bs-pds-oo Flashcards
(324 cards)
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
2
Q
Epiglottitis
A
high fever, drooling, “thumb” sign (swollen epiglotis)
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)
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
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)
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
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
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)
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!
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
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
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))
13
Q
Riboflavin deficiency
A
sore throat, edematous oropharyngeal mucous membranes, cheilitis, stomatitis, normocytic normochromic anemia, seborrheic dermatitis, photophobia
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
15
Q
Hypervitaminosis A
A
excessive Vitamin A = anorexia, pruritis, hepatomeagly, alopecia, irritability, increased ICP, seborrheic cutaneous lesions
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
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
18
Q
erythema multiforme
A
acute, self limited reaction, commonly from herpes simplex; targetoid papule/plaque; arcofacial distribution
19
Q
Nikolsky sign
A
gentle lateral pressure on the skin surface adjacent to blister causes slipping and detachment of superficial layer of skin)
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;
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
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
23
Q
erysipelas
A
bacterial infection of dermis; well demarcated indurated warm tender plaque; fever, chills, malaise, group A strep; face and lower extremities
24
Q
foreign body aspiration
A
cough, focal monophasic wheezing, inspiratory stridor; most common 6 months to 4 years; acute onset
25
laryngomalacia
4-8 months; increased laxity of supraglottic structures --> collapse during inspiratory phase; stridor LOUDest in SUPINE position; improves with upright or prone
26
croup
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
27
vascular rings
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
28
Intraventricular hemorrhage is most commonly seen in what type of patietns
premature and low birth weight (inversely proportional to birth weight)
29
Most common helminthic infection in the U.S.
Enterobiasis; etiology = pinworm Enterobius vermicularis; children age 5-10; perianal noctural itching; "scotch-tape" test; Tx = albendazole/mebendazole
30
blue sclera
osteogenesis imperfecta; type 1 collagen defect; numerous fractures in multiple stages of healing; short stature, blue sclera
31
bleeding disorders in children (coagulation versus platelet)
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);
32
pathologic cardiac murmurs in children
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
33
Measles
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
34
Type I hypersensitivity
IgE-mediated; allergen binds and crosslinks two IgE molecules attached to mast cell; E.g. = atopy, urticaria, anaphylaxis
35
Type II hypersensitivity
Ab-mediated; E.g. = immune hemolytic anemia, Rh hemolyic dz of newborn
36
Type III hypersensitivity
immune-complex meidated; E.g. serum sickness, arthus reaction
37
Type IV hypersensitivity
delayed cell-mediated; E.g. Allergic contact dermatitis
38
Turner syndrome (most common cardiac defect)
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;
39
Most common and 2nd most common congenital cardiac defect in adults
bicuspid aortic vavle is MC; 2nd is ASD (ostium secundum is most common type)
40
most common congenital heart defect
VSD
41
trachoma
major cause of blindness worldwide; follicular conjuncitivtis and neovascularization in cornea; Dx giemsa stain; Tx = tetracycline or azithromycin
42
herpes simplex keratitis
pain, photophobia, decreased vision, dendritic ulcer; minute clear vesicles in corneal epithelium
43
orbital cellulitis
abrupt onset fever, proptosis, restrction of EOM, swollen, red eyelids
44
viral conjunctivitis
red, copious watery discharge, contaminate swimming pools; adenovirus type 3
45
choanal atresia
cyanosis that is worse w/ feeding and improves w/ crying; most common nasal malformation; Dx = CT w/ intranasal contrast
46
"tet" spells
cyansosis from stressful conditions; charactersitics of TOF;
47
Transposition of Great Arteries
no murmur on exam but cyanosis at birth and continuous
48
total anomalous pulmoanry venous connection (TAPVC)
right to left shunt at atria llevel w/ systolic ejection mumur in pulmonic area
49
truncus arteriosus
cyanosis is absent/moderate at birth (depeds on severity of pulmonary outflow obstruction) and murmur is ALWAYS present
50
Chronic granulomatous disease
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
51
Digeorge syndrome
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
52
most common primary immunodeficiency
agammaglobulinemia: poor B-cell maturation --> risk of infection from encapsulated bacteria (Hib, s. pneumo, neisseria meningitidis, GBS, klebsiella, salmonella)
53
Howell-Jolly bodies
single, round, blue inclusions in RBCs on Wright stain; often means splenectomy or hyposplenism
54
hemoglobin precipitation
G6PD deficiency; Hgb becomes oxidized and forms Heinz bodies (insoluable precipitants)
55
Developmental milestones by age and category (gross motor, fine motor, language, social/cognitive)
see question id 4199
56
Dx of acute alkali ingestion
upper GI endoscopy
57
osteogenesis imperfecta type 2
autosomal dominant; defectin type 1 collagen;; type 2 is most severe w/ intrauterine and perinatal fractures and restrictive lung disease
58
Fetal alcohol syndrome
craniofacial abnormalities, poor prenatal growth, microcephaly, hypotonia, and poor feeding
59
phenytoin use during pregnancy
craniofacial abnormalities, fingernail hypoplasia, growth deficiency, developmental delay, cardiac defects, facial clefts
60
vitamin K def in newborns is due to: (3 things)
poor placental transre, absent gut flora, inadequate levels in breast milk
61
Reye syndrome
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
62
Systemic carnitine deficiency
similar picture to Reye's: hypoglycemia, hyperammonemia, hypoprothrombinemia, acute episodes of encephalopathy; BUT there is also ELEVATED acyl-carnitine
63
Enuresis treatment
1st line = desmopressin; 2nd line = TCA (imipramine); side effects of desmo = hyponatremia; of TCA is cardiotoxicity
64
Legg-Calve-Perthes disease
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
65
Slipped capital femoral epiphysis
classically obese adolescents (mean age 12 in girls and 13 in boys), also have limp and insidious onset hip pain
66
Chlamydial conjunctivitis
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)
67
side effect of oral erythromycin in newborn
hypertrophic pyloric stenosis
68
Chlamydial pneumonia time course
age 4-12 weeks
69
How to prevent chlamydial conjunctivitis or PNA?
maternal testing in 1st and 3rd trimester (if age >25 or >25 w/ risk factors) and Abx treatment
70
Erythromycin eye ointment is effective against
GONOCOCCAL conjunctivitis; not for chlamydial
71
Lesch-Nyhan syndrome
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
72
prophylaxis for sickle cell disease
twice daily penicillin until age 5
73
NEC
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
74
what nutrional interventions in first 6 months of life?
Iron and Vitamin D supplementation. Iron stores will be low so give iron for 1 year; Vitamin D when breastfeeding for first 6 months
75
Transient synovitis
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
76
Septic arthritis
fever >101, inability to bear weight, WBC > 12k, ESR > 40, CP >20mg/L; if more than 3 of these, do arthrocentesis
77
angiofibroma
epistaxis, localized mass, bony erosion on back of nose
78
doxycycline is contraindicated in
pregnant women and kids under age 8
79
Tx of Lyme disease in young kid
oral amoxicillin (cannot use doxycycline)
80
Tx of disseminated Lyme disease
IV ceftriaxone (for meningitis and heart block)
81
characterize fetal alcohol syndrome
MR, hypoplastic maxilla, long philtrum, microcephaly
82
characterize Down's syndrome
MR, microcephaly, flat occiput, Simian crease, endocardial cushion defects, prominent tongue, epicanthal folds w/ slanting eyes, Brush field spots on periphery of iris
83
characterize Fragile X syndrome
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
84
atopic dermatitis in infancy
pruritis and skin lesions on face, scalp, chest, and extensor surfaces of extremities
85
indications for evaluation of neonatal jaundice
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
86
erythroblastosis fetalis
unconj hyperbili and positive Coombs' test
87
Crigler-Najjar
inhereited deficiency of UDP-glucuronyl transferase; lack of enzyme entirely. die within years
88
Gilbert's
mild def of UDP-glucuronyl transferase
89
most common benign vascular tumor in kids
strawberry hemangioma (grow rapidly within 1 to 2 years then resolve by age 8)
90
most common benign vascular proliferation in adults
cherry hemangiomas; they do NOT regress
91
PANDAS
pediatric autoimmune neuropsychiatric disorders: can get OCD after recent streptococcal infection
92
Tx for OCD
SSRI (fluoxetine) or CBT
93
midgut volvulus
child less than 1 month; bilious vomiting, abdominal distension, bloody stools
94
duodenal atresia
bilious vomiting a few hours after birth; a/w Down's
95
meconium ileus
a/w cystic fibrosis; failure to pass meconium within first 24 hours
96
Pertussis
Bordetella pertussis is highly contangious even with immunization so PREVENTION is key by giving ALL contacts a 14 day supply of erythromycin
97
Tx of impetigo
topical mupirocin or oral erythromycin; cause is usually GABS or S. aureus
98
contraindications to breast-feeding
mother: active untreated TB, HIV infection, herpetic breast lesions, varicella infection, maternal meds, chemo/rads, active substance abuse; Child: galactosemia
99
Best step for rehydration for moderate to severe
20mL/kg of normal saline IV
100
most common predisposing factor for acute bacterial sinusitis
Viral upper respiratory infection
101
Neonatal rashes (list 4)
erythema toxicum neonatorium, neonatal herpes simplex virus, neonatal varicella, staphylococcal scalded skin syndrome
102
Erythema toxicum neonatorium (ETN)
asymptomatic, scattered erythematous macules, papules, and pustules over body; No Tx needed
103
Neonatal herpes simplex virus
Vesicular lcusters on skin, eyes, mucous membranes; CNS infection, dissemianted organ dz; Tx = acyclovir
104
Neonatal varicella
fever, vesicular clusters and disseminated dz; Tx = acyclovir
105
Staph scaled skin syndrome (SSSS)
fever, irritability, diffuse erythema, blistering exfoliation, positive Nikolsky's sign; Tx = oxacillin,nafcillin, vancomycin
106
VSD
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
107
Wolff-Parkinson-White syndrome
accessory pathway b/w atria and ventricle leading to increased risk of tachyarrhythmias; classic EKG findings = shortened PR, delta waves, widened QRS
108
Croup, appropriate Tx
laryngotracheobronchitis; try trial of racemic epinephrine before intubation
109
most common cause of congenital hypothyroidism in the U.S.?
thyroid disgenesis
110
indication for renal/bladder ultrasound in child
less than 24 months of age with a febrile UTI; give Abx and get U/S
111
common and dangerous causes of acquired torticollis
upper respiratory infections, minor trauma, cervical lymphadenitis; dangerous = retropharyngeal abscess and alantoaxial subluxation; so GET an XRAY
112
ciprofloxacin in children
contraindicated b/w may ause cartilage destruction and growth retardation
113
acute exacerbation of cystic fibrosis lung disease; organism and what to do
pseudomonas aeruginosa; give aminoglycoside (tobramycin) and antipseudomonal penicillin (piperacillin); in adults can give fluoroquinolone in place of the aminoglycoside
114
differential for T-wave inversion
myocardial infarction, myocarditis, old pericarditis, myocardial contusion, digoxin toxicity
115
Tularemia
Francisella tularensis = acute unilateral cervical lymphadenopathy, fever, chillds, headache, malaise; contact w/ rabbits, hamsters, blood-sucking arthropods
116
unilateral lymphadenitis differential in children
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)
117
if kid develops anaphylactic reaction, encephalopathy, or CNS complication to DTaP, what to do?
Give DT and leave out the pertussis part
118
talipes equinovarus
clubfoot: equinus and varus of calcaneum and talus, varus of midfoot, and adduction of forefoot
119
most common cause of primary amenorrhea
Turner syndrome (45 XO, short stature, webbed neck, hypogonadism (streak ovaries), lymphedema, high-arched palate, congenital bicuspid aortic valves, coarctationofthe aorta
120
differential for hematochezia in childhood
hemorrhoids, infectious colitis, intussusception, Meckel's diverticulum, inflammatory bowel disease
121
congenital rubella syndrome triad
cataracts, PDA, sensorineural hearing loss; can have growth retardation and purpura (blueberry muffin rash)
122
cause of childhood stroke
sickle cell anemia
123
most common cause of urinary tract obstruction in newbornboys
posterior urethral valves
124
Diamond-Blackfan syndrome
congenital hypoplastic anemia: macrocytic anemia, low retic count, congenital anomalies
125
foreign body ingestion Tx
flexible endoscopy
126
foreignb body aspiration Tx
rigid bronchoscopy
127
tinea corporis
superficial fungal infection characterized by erythematous, scaly, pruritic rash w/ central clearing; Tx = terbinafine (topical antifungal)
128
myxedema
congenital hypothyroidism: MR w/ phenotype at birth
129
what is Guthrie test?
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
130
most common urologic problem in children
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
recommendation for children with first UTI and recurrent febrile UTI
age 2-24 months get a renal ultrasound for first UTI; get a voiding cystourethrogram (VCUG) for any child with recurrent febrile UTIs
132
loop diuretics are indicated in children w/ …
congenital heart disease who have volume overload and pulmonary edema
133
Mycoplasma infection
dry cough, bullous myringitis, PNA, pharyngitis, mailaise, weakness; Tx is erythromycin
134
RSV more commonly affects
children with heart defects; Tx is aerosolized ribavirin
135
most common organism responsible for pericarditis
Coxsackie virus
136
Five major criteria and Three minor criteria for Rheumatic fever
Major = polyarthritis, carditis, chorea, subcutaneous nodules, erythema marginatum; Minor = fever, arthralgia, previous Rheum fever
137
Henoch-Scholein purpura
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
Mgmt of omphalocele/gastroschisis
cover with sterile dressing to prevent heat loss, insert orogastric tube (decompress stomach), stabilize airway, and establish peripheral IV access
139
most common renal malignancy in childhood
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
Denys-Drash syndrome
gonadal dysgenesis, nephropathy, Wilm's tumor; mutated WT1
141
Beckwith-Widemann (5 features)
macroglossia, macrosomia, midline abodminal defects, ear creases, neonatal hypoglycemia
142
confirmatory tests for SLE
anti-Smith Abs, and dsDNA (both are specific)
143
Transposition of great vessels
cyanosis within 24 hours of life, seen in infants of diabetic mothers and in males; includes single loud S2 and no murmur
144
Tx of rapidly enlarging fluctuant cervical lymph nodes
Incision and drainage + dicloxacillin (which covers MSSA and strep but NOT MRSA)
145
Tx for swallowed battery
depends on location: if in esophagus, do immediate endoscopic removal; if in stomach, then passes without problems in 90% of cases
146
what to do with isolated proteinuria in children?
repeat urine dipstick on 2 subsequetn occasions;
147
Common variable immunodeficiency (CVID)
acquired hypogammaglobulinemia; less severe Sx and usually presents aged 15 to 35 years old
148
Bruton's agammaglobulinemia
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
alantoaxial instability
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
choledocahl cyst
congenital anomaly of biliary ducts: dilation of intra-hepatic and extra-hepatic biliary ducts or both;
151
Caroli's syndomre
congenital disorder: intrahepatic dilation of bile ducts
152
recurrent noctural vulvar itching
Dx: scotch tape test and pinworm infection; Tx = mebendazole
153
Hydroxyurea
inhibits ribonucleotide reductase (which converts UDP to dUDP)
154
6-mercaptopurine (6-MP)
blocks de novo purine synthesis
155
5-FU
inhibits thymidylate synthase (dUMP to dTMP)
156
MTX
inhibts dihydrofolate reductase (humans)
157
TMP
inhibits bacterialdihydrofolate reductase
158
orotic aciduria
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
xeroderma pigmentosum
mutated nucleotide excision repair;
160
ataxia telangiectasia
mutated nonhomologous end joining
161
I-cell disease (inclusion cell disease)
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
Cri-du-chat syndrome
5p deletion; microcephaly, hypotonia, short stature, cat-like cry
163
Wolf-Hirschhorn syndrome
4p deletion; microcepahyl, bilateral epicanthral folds, "greek helmet" facies, ocular hypertelorism, frontal bossing, hypoplasia of eye socket, cardiac problems
164
osteoid osteoma
sclerotic cortical lesion, diaphysis commonly, central nidus of lucency; pain at night; relieved with NSAIDS
165
osteosarcoma
most common primary bone tumor in children; metaphyses of long bones; "sunburst" apperance and periosteal elevation; increased ESR, LDH, and alk phos
166
Friedreich ataxia
neurologic (ataxia, dysarthria), skeletal (scoliosis, feet deformaties (hammer toes)), and cardiac (concentric hypertrophic cardiomyopathy) problems; cause of death commonly cardiac/resp problems
167
rotavirus vaccination schedule
b/w 2 and 8 months; do NOT start after 15 weeks; and must FINISH by 8 months
168
earliest age for menicogocceal vaccine if functional asplenia
age 2; so think about for sickle cell anemia patients
169
Helmet cells RBCs --> DDx?
DIC, HUS, TTP
170
basophilic stippling
ribosomal precipitates (blue granules of various sizes throughou cytoplasm of RBC); seen w/ thalassemias, and with heavy metal poisoning (or lead)
171
Heniz bodies
aggregates of denatured Hgb; G6PD deficiency or thalassemia
172
constitutional growth delay
delayed growth spurt, delayed puberty, delayed bone age
173
osteonecross and african american
sickle cell disease; up to 50% who are homozygous will develop this problem; usually humeral and femoral heads;
174
turner syndrome a/w what long term complication
osteoporosis; because of low estrogen levels (streaked nonfunctional ovaries) and only one copy of genes on X chromsome involved in bone metabolism
175
developmental dysplasia of the hip (RF, Dx, Tx)
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
complications of mumps
orchitis (85% is unilateral); encephalitis, aseptic meningitis
177
most common cause of 2ndary HTN in kids
fibromusuclar dysplasia (20% of renal HTN cases); right renal artery often more affected than left; angiography = "string of beads"
178
studies to perform for neonatal bilious emesis
xray first; looking for malrotation w/ volvulus, meconium ileus (think CF), or hirschsprungs; then do contrast enema (water-souble gastrograpphin)
179
all vaccines administered according to chronological age with exception of ____
Hepatitsi B; wait until child is above 2kg (4lbs 6 ounces)
180
Dx and Tx of slipped capital femoral epiphysis
obese adolescent boys often w/ endocrinopathies (GH deficiency, hypothyroidism; get xrays of hip; Tx with screw fixation to prevent avascular necrosis of hip
181
Legg-Calve-Perthes disease
idiopathic AVN in boys age 5-7
182
infants treated w/ oral erythromycin are at increased risk of
infantile hypertrophic pyloric stenosis
183
ceftriaxone in infants is a/w
increased risk of kernicterus because it displaces bilirubin from albumin binding sites so exacerbates a hyperbilirubinemia
184
umbilical cord stump infection
tetanus; poor suckling and fatigue --> rigidity, spasms, opisthotonus --> death; look out for unimmunizied mothers
185
most common cause of intestinal obstruction in children ages 6 to 36 months
intussusception
186
intussusception a/w and test of choice to Dx and Tx
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
complications of prematurity
RPRBIN: respiratory distress syndrome, PDA, bronchopulmonary dysplasia, intraventricular hemorrhage, necrotizing enterocoltis, retinopathy of prematurity
188
Tx for intraventicular hemorrhage
antenatal corticiosteroids
189
clinical features of CF
Resp: bronchiectasis, recurrent PNA, chronic rhinosinusitis; GI: obstruction, panc dz; repro: infertility; MSK: osteopenia, kyphoscoliosis, digital clubbing
190
Mayer-Rokitansky-Kuster-Hauser syndrome
congenital uterine and vaginal aplasia (it is RARE even in CF patients)
191
normal weight loss for newborns
up to 7% of their birth weight is acceptable
192
never give water to child below age of
6 months; this can cause hyponatremia and seizures
193
drugs to give for scarlet fever / strep if allergic to PCN
erythromycin, clinda, and first gen cephalosporins
194
how to differentiate Kawasaki dz from scarlet fever
both have strawberry tongue, rash, lymphadenopathy; but Kawasaki has periperhal extremitiy involvement and/or conjunctival injection;
195
rash after amoxicillin in setting of fever, lymphadenopathy, and pharyngitis
infectious mononucleosis due to EBV
196
immune thrombocytopenia
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
white reflex in eye
retinoblastoma --> referal to ophthalmologist
198
serum sickness-like reaction
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
rheumatic fever
after strep pharyngitis if no Tx w/ Abx; Sx = polyarthritis, carditis, erythema marginatum, subcutaneous nodules, chorea
200
Tx of minimal change disease
prednisone
201
IgA deficiency
recurrent sinupulmonary and GI infections AND anaphylactic reaction; measure and look for low IgA and normal IgM and IgG
202
TCA toxicity
dry mucosa, dilated pupils, wide QRS; Tx = sodium bicarb for severe cases to narrow QRS; benzos for seizures; Physostigmine for mild anticholinergic intoxication`
203
sickle cell dz + hip pain + slow onset over weeks
aseptic necrosis; also in other hemoglobinopathies;
204
complications of kawasaki dz
coronary artery aneurysms, myocardial infarction
205
pediatric myocarditis
fever, lethargy, heart failure, viral prodrome; usually Cocksackie B or adenovrius; get a CXR, EKG, echo, and Bx; Tx = supportive with diuretics + inotropes
206
causes of niacin deficiency
alcohoics, long term users of INH, carcinoid syndrome, Hartnup disease
207
long term complications of bacterial meningitis
hearing loss, loss of cognitive functions, seizures, mental retardation, spasticity/paresis
208
aplastic anemia - acquired causes
drugs (NSAIDs, sulfonamides), toxic chemicals (benzene, glue), idiopathic, viral infections (HIV, EBV), immune disorders, thymoma
209
aplastic anemia - congential causes
Fanconi anemia (most common cause)
210
Fanconi anemia
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
RTA Type I, II, IV
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
manifestations of infant heart failure
diaphoresis w/ feeds, poor appetitie, lethargy, hyperactive precordium; loud S2, sys ejc murmur across pulm valve, holosys of VSD;
213
Key findings in Down Syndrome
upslanting palpebral fissures, epicanthal folds, brushfield spots (white spots on irides), single transverse palmar crease, "sandal" gap toes
214
Comorbidities of Down Syndrome
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
most common casues of bacterial meningitis in children > 1 month old
strep pneumo and N.meningitidis
216
indications for imaging PRIOR to LP in infants/children
Hx of hydrocephalus, Hx head trauma, Hx VP shunt, Comatose patient, focal neuro findings
217
Abx coverage for bacterial meningitis in neonates versus those older than 1 month
28 days = Ceftriaxone and Vancomycin (for S. pneumo and N. menigitidis)
218
most common manifestation of late untreated Lyme
lyme arthritis;
219
osteogenesis imperfecta
blue sclerae, hearing loss, recurrent bone fractures, opalescent teeth
220
laryngomalacia
inspiratory strider; worsens in supine position; peaks at 4-8 months; Dx with laryngoscopy; resolves by 18 months of age
221
% of down's that have duodenal atresia and % of duodenal atresia that have downs;
2.5% of down's have DA; 25% of DA have Down's
222
more than 50% of patients with duodenal atresia have…
polyhydramnios
223
intraperitoneal calcification
suggests prenatal perforation of gut and meconium periotonitus, likely meconium ileus
224
meglumine diatrizoate
gastrographin
225
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
aplastic anemia
226
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
osteoporosis
227
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
Hep A immune and hep b NOT immune
228
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
t lymphocytes
229
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
answer: myotonic dystrophy (hand relaxing problems)
230
9 month boy; can only sit with support; two blocsk together but no scribble; says mama and dada; dev assessment?
delayed motor; normal fine motor; normal language
231
3 y.o. boy w/ anorexia, apathy, irritiability, poor coordiantion, sporadic vomiting; hypochromic microcytic anemia
Lead poisoning (anemia + neuro signs) -
232
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?
blood cultures
233
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
silver stain of bronchoalvelar fluid (for fungi) - kid has HIV and PCP pneumonia - walking leads to dsats
234
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
HSV
235
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?
nasal polyps
236
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
MDD
237
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
hyperglycemia
238
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?
hypothermia
239
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
sinusitis
240
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
bacterial pneumonia
241
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
impaired humora immunity (right answer!)
242
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
Calcium
243
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
bronchoscopy for foreign body
244
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
b-hCG
245
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
inguinal hernia (right answer)
246
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
voiding cystourethrography (for BOYS!)
247
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
pulm art HTN
248
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
exocrine panc insufficiency (CF?)
249
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?
diet
250
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
obs
251
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
adam-stokes attack
252
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?
mixed met acid and resp alk (though not sure how long it takes for met acidosis to set in…)
253
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
humerus fx
254
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
cerebral edema
255
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
physio pubertal dev
256
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
serum lipid studies
257
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
post uretrhal valves
258
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
pyloric stenosis
259
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
iv PCN (right answer?)
260
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
impetigo contagiosa
261
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?
cooking meats (since believed to be salmonella from poorly cooked chicken)
262
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
strict hand washing
263
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
s. aureus
264
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
smears
265
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
venous hum
266
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
closure of ductus
267
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
phototherapy: is 20.8 lightable at 96 hours of life?
268
2410g newborn at 37w; mom hx of IV drug use no prenatal care; rapid HIV is positive; next step for kid?
oral AZT within 12 hours for 6 weeks
269
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
answer - shigella
270
newborn girl w/ "clunk" when hip is abducted, flexed and lifted forward; what is mechanism of disease?
shallow poorly developed acetabulum
271
4 y.o. with neutropenia and has been treated w/ amox, cefotaxime, and TMP-SMX. what is cause of neutropenia?
TMP-SMX
272
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?
lymphocytic infiltration
273
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
TS
274
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
Legg-calve-perthes dz - answer
275
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?
INH only
276
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?
acute otitis media
277
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?
hemolytic disease of newborn
278
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?
karyotype
279
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
spirometry
280
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
bronchopulm dysplasia
281
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
decreased excretion
282
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
neuroblastoma
283
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
Diazepam
284
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?
exchange transfusion
285
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
ekg and chrom analysis
286
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
NF
287
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
Recovery?
288
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
metabolic
289
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
x-linked agammaglobulinemia (B-lymphocytes)
290
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?
replication of live vaccine virus strain
291
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?
I think this is Kawasaki's so give IVIG (and aspirin if it was a choice)
292
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?
thinking viral illness given time course and absence of findings; so give Tylenol
293
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?
renal U/S
294
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
subglottic edema
295
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
hemolytic uremic syndrome (bloody diarrhea from campylobacter jejuni)
296
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
normal development
297
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
retinoblastoma
298
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
hydrolyzed casein
299
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
Addison's disease in which case "cortisol" should be the answer
300
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?
infertility (varicocele)
301
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
bone age determination (constitutional delay)
302
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
cholelithiasis
303
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
do not need parental consent for emergencies
304
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?
hmmm
305
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?
treponema pallidum
306
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
answer is RDS of newborn
307
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
abnormal caudal migration of ganglion cells
308
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
midgut volvulus
309
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
hypothyroidism
310
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
tibia vara
311
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
decrease insulin dose by 10-15%
312
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
air contrast enema (should be intussusception)
313
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
observation (strawberry hemangioma)
314
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
drug induced SLE from phenytoin (hydralazine, INH, phenytoin, procainamide)
315
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
Wilms
316
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
endoscopy
317
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
Tinea versicolor (malassesia furfur)
318
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
large VSD can have this presentation
319
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
penicillin
320
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
CMV
321
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
torsion
322
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
imparied renal perfusion
323
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
HSP
324
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
reactive arthritis
