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Uworld Shelf Questions DF > Peds > Flashcards

Peds Flashcards

1
Q

additional risk factors for slipped capital femoral epiphysis

normal age for SCFE

A

endocrinopathies like hypothyroid, GH deficiency, renal failure and radiation history

-these pts usually have bilateral disease and present at earlier age

normally 10-16

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2
Q

TB meningitis

WBC count
glucose
protein

A

wbc = 5-1000

glucose <10

protein >250

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3
Q

what test should be performed at the time of dx of Kawasaki disease

A

echocardiogram

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4
Q

the best diagnostic approach in sx children with rapidly increasing head circumference is what

A

CT scan

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5
Q

what bleeding disorder presents with predominatly easy mucosal bleeding, ecchymoses or petechiae

A

VWd

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6
Q

Foodborne botulism differes from infantile how

A

Prodrome of N/V, abdominal pain and diarrhea

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7
Q

absence seizures

muscle tone?
comorbidities?
treatmetn

A

preserved muscle tone

ADHD
anxiety

ethosuximide

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8
Q

McCune albright syndomre characterized by what

A

Precocious puberty, Pigmentation (cafe au lait spots) polyostotic fibrous dysplaiaand multiple bone defects

associated with endocrine disorders

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9
Q

deficiency of niacin vitamin ____ causes what

A

B3

pellagra = diarrhea, dermatitis, and dementia, can lead to death if sever

can be seen in people with bowel disease that interferes vitamin absorption

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10
Q

classic lab findings in sJIA

A

leukocytosis, thrombocytosis, and elevated inflammatory markers and anemia from chronic inlfmmation

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11
Q

chest x ray: diffuse reticulogranular appearance, air bronchograms, low lung volume

sx

A

RSD

tachypnea
grunting
nasal falring
retractions

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12
Q

complications of prematurity

A 
RSD
PDA
bronchopulmonary dysplasia
IVH
NEC
retinopathy of prematurity
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13
Q

second most common tumor in children and sx

A

medulloblastoma

infratentorial = comit, ha, ataxia

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14
Q

Dx?

Bilateral bulbar palsies (eg ptosis, sluggish pupillary response to light, poor suck and gag reflexes) then descending flaccid paralysis

Tx

A

Botulinism

Tx: botulinism immune globulin

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15
Q

biphasic stridor that improves with neck extension
can also have dsphagia, vomit, difficulty feeding

50% of pts also have what

A

vascular ring

aoritc arch developed wrong and wraps around trachea and esophagus

50% of pts have VSD or TOF

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16
Q

if untreated splenic sequestration can progress to what

lab findings

A

hypovolemic shock bc lots of total blood volume trapped in the spleen

normocytic anemia, elevated reticulocyte count, thrombocytopenia

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17
Q

newborns are born with a larger RV compared to LV bc the RV pumps systemic blood via the PDA which appears as what deviation and what waves

A

physiologic right axis deviation and R waves in V1-V3

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18
Q

girl with turner syndrome and bones

A

decreased bone mineral density bc of no estrogen

increased risk of osteoperosis

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19
Q

another name for osgood schlatters disease

path

pe

tx

A

traction apophysitis

periods of rapid growth in which quads tendon puts traction on apophysis of the tibial tubercle where patellar tendon inserts

PE: edema and tenderness over tibial tubercle
reproduce pain by extending knee against resistance

rest and nsaids

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20
Q

____ should be suspected in any pt with continued ear drainage for several weeks despite appropriate antibiotic therapy

description

can lead to what ominous things

A

cholesteatomas

retraction pocket in the tympanic membrane, which can fill with granulation tissue and skin debris

hearing loss and life threat brain abscesses or meningitis

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21
Q

when do you transfuse bili baby

A

at 20-25 total bili

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22
Q

if body is pink in newborn but extremities or cyanotic is this normal?

A

yes

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23
Q

when are parents not allowed to make medical decsiion for child

A

when there is a life saving treatment available for the child

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24
Q

intrinsic pathway factors and what pt or ptt

A

aptt

XII, XI, IX, VIII, X

extrinsic pt
7 and 10

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25
Q

abduction and internal rotation of hip limited in what disease

A

LCP

ABIR limited in LCP

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26
Q

x linked agammaglobulinemia

cx manifestations
dx
tx

A

cx: recurrent sinopulmonary and GI infections after age 6
absence of lymphoid tissue on exam (tonsils LNs)

dx: decreased Igs and B cells, normal T cells, no response to vaccines
tx: IG replacement therapy, prophylactic antibiotics if severe

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27
Q

____ is used to diagnose urinary refulx, what pt would need this

A

voiding cystourethrogram

pts with recurrent urinary tract infections

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28
Q

abnormal sexual behavior in preadolescents

A

repeated object insertion into vagina or anus

sex play

force threats or bribes in sex play

age inappropriate sexual knowledge

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29
Q

cyanosis, and dark chocolate colored blood
standard pulse ox readings are low

normal arterial partial pressure of oxygen**

> 5% difference btwn oxygen saturation on pulse ox and ABG

A

methemoglobenimea

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30
Q

methemoglobinemia is most commonly acquired after what

physiology of it or biochem of it not sure which subject it is but who cares

A

excessive exposure to an oxidizing agent like

dapsone, nitrites, and local/topical anesthetics

Fe2+ iron gets oxidized to ferric Fe3+ which has decreased affinity for oxygen but the remaining 3 ferrous sites have an increased oxygen affinity which leads to decreased oygen delivery to peripheral tissues

due to poor oxygen affinity of methemoglobin O2 supplementation does not help

bc arterial blood gas testing analyzes unbound arterial oxygen the PaO2 is normal and overestimates degree of true oxygen saturation

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31
Q

abnormal social development and extreme behavioral rigidity, with onset in early development. higher functioning individuals with normal language and intellectual ability may present later when deficits become more paparent with increased social demands

A

autism

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32
Q

what age group would you find breath holding spells

A

6 months to 6 years

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33
Q

strawberry hemangioma is also what name

tx

A

superficial hemangioma

regress spontaneously

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34
Q

what should be suspected in a cyanotic infant with left axis deviation and small or absent R waves in the precordial leads?

what is needed for survical

A

tricuspid valve atresia
lack of communication btwn right heart chambers = hypoplastic right ventricle and diminished RV forces on ECG

the lack of blood flow to RV and pulmonary outflow tract = underdevelopment of pulmonary valve and or artery = decreased pulmonary markings on chest x ray

associated ASD and VSD are needed for survival

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35
Q

gaucher disease is due to ___ deficiency

cx features

loss of milestones?

A

glucocerebrosidase def

HAT

HSM, anemia, thrombocytopenia

no loss of milestones or red macula

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36
Q

neonatal treatment for RSD

A

maternal antenatal glucocorticoids

continuous ppv

intubation if severe

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37
Q

what disease gives a maculopapular polymorphous rash after administration of ampicillin or amoxicillin

A

EBV

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38
Q

chest xray for transient tachypnea of newborn

path

resolves when

A

bilateral perihilar linear streaking

think liquid bc alveolar fluid clearance inadequate

resolves by day 2 of life

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39
Q

if pt with sinus infection gets periorbital edema, vision abnormalities or AMS then what management step

A

CT

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40
Q

cholesteatoma causes

sx
what you see

complications

A

genetic (younger pt)
acquired usually occur secondary to chronic middle ear disesease

cont ear drainage for sev weeks despite approp abx therapy

granulation tissue and skin debris

lead to hearing loss, CN palsies, vertigo, life threat brain infections

*new onset hearing loss or chronic ear drain with abx therapy

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41
Q

when a radiolucent foreign body is suspeected and not visualized on x-ray, ____ can be considered as the next diagnostic procedure

A

CT

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42
Q

which kind of cerebral palsy is most common in preterm infants

cx features

resistance to what

A

spastic diplegia

hypertonia, and hyperreflexia mainly LEs with both feet pointing down and inward (equijnovarus deformity)

resistance to passive muscle movement

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43
Q

optic pathway glioma in what dissease in children

A

NF type 1

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44
Q

colon in meconium ileus

A

narrow, underdeveloped colon (microcolon)

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45
Q

hurler syndrome is due to what deficiency?

age presentation?

cx features?

A

lysosomal hydrolase deficiency

age 6 months to 2 years

coarse facial features, inguinal or umbilical hernias, corneal clouding, and HSM

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46
Q

diagnostic features of acute bacterial rhinosinusitis

A

persistent sx 10 days of more without improvement
or
severe sx with 102 fever, purulent nasal discharge or face pain 3 days or more
or
worsening sx 5 days after initially improving viral URI

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47
Q

child with conjunctival injection, tarsal inflammation, and pale follicles are concerning for what

types

spreads in what conditions

what kind of conjunctivitis

concomitant what

last thing real bad

A

trachoma

chlamydia trachomatis serotypes A B and C which is leading cause of blindness worldwide

spreads in crowded conditions

follicaular conjunctiivits

concomitant nasophayngeal infection

scarring of eyelids

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48
Q

celiac disease extraintestinal sx

A

fatigue
IDA from poor absorption 2ndary to duodenal villous atrophy
dermatitis herpetiformis located on knees, elbows, forearms, and buttocks

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49
Q

legg clalve perthes disease is what and affects what pop

A

5-7 yrs in the LCP

idiotpathic AVN of hip

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50
Q

erythema tocicum neonatorum

A

healthy baby and asx papules and pustules
common in full term neonates
spares palms and soles in first 2 weeks of life

goes away on own

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51
Q

treatment for severe pneumonia in CF pts

A

empritic antistaph antibiotics like Cefepime (covers MSSA and psuedomonas)

MRSA coverage with IV vancomycin

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52
Q

treatment of sJIA

A

glucocorticoids, NSAIDs, biologic agents

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53
Q

NF1 inheritance what gene, what protein what cx features

A

AD
NF1
neurofibromin
cafe au lait macules, freckling and lisch nodules
low grade optic pathway glioma in 15% of pts under 6

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54
Q

ependymoma

what cells, location

sx

A

glial cell tumor that arise in ventricles and spinal cord 4th ventricle in posterior fossa common in children

tumor expansion leads to obstruction of CSF and increased ICP

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55
Q

asthma pts with respiratory failure despite escalation of medical treatment require

A

endotracheal intubation and mechanical ventilation

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56
Q

risk factors for cerebral palsy

A 
prematurity
IUGR
Intrauterine infection
antepartum hemorrhage
placental path
multiple gestation
maternal alcohol consumption and tobacco
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57
Q

disesae with low IgM IgA IgG in the normal B lymphocyte count

A

common variable immunodeficiency

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58
Q

what increases HCOM murmur

what inheritance and pop

A

valsalva bc decreases preload and abrupt standing and nitroglycerin

AD in blacks

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59
Q

how can OM with effusion be distinguished from AOM

A

om with effusion has lack of acute inflammaotry signs

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60
Q

ewings sarcoma

common sites

metastasis

age

appearance

A

metaphysis and diaphysis of the femur, followed by the tibia and humerus

lungs and LNs

white males, 0-20

lamellated appearance, o onion skin periosteal rxn, lytic cental lesion
moth eaten appearance

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61
Q

midgut volvulus classically presents in what pop and how

A

neonate under 1 month with bilious vomiting

can cause bloody stools, bowel perforation, abdominal distension and peritonitis

signs of ischemia or systemic decompensation are indication for emergency laparotomy

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62
Q

universal screening for dyslipidemia in children at what age

A

9-11 and 17-21

pre and post puberty

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63
Q

the thymus is normally visible on chest x rays in children under what age

A

3

sail sign bc looks triangular, scalloped and dense

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64
Q

dx pertussis

tx

A

pertussis culture or PCR
lymphocyte predominant leukocytosis

macrolides

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65
Q

bartonella henselae cx manifestations
tx

type of bacteria

A

papule at scratch/bite site
regional adenopathy
+/- fever of unknown origin for 14 days or more

azithromycin

gram neg bacillus

affected lns are enlarged, tender and have overlying erythema

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66
Q

cyanosis and respiratory distress during feeding that improves when infant cries

A

bilateral choanal atresia

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67
Q

dermal melanocytosis aka what occurs in what pop where are they located, what do they look like nad tx

A

mongolian spot

lower back and buttocks

native americans, africans, asians, hispanices

fades spontaneously

look like brusies but bruises are more varied in color and fade quickly

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68
Q

infected infants of toxo should receive what

A

pyrimethamine, sulfadiazine, and folate for a year

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69
Q

hepatitis B pt can have what renal disease

A

membranous nephropathy

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70
Q

pts with galactoinase deficiency present with what

A

cataracts only

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71
Q

evaluation of primary amenorrhea

A

pelvic US: uterus present—> serum FSH

  • increased = karyotype
  • decrease = cranial MRI

US: uterus absent—>karyotype serum testosterone too

  • if 46 xx and normal test = abnormal mullerian devel
  • if 46, xy and normal test level = AIS
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72
Q

Chronic granulomatous disease results from what

susceptible to what organisms

test

A

inability of phagocytes to produce hydrogen peroxide in their lysosomes

abscesses due to fungi or catalase positive bacteria like s aureus, serratia, burkholderia, aspergillus

dihydrohodamine 123 test
nitroblue terazolium test

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73
Q

idiopathic precocious puberty

gender
LH and GnRH

A

girls

basal levels of LH elevated and wil increase with GnRH stimulation

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74
Q

dx of Kawasaki:

A

pt should have fever for 5 consecutive days as well as 4 of 5 of the following

1) conjunctivitis, spares limbus
2) oral mucosal changes with erythema, fissured lips, strawberry tongue
3) rash
4) extremity changes, erythema, edema, desquamation of the hands and feet
5) cervical LAD: >1.5 cm, usually unilateral

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75
Q

major criteria acute RF

A

JONES

joins (migratory arthritis)
O (carditis)
nodules (subcut)
erythema marginatum
sydenham chorea
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76
Q

iron poisoning dx

A

iron is radiopaque and can be visualized on abdominal xray

aspirin similar but doesn’t show up on xray

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77
Q

truncus arteriosus is strongly associated with what

A

Digeorge syndrome

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78
Q

____ is characterized by severe pain, pallor, poikilothermia, paresthesias and late findings of pulselessness and paralysis

A

comparment syndrome

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79
Q

chronic oligoarthritis, daily fever and rash in child

what kind of disease

almost all pts with it present with what

A

systemic onset juvenile idiopathic arthritis sJIA

auto-inflammatory

2 weeks of fever occuring once a day, arthritis of 1 or more joints and a pink macular rash that worsens with fever

joint pain worse in morngin and better as day goes on

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80
Q

clinical manifestations of late onset CAH aka non classic

A

premature adrenarche/pubarche, severe chystic acne resistant to treatment, accelerated linear growth, advanced bone age

normal electrlyts

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81
Q

nephrotic syndrome in a pt with hepatitis B infection what kind

A

membranous nephropathy

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82
Q

posterior fossa tumor typically presents with what

A

cerebrellar dysfunction (ataxia, dysmetria)

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83
Q

how does neonatal polycythemia effect hematocrit which effects other stuff

A

hematorcrit rises with plycythemia which increases viscosity of the blood and impairs blood flow to various organs

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84
Q

celiac disease is associated with what other diseases and should be screened for in these ptws

how to screen

A

DM1 and thyroiditis

screen with anti-tissueTG antibody IgA followed by endoscopic duodenal biopsy for conifrmation

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85
Q

gold standard for testing for duchennes muscular dystrophy and what is finding

A

genetic testing
-will find muscle biopsy with absent dystrophin

deletion of dystrop[hin gene on Xp21

x linked recessive

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86
Q

infant or newborn with failure to thrive, bilateral cataracts, jaundice and hypoglycemia has what

pts at increased risk for what infection

tx

A

galactosemia from galactose-1-phosphate uridyl transferase deficiency (GPUT)

increased blood galactose levels

increased risk for E coli

elimante galactose from diet

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87
Q

angiokeratomas, perpiheral neuropathy, and asx corneal dystrophy

renal and heart failure possible to and risk thromboembolic events

A

fabrys diseae

FABRY ball CAP (corneal dys, angioker, periph neuorp)

KC (kidney and cardiac failure)

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88
Q

otitis externa

-micro infection
clinical manfest
tx

A

pseudomonas
staph aurues
-swimmers ear

pain with auricle manipulation, pruritus, discharge

tx: topical antibiotic (fluoroquinolone
topical glucocorticoid

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89
Q

testicles that have not descended by age what are unlikely to descend spontaneously and require surgery at the optimal age of what (orchiopexy)

A

6 months

1 year

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90
Q

beckwith wiedemann syndrome

pathogenesis (what chrom)
PE
complications
survellance

A

deregulation of imprinted gene expression in chrom 11

PE: fetal macrosomia, rapid growth unitl late childhood

  • omphalocele or umbiical hernia
  • macroglossia
  • hemihyperplasia

complications: wilms tumor or hepatoblastoma

surveillance: AFP and abdominal/renal US
monitor hypoglycemia, which is transient

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91
Q

treatment for HSP

A

supportive with hydration and NSAIDs for most pts

hospitalization and systemic glucocorticoids in pts with severe symptoms

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92
Q

orbital cellulitis

location, bugs, clinical manifestations

A

posterior to orbital septum

staph oaureus, strep pneumo and other streptococci

possible abscess within the orbit or brain, blindness, or sinus venous thrombosis

ophthalmoplegia, pain with extraocular movements, proptosis, virison impairment

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93
Q

inherited forms of QT prolongation and inheritance

sx

tx

standard
mild sx
sx with history of syncope

A

jervell lange nielsen AR

romano ward syndome AD

syncope, life threatening ventricular arrhytmias like torsades and sudden death

avoid exercise, certain meds, normal calcium, K+, and Mg+

beta blockers like propranolol shorten QT

if sx with history of syncope then need b blocker and pacemaker placement

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94
Q

treat diamond blackfan syndrome

A

corticosteroids, and transfucsion if needed

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95
Q

biliary atresia presents when and with what

A

early infancy with obstructive jaundice and acholic stools and is fatal without intervention

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96
Q

guillain barre syndrome pathology

sx

final stage

accompanying sx

tx

A

demyelination of the peripheral motor nerves

ascending weakness accompanied by feet tingling and neuropathic pain

final stage is flaccid paralysis with absent DTRs and nerve conduction velocities

accompanying autonomic sx can occur

pooled IG or plasmapheresis

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97
Q

carotid pulse with a dual upstroke is what

what other murmur

A

HCM

and also systolic ejection murmur along LSB with strong apical impulse bc LVOT

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98
Q

erythema multiforme is seen most commonly in what infection

A

herpes simples

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99
Q

all infants, regardless of maternal screen results should receive what for eyes and infections

A

topical prophylaxis within an hour of birth

prevents gonococcal disease

erythromycin ointment

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100
Q

maternal estrogen effects in mnewborns

A

breast hypertrophy in boys and girls

swollen labia

physiologic leukorrhea

uterine withdrawal bleed

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101
Q

medulloblastoma occurs where and causes what

A

posterior fossa in children

infratentorial in cerebrellar vermis and presents with vomiting, HA, ataxia

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102
Q

0758 and 590

A

reveiew

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103
Q

juvenile idiopathic arthritis cx features

poly vs oligo

which joints involved

A

symmetric arthritis for at least 6 weeks

polyarticular is 5 or more joints
oligoarticular is less than 5

knees, wrists, ankles, cervical spine and joints in hands and feet

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104
Q

what is a side effect of hydroxyurea (major)

A

myelosuppression, predisposes pt to infection

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105
Q

c trachomatis neonatal conjunctivits occurs when and what type of discharge

A

5-14 days after birth, watery discharge

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106
Q

granulosa cell tumor of the ovary cx featuers in child

A

precocious puberty

increased estrogen

pelvic US shows ovarian mass, thickened endometrium

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107
Q

edwards syndrome clinical features

head

limbs

organs

A

micrognathia

prominent occiput

low set ears

clenched hands with overlapping fingers

renal defects

heart defects (VSD)

limited hip abduction

rocker bottom feet

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108
Q

inspiratory stridor that worsens while supine

what helps

A

laryngomalacia

prone helps

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109
Q

marfanoid habitus has which direction displacement of lens? how about homocystinuria?

what protein?

inheritance?

A

upward (homocystinuria is AR that has displacement down)

fibrillin 1

AD

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110
Q

is prophylaxis recommended for all close contacts for pertussis

A

yes, use macrolides even if vaccinated

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111
Q

11B hydroxylase def

A

same as 21 but no salt wasting, have HTN and salt retention

from increased 11 deoxycorticosterone

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112
Q

normal features of LNs

feels
mobile or not mobile
size

A

soft
mobile
under 2 cm

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113
Q

cx presentation of measles

A

prodrome of cough coryza, conjunctivitis fever, koplik spots

maculopapular rash that starts head and goes down

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114
Q

wet diapers in neonates

A

how ever many days old is how many wet diapers should be up to first week.
after first week should have 6 or more

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115
Q

due to its location, supracondylar humerus fractures may be complicated by ___ injury or ____

A

neurovascular injury or compartment syndrome

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116
Q

prolonged pt and aptt in newborn from what

A

vitamin k deficiency

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117
Q

21 hydroxylase deficiency hormonal abnormalities

sx

A

decreased aldosterone and cortisol
increased testosterone
increased 17 0h progest

sx: ambig genitals in girls, salt wasting with hypotension and decreased na and increased K

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118
Q

caustic injury first and scond steps

A

remove contaminated clothing and then do upper GI endoscopy within 24 hours

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119
Q

type of strabismus that usually arises in children age <2 and is associated with eye deviation when trying to focus on objects

A

accommodative esotropia

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120
Q

causes of edema in turner syndrome pt

A

congenital lymphedema from abnormal development of the lymphatic netowrk

accumulation of pretein rich interstitial fluid in hands, feet and neck (webbed neck)

severe obstruction of lymphatic vessels can lead to cystic hygroma of neck and fetal hydrops

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121
Q

The most common congenital heart malformation is what

Murmur?

A

VSD

Holosystolic murmur at left lower sternal border

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122
Q

adolescent with a solitary breast mass most likely has what

what kind of tenderness is common

management

A

FAD

premenstrual

observation in adolescents
-check one cycle later then if decreased reassure
US for a persistent mass or older pt

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123
Q

using ___ in pregnancy causes long smooth philtrum, small palpebral fissures and thin upper lip and also ____

A

alcohol

CNS abnormalties like mental retardation

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124
Q

infants of diabetic mothers second trimester problems

A

fetal pancreas sufficient so now increased storage causes organomegaly, incrased GF (macrosomia) and increased oxygen consumption (polycythemia)

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125
Q

what is a GnRH stimulation test used for

A

evaluation of precocious puberty

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126
Q

pts with known or suspected measles should be what

when to give vaccine

A

isolated and placed in airbrne precautions

2 doses (age 1 and 4) give btwn age 6 and 11 momths if suspected travel

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127
Q

truncus arteriosus strongly associated with what syndrome and sx

A

digeorge

neonatal cyanosis, heart failure and systolic ejection murmur with a loud ejection click at LSB

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128
Q

anatomical injury mech with shaken baby syndrome

sx

dx

A

repetitive acceleration-deceleration forces cause shearing of the dural veins and coup-contrecoup injury with brain impact on the skull

subdural bleed can manifest as seizures, increased head circumference, bulging anterior fontanelles and AMS

also causes vitreoretinal traction and RETINAL HEMORRHAGES (pathognomonic)

dx: CT without contrast and skeletal survey

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129
Q

inattentive staring spell

A

occurs during boring activity

variable length over 1 minute

respond to stimulus

no automatisms

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130
Q

intussusception presents how

dx

tx

A

intermittent periodic pain associated with drawing the legs up toward the abdomen, emesis may follow

sausage shaped mass in RUQ

US to detect, use air enema to treat

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131
Q

what can cause neonatal polycythemia

A

increased EPO from intrauterine hypoxia: maternal diabetes, hypertension, or smoking: IUGR
pre-eclampsia

delayed cord clamp, t-t transfusion
hypothyroidism or hyper, trisomies

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132
Q

juvenile idiopathic arthritis laboratory findings

A

elevated inflammatory markers
hyperferritenemia, and hypergammaglobulinemia
thrombocytosis
ANEMIA (chronic inflammation and IDA), inflammatory markers increase hepcidin which prevents absoprtion of iron

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133
Q

milk or soy protein induced proctocolitis

pt pop

sx (triad)

from what

tx

A

infants

non IgE immune respone to proteins in formula or breast milk that cause rectal and colonic inflammation

eczema, regurgitation or vomit and painless bloody stools

mom elimate all dairy and soy from diet when breast feeding

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134
Q

evaluation of bilious emesis in the neonate

first thing you do then next thing

A

stop feeds
NG tube decompression
IV fluids

abdominal x-ray

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135
Q

positive nikolsky sign
erythema on face and generalizes within 24-48 hrs
superficial bullous

A

SSSS

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136
Q

xrays for osteosarcoma show what

A

mix of radiodense and radiolucent areas

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137
Q

weird GI thing that can happen in HSP

location

dx

tx

A

intussusception

small bowel or ileo ileal

dx by presence of a target sign on ulstrasound
-ileal ileal cannot usual be fixed with air enema and need surgery

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138
Q

bedwetting is normal before age what

A

5

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139
Q

treatment for

umbilical hernia
gastroschisis
omphalocele

A

umbilical hernia: monitor for spontanseous resolution by age 5

other 2 need immediate surgery after birth

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140
Q

polyhydramnios and vomiting with inital feeds with no RD

abdomen not distended and intestinal gas absent on xray

A

duodenal atresia

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141
Q

if have cryptorchid gonads what should you do in AIS

A

perform gonadectomy after puberty

can become dysgerminoma, gonadoblastoma after puberty

still want testosterone for normal height so do not removed till after puberty

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142
Q

breastfeeding failure jaundice

timing
pathophys
cx features

A

first week of life

lactation failure results in decreased billy elimination, increased enterohepatic circulation of billy

suboptimal breastfeeding and
signs of dehydration (brick red urate cystals, decreased urine output and losing weight)
jaundice

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143
Q

in congenital diaphragmatic hernia what is next step hfter airway has been secured

A

nasal or orogastric tube to provide continuous suction and prevent bowel distension against the lungs

also umbilical arterial line and venous cath should be placed

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144
Q

evaluation of primary amenorrhea steps

1) ___ or ____
if uterus absent then what

A

pelvic exam or US

uterus absent

karyotype and serum testosterone

  • 46 xy = AIS
  • 46 xx = Abnormal mullerian development
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145
Q

howell jolly bodies are what and seen in what

A

nuclear remnants within red blood cells that are typically removed by the spleen

single round blue inclusions on wright stain

presence of them indicates physical absense of spleen or functional hyposplenism due to splenic autoinfarction or splenic congestion

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146
Q

birth weight and loss in neonates

A

may lose up 7% of birth weight in first 5 days and it should be regained by age 10-14 days

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147
Q

differential for lytic bone lesion in a child

if have hypercalcemia narrows to what

A

infectious osteomyelitis

endocrine: hyperparathroid oseitis fibrosa cystica
neopalstic: ewing, langerhans cell histocytosis, metastasis

idiopathic

narrow to lytic bone neoplasm or hyperpatathyroid state (pituitary adenoma most common but occurs in pts over 50)

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148
Q

what kind of seizures have automatisms

length of these seizures?

A

absence seizures

last under 20 seconds

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149
Q

cx features of biliary atresia

A

well appearing at first followed by development of the following over 1-8 weeks

jaundice, acholic stool or dark urine, hepatomegaly, increased hyperbili, mild elevation transaminases

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150
Q

tx for AOM

A

initial is amoxicialin

2nd line is amox clav

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151
Q

evaluation of bilious emesis in the neonate

get abdominal x ray and see no evidence of free air and the bowel gas pattern is not suggestive of duodenal atresia or distal obstruction then

A

upper GI series (barium swallow)

fastest way of diagnosin midgut volvulus with malroation

ligmanet of treitz on right = malrotation

corkscrew pattern = volvulus

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152
Q

uric acid crystals in diapers with pink stains or brick dust in neonatal diapers abnormal?

A

no this is normal

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153
Q

most appropriate step in a 1 month old with VSD

A

echo to assess the size

if small spont close by age 2 usually with no long term sequelae

large defects have softer murmur bc of leess turbulence
-eisenmenger syndrome

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154
Q

pt with turner syndrome are at risk for decreased what due to what

A

bone mineral density bc lack estrogen

increased risk of osteoporosis

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155
Q

atresia of the jejunum or ileium is thought to occur due to what

sign

A

vascular accident in utero

vasoconstritive meds like coaine and tobacco

sign is triple bubble sign and gasless colon on xray

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156
Q

most common bufs for AOM

A

strep pneumo
H influeznae
moraxella catarrhalis

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157
Q

hemoglobin SC

A

type of sickle cell where you inherit C and S

similar complications like pain crises as SS but less often and less severity

splenic sequestration/infarction happens in adolescence rather than childhood

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158
Q

is lymphedema in turners pitting or non pitting

A

non pitting

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159
Q

treatment for radial head subluxation (nursemaid’s elbow)

A

hyperpronation of forearm or supination of forearm and flexion of elbow

160
Q

classic presentation of slipped capital femoral epiphysis

A

metaphysis and proximal femur slip relative to epiphysis at epiphyseal plate

obese adolescent male with complaints of pain

161
Q

kallman syndrome is what inheritance and cx features

A

x linked recessive

normal genotype

absence of GnRH = short, and delayed or absent puberty
amenorrhea, absent breast development
anosmia hyposmia

162
Q

most children are ready to begin toilet training at age what

A

2 or older

163
Q

laboratory findings in hemophilia A and B

PTT
platelet
bleeding time
PT

A

PTT prolonged

normal platelet, bleeding time and PT

164
Q

vesicoureteral reflux

grade I
II
III
IV
V
A

I: into nondilated ureter
II: into pelvis and calyces without dilation
III mild to mod dilation of ureter, renal pelvis and calyces with min blunting of fornices

IV moderate ureteral tortuosity and dilation of pelvis and calyces
V: gross dilation of ureter, pelvis and calyces, loss of papillary impressions, ureteral tortuosity

165
Q

management of primary nocturnal enuresis

which is most effective long term intervention of these

A 
lifestyle change
enuresis alarm (most effective long term intervention)
UA to r/o secondary causes

Desmopressin therapy if above does not work

TCAs only if desmopressin does not work bc of AEs

166
Q

painful vesicular rash with punched out erosions and hemorrhagic crusting

A

eczema herpeticum

superimposed primary herpes simplex virus infection

open area that is exposed to HSV type 1 can develop painful vesicles with erythemaouts base that evolve to punched out erosions with hemorrhagic crusting

167
Q

pt has abnormal uterine bleeding defined as what during adolescence. what causes it

A

menstrual bleeding under 21 days or over 45 days apart

immaturity of HPO axis fails to produce correct ratios of gonadotropin releasing hormone and therefore LH and FSH to induce ovulation

first few years post menarche, majority of menstrual cycles are anovulatory and present as painless, irreg, heavy bleeding

no progesterone in anovlulatory cycles

168
Q

normal sex beahvior in preadolescents: toddlers

A

EMU

explore own or other genitals
masturbate
undressing self or others

169
Q

purple or red eardrum +/- bulging

A

hemotympanum

170
Q

chest radiograph sowing diffuse reticulogranular pattern and air bronchograms is what

A

RDS

171
Q

optic pathway glioma found in what pts

A

NF1

can cause decreased visual acuity, alterations in color vision, optic nerve atophy and proptosis

172
Q

transient synovitis typically presents in what pop and cx features

tx

follows what
on exam hip is what

A

kids 2-8 with restricted ROM of hip joint, resolves with conservative therapy

follows viral infection or mild trauma

hip flexed, slightly abducted and externally rotated
FABER Tran Sexuals

173
Q

complications of prader-willi syndrome

feeding pattern: infancy and later

A

sleep apnea
DM
gastric distension/rupture
death by choking

poor suck and feeding problems in infancy followed by a life of compulsive binge eating

174
Q

acute lymphoblastic leukemia

other features with it

peripheral smear

degree of what

diagnostic

peroxidase positive granules

positive for what

A

30-50% pts present with infections
1/2 have splenomegaly of LAD
lymphoblasts on perioph smear
vary degree of anemia, neutropenia, thrombocytopenia

presence of more than 25% lymphoblasts in BM diagnostic

no peroxidase posigve granules

PAS positive

TdT positive

175
Q

fungal infection of scalp that causes pruritc patchy, fine, white scales that may resemble SD

does not involve what and not age

A

tinea capitits
no eyebrows or nasolabial folds
not common first year of life

176
Q

fanconi anemia

blood stuff
age

clinical sx

A

progressive pancytopenia and macrocytosis
age 8 is average age

cafe au lait spots, microcephaly, microphthalmia, short stature, horseshoe kidneys and absent thumbs

177
Q

what is the most common causes of chronic renal insufficiency/failure in children and occurs in what population

anatomy

A

posterior urethral valves causes obsturction

distended bladder, ureters and hydronephrosis

only in boys

178
Q

supratentorial tumor presents with what

A

seizures, weakness, and sensory changes

179
Q

minor criteria in acute RF

A

P FACE

Prolonged PR

Fever
Arthralgias
CRP up
ESR up

180
Q

what kind of blood penia should raise concern for malignancy

A

thrombocytopenia

181
Q

comorbidities with cerebral palsy

A

intellectual disability
epilepsy
strabismus
scoliosis

182
Q

congenital toxoplasmosis

what risk factors

A

undercooked meat
unwashed fruits/veggies
cat feces

cx: macrocephaly, intracranial calcifications, jaundice, HSM, blueberry muffin spots

183
Q

congenital diaphragmatic hernia is what

__ from esophag compresion
PE
tx

A

herniation of abdominal viscera into chest making pulmonary hypoplaia and PHTN

85% of cases on left

polyhydramnios can occur from esophageal compression

causes concave abdomen and barrel shaped chest on PE

emergecny intubation and cautious ventilation

184
Q

help preventing SIDS

A 
smoke avoidance during and after pregnancy
supine sleep
firm sleep surface
room sharing but not in same bed
pacifier use when sleeping
185
Q

PE with slipped capital femoral epiphysis

A

loss of abducrtion and internal rotation of the hip as well as external rotation of the thigh while the hip is being flexed

186
Q

PE: webbed neck, cleft lip, shielded chest, triphalangela thumbs, pale mucous membranes and conjunctivae

what else on labs

systolic ejction murmur

A

diamond blackfan anemia

macrocytic anemia, and low retic count
-no hypersegmented neutrophils

187
Q

osteonecrosis is a common complication of what disease

A

SCD

groin, buttock, or thigh pain that initially occurs with weight bearing but progresses to occuring at rest

pain and limited ORM with INTERNAL roation and abduction of hip

no erthemia or warmth of jionts, leukocytosis or elevated inflammatory markers

xrays of hip may appear normal early and MRI should be performed to confirm diagnosis

188
Q

what is the most common congenital heart defect in pts with down syndrome?

from what embryo stuff

what happens with blood flow

sx with feeding

A

complete atrioventricular septal defect
endocardial cushions don’t merge so both VSD and ASD

HF occurs from blood mixing and valve regurge = volume overload and excessive pulmonary blood flow

diaphoresis/dyspnea with feeds and crackles around age 6 weeks

189
Q

prophylaxis for measles

A

vitamin A

190
Q

what other workup with TE fistula

A

VACTERL

vertebral, anal atresia, cardiac, TE fistula, renal, limb

191
Q

heinz bodies are what and seen in what disease

A

hemoglobin precipitation is seen in G6PD deficiency

hemoglobin becomes oxidized and forms insoluble precipitants called heinz bodies

192
Q

light microscopy findings with minimal change disesae

tx

IF staining?

electron microscopy?

A

normal

tx: corticosteroids

no abnormality

diffuse effacement of foot processes on EM

193
Q

tuberous sclerosis associated with what

A

cortical tubers, hamartomas, subependymal giant cell astrocytomas, subep nodules

cardiac rhabdomyomas, renal angioleiomyomas, seizures, metbal retartdation
facial angiofibroas

194
Q

what is a complication of IVH in 1/3 of cases

those who survive may have what

A

communicating hydrocephalus

cerebral palsy if survive

195
Q

when does type 1 DM present

A

4-6 or at early puberty

196
Q

evaluation of bilious emesis in the neonate

get abdominal x ray and see double bubble sign then what is next

A

it is duodenal atresia

197
Q

intellectual disability, fair complexion, eczema and ___ body odor is what

A

musty

PKU

198
Q

who is at risk for meconium aspiration syndrome

A

neonates born after due date

199
Q

precocious puberty is defined as development of 2ndary sex characteristics before age what

A

8 in girls
9 in boys
accelerated bone growth is also comon

central
early HPO activation

peripheral: low fsh and lh
- from adrenal or gonadal relaease of excess sex hormones

200
Q

point tenderness at the inferior pole of the patella

A

patellar tendonitis

201
Q

medically emancipated minor

A

emergency care, sti, substance abuse, prenancy, contrapception

202
Q

drooling,tripod position (sitting up and leaning forwrd), sniffing position, stridor, high fever, dysphagia what infection

managment

A

h influenza type B

endotracheal intubation

antibiotics

203
Q

treatment for chlamydial neonatal conjunctivitis

A

PO macrolide

204
Q

congenital diaphragmatic hernia presents how and imaging

A

cyanosis and RD immediately after birth
polyhydramnios can occur crom esophageal compression

abdominal viscera into thorax shows schaphoid appearing abdomen

xray: displaced cardiac silhoutte, bowel in thorax and gasless abdomen

205
Q

why is human milk better than formula

A

it absorbs better and improves gastric emptying

  • 70% whey and 30% casein
  • whey digested easier

actually has less vitamin D so need to supplement with it if breast feeding

206
Q

emancipated minor

A

homeless, parent, married, military, financially independent, high school grad

207
Q

treatment of slipped capital femoral epiphysis

A

surgical pinning of the femoral head

208
Q

physiologic jaundice should be gone when

A

after 1-2 weeks

209
Q

question 9 on central vs peripheral precocious puberty

A

question 9

210
Q

evaluation of bilious emesis in the neonate

get abdominal x ray and see NG tube in misplaced duodenum then what next

what procedure

A

upper GI series

if see ligament of treitz on right side of abdomen then

maltoration

need surgery with Ladd procedure

211
Q

in pediatric pts the most common etiology of osteomyelitis is what

A

hematogenous seeding by s aureus

212
Q

enzyme def in niemann pick

A

sphingomyelinase

213
Q

definition of neonatal polycythemia

A

hematocrit over 65% in term infants

214
Q

what can cause subcutaneous emphysema with leaking from chest wall into subcut tissues

what else can occur this way

managment

A

severe coughing paroxysms

pneumothorax can occur this way too

get chest xray

215
Q

what should be obtained in all neonates with suspected sepsis and tx how

A

CSF culture

antibiotic therapy

216
Q

what is the best treatment for breastfeeding failure jaundice in otherwise healthy full term newborns

A

increase freq and duration of feeds to stimulate milk production, maintain hydration

breast feed every 2-3 hours for 10-20 mintues or more per feed in first month of life

217
Q

cx features adhd sx occur where

tx

A

sx must occur in at least 2 settings

tx: stimulants: methylphenidate, amphetamines
nonstimulants: atomoxetine, a-2 agonist
behavioral therapy

218
Q

treatment for neonatal polycythemia

A

IV fluids
glucose
partial exchange transfusion

219
Q

refeeding syndrome happens in what pts

A

anorexic

220
Q

what does a white reflex in the eye mean

metast where

A

aka leukocoria
considered retinoblastoma until proven otherwise, child needs prompt referral to an ophthalmologist

liver and brain metastases

221
Q

dry flaky peeling skin of the hands and feet of newborn problem?

A

not a problem, this is normal

222
Q

pertussis tx for close contact

under 1 month

1 month or older

A

under 1 = azithromycin 5 days (erythromycin can cause pyloric stenosis and clarithromycin not proven safe)

1 month or older

azithromycin 5 days or clarithromycin 7 days or erythromycin 14 days

223
Q

most common cause of hypothyroidism in infants

A

thyroid dysgenesis (hypoplasia, aplasia)

224
Q

monocular visual acuity assessment by snellen letters or numbers can begin when

A

at age 3

225
Q

iron poisoning sx

A

abdominal pain, NVD and hematemesis within 30 min to 6 hrs of ingestion

vasodilator too, so hypotensive shock and anion gap metabolic acidosis from poor perfusion

gastric scarring and pyloric stensosis can occur

226
Q

pts with complement def are at increased risk for what infections

A

disseminated bacterial with encapsulated bacteria, giardia wouldn’t effect these pts worse

227
Q

when does pyloric stenosis most commonly present

A

at age 3-6 weeks with nonbilious projectile vomiting

228
Q

esophageal coins in asx pts

what if sx or time of ingestion unknown

A

observe for up to 24 hrs

flexible endoscopy

229
Q

tx for hemophilia A and B

A

factor VIII for hemophilia A
factor IX for hemophilia B
desmopressin for mild hemophilia A

230
Q

how does neonatal herpes simplex virus infection of eye present

A

2nd or 3rd week of life with vesicular or ulcerative eye disease

231
Q

cessation of breastfeeding should be reserved for infants with what

A

suspected galactosemia

jaundice, lethargy, vomiting and hepatomegaly

232
Q

xray features with vit d def rickets

A

osteopenia
metaphyseal cupping and fraying
epiphyseal widening

233
Q

treatment for SCID

same tx in what other disease

A

stem cell transplantation

wiskott Aldrich syndrome too

234
Q

orchiopexy reduces risk of what but not what

A

testicular torsion and decreases risk of testicular cancer

not help subfertility

235
Q

blood smear with occasional RBCs with single, round, blue inclusions on wright stain are what and from what

A

howell-jolly bodies from splenectomy
or hyposplenism

usually removed by spleen

236
Q

acute unilateral LAD in children is most commonly caused by what

A

s aureus and s pyogenes

237
Q

respiratory isolation during first ___ days of antibiotic therapy for pertussis

A

5

238
Q

dx of homocystinuria based on elevated what and what is treatement

A

elevated homocysteine and methionine

tx: B6, folate, B12, antiplatelets or anticoags

239
Q

newborn with fTT, bilater cataracts, jaundice, and hypoglycemia has what and what enzype

A

galsctosemia and G 1PUT def

240
Q

impetigo

bacteria involved, cx features and tx

A

non bullous

  • s. aureus and group A strep
  • painful non pruritic pustules, honey crusted lesion

bullous

  • s aureus
  • rapidly enalrged flaccid bullae with yelow fluid, ruptured lesions in periphery

tx: limited skin involvement = mupirocin topical
extensive skin involvement = orals like cephalexin, dicloxacillin, clindamycin

241
Q

risk factor of intussusception

A

recent viral illness or rotavirus vaccine

pathological lead point
-meckel divert, HSP, celiac, intestinal tumor, polyp

242
Q

features of turner syndrome that aren’t as common

A

bicuspid aortic valve
coarctation of aorta

broad chest with widely spaced nipples

narrow high arched palate

243
Q

pt with sickle cell that has decreased reticulocytes and no splenomegaly what is it from

presents with what

tx

A

aplastic crisis

2ndary to parvovirus B19

pallor, weakness, fatigue (nonspecific for anemia) but funcitonal systolic murmur due to hyperdynamic blood flow

tx: blod transfusion

244
Q

when should the quadrivalent meningococcal vaccine be given and booster

when give it really early and what age

A

11-12

booster at 16

age 2 if asplenic, HIV postiive or complement deficiency

245
Q

accumulation of fluid in the inner ear that leads to hearing loss vertigo, and tinnitus

A

meniere’s diseae

246
Q

ADHD dx requires that several sx be present before age what

A

12

247
Q

hemoglobin precipitation is seen in what

A

G6PD deficiency, hemoglobin oxidized and forms insoluble precipitants called heinz bodies

248
Q

autosomal recesive polycystic kidney disease manifestations

A

large flank masses in infancy, leads to oligohydramnios

pulmonmary hypoplasia and potter faces

249
Q

inspiratory stridor infection

treatment?

A

parinfluenza virus

corticosteroids and racemic epinephrine for pts with stridor at rest

250
Q

____ children of all ethnicities are also at high risk for precocious develpoment

A

obese

251
Q

risk of desmopressin in notcturnal enureisis

A

relapse in up to 70% and hypnatremia

252
Q

central vs peripheral precocious puberty

look at what first

A

bone age

if advanced then basal LH

if low then GnRH test
if low then gonadotrophin indep prec

253
Q

x linked agammaglobulinemia is also called what

casued by defect in what

susceptible to what organisms

A

bruton agammaglob
Tyrosine Kinase that prevents development of mature B cells

encapsulated organisms liek H flu, strep pneumo, GIARDIA bc no IgA

254
Q

how do ulipristal, levonorgestrel and OCPs, stop pregnancy in emergency situation

A

delay ovulation

255
Q

child with deep cat bite what should management be and why

A

antibiotic prophylaxis and irrigation

give amox/clav for pasteurella multocida and aneorobic bacteria

256
Q

evaluation of bilious emesis in the neonate

get abdominal x ray and see dilated loops of bowel

what is next steps

A

contrast enema

if see microcolon–> meconium ileus

if see rectosigmoid transition zone–>hirschsprungs

257
Q

nec x ray shows what

what population

A

abdominal x ray with air in the bowel wall and portal veins

premies with bloody stool

258
Q

myotonic muscular dystrophy type 1 inheritance

A

AD trinucleotide repeat

delayed muscle relaxation

259
Q

phases of pertussis

A

catarrhal (1-2 weeks) mild cough rhinitis

paroxysmal (2-6 weeks) gough with inspiratory whoop, posttussive emesis

convalescent (weeks to months) symptoms reslove gradually

260
Q

Acute OM age groups most common

A

6-18 months and 5 yrs old

261
Q

causes of meningitis in children under 3

A

GBS (*****most common)
e coli
listeria
HSV

262
Q

serous liquid filled blisters on the typanic membrane

A

bullous myringitis

263
Q

the most common cause of syncope in children

A

vasovagal syncope

264
Q

organisms in septic arthritis in kids birth to 3 months

and older than 3 months

tx

A

staph, GBS, gram neg bacilli

nafcillin or vancoymicn plus gentamicin or cefotaxime

older than 3 months
-staph, group A strep, strep pneumoniae

nafcillin, clinda, cefazolin, vanco

need arthrocentesis as dx and tx

265
Q

medulloblastoma occurs where

cause what sx

A

posteior fossa in cerebella vermis which is important for balance and coordinaton

truncal and gain instability

can cause obstructive hydrocephalus

266
Q

auscultation in DS pt with complete AV septal defect

A

loud S2 due to pulmonary HTN

systolic ejection murmur from increased flow across the pulmonary valve from L to R shunt across the ASD

holosystolic murmur of VSD may be ther too

267
Q

stages of lyme disease

earl localized

early disseminated

late

A

EL: erythema migrans
fatigue, ha
mayalgias, arthralgias

ED: multiple erythema migrans
bells palsy
meningitis 
carditis
migratory arthralgias

lateL arthritis, encephalitis, peripheral neuropathy

268
Q

pyridoxine (vitamin ____) deficiency causes what

A

B6

irritability, depression, dermatitis, stomatitis

causes eleveated homocysteine concentration which is increase risk factor for VTE and atherosceleoris

DIDS you see that pyrate

269
Q

extraintestinal sx in celiac disease

A

IDA
dermatitis herpetiformis
-pruritic popular or vesicular rash located on knees, elbos, forearms and buttocks

270
Q

rickets (vit D def)

clinical manifestations

A 
craniotabes (soft skull(
delayed fontanel closure
enlalargred 
-skull, costochondral joints, wrist widening
genu varum
271
Q

hemophilic arthropathy in hemophilia a and b

tx

A

late complication of both types and is caused by iron/hemosiderin deposition leading to synovitis and fibrosis withing the joint

early prophylactic tx with factor concentrates

272
Q

complications of pediatric constipation

A

anal fissures
hemorrhoids
enuresis/UTIs
-fecal retention can cause rectal distension and can compress the bladder and prevent complete voiding (urinary stasis)

273
Q

definition of tourettes disorder

tx

A

both multiple motor and 1 or more vocal tics that persist for one year after initial onset

onset under age 18

tx: antipsychotics (1st gen approved second get being prescribed more often now), alpha-2 adrenergic receptor (clonidine and guanfacine) agonists, behavioral therapy

274
Q

iron studies in microcytic anemia

A

check uworld

275
Q

brutons agammaglobulinemia presents when and how

A

3-6 months after birth bc maternal ABs gone

sinopulmonary infection

GI (salmonella, campylobacter)

276
Q

withdrawal to ____ presents with irritability, a high pitched cry, poor sleeping, tremors, seizures, sweating, sneezing, tachypnea, poor feeds, vomiting, diarrhea

A

opiates

277
Q

patients with sickle cell disease are at risk for ____ disease due to increased ____ secondary to chronic anemia and what

A

cardiac disease, CO, infarction of myocardial microvasculature

278
Q

renal involvement in HSP

A

can occur 4-6 weeks after illness
most pts have hematuria and some proteinuria

severe can lead to HTN and acute renal fialure

279
Q

evaluation of primary amenorrhea steps

1) ____ or ____
uterus present then

A

pelvic exam or US

if uterus there then check FSH

if increased then get karyotype
if decreased then cranial MRI

280
Q

SS disease aplastic crisis

reticulocytes and key features

A

decreased retics

transient arrest of erythropoiesis
secondary to infection of parvovirus B19

281
Q

pruritic circular patch with central clearing and raised scaly border is what

what pathogen

A

tinea corporis

trichophyton rubrum

282
Q

seborrheic dermatitis

clinical features: age, location, description
tx:

what species

A

peaks first year of life and adulthood
erythemaotous plaques and or yellow greasy scales

on scalp, face, posterior ears, nasolabial folds, umbilicus and diaper area

treatment: first line–> emollients, nonmedicated shampoos
2nd: topical antifungals or low potenency glucocort

malassezia species

283
Q

wiskott aldrich syndrome

inheritance

increased risk of what infections

gene regualtes what

sx

tx

A

TIE

thrombocytopenia, Infections (recurrent) and eczema

x linked

infections of bacteria, viral and fungal

hematopoietic cells and regulates cytoskeleton remodeling, dysfunction of cell migration

cytoskeleton of platelets regulated by this too so can lead to petechiae or purpura, severe bleed, IC hem, hematemeis or hematochezia

tx: stem cell transplant

284
Q

neuroblastoma

what cells arise

A

Nc cells which become sympathetic chains and adrenal medulla

calcifications and hemorrhages are seen on plain xray and CT

HVA and VMA usually elevated but no pheo sx

285
Q

dep space neck infeciton most common in children under 4. high fever, muffled voice, and limited rotation of neck

A

retropharyngeal abscesses

286
Q

deficiency of riboflavin (vitamin ___)

A

B2

cheilosis, glossitis, seborrheic dermatitis, pharyhngitis, and edema or erythema of the mouth

287
Q

central lytic bone defect with surroudning sclerosis termed brodies abscess

A

osteomyelitis

288
Q

classic triad of brain abscess

A

fever, severe headaches and focal neurologic changes

289
Q

pasteurella mulocida caused by what

A

normal oral flora in dogs and cats that cuases cellullitis within 1-2 days of dog or cat bite

290
Q

intraventricular hemorrhage is a common complication in neonates born at ___ weeks gestation or ____ grams

A

30 weeks or 1500 grams (3.3 lbs)

291
Q

APGAR score under what may require further evaluation and resuscitation

A

7

292
Q

treatment of obsessive compulsive disorder

A

cognitive behavioral therapy and/or SSRI (fluoxetine)

clomipramine or antipsychotic augmentation for treatment nonresponse

deep brain stim for tx of severe or refractory cases

293
Q

most common spot for spondylolisthesis

A

L5 over S1

294
Q

xray in persistent pulmonary HTN

A

clear lungs with decreased pulmonary vascularity

295
Q

is it the toxin or the spore that is ingested in infantile botulinism?

how is it ingested

A

Spore

Environment, highest incidence in cali, penn, and utah. Leads to production and releaser of the toxin

Raw honey too

296
Q

immune thromboycytopenia most common in what pts age

A

2 to 5 (b rush Is The Prohphet)

297
Q

langerhans cell histiocytosis causes what

tx

A

solitary lytic long bone lesions
eosinophilic granuloma is least severe form and is in children and young adults as solitary bone lesion

usually resolve on own

298
Q

evaluation of bilious emesis in the neonate

get abdominal x ray and see

free air, hematemesis, unstable vital signs then whats next

A

surgery bc this means it is pneumoperitoneum, which is intestinal perforation and need immediate emergency surgery

299
Q

what should always be high on the differential for neonates (28 days or less) with decreased activity or poor feeding

temperature in these pts?

other signs

A

sepsis

may have either fever or hypothermia

mild jaundice, irritable, lethargic, hypotonic, abnormal WBC count high or low, left shift

300
Q

diagnosing biliary atresia

tx

A

US, absent or abnormal GB

failure of liver to excrete tracer into the small bowel on scintigraphy is suggestive

cholangiogram is diagnostic gold standard

tx: liver transplant

301
Q

why is bilirubin higher in newborns

A

hematocrit is 50-60% in newborns and has shorter life span so higher turnover

lower level of uridine diphosphogluconurate glucoronosyltransferase (UGT)

enterohepatic recycling is increased as the sterile newborn gut cannot break down bilirubin to urobilinogen for fecal excretion

302
Q

tx of ITP

A

self limited in children and spont recovery in 6 momths so observe

only treat in kids if have cutaneous symptoms, IVIG or glucocorticoids

303
Q

pt with AIS, what is treament of mass

A

cryptorchid gonads have 1-5% risk of devolping dysgerminoma or gonadoblastoma AFTER puberty

benefits from undergoing gonad stimulated puberty (attainment of adult height) outweigh low risk of malignancy

so wit to remove until after puberty

304
Q

complications of cryptorchidism

alsmost all of these pts have associated what

A

inguinal hernia
testicular torsiojn]subfertility
testicular cancer

processus vaginalis, repair to prevent inguinal hernia

305
Q

gonococcal conjunctivits

age presentation neonate

tx

prevention

A

age 2-5 days

one dose IM ceftriaxone or cefotaxime

erythromycin ointment

306
Q

homocystinuria inheritance, what deficiency, sx besides marfinoid

A

AR, systathione synthase

intellectual disability, thrombosis, downward lens dislocaiton, megaloblastic anemia, fair complexion

307
Q

adenitits due to m tuberculosis is strikingly what

A

nontender

308
Q

Erythema marginatum

A

Erythematous ring like rash that comes and goes associated with acute RF

309
Q

neonatal polycythemia

definition
causes
cx presentation
treatment

A

hematocrit over 65% in term infants

cause:
- increased EPO from intrauterine hypoxia: maternal DM, hypertension, smoking, IUGR
- erythrocyte transfusion: delayed cord clamping, t-t transfusion
- trisomy, hypo or hyperthyroid

sx:

  • mostly asx
  • respiratory distress, cyanosis, apnea, hypoglycemia, hyperbili

tx: IV fluids, glucose, partial exchange transfusion

310
Q

epiglottitis cx features

management

A

tripod position (sit up and lean forward), sniffing position, stridor

drooling

manage with intubation and abx

tracheotomy if attempts at intubation fail

311
Q

when give rotavirus vaccine

A

2-8 months

312
Q

treatment for enterobius vermicularis (what worm)

A

pinworm

albendazole or pyrantel pamoate

313
Q

Rash clasically begins with hearld patch, erythematous annular lesion on trunk which may increase in size and develop scaling around the edge. Within a week clusters of smaller oval red lesions appear on trunk. Scaly macules and papules distributed obliquely along the lines of tension in pattern on back

Can cause pruritis

What may precede this

Tx

A

Pityriasis rosea

Preceding viral prodrome

Self limited and resolves on its own, can use antihistamines and topical corticosteroids to help itching

314
Q

avascular necrosis of the femoral head is seen in what children age

A

legg calve perthes disease which is idiotpathic avascular necrosis of femoral head, presents with limp or hip pain and is common in kids age 4-12

sickle cell diseae can cause too

315
Q

nearly all pts with Cystic fibrosis dvlop what

A

sinopulmonary disease

316
Q

neurofibromatosis type 1 tumor types and what can they causae so management

A

optic pathway glioma

holocranial HA, vision change, vomiting, and HTN

get MRI of brain and orbits

317
Q

muscle biopsy with reduced dystrophin is what disease

A

becker MD

318
Q

hyper IgM syndrome
immuno level, inheritance

sx

A

low IgA and IgG with elevated IgM

x linked

CD40 lignad defect

recurrent sinopulmonary infections with encapsulated bacteria
increased viral infections and opportunistic infections like PCP

319
Q

what should you do if have urinary incontinence in child over 5

A

UA to screen for infection, DM, and DI

may consider acceleration of continence from alarm or desmopressin therapy

320
Q

any neoate with delayed passage of meconium for 48 hours should be considered to have what disesae

A

CF

hirschsprungs

321
Q

acute cervical adenitis in children

unilateral

bilateral

A

uni: staph, strep pyog, anaerobic bacteria, bartonella, mycobact avium
bilat: adenovirus, EBV/CMV

322
Q

infants of diabetic mothers first trimester problems

A

congenital heart disesae
NT defects
small left colon
spont abortion

323
Q

transient proteinuria in children is most common cause of what and how to evaluate

cause

A

most common cause of isolated proteinuria in children and should be reevaluated with a repeat urine dipstick test on 2 separate occasions to rule out persistent proteinuria

can be from fever, exercise, seizures, stress or dehydration

324
Q

osteoid osteoma commonly affects what bone

clinical features

f/u

tx

A

proximal femur

increasing pain that worsens at night without relation to physical activity

relieved by NSAIDs****

follow with serial exam and x rays every 4-5 months

most go away on own

325
Q

tay sachs or niemann pick has hyperreflexia

A

tay sachs

taylor allison is hyper what the hexosaminidase A is she doing?

326
Q

tracheoesophageal fistula can cause what

A

permits air entry into the GI tract and stomach and intestines and they can become quite distended with each breath

gastric fluid can also reflux into the distal esophagus through the fistula and into the trachea and lungs causing aspiration pneumonia —> crackles and x-ray inflitrates

327
Q

common variable immunodeficiency

manifestations

dx

management

A

manifestations: recurrent respiratory and GI infections
autoimmune disease
chronic lung disease and GI disorders

dx: decreased IgG and IgA/IgM, no response to vaccines

IG replacement therapy

328
Q

during the first week of life the normal number of wet diapers a day should be what

A

equal the amt of the days old the infant is

4 day old infant should have 4 or more wet diapers

329
Q

cat like cry is seen in what syndrome

characterisitc ____ present

other features

A

cri du chat 5p deletion

protruding metopic suture present

hypotonia, short, hypertelorism, wide and flat nasal bridge and dumb

330
Q

most life treatening finding in marfan syndrome is what

A

aortic root dilation and aortic regurgitation

monitor for aneurysms and aortic arch dissection

331
Q

common variable immunodeficiency abnormalty

increased risk what infections

other cell lines

growth and other disease

A

abnormal B cell differentiation results in def of multple IG classes

incrased risk encapsulated organisms and Giardia lambilia infection

sometime T cell immunity affected, so pts at increased risk for enteroviral encephalitis

FTT and chronic rep problems (asthma common)

332
Q

the majority of infants with congenital toxo are asx at birth but experience ___ in adulthood

A

chorioretinitis

333
Q

osteogensiss imperfecta is caused by mutations in what

A

type 1 collagen

334
Q

____is ubiquitous where and neonatal __ can result when at risk infants are exposed to it during unhygienic delivery or cord care practices

A

clostridium tetani, in soil

tetanus

335
Q

type of arthritis with lyme

A

monoarticular or asymmetric oligoarthritis most commonly involving the knee

336
Q

pyloric stenosis is most commonly seen in what pop and what kind of vomit and findings

how dx

A

boys age 1-2 months
non bilious projectile vomit
visible peristaltic waves and palpable abdominal mass

abdominal US

337
Q

biliary cyst is what

sx

pts by age

dx
can turn to what so tx

A

congenital dilation of the biliary tree

PAIN, JAUNDICE, PALPABLE mass (PAP got that bily cyst)

pts usually under 10

  • infants have jaundice and acholic stools
  • older kids can have pancreatitis

dx: US or ERCP if obstruction suspected

can turn to cholangiocarcinoma and need surgical resection

338
Q

normal sex behavior in school age kids

A

AIM to know the most

asking questions about sex and repro
Increased intrest in sex words and play
masturbatory movements

339
Q

in sickle cell pts the predominant hemoglobin type is HbS but HbF concentration is elevated at baseline to what? compared to healthy individuals

-so if give hydroxyurea the % of HbF should be above what

A

5-15% baseline

hydroxyurea should raise it above 15%

340
Q

cx features of HSP

A

palpable purpura, hematuria, abdominal pain, arthralgias, and occasionally scrotal swelling

341
Q

pt with a scrotal mass that is cystic and tranilluminate with light

what is this, embryology, and management

A

hydrocele

fluid in processus or tunica vaginalis (peritoneal projection that accompanies the testis during descent into scrotum. when it fails to obliterate, periotneal fluid may accumulate within making a communicating hydrocele. if fluid collects then obliteration then noncommunicating hydrocele)

differs from other testicular mases bc of transillumination

most resolve spontaneously by age of 12 months, if notthen may need surgery

342
Q

lead tox in houses built before what year

A

1978

only test if sx

343
Q

tetralogy of fallot consists of what

murmur?

A

VSD, RV outflow tract obstruction (pulmonic stenosis) overriding aorta and RV hypertrophy

DROP

harsh systolic ejeection murmur L upper sternal border

344
Q

infant with hypothermia, lethargy, and low white blood cell count with left shift is highly concerning for what

also full fontanelles and apnea is concerning for what

A

neonatal sepsis

meninigitis

345
Q

17a hydroxylase def hormonal abnormalities and sx

A

hormonal

decreased cort and test
increased mineral corticoids

all pts phenotypically femal, fluid and salt retention and HTN

346
Q

____ is used in combination with desmopressin to incrase bladder capacity in children with daytime incontinence

A

oxybutynin

347
Q

primary nocturnal enuresis definition

A

urinary incontinence that occurs at least twice a week age 5 or older

348
Q

things that increase risk of otitis externa

A

conditions that disrupt skin barrier (eczema, psoriasis)

retain foreign material and water in canal (headphones, hearing aids, diving caps)

349
Q

what is the most dangerous SM manifestation in myotonic muscular dystrophy

cardiac involvement?

other manifestations?

A

dysphagia can lead to aspiration pneumonia

cardiac involvement includes conduction problems and arrhytmias

cataracts, testicular atrophy/infertility, frontal baldness, and insulin resistance, foot drop, facial weakness

350
Q

congenital lymphedema in what pts and is what exactly

and can lead to what

A

turner, lead to cystic hygroma of neck and fetal hydrops

dysgenesis of the lymphatic network

protein rich interstitial fluid in the hands, feet, and neck

351
Q

diffuse intracranial calcifications in baby

dx

get how

tx

A

congenital toxo

undercooked meat

pyrimethamine, sulfadizine, folate

352
Q

aspergillus fumigatus is common in _____ and ____ with CF and treatment is what

A

older children and adults

itraconazole and voriconazole

353
Q

evaluation of neonate with suspected sepsis

A

CBC, culture, lumbar puncture, UA, and urine culture

empiric antibiotics

ampicillin and gentamicin after cultures

354
Q

ITP happens how and cx features

PS?

A

after viral infection get autoantiboides that bind to platelets and cause purpura and petechiae, sometimes muscosal bleeds

thromboyctopenia and megakaryocytes on PS

355
Q

how to confirm lyme disesae

organism

test

tx

A

spirochete

ELISA and Western blot testing

doxy or amoxicillin with no neuro sx

356
Q

triggers of generalized seizures

sc features

A

fever, hypoglycemia, sleep deprivation

+/- aura

LOC, convulsions, postictal state

357
Q

what is the threshold for phototherapy in a full term healthy 4 day old infant

A

total billy of 20 or more

transfusion if 25 or above and neuro dysfunction from the hyperbilly

358
Q

what is the most effective nonpharmacological treatment for tourettes disorder

A

habit reversal training

359
Q

the most common pathogen isolated from sputum cultures in infants and young children with CF is what

when is the other one most common

A

s aureus

pseudomonas is more common after age of 20

360
Q

h flu meningitis tx with what abx

reduces risk of what

A

dexamethasone

sensorineural hearing loss

361
Q

what is the leading cause of death in infants under 1

A

injury with abusive head trauma

362
Q

what reduces symptoms in TOF and how

A

squatting may reduce symptoms by increasing SVR and afterload which decreases the right to left shunt across VSD but increases flow across the right ventricular outflow tract obstruction, increasing intensity of systolic murmur

363
Q

preseptal cellulitis

A

mild infection of eyelid anterior to the orbital septub

eyelid edema, redness, tenderness, maybe fever and leuko

364
Q

most common brain tumors in children is what

sx

A

pilocytic astrocytoma

supratentorial location: seizures, weak, sensory change

posterior fossa location: cerebellar dysfunction

ICP too

365
Q

ependymomas found where and cause what

A

ventricles in posterior fossa

obstruction of CSF and increased ICP

366
Q

hemophilia A and B inheritance

A

x linked recessive

367
Q

antidote to methemoglobinemia

A

methylene blue

accepts electrons for NADPH and is reduced to leucomethylene blue which in turn reduces methemoglobin back to hemoglobin

368
Q

splenic sequestration crisis

A

acute anemia, splenic engorgement
cx: tachy, pallor, LUQ pain, splenomegaly
can progress to hypovolemi shock as lots of blood trapped in spleen

lab findings: normocytic anemia, compensatory elevated reticulocyte count, and THROMBOCYTOpenia

tx: fluid, transfusions, and eventual splenectomy

369
Q

cutis aplasia (absence of epidermis over the skull) and microphthalmia are both classically seen in what?

__ defects too

A

patau syndrome

midline defects like holoprosencephaly and omphalocele

370
Q

does cat scratch disease need antibiotic prophylaxis ?

A

not in immunocompetent people

in ICP yes

371
Q

comorbidities with duchenne musc dyst and becker and myotonic

A

DMD: scoliosis and cardiomyop

becker: cardiomyop
myotonic: MD: CAT Bald
- cataracts, arrhymias, testicular atrophy/infertility, baldiing

372
Q

deep brain stimulation for tx of severe or refractory cases of ___ target what area in brain

A

OCD, nucleus accumbens

373
Q

recurrent UTIs in infants and children is often from what

dx

what to evaluate long term renal scarring

follow renal function

monitor pts closely for what complications

A

vesicoureteral reflux

dx: voiding cystourehtrogram (VCUG), then renal US to see hydronephrosis

reanl scintigraphy with dimercaptosuccinic acid to evaluate renal scarring long term

renal function followed by seriel cr

pts monitored closely for complications of chornic renal insufficiency, like HTN and anemia

374
Q

what would you see on a muscle biopsy in someone with doucheanes muscular dystrophy

what about beckers

A

absent dystrophin

becker would show reduced dystrophin

375
Q

clinical manifestations of refeeding syndrome

A

arrhythmias
CHF
seizures
wernicke encephalopathy

phosphorus K,MG uptaken

phosphorus is primary deficient electrolyte for nrg

cardiac arrhythmias from mg and potassium

376
Q

most common cause of subacute unilateral LAD in young children is from what

A

mycobacterium avium

377
Q

risk factors for brain abscess

A

OM, mastoiditis

frontal or ethmoid sinusitis

dental infection

bacteremia from other sites of infection, cyanotic heart disease (TOF)

378
Q

Spinal muscular atrophy (werdnig-hofffman disease) cx by what

A

Symmetric proximal m weakness and hyporeflexia

Does not affect the pupils

Greater weakness in lower extremeties

379
Q

all sexually active women under age what should undergo testing for chlamydia and gonorrhoeae

A

25

380
Q

septic arthritis of the hip in children

tx

A

septic arthritis has fever of 101 or more

cannot bear weight

WBC count over 12,000
ESR over 40
CRP over 2

if have 3 or more of above then immediate arthrocentesisis

381
Q

what tumor can aphasia and hemiparesis occur in

A

pts with brain tumors in cerebral hemispheres

most common in children is low grade astrocytomas

382
Q

disease with low IgG, IgM, and IgA and low or absent B lymphocytes

A

bruton agammaglobulinemia

low MAG

383
Q

galactocerebrosidase deficiency

retard, blind, deaf, paralsis, neuorpathy, seizures

A

krabbe disease

id be krabbe if i was deaf, dumb, blind, and paralyzed

384
Q

treatment for cat scratch disease

another name
sx

A

bartonella henselae

azithromycin

localized popular or nodular skin lesion that may go unnoticed
fever of unknown origin and or regional LAD

385
Q

phsyical exam in older children with x linked agammaglob shows what

A

underdeveloped lymphoid tissue (tonsils and LNs)

386
Q

laryngomalacia is caused by what and sx

what makes worse

A

colapse of supraglottic structures during inspiration

choronic inspiratory stridor that begins in neonatal period and is worse in supine position

387
Q

hypertoiprhied peyer patch can lead to what

A

intussusception

happens in AGE

388
Q

treatment for croup (laryngotracheitis) and why it works

A

parinfluenza virus

causes tracheal inflammation leading to subglottic edema and narrowing

mild cases use single dose corticosteroids (dexamethasone)

if mod or severe use corticosteroids and nebulized epinephrine (constricts mucosal arterioles in upper airway and alters cap hydrostatic pressure)

389
Q

Breast milk jaundice

timing
pathophysi
cx features

A

starts days 3-5 and peaks at 2 weeks

high levels of b glucoronidase in breast milk deconjugates intestinal bilirubin and increases its circulation

adequate breast feed and normal exam

390
Q

pts with tourettes syndrome have high rates of comorbid conditions like what

A

ADHD and OCD

391
Q

precocious puberty uworld

A

look up

392
Q

risk factor for development of respiratory distress syndome

what decreases risk

A

prematurity

maternal diabetes mellitus, increased insulin antagonize cortisol and block the maturation of sphingomyelin

IUGR, mathernal HTN decrease risk

393
Q

what is the most common pathogenic organism in young children with CF? especially in the concurrence of ___ infection

A

staphylococcus aureus

influenza

394
Q

chediak-higashi syndrome inheritance and characteristics

A

AR

oculocutaneous albinism and recurerent cutaneous infections with staph aureus and srtrep pyogenes

395
Q

growing pains happens in what age group?

A

2-12

396
Q

Age group for osteod osteoma?

A

10-20

Uworld Shelf Questions DF (8 decks)

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