleep 5. Increasing headache frequency
289
Q

Primary treatment for intussusception

A

Air enema

290
Q

Most common cause for tinea capitis

A

trichopytan tonsuran

291
Q

Hodgkinson lymphoma presentation

A

–teenager? – Lymphadenopathy – Weight loss – Itching

292
Q

Humor shaft fracture leads to?

A

Radial nerve injury Inability to extend wrist/fingers

293
Q

Treatment for Gerd in a preemie

A

Fundoplication?

294
Q

Doll face/thin extremities association?

A

-Glucose – 6 –phosphatase deficiency – Hypoglycemia – Lactic acidosis – Hyperlipidemia – hyperuricemia

295
Q

Risk factors for cerebral palsy

A

– Prematurity – IUGR –placental pathology

296
Q

Clinical characteristics of cerebral palsy

A

– Progressive motor dysfunction –spastic VS. dyskinetic VS. ataxic –comorbidities: –intellectual disability – Epilepsy – Strabismus – Scoliosis

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