Pediatrics UWorld Flashcards

1
Q

what is dx in pt who refuses to speak in specific social situations but engages in normal communication when he/she feels comfortable >= 1 month

A

selective mutism

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2
Q

why is selective mutism not considered normal shyness

A

refusal to speak at school can impair both academic and social development and should not be considered normal shyness

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3
Q

what is dx in pt where social situations are avoided or endured with great distress due to fears of negative evaluation

A

social anxiety disorder

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4
Q

what is dx in pt who demonstrates impairments in social reciprocity and restricted or repetitive patterns of behavior during early development

A

children with autism spectrum disorder

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5
Q

when is stranger anxiety normal

A

usually begins in children 6-9 months old and generally subsides by 3yo

involves anxiety and distress when encountering unfamiliar people, even in the presence of a parent.

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6
Q

what is dx in pt who develops fever, urticaria, polyarthralgias, and lymphadenopathy ~1 week after penicillin therapy

A

serum sickness-like reaction

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7
Q

what type of hypersensitivity is serum sickness-like rxn

A

it’s a type 3 hypersensitivity rxn that occurs 1-2 weeks after admin of beta-lactams (penicillin, amoxicillin, cefaclor) or trimethoprim-sulfamethoxazole

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8
Q

what is management of serum-like sickness

A

removal of offending agent (penicillin d/c) resolves symptoms within 48 hrs

more severe cases may require glucocorticoid therapy

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9
Q

what is dx in systemic IgA-mediated vasculitis occurring after an URI
presents with fever, arthralgia, and palpable purpuric rash of buttocks and lower extremities

A

Henoch-Schonlein Purpura

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10
Q

what develops if mononucleosis is treated with amino penicillin

A

morbilliform rash on the trunk

rash typically spares the extremities (no arthralgias either)

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11
Q

what is dx in pt with fever and sandpaper rash following strep pharyngitis

A

scarlet fever

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12
Q

what is dx in pt who starts a medication then gets acute high fever, vesicular or bulls lesions, and painful hemorrhagic oral erosions

A

Stevens-Johnson syndrome

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13
Q

what is dx in infant who presents with easy bleeding/bruising born without prenatal care and/or at-home delivery

A

Vitamin K deficiency

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14
Q

what will labs look like in infant with Vitamin K deficiency

A

normal platelets

prolonged PT

prolonged PTT (possibly normal if mild deficiency)

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15
Q

what are 4 reasons infants are born Vitamin K deficient

A

poor placental transfer

absent gut flora

immature liver function

inadequate levels in breast milk

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16
Q

what is the most common inherited bleeding disorder

A

impaired synthesis of Von Willebrand factor

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17
Q

what is the most common congenital foot deformity, characterized by medial deviation of forefoot with normal/neutral position of the hind foot; usually bilateral

A

metatarsus adductus

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18
Q

what dx is characterized by flexible feet that overcorrect both passively and actively into lateral deviation (abduction)

A

metatarsus adductus

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19
Q

what is management for metatarsus adductus

A

reassurance

most forms of metatarsus adductus correct spontaneously

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20
Q

what is dx in infant with rigid medial and upward deviation of both forefoot and hind foot with hyper-plantar flexion of foot

A

congenital clubfoot

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21
Q

what is the management for congenital clubfoot

A

karyotyping should be considered due to risk of chromosomal abnormalities

requires ortho evaluation, and
serial manipulation and casting soon after birth

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22
Q

what should you be concerned with in a newborn with meconium ileus

A

cystic fibrosis

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23
Q

what is dx in infant with gradual growth failure and recurrent sinopulmonary infections with greasy stools

A

cystic fibrosis

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24
Q

how do CF pts develop pancreatic insufficiency?

A

pancreatic duct obstruction and distention due to viscous mucus and subsequent inflammation develop in utero, and
eventually lead to fibrosis

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25
what will a CF baby's growth chart reveal
normal birth measurements deceleration in weight followed by declaration in length
26
what is dx in pt who presents with decelerated velocity of height growth in infancy normalization of height growth rate after age 2-3 but have short stature and delayed puberty; and catch-up growth after puberty
constitutional growth delay
27
for a thalassemia, what is: MCV RDW total RBC
low MCV (microcytic) RDW normal total RBC count is normal or high
28
what is Mentzer Index, and when is it low vs high
Mentor index = MCV/RBC <13 typically occurs in a thalassemia (small cells but nl/high RBC count) > 13 in iron deficiency (due to decline in total RBC count)
29
what are contraindications to a rotavirus vaccine
anaphylaxis to vaccine ingredients history of intussusception history of uncorrected congenital malformation of GI tract (ex Meckel's diverticulum) SCID
30
what is most common cause of gastroenteritis in infants and young children worldwide
rotavirus
31
when is rotavirus vaccine administered
age 2-6 months
32
what type of vaccine is rotavirus vaccine
live attenuated virus vaccine
33
what is a risk when giving the rotavirus vaccine
intussusception
34
which is the only type of vaccines that should be administered 4 weeks apart due to possible interference of immune response
live -virus vaccines
35
what 2 types of injuries typically go along with shaken baby syndrome
shearing of dural veins and vitreoretinal traction (retinal hemorrhages)
36
what is the most common heart defect with Down Syndrome
complete atrioventricular septal defect
37
how does a complete AtrioVentricular septal defect present
heart failure in early infancy systolic ejection murmur due to increased pulmonary flow form the ASD holosystolic murmur due to the VSD
38
what causes a complete atrioventricular septal defect
failure of endocardial cushions to merge
39
what is coarctation of aorta associated with
Turner syndrome
40
what causes Ebstein anomaly
severe tricuspid regurgitation
41
what does auscultation reveal in Ebstein anomaly
"triple or quadruple gallop" (widely split S1 and S2 sounds plus a loud S3 and/or S4 and holosystolic or early systolic murmur at the LLSB
42
which murmur presents as continuous machine-like murmur
Patent ductus arteriosus
43
what is PDA strongly associated with
congenital rubella syndrome
44
what 2 heart defects are strongly associated with DiGeorge syndrome
Truncus arteriosus transposition of the great arteries (TGA)
45
which vaccine is associated with a decreased risk in developing hepatocellular carcinoma, esp in regions of Asia and Africa
Hepatitis B vaccine
46
what is the initial tx for hypernatremia
initial goal is to stabilize with fluid resuscitation | --only isotonic solutions such as 0.9% NS or LR should be used to slowly lower the Na levels
47
what type of solutions are 0.45% NS and 5% dextrose
hypotonic solutions
48
what is the classic triad for brain abscess
``` fever severe headaches (nocturnal or morning) focal neurologic changes ```
49
what should you give for treatment and post-exposure prophylaxis for pertussis
Macrolides (azithromycin, clarithromycin, erythromycin) all close contacts should be given the Abx regardless of age, immunization status, or symptoms --Pertussis is highly contagious
50
what are the doses of each macrolide for pertussis treatment and Post-exposure prophylaxis
< 1 mo: Azithromycin x 5 days ``` all others: Azithromycin x 5 days OR Clarithromycin x 7 days OR Erythromycin x 14 days ```
51
what should you suspect in pt with dermatitis herpetiformis and/or T1DM
Celiac disease
52
what does the work-up of Celiac disease involve
a high tissue transglutaminase (TTG) IgA antibody intestinal biopsy (villous atrophy)
53
what age is bedwetting normal up to
bedwetting is normal before age 5
54
what two options might help a pt with nocturnal enuresis >5yo?
enuresis alarm or desmopressin therapy
55
what is dx in pt who presents with persistent symptoms of nasal drainage, congestion, and cough, lasting 10-30 days without improvement; pt appears ill, high fevers, and purulent nasal drainage for at least 3 days
bacterial sinusitis
56
what is the most common predisposing factor for acute bacterial sinusitis
viral upper respiratory infection
57
why does a viral URI predispose a pt to bacterial sinusitis
contaminating bacteria cannot be cleared by mucociliary clearance due to mucosal inflammation from viral infection, leading to secondary bacterial infection
58
what is first-line treatment for acute bacterial sinusitis
amoxicillin plus clavulanic acid
59
what is dx in pt with rapid onset of: noninflammatory edema of the face, limbs, and genitalia laryngeal edema (could be life-threatening) edema of the intestines, resulting in colicky abdominal pain no evidence of urticaria usually follows an infection, dental procedure, or trauma
angioedema (hereditary)
60
what is pathogenesis of hereditary angioedema
C1 inhibitor deficiency, dysfunction, or destruction low C1 inhibitor leads to elevated levels of edema-producing factors C2b and bradykinin
61
what's the most common cause of acquired isolated angioedema
ACE inhibitor use, which results in elevated levels of bradykinin
62
what are C1q and C4 levels in hereditary vs acquired forms of angioedema
C1q is normal in hereditary C1q is low in acquired C4 levels are depressed in all forms of angioedema
63
what is the best way to dx phenylketonuria
quantitative amino acid analysis | --elevated phenylalanine
64
what is the deficiency in PKU
phenylalanine hydroxylase - -inability to metabolize phenylalanine into tyrosine - -accumulation in phenylalanine and its neurotoxic byproducts
65
what is the mutation in Marfan syndrome
auto dominant disorder that results from mutations in the fibrillin-1 gene
66
what is dx in pt with tall stature, long/thin extremities; arachnodactyly; joint hyper mobility; upward lens dislocation; aortic root dilation
Marfan syndrome | fibrillin-1 gene mutation
67
what is management of newborn with hydrocele
most will disappear spontaneously by 12 months --observation hydroceles that do not resolve spontaneously should be removed surgically due to risk of inguinal hernia
68
what is the most common type of brain tumor in children
astrocytoma
69
what is dx in young pt with recurrent, severe viral, fungal, or opportunistic (Pneumocystitis) infections, failure to thrive, and chronic diarrhea
SCID
70
what is etiology of SCID
gene defect leading to failure of T cell development B cell dysfunction due to absent T cells
71
what is treatment of SCID
stem cell transplant ASAP