Pediatrics: UWorld Flashcards
1
Q
Most common cause of sepsis in sickle cell patient
A
-pneumococcus (encapsulated organisms)
2
Q
Preventative management in sickle cell patients
A
-vaccination -penicillin (until age 5) -folic acid supplementation -hydroxyurea (pts w/recurrent pain events)
3
Q
Maternal estrogen effects in newborns
A
-breast hypertrophy -swollen labia -physiologic leukorrhea -uterine w/drawal bleed
4
Q
Indications for renal & bladder US in children
A
-infants/children with first febrile UTI @ 2-24mo.
5
Q
Complications of prematurity
A
-RDS -Patent ductus arteriosus -Bronchopulmonary dysplasia -Intraventricular hemorrhage -necrotizing enterocolitis -retinopathy of prematurity
6
Q
Causes of precocious puberty
A
-central ==> high FSH & LH -peripheral ==> low FSH & LH (e.g. excess peripheral conversion, estrogen-producing ovarian cyst, CAH)
7
Q
Contraindications to DTaP vaccine
A
w/prev. vaccine: -anaphylaxis -unstable neuro d/p -encephalopathy
8
Q
Clinical features of pineal gland mass
A
**parinaud syndrome: -limited upward gaze -ptosis -upper eyelid retraction -pupillary abnormalities **obstructive hydrocephalus -papilledema -HA, vomiting -ataxia **central precocious puberty
9
Q
Work-up for suspicion of pineal gland mass
A
-brain mri -serum/CSF alpha-fetoprotein & B-hcg
10
Q
Prevention/tx of neonatal chlamydial conjuctivitis
A
-routine maternal screening during pregnancy -tx = oral erythmromycin
11
Q
Erythromycin opthalmic ointment ==>
A
prevention of gonoccocal conjunctivitis
12
Q
Risk factors for cryptorchidism
A
-prematurity -SGA/low birth weight -in utero DES/pesticide exposure -genetic d/o -NTDs
13
Q
Cryptorchidism tx
A
-orchiopexy @
14
Q
Risks after successful orchiopexy
A
-No risk of testicular torsion -highest risk of subfertility -some testicular cancer risk
15
Q
III/IV harsh holosytolic murmur @ LLSB in infant ==> ?
A
VSD
16
Q
OCD criteria/characteristics and tx
A
-recurrent intrusive thoughts with repeptitive mental/physical rituals -tx = high-dose SSRIs
17
Q
Neonate w/failure to thrive, bilateral cataracts, jaundice, and hypoglycemia
A
-galactosemia -galactose-1-phosphate uridyl transferase defieciency - + hepatomegaly, convulsions -pt. at risk for E.coli neonatal sepssis
18
Q
Galactokinase deficiency ==>
A
bilateral cataracts only; otherwise asymptomatic
19
Q
Uridyl diphosphate galactose-4-epimerase deficiency ==>
A
-rare -same sx as transferase deficiency (failure to thrive, bilateral cataracts, jaundice, hypoglycemia, hepatomegaly) & hypotonia and nerve deafness
20
Q
Minimal change disease characteristics
A
-most common cause of nephrotic syndrome -usually @ 2-3yo;
21
Q
AOM causes/tx/complications
A
-risks: smoke exposure, URI, day care, formula -organisms: s. pneumo, H. infl, moraxella -tx: oral amox x 10 days -complications: mastoiditis, TM rupture, conductive hearing loss
22
Q
Well-apearing neonate w/painless, bloody stools ==>
A
milk- or soy-protein allergy
23
Q
Factor deficiencies in cystic fibrosis
A
-vitamin K related factors -II, VII, IX, X and protein C,S
24
Q
Legg-Calve-Perthes disease characteristics
A
-idiopathic osteonecrosis of femoral head -boys age 4-10yo -p/w hip or knee pain of insidious onset + antalgic gait (decreased time weight bearing on affected side)
25
SCFE characteristics
-slipped capital femoral epiphysis -p/w limp and insidious hip pain -obese adolescents
26
Tx for ringworm
-topical antifungals (e.g. terinafine)
27
Osteosarcoma vs. Osteoid osteoma vs. Ewings
-Osteosarcoma = most common primary bone tumor; large, tender mass @ metaphyses of long bones; "sunburst pattern" XR -Ewing's = second most common; "onion skin" XR -Osteoid osteoma = sclerotic, cortical lesion w/central lucency; p/w pain @ night, not worse w/activity, helped w/ NSAIDs
28
Common bacteria in CF pneumonia
-gram neg rods: pseudomonas, b. dolosa, stenotropomonas -gram neg coccobac: H. influenza -gram pos cocci chains: s. pneumo -gram pos cocci clusters: s. aureus
29
Cholesteatoma characteristics
-congenital or acquired from chronic middle ear dz -new-onset hearing loss or chronic ear drainage w/abx therapy -granulation tissue and skin debris w.in pockets of TM
30
Colic definition
-crying in otherwise healthy infant for \>3hrs daily (usually evening) - \>3x/week for \>3weeks -tx = soothing techniques
31
Risk factors for neonatal RDS
1. prematurity 2. male sex 3. perinatal asphyxia 4. maternal DM 5. C-section w/out labor
32
Presentation of RDS
-tachypnea, retractions, grunting, nasal flairing, cyanosis @ birth -CXR: diffuse reticulogranular pattern (ground-glass opacities) & air bronchograms
33
Tx of RDS
antenatal: corticosteroids postnatal: exogenous surfactant and respiratory support
34
Bilious emesis w/u
1. abdominal xray; pneumoperitoneum ==\> surgery 2. water-soluble contrast enema (if stable) ==\> find level of obstruction
35
Meconium ileus presentation
-bilious emesis -microcolon on contrast enema -pathognomonic for CF
36
Tx of Meconium ileus
1. hyperosmolar (gastrografin) enema to try to break up meconium 2. Surgery if not resolved 3. all pt.s receive sweat chloride testing after stabilized
37
Characteristics of Reye syndrome
-children who receive aspirin for virus-induced fever -hyperramonemia -transaminitis (w/microvesicular steatosis) -coagulopathy -emesis -AMS
38
Diarrheal illness + renal failure, hemolytic anemia, thrombocytopenia ==\> dx?
HUS
39
HUS causes
-E.Coli 0157:J& -Shigella -Salmonella -Yersinia -Campylobacter
40
Hallmark of HUS
microangiopathic hemolytic anemia
41
Peripheral smear in HUS
schistocytes and giant platelets
42
Transmission of lyme disease
Ixodes transmits Borrelia burgdorfer via bite
43
Presentation of eczema herpeticum
-HSV infection associated w/atopic dermatitis -umbilicated vesicles over area of healing atopic derm - + fever + adenopathy -can be life-threatening ==\> rapidly initiate tx
44
Impetigo causes
non-bullous: staph or strep pyogenes bullous: staph aureus
45
Appearance of bullous impetigo
-rapidly enlarging flaccid bullae w/yellow fluid -"collarette" of scale at periphery of ruptured lesions
46
Tx of non-bullous and bullous impetigo
-non-bullous: topical abx (mupirocin) -bullous: oral abx (cephalexin, dicloxacillin, clindamycin)
47
Management of pertussis cases
-macrolide abx for pt. -macrolide abx for all close contacts regardless of age, immunizations, sx
48
Pertussis prophylaxis abx
- 1 mo. old: Azithro x 5D vs. Clarithro x 5D vs. Erythro x 5D
49
Epidemiology of Meckel's diverticulum
Rule of 2s: -2% prevalence -2:1 male-to-female ratio -2% are sx at age 2 -Located w/in 2 ft. of the ileocecal valve
50
Clinical presentation of Meckel's diverticulum
-asymptomatic incidental finding -painless hematochezia -intussusception -intestinal obstruction -volvulus
51
Dx & Tx of Meckel's diverticulum
Dx: technium-99 scan Tx: surgery for sx patients
52
Characteristics of myotonic muscular dystrophy
-genetics: AD -onset: age 12-30 -presentation: facial weakness, hand grip myotonia, dysphagia -comorbidities: arrhythmias, cataracts, balding, testicular atrophy -prognosis: death from respiratory or heart failure
53
Duchenne M. dystrophy comorbidities
-scoliosis -cardiomyopathy
54
Becker m. dystrophy comorbidities
cardiomyopathy
55
CD3 = CD19 =
CD3=T lymph CD19 = B lymph
56
Tx for X-linked agammaglobulinemia
IVIG
57
Indications for neuroimaging in child w/HA
1. hx of coordination difficulties 2. numbness, tingling, focal neuro signs 3. H/A that awakens from sleep 4. increasing H/A frequency
58
Rheumatic fever features (major)
Joints O(carditis) Nodules Erythema marginatum Sydenham chorea
59
Rheumatic fever minor features
-fever -arthralgias -elevated ESR -prolonged PR
60
Red flag sx for intracranial pathology
-nocturnal headaches -morning vomiting
61
Dx features of acute bacterial rhinosinusitis
1. persistent sx \> 10 days OR 2. Severe sx, fever \>=39, purulent nasal drainage, face pain x 3days OR 3. Worsening sx \>=5 days after initial improvement from viral URI
62
Tx of acute bacterial rhinosinusitis
amox-clav PO
63
Characteristics of Waterhouse-Friderichsen syndrome
-infant w/meningococcemia -skin rash (large purpuric lesions on flanks) -sudden vasomotor collapse 2/2 adrenal hemorrhage
64
Unique CXR feature in children
thymic silhouette visible at superior right heart border
65
Characteristics of early disseminated lyme disease
-carditis: AV block, cardiomyopathy -neurologic: unilateral or bilateral CN n. defects (e.g. VII), meningitis, encephalitis -muscular: migratory arthralgias -conjunctivitis -skin: multiple erythema migrans
66
Complex partial seizures vs. partial seizure with generalization
-both: aura, LOC -complex partial ==\> motor automatisms (picking, chewing, etc.) during LOC -partial w/generalization ==\> tonic-clonic activity
67
Characteristics of simple partial seizure
-no LOC -deja-vu -can have aura -pt. may remember well
68
Firm, smooth unilateral abdominal mass that is asymptomatic w/hematuria ==\>
-Wilms tumor -most common childhood cancer -peak age 2-5yo
69
Dx & Tx of Wilms tumor
1. US to differentiate from other masses 2. CT w/contrast ==\> look for pulm mets 3. Tx = surgery + chemo; radiation for end-stage \*Good prognosis in early stages
70
Presentation of neuroblastoma
-3rd most common peds cancer -first year of life -typically: abdominal mass that crosses midline
71
Presentation of duodenal atresia
-prenatal US w/polyhydramnios -bilious vomiting w/in first 2 days of life -"double bubble" sign on abdXR -strongly associated w/Down's & assoc. heart defects
72
CV defects in Turner's
-bicuspid aortic valve -coarctation of the aorta -aortic root dilation
73
Renal defects in Turner's
horshoe kidney
74
Ataxia + scoliosis/foot deform + cardiomyopathy ==\> dx?
Friedrich ataxia
75
Presentation/characteristics of Friedrich ataxia
-most common spinocerebellar ataxia -AR; presents
76
Causes of neuro sx in Friedrich ataxia`
-degeneration of spinal tracts -spinocerebellar ==\> ataxia -posterior columns ==\> falling -pyramidal ==\> dysarthria
77
Complication of trauma to soft palate
==\> internal carotid artery disection ==\> stroke
78
Breast milk jaundice vs. BF failure: timing
-failure: w/in first week -BM: starts @ 3-5 days, peaks @ 2 weeks
79
Breast milk jaundice vs. BF failure: clinical features
-failure: suboptimal BF, signs of dehydration -BM: adequate BF, normal exam
80
Pathophysiology of BF failure jaundice
-decreased bilirubin elimination -increased enterohepatic circulation
81
Pathophysiology of BM jaundice
high levels of b-glucoronidase in BM deconjugate intestinal bilirubin and increase enterohepatic circulation
82
Threshold for phototherapy or exchange transfusion in neonatal jaundice
-phototherapy = ~w/in first week = 20 mg/dL+ -exchange transfusion = 25+
83
Normal/recommended newborn feeding patterns
-~8-12x/day for 10-20 min/breast -approx. every 2-3 hours
84
DDx of stridor
-croup (laryngotracheobronchitis) -laryngomalacia -FB aspiration -vascular ring
85
Distinguishing features of laryngomalacia
-most severe @ 4-8 mo. -worse in supine; improves in prone position
86
Distinguishing features of vascular ring (==\>stridor)
-presents
87
Drug of choice in PNA in young CF pt.
IV vancomycin
88
Tx of long-QT syndrome
-avoid vigorous exercise -avoid QT prolonging meds -maintain normal Ca, K, Mg -Beta-blockers -sx (e.g. lightheadedness, palpitations) or history of syncope ==\> pacemaker
89
NEC on abd. XR
-air visible in bowel wall ("double-line" or "train-track") ==\> pneumotasosis intestinalis -portal venous air
90
Common neuro complication in sickle cell
stroke 2/2 sludging and occlusion of vasculature
91
Tx of stroke in sickle cell pt.
exchange transfusion
92
Indication for intubation of asthmatic
-severe asthma unresponsive to meds w/: -fatigue -AMS -CO2 retention, worsening hypoxemia -poor air movement
93
Most common causes of viral meningitis
non-polio enteroviruses: echoviruses or coxsackieviruses
94
Signs/sx of intussusception
-colicky, severe abdominal pain -"currant jelly" stools (2/2 mesenteric ischemia) -"target sign" on US
95
Palpable purpura + arthritis/athralgia + abdominal pain + renal disease ==\> dx?
Henoch-Schonlein purpura = IgA mediated vasculitis
96
Lab findings in Henoch-Schonlein purpura
-normal platelets/coags -normal to increased Cr -hematuria +/- RBC casts +/- proteinuria
97
Tx of Henoch-Schonlein purpura
-most patients: hydration + NSAIds -severe: admission w/systemic steroids
98
Aplastic anemia causes
-drugs -toxins -viral infections ==\> pancytopenia
99
Presentation of Fanconi's anemia
-pancytopenia starting gradually at 4-12yrs. old -congenital anomalies: hyperpigmentation of trunk, neck, intertriginous areas; cafe-au-lait spots, short stature, upper limb abnormalities, hypogonadism, skeletal anomalies, eye/eyelid changes, or renal malformations-
100
Clinical presentation of rubella
-low-grade fever -conjunctivitis, coryza, cervical lymphadenopathy, Forscheimer spots (patchy erythema) on soft palate -cephalocaudal and centrifugal "pink" maculopapular rash from face to body
101
Clinical presentation of immune thrombocytopenia
-antecedant viral infection -asx petechiae & ecchymoses (msot commonly) -mucocutaneous bleeding
102
Tx of immune thrombocytopenia (children)
-skin manifestations only: observe -bleeding: IVIG or glucocorticoids
103
Riboflavin (B2) deficiency presentation
-angular cheilitis (fissures @ corners of lips) -glossitis -stomatitis (hyperemic/edematous oral mucosa) -normocytic-normochromic anemia -seborrheic dermatitis
104
Niacin deficiency presentation
-symmetric reddish rash on exposed skin, glossitis, diarrhea/vomiting -neuro disturbances
105
Thiamine (B1) deficiency presentaion
-dry beriberi: peripheral neuropathy -wet beriberi: cardiomyopathy -wernicke-korsakoff
106
Major advantages of breastfeeding
-absorbs better and improves gastric emptying -IgA and other proteins
107
Tx of pinworms
albendazole
108
Risk factors for intraventricular hemorrhage in newborn
1. preme 2. low birth weight 3. exposure to vascular perfusion injuries: hypoxia/ischemia, hypotension, reperfusion of damaged vessels, increased venous pressure, abrupt changes in cerebral flow
109
PKU pathophysiology
-autosomal recessive mutation in phenylalanine hydroxylase -failure to convert phenyl ==\> tyrosine results in hyperphenylalaninemia (elevated aa) and neuro injury
110
PKU Clinical features
-severe intellectual disability -seizures -musty body odor -hypopigment of skin, hair, eyes
111
Dx of PKU
a. newborn screen b. quantitiative amino acid analysis ==\> elevated phenylalanine
112
NF1: gene, chromosome, clinical features
-NF1 tumor suppressor ==\> neurofibromin -@ chromosome 17 -cafe-au-lait spots -multiple neurofibromas -lisch nodules
113
NF2: gene, chromosome, clinical features
-NF2 tumor suppressor ==\> merlin -@ chromosome 22 -bilateral acoustic neuromas
114
Pediatric viral myocarditis: etiology, clinical presentation
-coxsackie B vs. adenovirus -viral prodrome ==\> -heart failure: dyspnea, tachycardia, syncope, N/V, hepatomegaly
115
Dx of viral myocarditis
-CXR: cardiomegaly, pulmonary edema -ECG: sinus tachycardia -Echo: decreased EF, diffuse hypokinesis
116
Congenital diaphragmatic hernia presentation
-usually on left -polyhydramnios -concave abdomen + barrel shaped chest -absent lung sounds on affected side -can displace heart/heart sounds ==\> pulmonary hypoplasia nad pulm HTN ==\> respiratory distress as neonate
117
Management of congenital diaphragmatic hernia
1. intubate (NO bag-valve mask) 2. NG/OG tube 3. umbilical a. line for monitoring 4. umbilical v. line for fluids/meds
118
Tumor that produces estrogen
Granulosa-cell tumor
119
Sertoli-leydig cell tumor ==\>
androgen production ==\> masculinization
120
Glucose-6-phosphatase deficiency physical presentation
-doll-like face -thin extremities -short stature -protuberant abdomen
121
Glucose-6-phosphatase deficiency metabolic disturbances
-hypoglycemia ==\> seizures -lactic acidosis -hyperuricemia -hyperlipidemia
122
Hep B associated w/renal disease?
membranous nephropathy
123
Nutritional intervention for preterm infants
-iron supplimentation in exclusively breast-fed infants ==\> 1yo -all exclusively breastfed infants ==\> vit D supplement
124
Electrolyte abnormalities in refeeding syndrome
-decreased serum phosphorous, K, Mg -decreased serum thiamine -increased sodium and water retention
125
Hip pain but able to bear weight and w/out significant lab/vital abnormalities ==\> dx? tx?
-transient synovitis = most common hip pain in children (usually 3-10yo) -NSAIDs and rest, f/u in 1 wk.
126
Transposition of great vessels: exam/findings
-single S2 -+/- VSD murmur -CXR: "egg-on-a-string" heart (narrow mediastinum)
127
Tetrology of Falloy: exam/findings
-harsh pulmonic stenosis murmur -VSD murmur -CXR: boot-shaped heart (RVH)
128
Tricuspid atresia: exam/findings
-Single S2 -VSD murmur -minimal pulm. blood flow
129
Truncus arteriosus: exam/findings
-single S2 -systolic ejection murmur -increased pulm. flow, edema
130
Total anomalous pulm. venous return: exam/findings
-severe cyanosis -respiratory distress -pulm. edema; snowman sign (enlarged supracardiac veins and SVC)
131
Most common complication of sickle cell trait
painless hematuria
132
Evaluation of primary amenorrhea
1. pelvic US; if no uterus ==\> karyotype 2. FSH if no breast development 3. FSH low ==\> pituitary MRI 4. FSH high ==\> karyotyping
133
Jejunal atresia presentation and risk factors
-presentation: bilious vomiting and abdominal distention -abd. xr: "triple bubble" sign and gasless colon -risk factors: prenatal exposure to cocaine, tobacco, or vasoconstricive drugs
134
Risks for rickets
-exclusive BF -dark skin (?) -lack of sun exposure
135
Exam findings in rickets
-craniotabes ("ping-pong ball" skull) -delayed fontanel closure -enlarged: skull (frontal bossing), costochondral joints (rachitic rosary), long bone joints (wrist widening) -genu varum
136
Lab abnormalities in rickets
-Ca: normal to low -Ph: normal to low -alk phos: very elevated -PTH: elevated -vit D: low
137
Fragile X gene mutation
expanded CGG trinucleotide repeats and abberrant methylation @ FMR1
138
Risks if born SGA
-SGA =
139
Risks if born LGA
-hip sublux -talipes calcaneovalgus
140
Dx of kawasaki dz.
-fever 5+ days and 4+ of: -bilateral nonex. conjunctivitis -mucositis (including strawberry tongue) -cervical lymphadenopathy -erythematous polymorph rash -extremity changes (edema, erythema)
141
Tx of Kawasaki dz.
ASA + IVIG
142
Complications of Kawasaki dz.
-coronary a. aneurysms -MI or ischemia
143
Characteritics of osgood-schlatter disease
-common cause of knee pain, esp. @ adolescent athletes -traction apophysitis of the tibial tubercle @ the insertion of the paterllar tendon -PE: tenderness/edema @ tibial tubercle + pain with resisted extension -XR: anterior swelling, lifting of tubercle from shart, irregularity/fragmentation of tubercle
144
Tx of osgood schlatter
-activity restriction -stretching -NSAIDs
145
Presentation of homocytinuria
-AR -marfinoid body habitus -fair har/eyes -downward lens dislocation -developmental delay -hypercoaguability
146
Homocystinuria pathophys + tx
-cystathione synthase deficiency -tx: -vitamin supplementation -antiplatelet/anticoag
147
Tay-Sachs characteristics
-AR -decificency in B-hexosaminidase A -presents w/ID, weakness, seizures, cherry-red macula
148
Lab results in Vitamin K deficiency
-prolonged PT -prolonged PTT
149
Lab results in factor VIII deficiency
-prolonged PTT -normal PT
150
Risks of vitamin K defiency/prevention
-low vitamin k ==\> bruising, bloody stools, intracranial hemorrhage -prevention with vitamin K injection after delivery
151
Metatarsus adductus management
-Type I = correction to abduction w/movement ==\> reassurance -Type II = correct to neutral ==\> orthosis/corrective shoes (2nd line = casts) -Type III = rigid, no correction ==\> serial casting
152
Lab findings in measles
-leukopenia (t-cell cytopenia) -thrombocytopenia
153
Tx that reduces mortality in measles
-vitamin A
154
Management of caustic/alkali ingestion
1. secure airway, breathing, circulation 2. decontaminate: remove contaminated clothing and irrigate exposed skin 3. DO NOT attempt to neutralize w/acid and DO NOT pass blind NG tube 4. CXR if respiratory sx 5. endoscopy in 24 hours
155
Duration of PCN prophylaxis in rheumatic fever
-RH w/out carditis: until 21yo (or 5 years if \>16yo) -RH w/card but w/out valvular: until 21yo (or 10y if \>11yo) -RH w/card + valv: until 40yo (or 10y if \>30yo) \*PCN injection IM qmonth
156
Selective mutism criteria
-fail to speak in certain situations but not others - \> 1 mo. -interferes w/education or social communication -not 2/2 lang. barrier
157
self mutalation + neuro findings + elevated uric acid ==\> dx?
-Lesch-Nyan syndrome -HPRT (hypoxanthine-guanine phosphoribosyl transferase) deficiency
158
Sx of L-N syndrome
-self-mutilation -neuro: mental disability, dystonia, spasticity, choreoathetosis -gouty arthritis/tophi
159
Measles prodrome and exanthem
-prodrome: -fever, malaise, anorexia -coryza, conjunctivitis, koplik spots (clustered white spots on buccal mucosa) -exanthem: -blanching, reddish-brown maculopapular rash -cephalocaudal & centrifugal spread -spares palms/soles
160
GI Risk in H-S Purpura
ileo-ileal itussusception
161
Presentation/management of androgen insensitivity syndrome
-XY w/female phenotype -bilateral gonadectomy after completion of puberty
162
Contraindications to rotavirus vaccine
-anaphylaxis to ingredients -hx of intussusception -hx of uncorrected congenital malform. of GI tract (e.g. Meckel's) -SCID
163
Kallmann sydnrome presentation
-delayed/absent puberty -karytype matches phenotype -FSH/LH low
164
presentation of heriditary angioedema
-swelling/edema episodes after trauma, dental procedure, infections -non-inflammatory edema of face, limbs, genitalia -laryngeal edema can be life-threatening -edema of intestines ==\> abdominal pain -no urticaria
165
Pathophysiology of heriditary angioedema
-C1 inhibitor deficiency or dysfxn - ==\> elevated levels of C2b and bradykinin ==\> edema
166
Precocious puberty w/elevated LH ==\>
brain MRI to rule out intracranial pathology
167
Reproductive consequences of CF
-congenital bilateral absence of vas deferens in male ==\> infertility @ 95%+ -20% of women infertile
168
MSK consequences of CF
-osteopenia ==\> fx -kyphoscoliosis -digital clubbing
169
Omphalocele associated with...
-NTDs -cardiac malformations -trisomy
170
DDx of regurgition/vomiting in infants
-physiologic reflux -milk protein allergy -GERD -pyloric stenosis
171
Infant GERD presentation
-FTT -significant irratibility -sandifer syndrome (intermittent opisthotonic posturing = arched back, rigid neck)
172
Management of infant GERD
-thickened feeds -antacid therapy -esophageal monitoring with pH probe -upper endoscopy
173
Lab findings in hereditary spherocytosis
-increased MCHC -spherocytes on peripheral smear -negative coombs -increased osmotic fragility of acidified glycerol lysis test -abnormal eosin-5-maleimide binding test
174
parvovirus B19
xx
175
Acute lymphadenitis cause/presentation
-acute, unilateral cervical lymphadenitis = red, warm, swollen -usually 2/2 staph aureus
176
Pathogen for acute, unilateral cervical lymphadenopathy w/: -rabbit/animal contact -periodontal disease -violaceous color
-rabbit = tularemia -periodontal = peptostreptococcus -color = nonTB myco (e.g. MAC)
177
Infant botulism tx
human-derived botulism IG
178
foodborne botulism tx
equine-derived botulism antitoxin
179
Labs in thalassemis
-MCV: very low -Iron: high -TIBC: low -Ferritin: high -Transferrin sat: very high
180
Dx test for CF
sweat chloride testing via quantitative pilocarpine iontophoresis
181
infant w/macrocytic anemia, low retic count, congenital anomalies ==\> dx?
diamond-blackfan syndrome
182
avg. age of dx of fanconi's anemia?
8 yo
183
congenital anomalies in diamond-blackfan syndrome
-short stature -webbed neck -cleft lip -shielded chest -triphalangeal thumbs
184
congenital anomalies in fanconi's anemia
-hyperpigmentation of trunk, neck, intertriginous areas -cafe-au-lait spots -short stature -upper limb abnormalities/absent thumbs -hypogonadism -skeletal anomalies -eye/eyelid changes -renal malformations
185
Transient erythroblastoma presentation
- \>1yo -pure red cell aplasia w/out macrocytosis -no congenital anomalies
186
Congenital rubella presentation
-sensorineural hearing loss -intellectual disability -cardiac anomalies -cataracts, glaucoma
187
Wolff-Parkinson-White syndrome characteristics
-shortened PR -delta waves (slurred initial portion of QRS) -widened QRS -2/2 accessory pathway conducts from atria to ventricles faster than through AV node ==\> supraventricular tachycardia
188
Characteristics of serum sickness-like rxn
1-2 wks after B-lactam or TMP-SMX therapy -Type III hypersensitivity rxn -fever, urticaria, polyarthralgia -labs: nonspecific hypocomplementemia, elevated inflammatory markers (ESR, CRP)
189
Tx of serum sickness rxn
1. remove offending agent 2. if severe ==\> steroids \*avoid in future (even though not true allergy)
190
Chronic granulomatous disease presentation
-recurrent/unusual lymphadenitis, abscesses, osteo -infections w/catalase-positive organisms: s. aureus, aspergiluus, serratia marcescens, burkholderia cepacia -positive nitroblue tetrazolium test
191
Wiskott-Aldrich syndrome presentation
-X-linked recessive -eczema, thrombocytopenia, recurrent infections w/encapsulated organisms -@ birth: petechaie, bruises, bleeding w/circumcision, bloody stools -immune: low IgM, high IgA and IgE
192
Chediak-Higashi syndrome characteristics
-decreased degranulation, chemotaxis/fxn of PMNs -coagulopathy, neuropathology, hepatosplenomegaly, pancytopenia -frequent bacterial infections (usually staph) -==\> neutropenia + giant lysosomes in PMNs
193
Tx of chediak-higashi
-daily TMP-SMX -daily ascorbic acid
194
Leukocyte adhesion deficieny presentation
-neutrophilia w/out polymorph in infected pus -delayed separation of umbilical cord -recurrent bacterial infections -necrotic skin lesions -dental/oral decay
195
CSF in guillan-barre
-elevated protein -normal cell count
196
Tx of guillan-barre
-pooled immunoglobulin -plasmaphersis
197
Anterior horn diseases
-polio -spinal muscular atrophy -ALS
198
NMJ diseases
-botulism -myasthenia gravis -lambert-eaton (proximal m. weakness) -organophosphate poisoning
199
Vitamin B12 deficiency neuro sx
-subactue degeneration of dorsal and lateral columbs (upper motor neurons) - ataxia, bilateral paresthesia, loss of vibration/position sense
200
Most common leukemia in 2-10yo
ALL
201
ALL presentation
-children 2-10yo -fever, infection vs. lymphadenopathy vs. splenomegaly -PAS+ -TdT +
202
Chlamydial vs. Gonoccocal conjunctivitis
-gonoccocal: @ 2-5 days old w/purulent discharge -chlamydial: @ 5-14 days w/watery discharge
203
Dx of hereditary spherocytosis
-presents w/coombs-neg. hemolytic anemia, jaundice, splenomegaly -positive Eosin-5-maleimide binding & acidified glycerol test
204
Spondylolisthesis definition
-developmental d/o w/forward slip of vertebra -usually L5 over S1
205
Trachoma presentation
-follicular conjunctivitis + neovascularization @ cornea -concurrent infection of nasopharynx nasal d/x -dx: giemsa stain of conjuctival scrapics
206
Tx of trachoma
-topical tetracycline or oral azithromycin
207
Causes of secondary enuresis
-psych stress -UTI -DM -Diabetes insipidus -OSA
208
Dx of cyclic vomiting syndrome
-\>3 episodes in 6 mo. Period -family recognition -lasts 1-10 days -vomiting \>4x/hour @ peak -no sx in between episodes -no underlying condiditon -often hx of migraines in parents
209
Method of removal of small vaginal foreign body in young child?
-calcium alganate swab -warm irrigation w/topical anesthetic -large FB ==\> may need to remove under anesthesia
210
sleep stage for night terrors
non-rem
211
head trauma w/LOC ==\> ?
CT w/out contrast
212
Pathophys/renal complications in sickle cell trait + painless hematuria?
-renal papillary necrosis -inability to concentrate urine -distal renal tubular acidosis
213
Thrombosis-related complications in sickle cell trait
-splenic infarct (esp. @ high altitude) -VTE -priapism
214
Most common cardiac malformation in Down's + presentation
-complete AV septal defect ==\> HF @ 6wks old -HF ==\> poor feeds, diaphoresis, dyspnea -loud S2 + systolic ejection murmur +/- VSD
215
One of earliest manifestations of sickle cell anemia
dactylitis (hand-foot syndrome) = vaso-occlusion/crisis @ hands and feet ==\> lytic lesions on xr
216
Common neonatal rashes
-erythema toxicum neonatorum = asx, scattered red macules, papules, pustules = benign -neonatal HSV = vesicles -neonatal varicella = fever + clustered vesicles -staph scalded skin = fever, irritable + erythema =\> blister =\> exfoliation
217
McCune-Albright characteristics
-3 P's: -Precocious puberty -Pigmentation (cafe-au-lait spots) -Polyostotic fibrous dysplasia = multiple bone defects -assoc. w/cushing syndrome
218
Common predisposing factor for orbital cellulitis
bacterial sinusitis
219
Bilious vomiting + abdominal distension + gasless on abdominal xray ==\> ?
upper GI series
220
bilious vomiting + dilated loops of bowel on abd xray ==\>
-contrast enema -microcolon on contrast enema ==\> mec ileus -rectosigmoid transition zone ==\> hirschsprung
221
Fetal Alcohol Syndrome dysmorphic features
1. small palpebral fissures 2. smooth philtrum 3. thin vermilion border (thin lip)
222
Maternal benefits of breast feeding
-decreased PP bleeding -weight loss -child spacing -bonding -reduced breast & ovarian cancer
223
Contraindications to breastfeeding
-MOC w/TB -Maternal HIV -herpetic breast lesions -varicella infections
224
Lab findings in thallasemias
-MCV: low -RDW: normal (vs. high in iron def.) -RBCs: normal (vs. low in iron def.) -normal/increased iron and ferritin -smear: target cells (vs. microcytosis, hypochromia in iron def.)
225
Hb electrophoresis in iron def. vs. alpha thal minor vs. b-thal minor
-iron = normal -a-thal minor = normal -b-thal minor = increased hb A2
226
Congenital hypothyroid presentation/timeline
-normal @ birth; ==\> apathy, weakness, hypotonia -sluggish, large tongue, abdominal bloating, umbilical hernia \*\*usually screened for @ birth in US
227
Condition in which pt.s may have anaphylactic rxn to blood transfusion?
IgA deficiency 2/2 abs formed against IgA
228
Timing for meningococcal vaccine
-initial dose @ 11-12yo -booster @ 16yo
229
Edward's syndrome characteristics
-Trisomy 18 -micrognathia, microcephaly -rocker bottom feet, overlapping fingers, absent palmar creases -VSD = most common defect
230
Cause of croup
parainfluenza virus
231
UMN findings in Down's syndrome child ==\> dx?
atlantoaxial instability
232
Nikolsky sign (positive) ==\> ?
-Nikolsky sign = gentle lateral pressure @ skin surface adjacent to blister leads to slip/detachment of superficial layer of skin -positive ==\> staph scalded skin syndrome
233
Toxin-mediated skin rashes
-Staph scalded skin syndrome -Scarlet fever
234
Characteristics of scarlet fever
-group A beta-hemolytic strep toxin-mediated -viral prodrome: fever, H/A, vomiting, sore throat -12-48h ==\> fine pink blanching papules @ neck, trunk ==\> flexor surfaces
235
Pathophysiology of physiologic jaundice of newborn
-unconjugated hyperbili 1. high hb turnover in first few days of life 2. UGT is slow in newborn (asians have decreased activity compared to other ethnicities) 3. enterohepatic recycling is increased in newborn gut
236
Phototherapy to prevent ...?
kernicterus
237
Adenosine deaminase deficiency = characteristic of which dz?
SCID
238
Dysmorphogenesis of third/fourth pharyngeal pouch = ?
digeorge ==\> t cell dysfxn
239
CGD 2/2 to ?
inability to oxidize pathogens w/in phagocytes
240
Elevated screening lead ==\> ?
1. draw venous lead level (2/2 false + on fingerstick) 2. mild elev. (5-44) ==\> no meds, repeat w/in 1 mo. 3. mod elev. (45-69) ==\> meso-2,3-dimercapto-succinic acid (DMSA) 4. severe (70+) ==\> dimercaprol + ca disodium edetate (EDTA)
241
Most common brain tumors in peds
1. benign astrocytoma 2. medulloblastoma
242
Most common causes of bacterial sinusitis
1. strep pneumo 2. h. influenza
243
Sx/risk factors/definition of polycythemia in neonate
-Hct \> 65% @ term neonate -delayed cord clamp, maternal HTN, maternal DM -respiratory distress, hypoglycemia, neuro problems
244
Examination findings in endometriosis
-uterosacral nodularity -adnexal tenderness
245
Examination findings in adenomyosis
-uterine tenderness/enlargement
246
Common causes of acquired torticollis + inititial evaluation
-URI -minor trauma -cervical lymphadenitis -retropharyngeal abscess -C-spine Xray to rule our c-spine fx/dislocation
247
NF1 features
-cafe-au-lait -macrocephaly -feeding problems -short stature -learning disabilities ==\> fibromas, neurofibromas, other tumors
248
Congenital syphillis presentation
-HSM -cutaneous lesions -jaundice -anemia -rhinorrhea -On xray: metaphyseal dystrophy and periostitis
249
Sturge-Weber features
-unilateral hemangioma along trigeminal distribution -intracranial calcifications that resemble a tramline
250
Absence seizure tx
-ethosuximide -valproic acid
251
Tx of battery ingestion distal vs. proximal to esophagus
-proximal ==\> immediate endoscopic removal -distal ==\> observe w/stool exam/f/u radiographs
252
Bug/Tx of typical acute unilateral cervical adenitis
-usually 2/2 staph aureus vs. strep pyogens -tx = clindamycin
253
Bug/Tx of acute unilateral cervical adenitis in child w/dental disease
-anaerobic -tx = clinda vs. amox/clav
254
Tx of acute unilateral cervical adenitis in child w/non-TB myco
-excision -marcolide +/- rifampin
255
Neuroblastoma characteristics
-3rd most common cancer in peds -NC cells ==\> sympathetic chains/adrenal medulla -common site is abdomen -calcification/hemorrhage on xray or CT -elevated homovanillic acid or vanillylmandelic acid
256
Recurrent episodes of hematuria + sensorineuronal deafness + fhx of renal failure ==\> dx?
Alport syndrome
257
EM in Alport syndrome
-thinned and thickened cap loops -splitting of glomerular basement membrane
258
Tx of lyme disease in children
- amox - \>9yo ==\> doxy
259
Lower intestinal pathology assoc. w/Down's
Hirschsprung
260
Fluid for correction of hypernatremia in infants
NS
261
Source of delayed/absent puberty in turner's
-streak ovaries ==\> decreased estrogen - ==\> elevated FSH
262
Initial management of epiglottitis
1. to OR for intubation vs. tracheostomy 2. admission + IV antibiotics NO racemic epi or steroids
263
Late-onset CAH presentation/cause
-21-hydroxylase deficiency -presents in late childhood -leads to signs of androgen excess ==\> premature adrenarche/pubarche, severe cystic acne, accel linear growth, advanced bone age -NORMAL ELECTROLYTES
264
Management of abnormal hip exam in child
- (+) ortolani/barlow ==\> ortho -asymmtery w/ (-) o/b @ 2wks - 6mo. ==\> hip US -asymmtery w/ (-) o/b @ 4 - 6mo. ==\> hip xray
265
Gold standard for Duchenne MD dx?
genetic testing
266
Iron poisoning presentation
-abdominal pain -hematemsis -shock -metabolic acidosis -xray w/radioopaque pills
267
Iron poisoning tx
-deferoxamine -IV fluids if needed
268
Management of neonatal weight loss
-may lose up to 7% BW w/in first 5 days; should regain BW by 10-14 days - exclusive BF can continue, f/u for weight recheck - \>7% ==\> assess for oromotor dysfxn, assess for lactation failure, daily weights, consider supplementing w/formula
269
Breath-holding spell work-up
CBC to eval for anemia
270
Cause of hemophilic arthropathy
-2/2 recurrent hemarthrosis - ==\> hemosiderin deposition ==\> synovitis and fibrosis in joint
271
Cat bite tx/ppx
amox-clav
272
Cause/metabolic consequence of RTA
-defect in H excretion or HCO3 resorption in kidney - ==\> FTT 2/2 chronic, normal anion-gap metabolic acidosis -tx = oral bicarb replacement
273
Niemmann Pick vs. Tay Sachs
-Both: cherry-red macula, loss of motor milestones, feeding difficulties -Tay-Sachs: hyperreflexia, b-hexos deficiency -NP: HSM, areflexia, sphingomyelinase
274
Complication of neonatal heart surgery
-pericardial effusion -aka "postpericardiotomy" ==\> fluid accumulation ==\> distant heart sounds, hypotension, distended jugular veins
275
Scalp swelling limited to one cranial bone in newborn ==\> dx?
-cephalohematoma = subperiosteal hemorrhage -w/in a few hours after birth -most do not require tx -resorb spontaneously w/in a few months
276
Cranial menigocele presentation
-scalp swelling w/pulsations -increased pressure w/crying -evidence of bony defects
277
Caput succedaneum presentation
-diffuse, ecchymotic, swelling of the scalp -can extend across suture lines
278
infant w/intermittent cyanosis during feeding and relief w/crying ==\> dx?
-choanal atresia ==\> inability to pass catheter through the nose -CT: narrowing @ pterygoid plate in post. nasal cavity
279
Herpangina: etiology, age, seasonality, clinical features, tx
-Coxsackie A virus -3-10y -summer/fall -fever, pharyngitis, gray vesicles/ulcers on post. oropharynx -supportive tx
280
Herpetic: etiology, age, seasonality, clinical features, tx
-HSV-1 -6 mo - 5yrs -no season -fever, pharyngitis, erythematous gingiva, clusters of small vesicles on anterior oropharynx -tx: oral acyclovir
281
Strawberry hemangioma characteristics/tx
-composed of capillaries separated by connective tissue -generally observation/reassurance because most regress spontaneously -beta-blockers if disfiguring or dangerous
282
Fixed split S2 ==\> ?
-ASD -fixed split is 2/2 to increased flow in R. heart leading to delay - + SEM 2/2 to increased flow across pulmonic valve
283
Diastolic rumble = ?
increased flow across mitral valve (e.g. eisenmenger syndrome) vs. mitral stenosis
284
Fanconi anemia
-AR -congenital marrow failure -poor growth -morphologic abnormalities (microcephaly, abnormal thumbs, hypogonadism) -macrocytic anemia
285
Acquired causes of aplastic anemia
-drugs (NSAIDs, sulfonamides) -toxic chemicals -idiopathic -viral infections (HIV, EBV) -immune disorders -thymoma
286
fetal macrosomia, umbilical hernia, macroglossia, hemihyperplasia ==\> Dx?
-Beckwith-Wiedemann syndrome -deregulated imprinted gene on 11p -can lead to Wilms tumor/heaptoblastoma -surveillance alpha-feto and abdominal/renal US
287
Diseases that present w/fever, pharyngitis, and oral lesions in young children
-Herpangina (coxsackie A virus) -Herpetic gingivostomatitis (HSV-1)
288
Indications for imaging in headache
1. Ataxia/ coordination difficulties 2. Numbness/tingling 3. Focal Nero signs 4. History of headache awakening from sleep 5. Increasing headache frequency
289
Primary treatment for intussusception
Air enema
290
Most common cause for tinea capitis
trichopytan tonsuran
291
Hodgkinson lymphoma presentation
–teenager? – Lymphadenopathy – Weight loss – Itching
292
Humor shaft fracture leads to?
Radial nerve injury Inability to extend wrist/fingers
293
Treatment for Gerd in a preemie
Fundoplication?
294
Doll face/thin extremities association?
-Glucose – 6 –phosphatase deficiency – Hypoglycemia – Lactic acidosis – Hyperlipidemia – hyperuricemia
295
Risk factors for cerebral palsy
– Prematurity – IUGR –placental pathology
296
Clinical characteristics of cerebral palsy
– Progressive motor dysfunction –spastic VS. dyskinetic VS. ataxic –comorbidities: –intellectual disability – Epilepsy – Strabismus – Scoliosis
