bs-pds-oo Flashcards

1
Q

Retropharyngeal abscess (clinical sx/sign and test to order and organisms involved)

A

fever, dysphagia, inability to extend neck, muffled voice, lateral x-ray with widened prevertebral space; get a CT w/ contrast; polymicrobial (s. pyogenes, s. aureus, anaerobes); most commonly children 6 months to 6 years

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2
Q

Epiglottitis

A

high fever, drooling, “thumb” sign (swollen epiglotis)

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3
Q

Henoch-Schonlein purpura (a/w with what complication)

A

immune-mediated leukocytoclastic vasculitis a/w IgA deposition; purpura, arthritis, abd pain, renal dz, scrotal swelling/pain; normal platelets/coags, hematuria, inc. Cr; Tx = NSAIDs, supportive, steroids; INTUSSUSCEPTION (ileo-ileal or small bowel, look for “target sign” on ultrasound since these do not show up on contrast enema)

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4
Q

DDx of hyperandrogenemia and oligo-ovulation

A

PCOS (most common but need to rule out others), ovarian/adrenal tumors, late-onset CAH, hyper-prolactinemia, acromegaly, Cushing’s Dz

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5
Q

Partial deficiecny of 21-hydroxylase in female patients

A

adolescence/adulthood presentation w/ hirsuitism, virilism, elevated 17-hydroxyprogesterone, varying degrees of salt wasting (depending on degree of enzyme deficiency)

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6
Q

PCOS

A

oligo-ovulation, hyperandrogenemia, polycystic ovries on imaging (2 out of 3 of these); High LH:FSH ratio is nonspecific; 17-hydroxyprog is usually NORMAL

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7
Q

Acute otitis media (presentation, causative organisms, complications, treatment)

A

6-36 months, fever, fussiness, irritability, ear complaints; Strep pneumo (40%), H. influenza (30%), viral (RSV and rhino - 30%), Moraxella catarrhalis (10%), hearing loss, mastoiditis, labrynthitis, TX = amoxicillin 10 days, or IM ceftriaxone

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8
Q

pyloric stenosis

A

boys, projectile nonbilious vomiting, palpable olive-shaped mass in RUQ, hypochloremic, hypokalemic metabolic alkalosis; Dx = abdominal ultrasound; Tx = pyloromyotomy (after hydration and stabilization of e-lytes)

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9
Q

Necrotizing enterocolitis (NEC)

A

most common GI emergency in NICU; prematurity and low birth weight are RFs; air in bowel wall (“train-track”, pneumatosis intestinalis on xray and portal venous air (look at liver); can cause perforation and pneumoperitoneum); labs: leukocytosis and metabolic acidosis from inflammation and intestinal ischemia; Tx = supportive and also BREASTMILK is best!

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10
Q

Hemophilia A & B

A

Factor VIII, IX deficiences; X-linked recessive; males, hemarthroses, intramuscular hematomas, mucosal bleeding, GI/GU bleeding, intracranial hemorrhage; late complications = hemophilic arthropathy, blood-borne infection, inhibitory Abs; tests: Coags (PT, INR, PTT); Tx = Factor VIII/IX

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11
Q

Vitamin A deficiency

A

age 2 or 3, dry conjunctiva, dry cornea, scaly skin, impaired adaptation to darkness, keratomalacia (wrinkled, cloudy cornea), Bitot spots (dry, silver-gray plaques on bulbar conjunctiva), follicular hyperkeratosis

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12
Q

Thiamine defiency

A

a/w infantile/adult beriberi and Wernicke-Korsakoff; infantile beriberi = cardiomegaly, tachycardia, cyanosis, dyspnea, vomiting; adult beriberi = dry/wet, dry = symmetrical peripheral neuropathy w/ sensory and motor impairments (distal extremities), wet = neuropathy + cardiac (CHF, cardiomegaly, edema))

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13
Q

Riboflavin deficiency

A

sore throat, edematous oropharyngeal mucous membranes, cheilitis, stomatitis, normocytic normochromic anemia, seborrheic dermatitis, photophobia

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14
Q

Scurvy

A

Ascorbic acid defiency: impaired collagen synthesis; ecchymoses, petechiae, bleeding gums, hyperkeratosis, Sjogren’s syndrome, arthralgias, impaired wound healing; weakness, malaise, coiled hair, depression, neuropathy, dry skin, dry eyes

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15
Q

Hypervitaminosis A

A

excessive Vitamin A = anorexia, pruritis, hepatomeagly, alopecia, irritability, increased ICP, seborrheic cutaneous lesions

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16
Q

Jervell-Lange-Nielson syndrome

A

congenital long QT syndrome: congenital deafness, syncopal episode WITHOUT following disorientation (so Torsades or other arrhythmia is likely), normal physical exam, and history of sudden cardiac death; TX = propranolol

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17
Q

acute bacterial rhinosinusitis

A

cough, nasal discharge, swollen turbinates; Sx >10 days or severe Sx, fever > 39C, face pain for > 3 days; or worsening Sx >5 days after initial improvement; Tx = amoxicillin + clavulanic acid; most common causes; s. pneumo and h. influ

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18
Q

erythema multiforme

A

acute, self limited reaction, commonly from herpes simplex; targetoid papule/plaque; arcofacial distribution

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19
Q

Nikolsky sign

A

gentle lateral pressure on the skin surface adjacent to blister causes slipping and detachment of superficial layer of skin)

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20
Q

Staphylococcal scalded skin syndrome

A

exfoliative toxin producing strains of S. aureus. targets desmoglein-1 (keratinocyte adhesion in superficial epidermis; prodrome of fever, irritiability, skin tenderness; erythema starts on face –> generalizes over 24-48 hours; Nikolsky sign positive; Scaling and desquamation for 5 days, resolves w/in 2 weeks;

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21
Q

impetigo

A

localized epidermal infection causes by S. aureus or group A beta hemolytic strep; more common in kids than adults; bullous = flaccid, honey-colored crust

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22
Q

scarlet fever

A

toxin-related process; (group A beta-hemolytic strep; children; usually after tonsilitis, pharyngitis; prodrome of fever, headache, vomiting, sore throat; rough-sandpaper-like texture of eruption

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23
Q

erysipelas

A

bacterial infection of dermis; well demarcated indurated warm tender plaque; fever, chills, malaise, group A strep; face and lower extremities

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24
Q

foreign body aspiration

A

cough, focal monophasic wheezing, inspiratory stridor; most common 6 months to 4 years; acute onset

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25
Q

laryngomalacia

A

4-8 months; increased laxity of supraglottic structures –> collapse during inspiratory phase; stridor LOUDest in SUPINE position; improves with upright or prone

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26
Q

croup

A

laryngotracheobronchitis; most common cause of inspiratory stridor in age group 6 months to 3 years; caused by parainfluenza virus; “barky” cough, rhinorrhea, congestion, low-grade fevers, inspiratory sridor; RESPONDS to epinephrine and corticosteroids

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27
Q

vascular rings

A

BEFORE age 1; respiratory/esophageal Sx (stridor, wheezing, cough, dysphagia); stridor IMPROVES w/ neck extension and do NOT improve w/ epi or corticosteroids; Dx = barium esophagogram, bronchoscopy, CT; Tx = surgery

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28
Q

Intraventricular hemorrhage is most commonly seen in what type of patietns

A

premature and low birth weight (inversely proportional to birth weight)

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29
Q

Most common helminthic infection in the U.S.

A

Enterobiasis; etiology = pinworm Enterobius vermicularis; children age 5-10; perianal noctural itching; “scotch-tape” test; Tx = albendazole/mebendazole

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30
Q

blue sclera

A

osteogenesis imperfecta; type 1 collagen defect; numerous fractures in multiple stages of healing; short stature, blue sclera

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31
Q

bleeding disorders in children (coagulation versus platelet)

A

easy brusing, prolonged bleeding, mucosal bleeding, hemarthrosis, soft tissue bleeding; DDx: hemophilia A/B, vWD, platelet function disorder (Glanzmann thrombasthenia, Bernard Soulier syndrome); coag disorders: hemarthrosis, soft tissue and intramuscular hematomas; platelet disorders: ecchymoses and petechiaeeasy brusing, prolonged bleeding, mucosal bleeding, hemarthrosis, soft tissue bleeding; DDx: hemophilia A/B, vWD, platelet function disorder (Glanzmann thrombasthenia, Bernard Soulier syndrome);

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32
Q

pathologic cardiac murmurs in children

A

infants = diaphoresis or tiring w/ feeds, poor weight gain; Children = chest pain, dizziness, syncope, SOB, fatigue; Phys exam: holosystolic, diastolic, 3/6 or higher, increases w/ standing, abnormal S2, decreased/absent femoral pulses; workup = CXR, EKG, echo

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33
Q

Measles

A

Rubeola: cough, coryza, conjunctivitis, Koplik spots (bluish specks on buccal mucosa), maculopapular rash that spread cranio-caudal and SPARES palms/soles; transmission: resp droplets; Tx = supportive + Vitamin A; Complications = encephalitis, acute disseminated encephalopmyelitis; subacute sclerosing panencephaliti; prevention = live attenuated vaccine

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34
Q

Type I hypersensitivity

A

IgE-mediated; allergen binds and crosslinks two IgE molecules attached to mast cell; E.g. = atopy, urticaria, anaphylaxis

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35
Q

Type II hypersensitivity

A

Ab-mediated; E.g. = immune hemolytic anemia, Rh hemolyic dz of newborn

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36
Q

Type III hypersensitivity

A

immune-complex meidated; E.g. serum sickness, arthus reaction

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37
Q

Type IV hypersensitivity

A

delayed cell-mediated; E.g. Allergic contact dermatitis

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38
Q

Turner syndrome (most common cardiac defect)

A

short stature, webbed neck, broad chest w/ widely spaced nipples; phys exam: hypertension in upper extremities and delay in radial-femoral pulse; most common defect is coarctation of the aorta; also bicuspid aortic valve;

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39
Q

Most common and 2nd most common congenital cardiac defect in adults

A

bicuspid aortic vavle is MC; 2nd is ASD (ostium secundum is most common type)

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40
Q

most common congenital heart defect

A

VSD

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41
Q

trachoma

A

major cause of blindness worldwide; follicular conjuncitivtis and neovascularization in cornea; Dx giemsa stain; Tx = tetracycline or azithromycin

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42
Q

herpes simplex keratitis

A

pain, photophobia, decreased vision, dendritic ulcer; minute clear vesicles in corneal epithelium

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43
Q

orbital cellulitis

A

abrupt onset fever, proptosis, restrction of EOM, swollen, red eyelids

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44
Q

viral conjunctivitis

A

red, copious watery discharge, contaminate swimming pools; adenovirus type 3

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45
Q

choanal atresia

A

cyanosis that is worse w/ feeding and improves w/ crying; most common nasal malformation; Dx = CT w/ intranasal contrast

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46
Q

“tet” spells

A

cyansosis from stressful conditions; charactersitics of TOF;

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47
Q

Transposition of Great Arteries

A

no murmur on exam but cyanosis at birth and continuous

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48
Q

total anomalous pulmoanry venous connection (TAPVC)

A

right to left shunt at atria llevel w/ systolic ejection mumur in pulmonic area

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49
Q

truncus arteriosus

A

cyanosis is absent/moderate at birth (depeds on severity of pulmonary outflow obstruction) and murmur is ALWAYS present

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50
Q

Chronic granulomatous disease

A

inherited immunodef; inability to oxidize pathogens b/c deficient NAPDH oxidase enzyme; recurrent bacterial infections (S. aureus), neutrophils filled w/ bacteria; pneumonia and suppurative adenitis are common

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51
Q

Digeorge syndrome

A

dysmorphogenesis of 3rd and 4th pharyngeal pouches; hypoCa+2 2/2 parathyroid hypoplasia; defective T cell function 2/2 thymic hypoplasia; fungal/viral infections are common

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52
Q

most common primary immunodeficiency

A

agammaglobulinemia: poor B-cell maturation –> risk of infection from encapsulated bacteria (Hib, s. pneumo, neisseria meningitidis, GBS, klebsiella, salmonella)

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53
Q

Howell-Jolly bodies

A

single, round, blue inclusions in RBCs on Wright stain; often means splenectomy or hyposplenism

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54
Q

hemoglobin precipitation

A

G6PD deficiency; Hgb becomes oxidized and forms Heinz bodies (insoluable precipitants)

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55
Q

Developmental milestones by age and category (gross motor, fine motor, language, social/cognitive)

A

see question id 4199

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56
Q

Dx of acute alkali ingestion

A

upper GI endoscopy

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57
Q

osteogenesis imperfecta type 2

A

autosomal dominant; defectin type 1 collagen;; type 2 is most severe w/ intrauterine and perinatal fractures and restrictive lung disease

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58
Q

Fetal alcohol syndrome

A

craniofacial abnormalities, poor prenatal growth, microcephaly, hypotonia, and poor feeding

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59
Q

phenytoin use during pregnancy

A

craniofacial abnormalities, fingernail hypoplasia, growth deficiency, developmental delay, cardiac defects, facial clefts

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60
Q

vitamin K def in newborns is due to: (3 things)

A

poor placental transre, absent gut flora, inadequate levels in breast milk

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61
Q

Reye syndrome

A

aspirin (salicylates) to kids under age 15; vomiting, agitation, lethargy, stupor; labs = hyperammonemia, elevated bilirubin, Alk phos, PT, AST, ALT, LDH, low glucose; Bx = microvesicular steatosis

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62
Q

Systemic carnitine deficiency

A

similar picture to Reye’s: hypoglycemia, hyperammonemia, hypoprothrombinemia, acute episodes of encephalopathy; BUT there is also ELEVATED acyl-carnitine

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63
Q

Enuresis treatment

A

1st line = desmopressin; 2nd line = TCA (imipramine); side effects of desmo = hyponatremia; of TCA is cardiotoxicity

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64
Q

Legg-Calve-Perthes disease

A

osteonecrosis of femoral head; boys, age 4-10 years (mean 7), insidious onset w/ hip and/or knee pain and antalgic gait (shorter time weight bearing on affected side due to pain); Xrays may not show; get MRI

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65
Q

Slipped capital femoral epiphysis

A

classically obese adolescents (mean age 12 in girls and 13 in boys), also have limp and insidious onset hip pain

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66
Q

Chlamydial conjunctivitis

A

develops 5-14 days afer birth, presents w/ chemosis, mild eyelid swelling and watery/mucopurlent discharge; Tx = 14 days course oral erythromycin (SIDE EFFECT hypertrophic pyloric stenosis)

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67
Q

side effect of oral erythromycin in newborn

A

hypertrophic pyloric stenosis

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68
Q

Chlamydial pneumonia time course

A

age 4-12 weeks

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69
Q

How to prevent chlamydial conjunctivitis or PNA?

A

maternal testing in 1st and 3rd trimester (if age >25 or >25 w/ risk factors) and Abx treatment

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70
Q

Erythromycin eye ointment is effective against

A

GONOCOCCAL conjunctivitis; not for chlamydial

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71
Q

Lesch-Nyhan syndrome

A

x-linked recessive; males; deficiecny of hypoxanthine-guanine phosphoribosyl transferase (HPRT), involved in purine mteabolism; increased uric acid; 6 moths, hypotnia, gout, persistent vomtiing, MR, spasticity, compulsive self injury (biting); Tx is allopurinol, control fluid intake

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72
Q

prophylaxis for sickle cell disease

A

twice daily penicillin until age 5

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73
Q

NEC

A

vital sign instability, vomiting, bloody stols, abdominal distension/tenderness; Tx is bowel rest + hydration + broad spectrum Abx; at risk for septic shock, stricures, short bowel snydrome, death

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74
Q

what nutrional interventions in first 6 months of life?

A

Iron and Vitamin D supplementation. Iron stores will be low so give iron for 1 year; Vitamin D when breastfeeding for first 6 months

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75
Q

Transient synovitis

A

most common cause of hip pain in children; boys age 3 to 10; often follows viral infection; on exam, hip typically flexed, abducted and externally rotated; Tx is NSAIDs and rest; get Xrays to r/o LCP

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76
Q

Septic arthritis

A

fever >101, inability to bear weight, WBC > 12k, ESR > 40, CP >20mg/L; if more than 3 of these, do arthrocentesis

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77
Q

angiofibroma

A

epistaxis, localized mass, bony erosion on back of nose

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78
Q

doxycycline is contraindicated in

A

pregnant women and kids under age 8

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79
Q

Tx of Lyme disease in young kid

A

oral amoxicillin (cannot use doxycycline)

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80
Q

Tx of disseminated Lyme disease

A

IV ceftriaxone (for meningitis and heart block)

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81
Q

characterize fetal alcohol syndrome

A

MR, hypoplastic maxilla, long philtrum, microcephaly

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82
Q

characterize Down’s syndrome

A

MR, microcephaly, flat occiput, Simian crease, endocardial cushion defects, prominent tongue, epicanthal folds w/ slanting eyes, Brush field spots on periphery of iris

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83
Q

characterize Fragile X syndrome

A

low IQ w/ learning disability, language disability, short attention span, autism, large head, prominent jaw, low set large ears, macroorchidism; CGG trinucleotide repeats, mutation in FMR1 gene

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84
Q

atopic dermatitis in infancy

A

pruritis and skin lesions on face, scalp, chest, and extensor surfaces of extremities

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85
Q

indications for evaluation of neonatal jaundice

A

1) conjugated hyperbili > 2mg/dL; 2) jaudice w/in first 24-36 hours; 3) serum bili rising faster than >5mg/dL/day; 4) serum bili >12 in full term; 5) jaundice lasting longer than 14 days; 6)signs/Sx

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86
Q

erythroblastosis fetalis

A

unconj hyperbili and positive Coombs’ test

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87
Q

Crigler-Najjar

A

inhereited deficiency of UDP-glucuronyl transferase; lack of enzyme entirely. die within years

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88
Q

Gilbert’s

A

mild def of UDP-glucuronyl transferase

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89
Q

most common benign vascular tumor in kids

A

strawberry hemangioma (grow rapidly within 1 to 2 years then resolve by age 8)

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90
Q

most common benign vascular proliferation in adults

A

cherry hemangiomas; they do NOT regress

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91
Q

PANDAS

A

pediatric autoimmune neuropsychiatric disorders: can get OCD after recent streptococcal infection

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92
Q

Tx for OCD

A

SSRI (fluoxetine) or CBT

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93
Q

midgut volvulus

A

child less than 1 month; bilious vomiting, abdominal distension, bloody stools

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94
Q

duodenal atresia

A

bilious vomiting a few hours after birth; a/w Down’s

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95
Q

meconium ileus

A

a/w cystic fibrosis; failure to pass meconium within first 24 hours

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96
Q

Pertussis

A

Bordetella pertussis is highly contangious even with immunization so PREVENTION is key by giving ALL contacts a 14 day supply of erythromycin

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97
Q

Tx of impetigo

A

topical mupirocin or oral erythromycin; cause is usually GABS or S. aureus

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98
Q

contraindications to breast-feeding

A

mother: active untreated TB, HIV infection, herpetic breast lesions, varicella infection, maternal meds, chemo/rads, active substance abuse; Child: galactosemia

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99
Q

Best step for rehydration for moderate to severe

A

20mL/kg of normal saline IV

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100
Q

most common predisposing factor for acute bacterial sinusitis

A

Viral upper respiratory infection

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101
Q

Neonatal rashes (list 4)

A

erythema toxicum neonatorium, neonatal herpes simplex virus, neonatal varicella, staphylococcal scalded skin syndrome

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102
Q

Erythema toxicum neonatorium (ETN)

A

asymptomatic, scattered erythematous macules, papules, and pustules over body; No Tx needed

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103
Q

Neonatal herpes simplex virus

A

Vesicular lcusters on skin, eyes, mucous membranes; CNS infection, dissemianted organ dz; Tx = acyclovir

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104
Q

Neonatal varicella

A

fever, vesicular clusters and disseminated dz; Tx = acyclovir

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105
Q

Staph scaled skin syndrome (SSSS)

A

fever, irritability, diffuse erythema, blistering exfoliation, positive Nikolsky’s sign; Tx = oxacillin,nafcillin, vancomycin

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106
Q

VSD

A

most common congenital heart dz (26%); of these, 40% close by age 3; and 75% close by age 10. Louder holosystolic murmur often means smaller VSD so more likely to close; can do surveillance w/ EKG and echo

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107
Q

Wolff-Parkinson-White syndrome

A

accessory pathway b/w atria and ventricle leading to increased risk of tachyarrhythmias; classic EKG findings = shortened PR, delta waves, widened QRS

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108
Q

Croup, appropriate Tx

A

laryngotracheobronchitis; try trial of racemic epinephrine before intubation

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109
Q

most common cause of congenital hypothyroidism in the U.S.?

A

thyroid disgenesis

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110
Q

indication for renal/bladder ultrasound in child

A

less than 24 months of age with a febrile UTI; give Abx and get U/S

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111
Q

common and dangerous causes of acquired torticollis

A

upper respiratory infections, minor trauma, cervical lymphadenitis; dangerous = retropharyngeal abscess and alantoaxial subluxation; so GET an XRAY

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112
Q

ciprofloxacin in children

A

contraindicated b/w may ause cartilage destruction and growth retardation

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113
Q

acute exacerbation of cystic fibrosis lung disease; organism and what to do

A

pseudomonas aeruginosa; give aminoglycoside (tobramycin) and antipseudomonal penicillin (piperacillin); in adults can give fluoroquinolone in place of the aminoglycoside

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114
Q

differential for T-wave inversion

A

myocardial infarction, myocarditis, old pericarditis, myocardial contusion, digoxin toxicity

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115
Q

Tularemia

A

Francisella tularensis = acute unilateral cervical lymphadenopathy, fever, chillds, headache, malaise; contact w/ rabbits, hamsters, blood-sucking arthropods

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116
Q

unilateral lymphadenitis differential in children

A

EBV (often bilateral and sub-acute to chroinc w/ systemic sx (fever, pharyngitis, hepatosplenomegaly)), tularemia (animal contact), peptostreoptococcus (periodontal disease), non-TB mycobacteria (MAC: subacute and no fever/tenderness)

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117
Q

if kid develops anaphylactic reaction, encephalopathy, or CNS complication to DTaP, what to do?

A

Give DT and leave out the pertussis part

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118
Q

talipes equinovarus

A

clubfoot: equinus and varus of calcaneum and talus, varus of midfoot, and adduction of forefoot

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119
Q

most common cause of primary amenorrhea

A

Turner syndrome (45 XO, short stature, webbed neck, hypogonadism (streak ovaries), lymphedema, high-arched palate, congenital bicuspid aortic valves, coarctationofthe aorta

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120
Q

differential for hematochezia in childhood

A

hemorrhoids, infectious colitis, intussusception, Meckel’s diverticulum, inflammatory bowel disease

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121
Q

congenital rubella syndrome triad

A

cataracts, PDA, sensorineural hearing loss; can have growth retardation and purpura (blueberry muffin rash)

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122
Q

cause of childhood stroke

A

sickle cell anemia

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123
Q

most common cause of urinary tract obstruction in newbornboys

A

posterior urethral valves

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124
Q

Diamond-Blackfan syndrome

A

congenital hypoplastic anemia: macrocytic anemia, low retic count, congenital anomalies

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125
Q

foreign body ingestion Tx

A

flexible endoscopy

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126
Q

foreignb body aspiration Tx

A

rigid bronchoscopy

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127
Q

tinea corporis

A

superficial fungal infection characterized by erythematous, scaly, pruritic rash w/ central clearing; Tx = terbinafine (topical antifungal)

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128
Q

myxedema

A

congenital hypothyroidism: MR w/ phenotype at birth

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129
Q

what is Guthrie test?

A

for PKU: mousy odor in urineR; most common test for PKU is blood phenyalanine levels; Guthrie test is qualitative; 1 in 10,000; whites and asians

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130
Q

most common urologic problem in children

A

vesicoureteral reflux (30-45% of kids with UTI) and is risk factor for both pyelonephritis and recurrent UTI; long term complication is renal scarring

131
Q

recommendation for children with first UTI and recurrent febrile UTI

A

age 2-24 months get a renal ultrasound for first UTI; get a voiding cystourethrogram (VCUG) for any child with recurrent febrile UTIs

132
Q

loop diuretics are indicated in children w/ …

A

congenital heart disease who have volume overload and pulmonary edema

133
Q

Mycoplasma infection

A

dry cough, bullous myringitis, PNA, pharyngitis, mailaise, weakness; Tx is erythromycin

134
Q

RSV more commonly affects

A

children with heart defects; Tx is aerosolized ribavirin

135
Q

most common organism responsible for pericarditis

A

Coxsackie virus

136
Q

Five major criteria and Three minor criteria for Rheumatic fever

A

Major = polyarthritis, carditis, chorea, subcutaneous nodules, erythema marginatum; Minor = fever, arthralgia, previous Rheum fever

137
Q

Henoch-Scholein purpura

A

IgA-mediated vasculitis of small vessel; common in cihldren; clinica = palpable purpura on lower extremities, renal disease, abdominal pain, arthralgias; Renal Bx = IgA deposition

138
Q

Mgmt of omphalocele/gastroschisis

A

cover with sterile dressing to prevent heat loss, insert orogastric tube (decompress stomach), stabilize airway, and establish peripheral IV access

139
Q

most common renal malignancy in childhood

A

Wilm’s tumor: age 2-5, fourth most common childhoood cancer; sporadic; associated syndromes WAGR (Wilm’s, aniridia, genitourinary anomalies, retardation), Beckwith-Widemann, Denys-Drash syndrome

140
Q

Denys-Drash syndrome

A

gonadal dysgenesis, nephropathy, Wilm’s tumor; mutated WT1

141
Q

Beckwith-Widemann (5 features)

A

macroglossia, macrosomia, midline abodminal defects, ear creases, neonatal hypoglycemia

142
Q

confirmatory tests for SLE

A

anti-Smith Abs, and dsDNA (both are specific)

143
Q

Transposition of great vessels

A

cyanosis within 24 hours of life, seen in infants of diabetic mothers and in males; includes single loud S2 and no murmur

144
Q

Tx of rapidly enlarging fluctuant cervical lymph nodes

A

Incision and drainage + dicloxacillin (which covers MSSA and strep but NOT MRSA)

145
Q

Tx for swallowed battery

A

depends on location: if in esophagus, do immediate endoscopic removal; if in stomach, then passes without problems in 90% of cases

146
Q

what to do with isolated proteinuria in children?

A

repeat urine dipstick on 2 subsequetn occasions;

147
Q

Common variable immunodeficiency (CVID)

A

acquired hypogammaglobulinemia; less severe Sx and usually presents aged 15 to 35 years old

148
Q

Bruton’s agammaglobulinemia

A

X-linked; defect in tyrosine kinase; usually well til 6-9 months of age when maternal IgG antibodies go away and kid has low concentrations of IgG, IgA, IgM, IgE and absent B cells; it’s X-linked so MALES

149
Q

alantoaxial instability

A

Down syndrome; suspect in patient with upper motor neuron findigns that progress over weeks; seen in 10-15% of down syndrome and caused by excessive laxity in posterior transverse ligament

150
Q

choledocahl cyst

A

congenital anomaly of biliary ducts: dilation of intra-hepatic and extra-hepatic biliary ducts or both;

151
Q

Caroli’s syndomre

A

congenital disorder: intrahepatic dilation of bile ducts

152
Q

recurrent noctural vulvar itching

A

Dx: scotch tape test and pinworm infection; Tx = mebendazole

153
Q

Hydroxyurea

A

inhibits ribonucleotide reductase (which converts UDP to dUDP)

154
Q

6-mercaptopurine (6-MP)

A

blocks de novo purine synthesis

155
Q

5-FU

A

inhibits thymidylate synthase (dUMP to dTMP)

156
Q

MTX

A

inhibts dihydrofolate reductase (humans)

157
Q

TMP

A

inhibits bacterialdihydrofolate reductase

158
Q

orotic aciduria

A

inability to convert orotic acid to UMP; autosomal recessive; inrease orotic acid in urine, megaloblastic anemia which does NOT improve w/ B12, no hyperammonemia; Tx is oral uridine

159
Q

xeroderma pigmentosum

A

mutated nucleotide excision repair;

160
Q

ataxia telangiectasia

A

mutated nonhomologous end joining

161
Q

I-cell disease (inclusion cell disease)

A

inherited lysosomal storage disorder; failure of addition of mannose-6-phosphate –> coarse facial features, cloded corneas, restricted joints, high plasma levels of lyosomal enzymes; fatal in childhood

162
Q

Cri-du-chat syndrome

A

5p deletion; microcephaly, hypotonia, short stature, cat-like cry

163
Q

Wolf-Hirschhorn syndrome

A

4p deletion; microcepahyl, bilateral epicanthral folds, “greek helmet” facies, ocular hypertelorism, frontal bossing, hypoplasia of eye socket, cardiac problems

164
Q

osteoid osteoma

A

sclerotic cortical lesion, diaphysis commonly, central nidus of lucency; pain at night; relieved with NSAIDS

165
Q

osteosarcoma

A

most common primary bone tumor in children; metaphyses of long bones; “sunburst” apperance and periosteal elevation; increased ESR, LDH, and alk phos

166
Q

Friedreich ataxia

A

neurologic (ataxia, dysarthria), skeletal (scoliosis, feet deformaties (hammer toes)), and cardiac (concentric hypertrophic cardiomyopathy) problems; cause of death commonly cardiac/resp problems

167
Q

rotavirus vaccination schedule

A

b/w 2 and 8 months; do NOT start after 15 weeks; and must FINISH by 8 months

168
Q

earliest age for menicogocceal vaccine if functional asplenia

A

age 2; so think about for sickle cell anemia patients

169
Q

Helmet cells RBCs –> DDx?

A

DIC, HUS, TTP

170
Q

basophilic stippling

A

ribosomal precipitates (blue granules of various sizes throughou cytoplasm of RBC); seen w/ thalassemias, and with heavy metal poisoning (or lead)

171
Q

Heniz bodies

A

aggregates of denatured Hgb; G6PD deficiency or thalassemia

172
Q

constitutional growth delay

A

delayed growth spurt, delayed puberty, delayed bone age

173
Q

osteonecross and african american

A

sickle cell disease; up to 50% who are homozygous will develop this problem; usually humeral and femoral heads;

174
Q

turner syndrome a/w what long term complication

A

osteoporosis; because of low estrogen levels (streaked nonfunctional ovaries) and only one copy of genes on X chromsome involved in bone metabolism

175
Q

developmental dysplasia of the hip (RF, Dx, Tx)

A

Do physical exam at each well-child visit; RFs = white, female, FHx, breech presentation; Dx is using Barlow (adduction w/ posterior pressure) and Ortolani (abduction) maneuvers; also look for inguinal skin folds; if child < 6 months and there is joint laxity, get an Ultrasound; if > 6 months, get an xray; Tx for kids < 6 months if Pavlik harness

176
Q

complications of mumps

A

orchitis (85% is unilateral); encephalitis, aseptic meningitis

177
Q

most common cause of 2ndary HTN in kids

A

fibromusuclar dysplasia (20% of renal HTN cases); right renal artery often more affected than left; angiography = “string of beads”

178
Q

studies to perform for neonatal bilious emesis

A

xray first; looking for malrotation w/ volvulus, meconium ileus (think CF), or hirschsprungs; then do contrast enema (water-souble gastrograpphin)

179
Q

all vaccines administered according to chronological age with exception of ____

A

Hepatitsi B; wait until child is above 2kg (4lbs 6 ounces)

180
Q

Dx and Tx of slipped capital femoral epiphysis

A

obese adolescent boys often w/ endocrinopathies (GH deficiency, hypothyroidism; get xrays of hip; Tx with screw fixation to prevent avascular necrosis of hip

181
Q

Legg-Calve-Perthes disease

A

idiopathic AVN in boys age 5-7

182
Q

infants treated w/ oral erythromycin are at increased risk of

A

infantile hypertrophic pyloric stenosis

183
Q

ceftriaxone in infants is a/w

A

increased risk of kernicterus because it displaces bilirubin from albumin binding sites so exacerbates a hyperbilirubinemia

184
Q

umbilical cord stump infection

A

tetanus; poor suckling and fatigue –> rigidity, spasms, opisthotonus –> death; look out for unimmunizied mothers

185
Q

most common cause of intestinal obstruction in children ages 6 to 36 months

A

intussusception

186
Q

intussusception a/w and test of choice to Dx and Tx

A

preceding viral infections (gastroenteritis), Meckel’s, Henoch Schonlein Purpura (hematomas); Dx with ultrasound (100% sens and spec if good tech); Tx is air enema

187
Q

complications of prematurity

A

RPRBIN: respiratory distress syndrome, PDA, bronchopulmonary dysplasia, intraventricular hemorrhage, necrotizing enterocoltis, retinopathy of prematurity

188
Q

Tx for intraventicular hemorrhage

A

antenatal corticiosteroids

189
Q

clinical features of CF

A

Resp: bronchiectasis, recurrent PNA, chronic rhinosinusitis; GI: obstruction, panc dz; repro: infertility; MSK: osteopenia, kyphoscoliosis, digital clubbing

190
Q

Mayer-Rokitansky-Kuster-Hauser syndrome

A

congenital uterine and vaginal aplasia (it is RARE even in CF patients)

191
Q

normal weight loss for newborns

A

up to 7% of their birth weight is acceptable

192
Q

never give water to child below age of

A

6 months; this can cause hyponatremia and seizures

193
Q

drugs to give for scarlet fever / strep if allergic to PCN

A

erythromycin, clinda, and first gen cephalosporins

194
Q

how to differentiate Kawasaki dz from scarlet fever

A

both have strawberry tongue, rash, lymphadenopathy; but Kawasaki has periperhal extremitiy involvement and/or conjunctival injection;

195
Q

rash after amoxicillin in setting of fever, lymphadenopathy, and pharyngitis

A

infectious mononucleosis due to EBV

196
Q

immune thrombocytopenia

A

ages 2 to 6; Antibodies to platelets; preceded by viral infection; often has purpura, petechia, nosebleeds; Tx is corticosteroids if platelets less than 30k

197
Q

white reflex in eye

A

retinoblastoma –> referal to ophthalmologist

198
Q

serum sickness-like reaction

A

1 to 2 weeks after PCN, amox, cefaclor in setting of viral illness; Sx = fever, urticarial rash, polyarthralgia, and lymphadenopathy; it does NOT represent a TRUE drug allergy

199
Q

rheumatic fever

A

after strep pharyngitis if no Tx w/ Abx; Sx = polyarthritis, carditis, erythema marginatum, subcutaneous nodules, chorea

200
Q

Tx of minimal change disease

A

prednisone

201
Q

IgA deficiency

A

recurrent sinupulmonary and GI infections AND anaphylactic reaction; measure and look for low IgA and normal IgM and IgG

202
Q

TCA toxicity

A

dry mucosa, dilated pupils, wide QRS; Tx = sodium bicarb for severe cases to narrow QRS; benzos for seizures; Physostigmine for mild anticholinergic intoxication`

203
Q

sickle cell dz + hip pain + slow onset over weeks

A

aseptic necrosis; also in other hemoglobinopathies;

204
Q

complications of kawasaki dz

A

coronary artery aneurysms, myocardial infarction

205
Q

pediatric myocarditis

A

fever, lethargy, heart failure, viral prodrome; usually Cocksackie B or adenovrius; get a CXR, EKG, echo, and Bx; Tx = supportive with diuretics + inotropes

206
Q

causes of niacin deficiency

A

alcohoics, long term users of INH, carcinoid syndrome, Hartnup disease

207
Q

long term complications of bacterial meningitis

A

hearing loss, loss of cognitive functions, seizures, mental retardation, spasticity/paresis

208
Q

aplastic anemia - acquired causes

A

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

209
Q

aplastic anemia - congential causes

A

Fanconi anemia (most common cause)

210
Q

Fanconi anemia

A

aplastic anemia, progressive bone marrow failure, short stature, microcephaly, abnormal thumbs, hypogonadism, macrocytic anemia, skin changes, and eye/ear problems (strabismus, low-set ears, middle ear abnromalities)

211
Q

RTA Type I, II, IV

A

1 = defect in hydorgen ion secreiton in distal tubule –> hypokalemic acidosis with inc. urinary pH, type 2 = decreased bicarb resorption and problem in prox tubule, Fanconi syndrome is a common cause; type 4 = defect in Na/K pump –> hyperK, hyperCl acidosis

212
Q

manifestations of infant heart failure

A

diaphoresis w/ feeds, poor appetitie, lethargy, hyperactive precordium; loud S2, sys ejc murmur across pulm valve, holosys of VSD;

213
Q

Key findings in Down Syndrome

A

upslanting palpebral fissures, epicanthal folds, brushfield spots (white spots on irides), single transverse palmar crease, “sandal” gap toes

214
Q

Comorbidities of Down Syndrome

A

cardiac: complete AV canal, VSD, ASD; GI: duoedenal satresia, Hirschsprung; Neuro: MR, early Alzheimers; Heme: acute leukemia; Endocrine: hypothyroidism, type 1 DM; MSK: Alantoaxial instability

215
Q

most common casues of bacterial meningitis in children > 1 month old

A

strep pneumo and N.meningitidis

216
Q

indications for imaging PRIOR to LP in infants/children

A

Hx of hydrocephalus, Hx head trauma, Hx VP shunt, Comatose patient, focal neuro findings

217
Q

Abx coverage for bacterial meningitis in neonates versus those older than 1 month

A

28 days = Ceftriaxone and Vancomycin (for S. pneumo and N. menigitidis)

218
Q

most common manifestation of late untreated Lyme

A

lyme arthritis;

219
Q

osteogenesis imperfecta

A

blue sclerae, hearing loss, recurrent bone fractures, opalescent teeth

220
Q

laryngomalacia

A

inspiratory strider; worsens in supine position; peaks at 4-8 months; Dx with laryngoscopy; resolves by 18 months of age

221
Q

% of down’s that have duodenal atresia and % of duodenal atresia that have downs;

A

2.5% of down’s have DA; 25% of DA have Down’s

222
Q

more than 50% of patients with duodenal atresia have…

A

polyhydramnios

223
Q

intraperitoneal calcification

A

suggests prenatal perforation of gut and meconium periotonitus, likely meconium ileus

224
Q

meglumine diatrizoate

A

gastrographin

225
Q

3 y.o. girl - 3 weeks fever, pallor, decreased appetite; taking acetaminophen; bruises on legs over past week; fever, pulse 120; rr 24; ecchymoses; oral ulcers; no lmyphadenoapthy; Hgb 4.5; leukocytes 300 (99% lymphocytes); platelets 2k; most likely Dx: aplastic anemia, Chediak higashi, abuse, CML, cong agranulocytosis, cyclic neutropenia, drug induced neutro, Leukocyte adh def, viral-med neutro

A

aplastic anemia

226
Q

16 y.o. girl; LMP 18 months ago; not sexually active; 5ft 5 in; BMI 17; At risk for: endometrial cancer, hypothyroidism, osteoporosis, PCOS, rheum arth

A

osteoporosis

227
Q

13 mo girl adopted from china; 10% for length, weight, head; Hep A antibody postive; Hep B surface antigen positive and antibody negative; what is immune status

A

Hep A immune and hep b NOT immune

228
Q

8 mo girl diarrhea for 1 month; wt loss, multiple thrush episodes, hospitalized for parainfluenza virus; 5th% weight; thick white plaques on buccal mucosa; harsh 3/6 systolic murmur; what kind of deficiency: mucosal Ig, NADPH oxidase, segmented PMNs, serum complement, T lymphocytes

A

t lymphocytes

229
Q

4 y.o. girl; intermittentn muscle cramps x 3 mo; mild dev motor delay; father also has muscle cramping; difficulty relaxing grip after squeezing doc’s fingers; impaired enunciation; likely dx: cerebral palsy, congenital muscular dystrophy, duchenne’s, myasthenia gravis, myotonic dystrophy

A

answer: myotonic dystrophy (hand relaxing problems)

230
Q

9 month boy; can only sit with support; two blocsk together but no scribble; says mama and dada; dev assessment?

A

delayed motor; normal fine motor; normal language

231
Q

3 y.o. boy w/ anorexia, apathy, irritiability, poor coordiantion, sporadic vomiting; hypochromic microcytic anemia

A

Lead poisoning (anemia + neuro signs) -

232
Q

2 y.o. boy w/ 5 days of fever (40 C). pulse 130; bp 90/60; 3/6 systolic on LLSB; splenomegaly; they are getting echo; what to confirm dx: ANA, ESR, serum cardiac enzymes, blood cultures, EKG?

A

blood cultures

233
Q

2 y.o. with 30 min of severe respiratory distress w/ retractions; 5 day history of fever/cough; FTT since 5 months; frequent thrush, recurrent diarrhea; 5th percentile height/weight; fever, pulse 125, RR 65; o2 sat 82%; diffuse crackles; hepatosplenomegaly; CXR shows diffuse interstitial infiltrates; what is next step in Dx: sputum Cx, blood Cx, serologic VDRL, silver stain of bronchoalveolar fluid; CT chest

A

silver stain of bronchoalvelar fluid (for fungi) - kid has HIV and PCP pneumonia - walking leads to dsats

234
Q

14 y.o. girl in ED 4 hours after 3 min gen tonic-clonic; began w/ bizarre behavior; low grade fever/cough x 3 days; on arrival, confused, unable to follow commands; fever 102; mild resistance to neck flexion; brisk DTRs; + babinski; CT head = mixed areas hyper and hypodens in righ temporal lobe; CSF fluid w/ 80% lymps; ESR 1250; causative agent? arrbovirus, borrelia, CMV, entero, HSV, HIV, listeria, rickettsia, toxo, VZV

A

HSV

235
Q

12 y.o. boy with loud snoring; hx of URI and steatorrhea; what explains new Sx? branchial clef cyst, deviated septum, nasal polyps, respiratory muscle weakness, tonsillar hypertrophy?

A

nasal polyps

236
Q

13 y.o. girl with 6 weeks of tired/irritable; school is “overwhelming”; trouble concentrating; lost interest in friends; wt loss; what is dx: MDD, dysthymia, adjustment disorder, substance abuse

A

MDD

237
Q

16 y.o. with T1DM who is 1 day post op; got 3.5 L of LR; on cefepime, morphine, insulin; afeb and VSS; Na 128, K 3.9, Cl 96, Hco3 22, BUN 14, glucose 642, Cr 0.9; likely cause of low sodium? ATN, anemia, CGD, excessive H2O, hyperglycemia, sepsis

A

hyperglycemia

238
Q

17 y.o. girl found lying on the street; temp outside 40 F; EKG showed J-wave pattern; lethargic; poorly responsive to verbal commands; temp is 89 F; pulse 60; rr 12; bp 90/60; odor of ethanol on breath; explanation of cardiac findings: cocaine, ethanol, hyperK, hypoCa, hypothermia, inc. ICP, MI?

A

hypothermia

239
Q

11 y.o. with 14 day hx fever, headache, yellow-green nasal discharge; noctural cough; temp 39, pulse 100; rr 18; posterior pharygneal wall is erythematous and covered with thin gray mucus

A

sinusitis

240
Q

5 y.o. boy w/ 2-day hx fever, cough, abdominal pain; No N/V; temp 39.2; RR 32; o-sat 92%; decreaesd breath sounds at right lung base; has abomdinal tenderness without guarding/rebound

A

bacterial pneumonia

241
Q

16 y.o. boy w/ 3 days pain/pressure left cheek; Hx of s. pneumo at 6 and 10; two episodes sinusitis over past 2 years; fever 38.1; bilateral tender maxillary sinuses and boggy turbinates; sputum grows H. influenzae; most likely cause of patients’ recurrent infections: combind immunodef, complement def, impaired cell mediated immunity, impaired chemotax, impaired humoral immu

A

impaired humora immunity (right answer!)

242
Q

12 hour old girl w/ irratibility, difficult breathing, 2 min gen ton-clon seiz; born at 36w by CS for breech; poorly controlled DM in mom; what should you measure (in addition to glucose): bicarb, bili, Ca, Mg, TSH

A

Calcium

243
Q

3 y.o. boy to ED for rapid breathing x 1 hour; afeb; R 30; clear lungs; hyperreson on right chest; dec breath sounds on R; overexpansion of R lung on CXR: what next? hyperbaric O2, CT chest, bronchoscopy, thoracotomy, tube thoracostomy

A

bronchoscopy for foreign body

244
Q

13 y.o. girl w/ 6 weeks constant abdominal pressure, breast tenderness, wt gain; no menstrual period yet; Tanner 3 breast/pubic; exam shows nontender mass below umbilicus; next step in Dx? U/A, serum AFP, b-hCG, xray of abdomen, CT pelvis

A

b-hCG

245
Q

16 y.o. boy w/ painless mass in scrotum; mass can be “milked” into abdomen through inguinal ring: epidymitis, hydrocele, inguinal hernia, leydig cell tumor, teratoma, torsion, varicocele

A

inguinal hernia (right answer)

246
Q

6 month old boy w/ 1 day fever/foul smelling urine; UA = 50 WBC, pos nitrities, postive LE; e. coli; renal U/S shows no abnromalities; next step? obs, repeat UA in 3 months, IVP, voiding CUR, cystoscopy

A

voiding cystourethrography (for BOYS!)

247
Q

14 y.o. girl w/ Downs with polycythemia; HgB 16.8; mild-mod cyanosis + digital clubbing; loud S2; no murmurs; echo shows large VSD and dilated main pulmonary artery; likely cause of polycythemia? tamponade, dec EPO, iron def anemia, pulm artery HTN, tetrology of fallot

A

pulm art HTN

248
Q

3 y.o. girl with progressive FTT; large greasy stools and farts; did not pass meconium until 72 hours; 5th % weight/heigh; dec. soft tissue mass on exam

A

exocrine panc insufficiency (CF?)

249
Q

16 y.o. boy w/ fam history of premature CAD, HTN, HLD; 5 ft 8 in, 106kg, BMI 35; BP 136/84; total chol 214 (HDL 32 LDL 144), triglyc 187; next step? reduced calorie diet, wt training, B-blocker, chol binding resin, statin?

A

diet

250
Q

14 y.o. girl w/ monthly lower abomdinal pain during past 6 months; normal BMI; tanner 4 breast/pubic; suprapubic tenderness; bluish red smooth bulge between labia; next step? obs, hysteroscopy, lap, incision of hymen, ex laparotomy14 y.o. girl w/ monthly lower abomdinal pain during past 6 months; normal BMI; tanner 4 breast/pubic; suprapubic tenderness; bluish red smooth bulge between labia; next step? obs, hysteroscopy, lap, incision of hymen, ex laparotomy14 y.o. girl w/ monthly lower abomdinal pain during past 6 months; normal BMI; tanner 4 breast/pubic; suprapubic tenderness; bluish red smooth bulge between labia; next step? obs, hysteroscopy, lap, incision of hymen, ex laparotomy14 y.o. girl w/ monthly lower abomdinal pain during past 6 months; normal BMI; tanner 4 breast/pubic; suprapubic tenderness; bluish red smooth bulge between labia; next step? obs, hysteroscopy, lap, incision of hymen, ex laparotomy14 y.o. girl w/ monthly lower abomdinal pain during past 6 months; normal BMI; tanner 4 breast/pubic; suprapubic tenderness; bluish red smooth bulge between labia; next step? obs, hysteroscopy, lap, incision of hymen, ex laparotomy14 y.o. girl w/ monthly lower abomdinal pain during past 6 months; normal BMI; tanner 4 breast/pubic; suprapubic tenderness; bluish red smooth bulge between labia; next step? obs, hysteroscopy, lap, incision of hymen, ex laparotomy

A

obs

251
Q

3 y.o. girl with syncope after GTC seizure (30 sec); has 2nd seziure (focal) in ED; no QRS complexes; then normal sinus resumes in 20 seconds; then 1 minute later she is fully alert: absence, adams stokes, med effect, breath holding, nacrolesy-cataplexy, carotid artery trauama, vasovagal syncope, ventricular tachyarrhythmia

A

adam-stokes attack

252
Q

2 y.o. girl in ED for looking ill and breathing rapidly; open aspirin in play area (unclear how many tablets missing); it’s been 20 minutes; HR 130, RR 46; what is ABG going to look like?

A

mixed met acid and resp alk (though not sure how long it takes for met acidosis to set in…)

253
Q

5 year old with an xray that shows fracture in humerus: what is cause of pain? acromioclav separation, clav fx, glenohumeral dislocation, humerus fx, unicameral bone cyst

A

humerus fx

254
Q

12 y.o. girl w/ headache and change in mental status; newly Dx T1DM and admitted for DKA; got 3 NS boluses in past 2 hours; glucose down to 400; HR 56, fundoscopic exam shows absence of venous pulsations; cause of altered state? cerebral edema, hypoglyc, hypovol, met acid, persistent hyperglyc

A

cerebral edema

255
Q

16 y.o. boy with 2 months of painless lump in right breast; 1cm smooth, firm mass under right nipple; no nipple or skin retraction, no axillary lymphadenopathy; what is it? accessory breast tissue, carcinoma, cystic mastopathy, fibroadenoma, physio pub dev

A

physio pubertal dev

256
Q

17 y.o. girl w/ 3 years smoking, 1 oz EtOH weekly, mom w/ breast cancer, dad and granddad died of heart disease in 30s. most appropriate screening test? serum lipids, U/A, mammography, xray, exercise stress test

A

serum lipid studies

257
Q

2 day old newborn w/ decreased UOP; 6cm round, midline, suprapubic pelvic mass palpated; Na 137, Cl 102, K 4.2, Hco3 21, Cr 0.5; Urine pH 6, spec grav 1.009, trace protein; reaso for oliguria? chronic glomerulonephritis, phimosis, pos urethral valves, vesicoureteral reflux, UTI

A

post uretrhal valves

258
Q

2 mo old boy w/ 3 days vomiting; drinks cow milk but vomits after each feeding; increasing amount in force; Nonbloody and non bilious; appears dehydrated; Na 130, Cl 85, HCo3 34

A

pyloric stenosis

259
Q

12 month old african american girl w/ SCD with high fever, irritability, tachypnea of 6 hours; misses some PCN prophylaxis doses; fever 102.2, HR 180, RR 48; conjunctival pallor; 2/6 syst murmur; Hct 23%, WBC 23; initial step in mgmt? oral PCN, IV cefotaxmine, IV steroids, IVIG, IV naf, IV PCN

A

iv PCN (right answer?)

260
Q

16 y.o. with facial rash. started on chin and spread over past 2 weeks; Dx? allergic contact derm, herpes simplex, impetigo contagiosa, molluscum contagiosum, tinea faciei

A

impetigo contagiosa

261
Q

4 y.o. girl w/ fever, abdominal cramps, diarrhea for 2 days; attends day care and other kids are sick; hx of picnic w/ chicken and salads (all types); WBC 10.4 with 60 L and 33 N and 7% bands; stool cutlure shows Salmonella; what public health measure prevents infection?

A

cooking meats (since believed to be salmonella from poorly cooked chicken)

262
Q

18 month old w/ diarrhea x 3 days; other day care kids have it; water source is private well; center has turtles and birds; WBC 8.4 w/ 60 L 39 N; RSV positive

A

strict hand washing

263
Q

16 y.o. boy w/ fever and right foot pain; 1 week itching rash b/w 2nd and 3rd toes on both feet; fever 101.1, induration from interdigital spaces to medial malleolus; causitive agent? e. coli, mTB, pseudomonas, s. aurues, trichophyton rubrum

A

s. aureus

264
Q

5 y.o. boy w/ 3 weeks fatigue; acute onset fever/chills 2 hours ago; travel to asia 1 month ago; got chloroquine prophylaxis; exam shows pallor and splenomegaly; Hct 22%; WBC 18k, platelets 80k; next step in Dx? ASO, heterophile Ab, PT and PTT, AST/ALT, thick and thin blood smears

A

smears

265
Q

8 y.o. with split S1 that widens during inspiration; sinus bradycardia; low-pitched vibratory murmur throughout cardiac cycle best at LUSB when child is sitting and murmur disappears when child in supine and when neck is rotated in sitting position; ASD, coarc, PDA, periph pulmonic stenosis, venous hum

A

venous hum

266
Q

1 week newborn w/ 1 day difficulty breathing and discoloration of extremities; afebrile; HR 160, RR 52; BP 60/36 in uppers and unobtainable in lowers; skin/membranes appear dusky, mottled discoloration; 3/6 holosystolic murmur LSB; hepatomegaly; ABG 7.15/28/98; no improvement w/ intubation, IV fluids; CXR shows cardiomeg and pulm congestion; what explains? closure of ductus arteriosus, dec pulm vasc resist, inc pulm vasc resist, intracardiac L to R shunting, opening of ductus

A

closure of ductus

267
Q

4 day old female w/ cephalohematoma and yellow skin; newborn is O+ and mom is A+; Coombs test was negative; serum bili 20.8; next step? stop breast feeding, supplement with formula, get another bili in 6 hours, phototherapy, iv bolus, partial exchange transfusion, no intervention

A

phototherapy: is 20.8 lightable at 96 hours of life?

268
Q

2410g newborn at 37w; mom hx of IV drug use no prenatal care; rapid HIV is positive; next step for kid?

A

oral AZT within 12 hours for 6 weeks

269
Q

30 month old girl w/ abdominal pain, loose stools, temp 38.3; malaise/fever; loose BLOODY stools; at night has GTC seizure; temp 103.5, dec skin turger; dx? HUS, HSP, idiopathic seizures, shigellosis, toxic megacolon

A

answer - shigella

270
Q

newborn girl w/ “clunk” when hip is abducted, flexed and lifted forward; what is mechanism of disease?

A

shallow poorly developed acetabulum

271
Q

4 y.o. with neutropenia and has been treated w/ amox, cefotaxime, and TMP-SMX. what is cause of neutropenia?

A

TMP-SMX

272
Q

14 y.o. girl w/ enlarged neck over past 5 months; T4 3 ug/dL; TSH of 15 uU/mL; patchy irregular uptake on throid; biopsy will show? EBV DNA, granulomas, lymphocytic infiltration, medullary carcinoma, microabscesses?

A

lymphocytic infiltration

273
Q

6 month old boy w/ 1 week unprovoked startle-like movements; sudden quick flexion of head, arms, legs; occur in clusters; awaken from sleep; had heart murmur since birth; 3 areas of hypopigmentation; periventricular nodules; what is Dx? duchennes, myotonic dystrophy, NF, tuberous sclerosis, facioscapulohumeral musc dys, hepatolenticular degeneration

A

TS

274
Q

5 year old boy w/ painful limp x 3 weeks; no illness/trauam; flexion/int rotation on R hip are decreased; xray shows dense, contracted right femoral cpital epiphysis; Dx = congenital hip dysplasia, diasteomatomyelia, femoral anteversion, fibular hemimelia, jumper’s knee, LCP dz, metatarsus adductus, osgood-schlatter, osteosarc, prox focal fem def, septic arthritis, SCFE, spondylolisthesis L4 on L5, tibial hemimelia

A

Legg-calve-perthes dz - answer

275
Q

asymptomatic 2 y.o. girl after 2 month visit with g-father who has active TB; PPD is 25mm at 72 hours; normal CXR; what is mgmt? BCG, INH only, rifampinonly, INH + Rif + etham, INH + rif + Pyrazin + streptomycin?

A

INH only

276
Q

5 month old girl w/ 1 day fever; ear tugging; nasal congestion; 38.3 C, HR 124, 26/min; bulging, diffusely erythema left TM; no movement with pneumatic otoscopy; Dx = AOM, bullous myringitis, cholestatoma, otitis externa, otitis media w/ effusion?

A

acute otitis media

277
Q

18 hour old female w/ jaundice; mother is O+; newborns urine is dark yellow; difficulty latching; jaundice down to chest; TBili 11.1 w/ Dbili 0.1; likely Dx? breast milk jaundice, galactosemia, hemolytic disease of newborn, physiologic jaundice, SCD?

A

hemolytic disease of newborn

278
Q

14 y.o. girl without no Hx of menstrual period; first pubic/axillary then thelarche at 12; height is like an 8 year old; BP 140/100; tanner 2 breast; pubic tanner 4; weak fem pulses; (she has turners?); next step? Karyotype, insulin level, prolactin level, xray, eeg?

A

karyotype

279
Q

8 y.o. boy w/ 9 months nonproductive cough worse at night and SOB w/ activity; NOT had unusual number of colds but colds seem to linger in chest; CXR shows mild hyperinflation; Next step in Dx? sweat chloride, IgA level, IgE level, allergy testing, spirometry

A

spirometry

280
Q

4 month old girl; preterm 27 weeks; birth weight 1200 grams; 3 month NICU; on O2 and diuretics at home; 5th% length/weight; afeb, pulse 104, RR 32, BP 115/67 in right arm and 105/67 in left leg; pronounced S2 and pericardial heave and hepatomegaly; RVH on EKG; normal kidneys; most likely cause of increased BP? bronchopulm dysplasia, coarctation of aorta, essentialHTN, pheo, renal art thromb

A

bronchopulm dysplasia

281
Q

3 week old boy w/ yellowing of skin over past 6 days; stools have become lighter in color over past 3 days; formula fed since birth; Tbili 14; direct 6.0; likely mechanism for jaundice? decreased conjugation, decreased excretion, hepatic enzyme def, increased enterohepatic circ, increased production

A

decreased excretion

282
Q

2 y.o. girl w/ 2 week history of irritability, low appetite, cough, reluctance to walk; 2kg weight loss over past 6 months; no history of illness, constipation, vomiting, diarrhea; 75th% height; 25th% weight; 100.2 F, pulse 140, RR 24, BP 145/100; bluish discoloration under both eyelids; Hgb10.5, WBC 8.3k (55% lymphocytes), Plt 240k; mass in posterior mediastinum on CXR: DDx? anthrax, cong heart dz, CF, dermatomyositis, neuroblastoma, pulm sequestration, thymoma, TB

A

neuroblastoma

283
Q

7 month boy presents 35 min after seizure started; jerking started left arm then progressed to right arm and both legs; no PMHx; 25th% length; 10th% weight; cyanotic (doesn’t improve on his side); temp 103.5, pulse 160, RR 30, BP 90/60; upward dev of eyes; rigid, hyperextended neck, back; clonic jerking; O2 given; next step? acetaminophen, diazepam, glucose, naloxone, Vit B1

A

Diazepam

284
Q

36 hour old boy w/ jaundice 12 hours after birth; on phototherapy last 24 hours; mom is O+ and newborn is A+; jaundice below knee; spleen 1cm below costal margin; Hct is dropping and Tbili is rising; next step? obs, more phototherapy, exchange transfusion, IV fluids, splenectomy?

A

exchange transfusion

285
Q

newborn male 3400 grams with decreased muscle tone; afeb, HR 150, RR 60; O2sat 92%; epicanthal folds, white stellate spots on irises, single palmar crease, sandal gap, truncal hypotonia; next step? measure CK and MRI brain, CXR and BCx, ECG and chrom analysis, alprostadil (prostaglandin E1) and O2, surfactant + IV amp/gent

A

ekg and chrom analysis

286
Q

14 y.o. boy w/ 1 year hyperactivity and learning problems; cannot concentrate per teacher and mom; GTC seizure at 8 years; 9 lesions w/ coffee-stain-like apperance on chest/abdomen; increased pigmentation in axillae and small skin tags; likely Dx? herediatry hemorrhagic telangiectasia, NF type 1, sturge-weber, tuberous sclerosis, von hippel dz

A

NF

287
Q

3 weeks post cellulitis, 11 y.o. girl has blood urine w/ RBC casts; BP is increased; pedal edema is present; ASO titer is 500 (normal

A

Recovery?

288
Q

22 month old w/ bowed legs; drinks goat milk-based formula and table food; family lives in old farmhouse w/ peeling paint; most likely mechanism of deformity (rickets on xray) is: degenerative, infectious, metabolic, neoplastic, toxic

A

metabolic

289
Q

2 y.o. boy w/ 3 days fever and right elbow pain 1 day; had GBS meningitis at 5 months and septic arth from H. influ at 9 months; brother and 3 uncles died in infancy from septicemia; immunizations up to date; temp 40.3 C, red/swollen elbow; WBC 34k (60 N, 15% bands, 25 L); strep pneumo postive blood culture; cause of these findings is def of: b-lymphocyte function, complement function, segmented PMN adhesion, segmented PMN chemotaxis, T-lymphocyte function

A

x-linked agammaglobulinemia (B-lymphocytes)

290
Q

15 month old girl w/ 1 day rash; fever past 3 days; had MMR vaccine 10 days ago; temp 38.4 C; erythematous maculopapular rash over face, trunk, extremities; most likely explanation? arthurs reaction to pre-existing Ab to rubella virus, delayed-type HSN rxn to rubella antigen, immune complex dz from vaccine preservatives, replication of live vaccine virus strain; viral dissemination in immunocompromised host?

A

replication of live vaccine virus strain

291
Q

9 month old boy w/ 40 C temp, fussy, dec oral intake over past 5 days; no sick contacts; Temp 103.3; fissured lips, red oral mucosa and conjunctiva, single enlarged cervical lymph node; maculopapular rash over trunk, extremities and dorsal edema of hands; Hct 32, WBC 15k, Plt 550k; Next step mgmt? acetaminophen, IV Abx, IVIG, IM ceftriaxone, oral Abx, oral steroids, CXR?

A

I think this is Kawasaki’s so give IVIG (and aspirin if it was a choice)

292
Q

3 y.o. boy w/ fever, sore throat, malaise, poor appetite for 2 days; no V/D/rhinorrhea; temp 101.7, no abnormalities of tympanic membranes or pharynx; WBC 9.5k; next step mgmt? acetaminophen, IV Abx, IVIG, IM ceftriaxone, oral Abx, oral steroids, CXR?

A

thinking viral illness given time course and absence of findings; so give Tylenol

293
Q

3 month old boy w/ 2 days fever; temp 100.6 F, fussy; spec grave 1.015, WBC 20-50, gram neg rods; UCx 100k E. coli; started Abx; next step in Dx? IVP, renal digitatl subtraction angiography, renal U/S, CT abdomen, renal dimercaptosuccinic acid scan?

A

renal U/S

294
Q

2 y.o. boy presents 2 hours after acute onset fever/dif breathing; had rhinorrhea past 24 hours; noisy breath when inhales; improved when he went outside; temp 100.4, pulse 120, RR 30; supraclav retractions, inspiratory stridor; likely mechanism? alveolar atelectasis, edema of epiglottis, narrowing of mod sized airways, pulm parenchymal inflammation, subglottic edema

A

subglottic edema

295
Q

7 y.o. girl w/ 7 days bloody diarrhea; fatigue x 2 days; temp 98.6, palor and scleral icterus; liver is NOT enlarged; Hgb 6, MCV 80, WBC 18k, Retic 12, Plt 50k; most likely DDx: ALL, bone marrow suppression, G6PD def, Hgb SS, HUS, iron def, thalassemia

A

hemolytic uremic syndrome (bloody diarrhea from campylobacter jejuni)

296
Q

11 y.o. girl w/ early/rapid onset sexual development; thelarche age 9, pubic hair 9.5 yrs; menarche 2 days ago; tanner 4; most likely Dx: adrenocortical tumor, idiopathic precocious puberty, pituitary tumor, true precocious puberty, normal development

A

normal development

297
Q

10 month old girl w/ recent lazy eye; eye shows hyphema and esotropia; conjunctiva are NOT injected; no discharge; pupil appears WHITE on reflex testing; cause of findings? congenital cataract, glaucoma, retinoblastoma, retinopathy of prematurity, retinitis pigmentosa

A

retinoblastoma

298
Q

6 wk girl w/ 2 wk persistent irratibility and stools w/ mucus and blood; no vomiting; switching 2 weeks ago from cow’s milk to soy milk formula; 25th% length, wt, head; wt unchanged from 2 weeks ago; next step? electrolyte rehydration for 24 hrs, formula w/ evaporated milk, H2O, corn syrup, formula w/ hydrolyzed casein, oral amox, oral ranitidine

A

hydrolyzed casein

299
Q

7 y.o. girl who fainted; progressively lethargic over past winter; complexion darkened; height/weight at 50th%; BP 80/40; best lab test? plasma cortisol, serum glucose, serum gonadotropin, serum PTH, serum TSH

A

Addison’s disease in which case “cortisol” should be the answer

300
Q

14 y.o. boy w/ 3 months draggin sensation of left scrotum; had left scrotal hydrocele during infancy that resolved; both testes descended; when standing, left scrotum hangs lower than right; feels like “bag of worms.” at risk for: mets, incarceration, infertility, testicular carcinoma, torsion, torsion of appendix testis?

A

infertility (varicocele)

301
Q

12 y.o. boy; shortest in class; father 6 ft 1 in, mom is 5 ft 6; father was ALSO the shortest until high school; patient is 5th% hieght; growth velocity within normal limits; Tanner stage 1; what confirms Dx? determination of bone age, serum cortisol, serum GH, thyroid function studies, MRI brain

A

bone age determination (constitutional delay)

302
Q

8 y.o. girl w/ pallor and fatigue x 2 months; began after URI; adopted and FHx unknown; spleen tip 2cm below costal margin; Hct 28%, retic 4% w/ 3+ spherocytes; splenectomy is likely to prevent? cholelithasis, esophageal varices, sepsis, pain crises, pancreatitis

A

cholelithiasis

303
Q

9 y.o. girl w/ acute appy; neigh offers to sign consent since parents cannot be contacted; what to do? perform operation b/c it is emergency and consent is not required, perform b/c an adult has given consent, perform if child consents, delay operation until parent contacted while closely monitoring child’s status

A

do not need parental consent for emergencies

304
Q

3 y.o. girl w/ 2 month Hx right-sided limp; most obvious when awakens and less noticeable throughout day; appears well but has limp; no rash; right knee swoellen and warm, no erythema or tenderness; holds R lower ext in flexed and will not extend; no rash; ESR 64, ANA pos; neg RF; neg ASO; neg Lyme; in addition to naproxen, what else needed? slit lamp, bartonella henselae titer, upper GI, arthroscopy, surgical aspiration of right knee?

A

hmmm

305
Q

16 y.o girl from Africa; painless lesion on vulva for 4 days; sexually active, no contraception; 10mm nontender sharply demarcated elevated round lesion on right labium majur; base is smooth and nonpurulent; likely causal organism? chylamydia trach, gardnerella, H. ducreyi, HSV, HPV, N. gon, Trep pal, trich?

A

treponema pallidum

306
Q

newborn 1 hour after delivery; in resp distress; 28 weeker; initially baby pink and active; temp 36.4, pulse 160, RR 60 and shallow; BP 68/44; grunting, nasal flaring, retractions; CXR graunlar appearance of parenchyma w/ air bronchograms; likely Dx? meconium asp PNA, RDS of newborn, GBS infection, TAPVR, transient tachypnea of newborn

A

answer is RDS of newborn

307
Q

1 day old 2460 gram w/ abdominal distention; bilious vomiting x 2; no meconium yet; xray shows several dilated loops w/ air-fluid levels; dilated proximal to the mid-sigmoid colon; after enema has explosive BM; what causes this in utero? abnormal caudal migration of ganglion cells, abnormal pancreatic/intestianl gland secretion, failure of normal bowle rotation, patency of processus vaginalis, vascular accident of bowel

A

abnormal caudal migration of ganglion cells

308
Q

1 month old boy; 2 hours of bilious vomiting; last BM 1 day ago; temp 100.4; RR 60, pulse 180; BP 70/40; abdomen firm/distended; decreased sounds; normal rectal tone; occult blood is positive; DDx: hirschsprungs, gastroenteritis, pyloric stenosis, intussusception, midgut volvulus

A

midgut volvulus

309
Q

14 y.o. girl w/ progressive tiredness; T1DM dx 4 years ago; A1c is 6; last 3 months menses have been longer and heavier flow; LMP 4 weeks ago; tanner 4; thyroid gland easily palpable; in addition to T1DM, what else does she have? addisons, hypothyroidism, pituitary adenoma, PCOS, sjogren’s

A

hypothyroidism

310
Q

9 y.o. girl w/ bowing right leg; been bowed since she started to walk and gotten worse; 95th% for weight, 50th% ehight; Xrays show collapse of medial aspect of metaphysis of proximal tibia; likely Dx? osteogenesis imperfecta, osteomyelitis, rickets, physiologic bowleg deformity, Tibia vara

A

tibia vara

311
Q

15 y.o. boy w/ 5 years of T1DM; a1c 6.5; how to decrease risk of DM-complications during basketball? decreae insulin by 10-15% on practice days, limit exercise to 30 min at a time, measure urine glucose every 30 min; only noncontact sports; switch to short acting insulin only

A

decrease insulin dose by 10-15%

312
Q

18 month boy w/ 4 hours lethargy; intermittent abd pain x 24 hours; no D/V; mass palpated in RLQ; dec bowel sounds; red stool and occult blood positive; xray shows no air in ascending or transverse colon; next step? air contast enema, upper GI, Meckel scan, upper endoscopy, laparatomy

A

air contrast enema (should be intussusception)

313
Q

6 month girl w/ hemangioma on neck enlarging since 6 weeks; raised erythematous and blanches w/ pressure, no ulceration; next step? obs, intralseional antiangiogenic factor, intralesional interferon, oral steroids, lasers therapy

A

observation (strawberry hemangioma)

314
Q

10 y.o. girl with GTC seizure; 1 month Hx behaivor changes; 2 weeks fever, weakness, painful swollen left knee; Hgb 9; WBC 3.8, Plt 65; Coombs positive; U/A has protein and microscopic blood; Dx is: Hodgkins, HSP, Mono, SLE, viral encephalitis

A

drug induced SLE from phenytoin (hydralazine, INH, phenytoin, procainamide)

315
Q

30 month girl w/ firm mass left flank; no other Sx; pale;U/A has 20-50 RBCs; Dx: ADPKD, multicystic dysplastic kidney, nephroblastoma (Wilms), neuroblastoma, RCC

A

Wilms

316
Q

28 month old boy; 20 min after found crying, drooping, open container drain cleaner; temp 100.4, pulse 124, RR 40; 1 to 2cm erythematous blistrs on lips and tongue; stridor and retractions; next step (after airway stabilization)? Lat xray of neck, EKG, CT head neck, esophagography, fiberoptic endoscopy

A

endoscopy

317
Q

15 y.o. w/ 2 weeks with multiple areas that are lighter than rest of skin; mild itching when playing sports; flat, oval, hypopigmented lesions; likely Dx? actinic keratosis, atopic derm, contact derm, discoud lupus erythematosus, impetigo, rosacea, seborrheic derm, tinea corporis, tinea versicolor, urticaria, vitiligo

A

Tinea versicolor (malassesia furfur)

318
Q

6 wk girl w/ murmur since 2 weeks; 25th% length; 5th% weight; grade 4/6 holosystolic along LLSB; P2 acentuated; 2/6 mid diastolic murmur at apex; liver edge 3cm below costal margin

A

large VSD can have this presentation

319
Q

female newborn w/ cyanosis and cardiac murmur; 4 hours of life; heart sounds best heard on RIGHT; xray shows dextrocardiac; echo shows regurgitant atrioventricular valve; blood smear shows howell-jowel bodies; most appropriate prophylaxis for this newborn? aspirin, factor VIII, iron, penicillin, warfarin

A

penicillin

320
Q

26 y.o. woman with 1800 gram newborn at 42 weeks; fetal grwoth restriction in 3rd tri; no smoking, drugs, or pets; exam shows hepatosplenomeg; xrays shows periventricular intracranial calcifications; CMV, HSV, parvo, rubella, syphilis

A

CMV

321
Q

7 y.o. boy w/ 6 hours severe lower abd pain and nausea; no vomiting; no F/D/const; no trauma; temp 100.2; pulse 125, RR 20; exam is soft, nontender abdomen; edema and erythema of right scrotal sac (appears full); palpation of right testis prouces pain; absent cremasteric reflex; likely Dx? appy, epididymitis, hydrocele, incarc inguinal hernia, torsion

A

torsion

322
Q

3 y.o. boy w/ 4 days lethargy and severe vomting/diarrhea; no UOP past 24 hours; 75th% height, 35th% weight; lost 1 pound since last visit; temp 101.8, pusle 135, RR 30, BP 85/60; sunekn eyes, tacky oral mucosa, dry lips; no rash; Na145, Cl 105, K 4.5, CO2 12, BUN 40, Cr 1.3; Spec grav 1.035; cause of renal failure? bacterial toxin, immune complex nephroaphty, impaired renal perfusion, obstructive uropathy, renal vein thrombosis

A

imparied renal perfusion

323
Q

7 y.o. boy w/ 1 week low grade fever/fatigue; 3 days rash and moderate pain/swelling ankles; first appeared on ankesl then spread over legs; temp 38.2; palpable petechia and confluent purpuric areas over lower extremities; ankles are swollen and mildly tender; likely cause of rash: Ank spon, Behcet, dermato, HSP, Juv rheum arth, Kawsaki, psoriatic arthr, reactive arth, sarcoid, sjogren, SLE, scleroderm

A

HSP

324
Q

16 y.o. boy w/ 2 week pain, swelling, rash over right knee, 1 week mod pain/redness in both eyes; temp 100 F; injected conjunctivae w/ mucopurulent discharge; diffuse macular erythematous rash over lower extremtiies and sweeling of right knee; erythema/edema of urethral meatus

A

reactive arthritis