Pretest Questions Flashcards

1
Q

HSP also known as?

A

anaphylactoid purpura

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2
Q

treatment for retentive encopresis

A

clear fecal impaction and use lots of stool softeners

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3
Q

unilateral bowing of leg past 2 years of age =

A

blount disease aka idiopathic tibia vera. Abnormality in medial aspect of tibia

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4
Q

What is legg-Calve perthes disease

A

avascular necrosis of the femoral head

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5
Q

painless limp think

A

legg-calve perthes disease

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6
Q

overweight patient with a limp think

A

slipped capital femoral epiphysis

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7
Q

What are two types of breath holding spells

A

cyanotic - kid misbehaves and wants something

pallid - painful experience, kid stops breahting and passes out

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8
Q

bedwetting and fat think

A

diabetes

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9
Q

bitter almond odor think

A

poisoning w/ hydrogen cyanide

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10
Q

treatment for hydrogen cyanide poisoning

A

high level oxygen, and hydroxocobalamin antidote

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11
Q

headache, agitated and confused, works with plastics, gets dysrhythmia

A

cyanide poisoning

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12
Q

rash that peaks at 2-4 weeks of life and is harmless

A

neonatal acne from mothers hormones

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13
Q

white keratin plugs on nose, cheeks, chin

A

milia - benign

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14
Q

treatment for scabies

A

permethrin cream

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15
Q

how is scabies different in kids and adults

A

in kids it can occur on palms, soles,face, and head where as it is normally spared in adults

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16
Q

pruritic papular rash that is in many family members

A

scabies

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17
Q

diffuse infiltrates in newborn bilaterally, rapid onset, low WBC, left shift

A

group B strep

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18
Q

Most common way that kids get orbital cellulitis

A

direct extension from nearby site like paranasal sinuses

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19
Q

Common causes of orbital celluliits

A

MRSA, strep, Haemophilus

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20
Q

orbital vs periorbital cellulitis

A

orbital has restriction of eye movement,. Periorbital can be managed as outpatient with oral antbioitcs

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21
Q

general direction of motor development in kids?

A

cephalocaudal, trunk then arms then fingers

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22
Q

How do you test for HIV in neonate and why?

A

use an antigen test like HIV PCR. Can’t do ELISA bc it will reflect maternal antibodies

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23
Q

Where is eczema located when younger than 2

A

cheeks, wrists, scalp, arms and legs.Afterwards goes to the extensor surfaces

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24
Q

Unique contraindication to DTaP

A

encephalopathy - coma, AMS, prolonged seizure within 7 days of administration of last dose

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25
Q

What is the problem with 1% lidane?

A

It can cause neurotoxicity from transdermal absorption. Especially when under 2 months?

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26
Q

3 weeks of progressive cognitive decline, listlessness, vomiting think

A

tuberculous meningitis

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27
Q

whitish polyp extending through TM in patient with recurrent OM

A

cholesteatoma - from recurrent infection or chronically draining ear

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28
Q

Once a child has low blood pressure how what percent dehydration has occurred?

A

10-15%

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29
Q

umbilical cord intact after 1 month think?

A

leukocyte adhesion deficiency type 1 - CBC with marked leukocytosis

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30
Q

How do you diagnose leukocyte adhesion deficiency type 1

A

measure surface CD11b with flow cytometry.

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31
Q

What can form after umbilical cord has separated

A

umbilical granulomas

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32
Q

how do you treat umbilical granulomas

A

silver nitrate

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33
Q

Most common cause of septic arthritis is?

A

S Aureus

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34
Q

Td 10 years ago what do you give now?

A

Tdap to help reduce pertussis

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35
Q

Mammalian bite how do you care for it?

A

Lots of irrigation. Unsure whether to do antibiotics. Definitely if monkey, less so if cat or dog

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36
Q

deferoxamine treats?

A

iron overdose

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37
Q

N acetylcysteine treats

A

acetaminophen ingestion

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38
Q

Dimercaptosuccinic acid treats

A

arsenic poisoning

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39
Q

CroFab treats

A

western diamond back rattlesnake

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40
Q

developmental delay, autism, abnormal temperament test for?

A

fragile X syndrome

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41
Q

long face, large ears, prominent jaw, macroorchidism, hypotonia, repetitive speech, hand flapping =

A

fragile X syndrome

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42
Q

What are normal transmitting males

A

guys with fragile X but they are normal and it doesn’t show up on karyotypes. Pass it on to all daughters and then also to those grandchildren.

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43
Q

Genetics of fragile X syndrome

A

Dominant X-linked disorder with reduced penetrance

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44
Q

rocker bottom feet think

A

trisomy 18

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45
Q

erythematous macules with central yellow-white pustule,. What does fluid from lesions show?

A

This is erythema toxicum benign and found in newborns. Will show eosinophils

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46
Q

rash on neck and sometimes eyelid that gets worse with emotion and activity.

A

salmon patches or nevus simplex/nevus flammeus. The ones on face go away ones on nuchal and occcipital areas persist.

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47
Q

african america, present at birth, small pustules with hyperpigmented lesions encircled by a collarette of scale when u rupture the pustule

A

pustular melanosis- pustules first few days and then hyperpigmentation for weeks to months

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48
Q

Scalp area without hair and scaly yellow-orange lesions

A

sebaceous nevi. They can become malignant in adolescence

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49
Q

What are epstein pearls

A

epithelial cysts on the palage associated with milia

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50
Q

nonerythematous fine yellowish-white lesions on face of neonate think

A

milia

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51
Q

erythematous, ulcerative lesions on his fingertips. Painful and itchy - was in the snow all day

A

chillbain

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52
Q

girl with erythematous, firm, slightly swolen skin at edge of mouth extending to cheek in the summer

A

popsicle paniniculitis - destruction of fat cells by exposure to cold weather.

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53
Q

small, firm, white cold patches of skin after cold =

A

frostnip

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54
Q

cold exposure then stinging in tisseu followed by aching and then numb .Area becomes red, blotchy, and painful when you warm it back up

A

frostbite

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55
Q

scaling of lips with fissures at corners, glossitis, ocular problems, seborrheic dermatitis, poor growth, burning and itching of eyes =

A

riboflavin deficiency

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56
Q

seizure, peripheral neuritis, dermatitis, hypochromic anemia =

A

B6 deficiency

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57
Q

thiamine deficiency think besides korsakoff or wernicke

A

beriberi

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58
Q

NIacine deficiency think

A

dermatitis, diarrhea, dementia, death

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59
Q

Biotin deficiency think

A

dermatitis/seborrhea

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60
Q

High pitched cry, small for age, microcephalic

A

Cri du chat syndrome - abnormal larynx. delete short arm of 5

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61
Q

Deleted segment in angelman and prader willi

A

15q11-13

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62
Q

painful limp, WBC count and ESR are normal. Ruled out osteo and septic hip

A

transient synovitis

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63
Q

progressive neuro symptoms in kid in new house being redone think what and treat with what?

A

Lead poisoning, if symptomatic give dimeraprol and calcium EDTA assuming its severe. One or the other in a more mild form

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64
Q

When is N acetylcysteine ineffective?

A

after 36 hours of acetaminophen overdose

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65
Q

aspirin overdose what will help?

A

sodium bicarb helps in excretion

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66
Q

organophosphate poisoning treat with

A

washing, emesis, atropine followed by pralidoxime (2-PAM)

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67
Q

Megaloblastic anemia not from deficiency think?

A

infection with tapework diphyllobothrium latum - longest tapeworm. Treat with Praziquantel

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68
Q

Mother on lithium - kid gets

A

Ebstein anomaly - heart defect where tricuspid valve is in the right ventricle with attachment to wall instead of annulus

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69
Q

mother on ACE i - baby gets

A

renal sygenesis, oligohydramnios, skull ossification

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70
Q

mother on isoretinoin baby gets

A

teratogenic effects = hydrocephalus, CNS porb, microtia, smallor missing thymus, conotruncal heart defect, micrognathia

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71
Q

low leafy greens and stuff in mom, baby gets

A

neural tube defects like spina bifida

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72
Q

mom gets 3 day measles, baby gets

A

(mom got rubella) deafness, cataracts, mental retardation, heart defects

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73
Q

Guy at the beach gets intensely ithy lesion on feet that progresses up- raised, red, serpiginous.

A

called creeping eruption or cutaneous larva migrans. from dog and cat hookworms. Treat with ivermectin

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74
Q

what is ivermectin

A

anti-helminth medication

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75
Q

treatment for seborrheic dermatitis

A

topical steroids or selenium-sulfide compounds

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76
Q

people out at barbecue and they get photosensitive rash

A

can happen from lime juice

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77
Q

to cause heart block, SLE mothers must have antibodies to?

A

SSA/RO or SSB/La

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78
Q

Isoniazid therapy needs what vitamin

A

pyridoxine or B6 supplements

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79
Q

kids on pheytoin need?

A

folate supplementation

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80
Q

Kids with measles in developing countries need what vitamin

A

Vitamin A

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81
Q

Baby who is breast fed needs vitamin

A

D

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82
Q

Patients with sickle cell disease need supplementation wtih

A

folate

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83
Q

1 hour old newborn may need what supplement

A

Vitamin K to prevent hemorrhagic disease

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84
Q

Kid with repeated abscesses and a liver abscess think

A

chronic granulomatous disease - use NBT to assess

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85
Q

Kid with severe eczema and unusual infections think of?

A

Wiskott-Aldrich syndrome

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86
Q

Wha tis an easy way to rule out Wiskott aldrich syndrome in kid with severe eczema and unusual infection

A

check platelets. Wiskott-Aldrich syndrome unlikely if platelets are normal

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87
Q

How can you test for T cell deficiencies,

A

You could do an intradermal skin test using candida albicans, Won’t get response

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88
Q

Kid with repeat meningitis from neisseria and then strep worry about?

A

asplenia - will see howell-Jolly bodies

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89
Q

children of diabetic mothers have what GI problem

A

small left colon

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90
Q

Baby becomes hypoxic when feeding but is ok when crying. What is happening

A

choanal atresia - need to stick an NG tube down

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91
Q

What is the CHARGE association with choanal atresia?

A

coloboma, heart disease, atresia of choanae, retarded grwoth, genital hypoplasia, ear abnormalities

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92
Q

How do you manage baby with mother who has VZV close to birth

A

VZV 5 days before delivery or within 2 days after need to give VZIG and isolation. If after 2 days you don’t need to do anything

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93
Q

True contraindications to breastfeeding

A

active pulmonary TB, HIV, antineoplastic agents, malaria, typhoid fever, septicemia

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94
Q

What happens to second kid of a mother who had CMV during pregnancy

A

protected by mother IgG

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95
Q

What trimester is worst to get CMV infection

A

first

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96
Q

What is the apt-downey test and what is it used for?

A

Its a test that differentiates fetal from adult hemoglobin in bloody specimen since fetal hgb is alkali resistant. Used when baby has some blood stools bc if mothers blood then don’t need to do anything

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97
Q

Safe medicine for breastfeeding

A

safe medicine for breastfeeding

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98
Q

Infants born to diabetic mothers have sugars that are initially?

A

hypoglycemic

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99
Q

What electrolyte abnormalities occur in neonates of diabetic mothers at 2-4 days?

A

Hypocalcemia and hypomagnesemia bc delayed PTH synthesis

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100
Q

Which is more severe Rh incompatibility or major blood group incompatibility

A

Rh is more severe

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101
Q

how is direct coombs test different in Rh and ABO incompatibility

A

in Rh its strongly positive. In ABO it can be weakly positive or not at all

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102
Q

Describe why babies get cold easily and how they respond

A

Going to colder environment, wet, with large surface area, no fat. To bring up temp start increasing metabolic rate, ventilate faster, they DON”T sweat, and often build up lactic acid

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103
Q

In premature infants what is the cause of apnea

A

icnompletely developed respiratory center

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104
Q

What defines an apneic episode

A

absence of respirations for more than 20 seconds

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105
Q

Mass on the right neck at 1 week of age

A

borken clavicle that is only now apparent with callus formation

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106
Q

Erbe-Duchene paralysis which nerves are affected and how do they present

A

cervical 5,6. Present with pronated forearm that is extended. With arm that is internally rotated and at side. CAN”T abduct or externally rotate at shoulder

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107
Q

Klumpke paralaysis which nerves are affected and presentation

A

cervical 7,8, T1. palsy of hand and can produce horner syndrome.

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108
Q

When there is paralysis of upper extremity from brachial plexus also worry about?

A

phrenic nerve damage. Will see elevated diaphragm on CXR

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109
Q

newborn with enlarging head and fluid wave in the skull worry about

A

subgaleal or subaponeurotic hemorrhage - need to observe in ICU.
LIke a cephalohematoma that crosses midline

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110
Q

Elevated hematocrit in premature baby can cause?

A

seizure from hyperviscosity syndrome

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111
Q

Complication of erythromycin in newborn?

A

increased incidence of hypertrophic pyloric stenosis

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112
Q

What do you give to infants of HIV mothers

A

Ziduvodine

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113
Q

Two things always given to neonates at birth

A

Hep B vaccine and Vitamin K supplements

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114
Q

congenital cystic adenomatoid malformation vs diaphragmatic hernia

A

in diaphragmatic hernia the ng tube will go into chest, whereas normal location in CCAM

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115
Q

Baby born to Hep B ag positive mother what do you do

A

give vaccine and immunoglobulins

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116
Q

Why are beta blockers dangerous at pregnancy

A

When throughout pregnancy cause growth retardation

but near birth they prevent baby from increasing heart rate if asphyxiated. Can also get hypoglycemia and apnea

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117
Q

If there is bilious fluid in a neonates vomit you know that the GI anomaly is not?

A

Esophageal atresia or pyloric stenosis

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118
Q

Cleft lip and palate predisposes you to?

A

recurrent otitis media and hearing loss and speech deficits

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119
Q

How do you make prenatal diagnosis of neural tube defects?

A

detection of elevated alpha feto-protein in the amniotic fluid

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120
Q

CXR in newborn showing pulmonary gascular markings with fluid in fissures and hyperexpansion of lungs - has difficulty breathing and grunting. Csection

A

transient tachypnea of the newborn

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121
Q

How do you evaluate a fifth-finger polydactyly in whites and blacks

A

In black its common and familial
in whites need to do a cardiac exam.
thumb sided polydactyly is always bad!

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122
Q

What is harlequin syndrome?

A

transient change in skin color of asymptomatic newborn. dependent side turns red and upper side remains pale

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123
Q

Two wtins have hematorcrit that differ by more than 15 what happened/

A

twin-to-twin transfusions that happens in monochorionic

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124
Q

What happens to donor in twin-to-twin transfusions? recepient?

A

donor gets oligohydramnios, anemia, hypovolemia

recipient gets polyhydraminios, larger, hyperviscosity syndrome if hematocrit goes over 65%

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125
Q

What do you do with natal tooth that is loose

A

have to remove it because worried about aspiration

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126
Q

longitduinal striations in the metaphysis charactersitic of

A

congenital rubella

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127
Q

osteochondritis or periostitis indicates

A

congenital syphilis

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128
Q

Triad of toxo congenital infection

A

hydrocephalus, chorioretinitis, intracranial calcifications

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129
Q

Which is most common TORCH infection

A

CMV

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130
Q

Cardiac defects make which TORCH infection more likely?

A

congenital rubella

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131
Q

Stable pneumothorax in a neonate can be treated with?

A

100% oxygen to cause a “nitrogen washout”

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132
Q

widely spaced eyes, low set ears, broad nose, bilateral renal agenesis and you get?

A

Potter sequence with pulmonary hypoplasia

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133
Q

What proteins do you care about in maple urine syrup disease

A

leucine, isoleucine, valine

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134
Q

hemihypertrophy and aniridia think

A

Wilms tumor

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135
Q

lateral displacement of medial canthi, broad nasal bridge, single eyebrow, partial albinism (white forelock or heterochromia, deafness

A

Waardenburg syndrome - AD inheritance

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136
Q

flat vascular malformation in distribution of Cn III think

What else do you get?

A

Sturge-Webber syndrome

malformation involves meninges, get atrophy of cerebral cortex, seizures, mental deficiency, hemiparesis, hemianopsia

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137
Q
A

tuberous sclerosis - can get them in brain and also have seizures

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138
Q

What brain abnormalitiy do you get with shaken baby syndrome?

A

subdural hematoma

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139
Q

What is caput succedaneum

A

soft-tissue swellin go fscalp where the baby was delivered. Can be ecchymotic and cross sutures. Resolves in first few days

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140
Q

preterm infant with enlarging head and lethargy worry about?

A

intraventricular hemorrhage aka germinal matrix hemorrhage. - in gelatinous subependymal germinal matrix

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141
Q

cephalohematomas vs caput succedaneum

A

cephalohematomas never cross the suture line.

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142
Q

Treatment for tricyclic antidepressant overdose when you have prolonged QT =

A

sodium bicarbonate to alkanize the blood.

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143
Q

Jones major criteria for ARF

A

carditis, polyarthritis, erythema marginatum, chorea, subcutaneous nodules

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144
Q

What one symptoms is enough to make diagnosis of rheumatic fever

A

Syndenham chorea

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145
Q

What is most common symptom of acute rheumatic fever

A

polyarthritis

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146
Q

Medical treatment for tet spells

A

propranolol and morphine to calm kid down and improve blood flow into the lungs

147
Q

What do you do with child with FH of early heart disease

A

Test for lipid levels after 2 years of age to see if there are any lipid lowering therapy that you will need to do

148
Q

swelling in feet and hands for a few days in kid with other family members with similar problem =

A

Hereditary angioedema - AD
Can get abdominal pain, vomiting, diarrhea if the edema happens in the GI tract. Can also get in larynx and cause problems breathing

149
Q

What is wrong in hereditary angioedema

A

deficiency of C1-esterase inhibitor - get excessive vasoactive kinin

150
Q

Common cardiac defect in turner’s syndrome

A

coarctation of the aorta

151
Q

Risk in kid with another child in family with congenital heart disease?

A

2-6%

152
Q

right ventricular systolic lift. Fixed S2 think

A

atrial septal defect

153
Q

conjunctivitis in someone with rash, arthritis, and other constitutional symptoms think

A

Kawasaki’s disease - conjunctivitis is more unique to Kawasaki than ARF or other things

154
Q

Treatment for Kawasaki disease

A

IVIG and high dose aspirin

aspirin stays until the thrombocytosis comes down

155
Q

Most common causes of myocarditis in babies

A

coxsackievirus B and adenovirus

156
Q

3 groups most at risk of endocarditis

A

unrepaired cyanotic heart disease
prosthetic material
prior endocarditis

157
Q

Organisms that cause endocarditis in children

A

Most are strep viridians and staph aureus

also think about HACEK group, strep pneumo, coag neg staph,

158
Q

first line treatment for SVT in infant

A

carotid massage, face in cold water, voluntary straining

159
Q

Verapamil or adenosine for SVT treatment in infant

A

Can’t use verapamil bc causes hypotension cardiac arrest

160
Q

quadruple heart sounds with right atrial enlargement, right conduction defect with systolic murmur and diastolic rumble =

A

ebstein’s anomaly

161
Q

girl becomes pale while running and then returns to normal after 30 minutes. No evidence of structural heart disease

A

WPW

162
Q

spiking fevers, spindle shaped swelling of fingers and joints. Salmon colored rash that develops with fever

A

Systemic juvenile idiopathic arthritis

163
Q

Cyanotic newborn with LVH and LAD. holosystolic murmur

A

tricuspid atresia - have hypoplastic right ventricle so shows LVH

164
Q

Transposition of the great arteries

A

Transposition of the great vessels with an intact ventricular septum presents with early cyanosis, a normal-sized heart (classic “egg on a string”
radiographic pattern in one-third of cases), normal or slightly increased pulmonary vascular markings, and an ECG showing right-axis deviation and
right ventricular hypertrophy. In tetralogy of Fallot, cyanosis is often not seen in the first few days of life. Tricuspid atresia, a cause of early
cyanosis, causes diminished pulmonary arterial blood flow; the pulmonary fields on x-ray demonstrate a diminution of pulmonary vascularity, and
left-axis and LVH are shown by ECG. Total anomalous pulmonary venous return below the diaphragm is associated with obstruction to pulmonary
venous return and a classic radiographic finding of marked, fluffy-appearing venous congestion (“snowman”). In pulmonic atresia with an intact
ventricular septum, cyanosis appears early, the lung markings are normal to diminished, and the heart is large.

165
Q

Cyanotic patient hours after birth and not responding to oxygen. Give them?

A

prostaglandin E1

166
Q

Thrombocytopenia-absent radius has what heart defects

A

TOF and ASD

167
Q

Male with turner syndrome like stuff has what disease and what cardiac symptoms

A

Noonan syndrome think right sided heart stuff like pulmonic stenosis

168
Q

Ehlers-Danlos syndrome associated with what heart defect

A

Mitral valve prolapse is seen in patients with Ehlers-Danlos syndrome. Patients with Ehlers-Danlos syndrome have hyperextensibility and easy
bruising, joint hypermobility (leading to joint dislocations and sprains), skin that is velvety to touch, and tissue fragility. Six different kinds are
described with “classic” and “hypermobility” forms predominating.

169
Q

Adolescent with syncopal episodes and deafness

A

QT elongation and deafness = Jervell-Lange-Nielsen syndrome

170
Q

child with difficulty breathing, right arm palsy, but normal CXR. What do you do next

A

Do a chest ultrasound to look for hemidiaphragm paralysis

171
Q

1 year old runny nose and sneezing then with worsening cough and wheezing on exam? What is it and what to do

A

Bronchiolitis - monitor oxygen and fluid status. Most things don’t really help

172
Q

ingestion of gasoline, kerosene, or furniture polish, what do you do?

A

symptomatic management. Don’t want NG, charcoal etc

173
Q

At what age does a PPD become effective

A

3 months. Before that treat a child empirically if you are worried about exposures.

174
Q

Repeat pneumonia with hemoptysis. Rapid resolution each time. think about?

A

Idiopathic pulmonary hemosiderosis

175
Q

What are typical findings in idiopathic pulmonary hemosiderosis

A

recurrent infections w hemoptysis. Microcytic, hypochromic anemia, low serum iron levels, occult blood in stool (swallowing pulmonary secretions)

176
Q

How do you diagnoise idiopathic pulmonary hemosiderosis

A

bronchalveolar lavage showing hemisoderin laden macrophages.

177
Q

Some people with pulmonary hemosiderosis have what other hypersensitivity

A

hypersensitivity to cows milk. called Heiner syndrome.

178
Q

Staph pneumonia with acutely worsening respiratory symptoms worry about?

A

Tension pneumothorax. Need large bore needle and chest tube aspiration kit

179
Q

Biphasic stridor following a few days of URI. Looks toxic and has difficulty breathing. Normal epiglottis

A

bacterial tracheitis

180
Q

Kid who eats a lot of dirt with first episode of pneumonia. Found to have a lot of eos

A

Infection from dog parasite toxocara canis. Need to test with ELISA bc the larvae go from gut to organs but don’t return to gut

181
Q

Pus in middle meatus suggests what source?

Superior meatus

A

Middle meatus - maxillary, frontal, anterior ethmoid

Superior meatus - sphenoid or posterior ethmoid

182
Q

How do sinuses change over time in terms of infectio

A

From birth maxillary and ethmoid can have infection
sphenoid large enough at 3-5 years
frontal large enough at 6-10 years

183
Q

Which organism causing pneumonia can also cause a number other upper respiratory infections?

A

Mycoplasma pneumonia

184
Q

reversed heart, chronic sinuopulmonary infections

A

Kartagener syndrome - have primary ciliary dyskinesia

185
Q

When and what causes congenital cystic adenomatoid malformation

A

embryonic disruption before 35th day - improper bronchiole development

186
Q

Fluctuant mass on the posterior wall of pharynx after few days of low grade fever and sore throat

A

retropharyngeal abscess

187
Q

2-12 week old who had conjunctivitis earlier now with afebrile pneumonia think

A

chlamydia trachomatis

188
Q

Young child with decreased breath sounds on one side where you hear normal inspiration but don’t hear expiration

A

Think of foreign body causing air trapping

189
Q

stool filled rectal vault with poor anal sphincter tone

A

functional constipation

Hirschrung disease would have normal or excess sphincter tone

190
Q

systemic signs of eosinophilic esophagitis

A

peripheral eosinophilia or elevated IgE levels

will see food allergies and eczema etc.

191
Q

When do children start getting intussuception?

A

After 6 months of age

192
Q

echogenic bowel on prenatal ultrasound hints at

A

CF meconium ileus developing

193
Q

What is sandifer syndrome

A

Bending or arching of neck caused by reflux - can be confused for infantile spasm

194
Q

Triad of crohns disease

A

diarrhea, abdominal pain, weight loss

195
Q

What do you do with a child who regurgitates food and then swallows some of it agtain

A

behavioral modification. its rumination and related to an attention seeking behavior

196
Q

How do you diagnose meckel’s diverticulum

A

technetium 99m

197
Q

diagnostic test for lactose intolerance

A

breath hydrogen or acidic stool pH in the presence of a reducing substance

198
Q

When do you consider it to be direct obstructive jaundice?

A

When dbili is more than 20% of the increase

199
Q

When can ABO and Rh incompatibility lead to direct hyperbilirubinemia

A

if there is significant hemolysis at birth that causes inspissated bile syndrome

200
Q

How is hypertonic dehydration different in presentation

A

skin can be doughy and you get less tachycardia and hypotension bc the high sodium means a lot of the fluid loss is from intracellular compartment rather than the extracellular compartment

201
Q

Maxillary frontal teeth decay but normal mandibular frontal teeth

A

bottle feeding esp with things with high concentration of sugar

202
Q

If you break your teeth how should you transport them?

A

In saliva or milk

203
Q

Management after ingestion of drain cleaner

A

Don’t try to vomit bc re-expose esophagus again. Just do endoscopic exam in 12-24 hours

204
Q

What are some drugs that charcoal is actually useful for

A

pnenobarbital and tricyclic antidepressants. sustained release theophylline

205
Q

How do you diagnose EHEC?

A

Need special sorbitol-MacConkey agar to isolate. Or you can now look for shiga toxin

206
Q

What laboratory test can show vitamin C deficiency

A

plasma ascorbate level <.2mg/dl

207
Q

CXR with generalized bone atrophy and epiphyseal separation?

A

Vitamin C deficiency

208
Q

how do you treat labial adhesions?

A

can just observe and wait for estrogen to kick in during puberty or put topical estrogen cream. Then need petroleum after separation to ensure it doesn’t stick together again

209
Q

gray and bloody vaginal discharge since birth with normal exam?

A

physiologic trauma related to estrogen withdrawal

210
Q

lax, wrinkled abdominal wall, dilated urinary tract, inta-abdominal testicular tissuse. Oligohydramnios, pulmonary problems, other renal problems. Could have club feet

A

prune belly syndrome

get lack of abdominal wall muscles, cryptorchidism, and urinary tract abnormalities

211
Q

Baby who is always thirsty and peeing all the time

A

congenital nephrogenic diabetes insipidus

212
Q

Review fanconi syndrome

A

Fanconi
syndrome can be hereditary or acquired; hereditary forms are usually secondary to a genetic abnormality such as cystinosis, galactosemia,
Wilson disease, and some mitochondrial abnormalities. A number of agents can cause Fanconi syndrome, including gentamicin (or other
aminoglyco-sides), outdated tetracycline, cephalothin, cidofovir, valproic acid, strepto-zocin, 6-mercaptopurine, azathioprine, cisplatin, ifosfamide,
heavy metals (eg, lead, mercury, cadmium, uranium, platinum), paraquat, maleic acid, and toluene (from sniffing glue). The mechanism of action
of these agents is through acute tubular necrosis, alteration of renal blood flow, intratubu-lar obstruction, or allergic reactions within the kidney
itself. Many of these toxic effects are reduced or eliminated with removal of the offending agent.

213
Q

Asian boy with pain radiating from his abdomen down to his groin

A

urolithiasis

214
Q

large bladder, large kidneys, weak urinary stream in a male

A

posterior urethral valve - an obstructing membrane in the posterior urethra

215
Q

what is a pyelonephritis that is taking longer to respond to antibiotics and a wedge shaped focal enlargement of one lobe that has minimal contrast enhancement

A

acute lobar nephronia - need to do prolonged IV and PO antibiotics
On CT has no cortical rim sign just an area of decreased blood flow

216
Q

Fixing an undescended testis only helps with what potential complication

A

can help prevent testicular torsion through orchiopexy

217
Q

3 day old infant with blood found in diaper, new abdominal mass. Baby was large for gestational age when born

A

renal vein thrombosis - kid could also be dehydrated and have polycythemia making it more likely

218
Q

how are rheumatic strains and nephritogenic strains of strep different?

A

Rheumatic disease only comes from group A strep of pharynx

post strep nephritis can come from pharyngeal or skin infection (impetigo)

219
Q

coombs test in HUS?

A

negative

220
Q

Epididymitis is different than testicular torsion how?

A

Normal cremasteric reflex, pain reduces when you elevated testicle, posterior pain

221
Q

rash on buttocks and extensor surfaces, with renal failure, and abdominal pain - not on face, soles, trunk, and palms

A

HSP

222
Q

HSP is what type of disease

A

An IgA mediated systemic vasculitis

223
Q

Unilateral lymphadenopahty is part of what disease?

A

Kawasaki’s disease

224
Q

Initial management of varicocele

A

Education and wait

225
Q

Management for hydrocele found in first year of life

A

Just observe

226
Q

Asymptomatic microscopic hematuria - that becomes gross hematuria after respiratory infections is what? and what else is it associated with

A

Alport syndrome - also get ocular abnormalities and hearing abnormalities

227
Q

What is inheritance of Alport syndrome?

A

X linked dominant - so guys get it worse

228
Q

Idiopathic hypercalciuria initially presents as?

A

hematuria, often persistent. dysuria, abdominal pain, don’t always get stones formed early

229
Q

What do you have to worry about with acute glomerulonephritis?

A

Get oliguria (looking like dark,cola urine). Need to sometimes restrict fluids to make sure you don’t get volume overload

230
Q

Where do you normally get edema in nephrotic syndrome

A

periorbital and scrotal in boys

231
Q

Berger nephropathy associated with?

A

HSP

232
Q

prevention/treatment for malignant hyperthermia is

A

dantrolene

233
Q

How to identify patients at risk for malignant hyperthermia?

A

caffeine contracture test

234
Q

Headache, seizure, atazia, fatigue 2 weeks after infection with multiple white lesions on MRI think

A

ADEM - acute disseminated encephalomyelitis

235
Q

What do you use instead of edrophonium to test for MS in young kids

A

neostigmine. Avoid bc can cause cardiac arrhythmias in kids

236
Q

hemisyndrome with eyes looking away from the paralyzed side makes you think of?

A

infantile hemiplegia

237
Q

developmental deterioration, macular cherry-red spots, sensitivity to sound is what disease and what mutation

A

Absence of Beta hexosaminidase A activity in WBCs and is Tay Sach’s disease

238
Q
A

Fabry disease - absence of alpha galactosidase

239
Q

myotonic muscular dystrophy is different than most other muscular dystrophies in that it?

A

affects distal muscles more than proximal

240
Q

What is common problem that occurs alongside myotonic muscular dystrophy?

A

cataracts

241
Q

Tuberculous meningitis findings on CT scan with contrast

A

exudate on basal cisterns that enhances

242
Q

tuberous sclerosis sometimes treated with?

A

rapamycin

243
Q

cranial nerve abnormalities, long-tract signs, unsteady gate and behavioral changes

A

pontine glioma

244
Q

Patient with tetrology of fallot and an infection suddenly has neuro symptoms why?

A

He has a brain abscess bc tetralogy of fallot allows for right to left shunts

245
Q

unilateral pupillary defect, hemiplegia, seizures after a fall worry about

A

an epidural hematoma causing downward herniation of temporal lobe compressing ipsilateral CN III and brainstem

246
Q

triplet repeat expansion on chromosome 9

A

Friederich’s Ataxia

247
Q

What is more common infratentorial or supratentorial tumors in kids

A

Until 1 year of life supratentorial more common
1-10 get more infratentorial tumors
>10 supratentorial tumors become more common again

248
Q

constipation is presenting first symptom of?

A

Botulism

249
Q

CNS degeneration, liver/spleen elargement, macroglossia, coares facial features, cherry-red spot on eye at birth

A

GM1 gangliosidosis type 1 with complete absence of acid Beta galactosidase activity

250
Q

Normal children at birth with rapid decline in motor and cognitive function, btwn 16-18 mo. In girls lose control of their hands and get social withdrawal

A

Rett’s syndrome

251
Q

How long do febrile seizures normally last?

A

Only 2-5 minutes. Longer and you should be worried

252
Q

peroneal and intrinsic foot muscle atrophy, then intrinsic hand muscle atrophy, then legs?

A

Charcot-Marie-Tooth disease

253
Q

Periventricular calcifications classic for

A

congenital CMV infection

254
Q

scattered and soft appearing calcifications in cortex classic for?

A

congenital toxo infection

255
Q

achromic skin patches and infantile spasms

A

tuberous sclerosis

256
Q

decreased fetal movement, general hypotonia and arefelxia with fasciculations

A

degeneration of anterior horn cells in SMA type 1

Normally you have selective degeneration bc functional gene

257
Q

When does duchenne’s muscular dystrophy usually start to present itself?

A

Kid reaches normal milestones but starts showing the weakness around 2 years. by 7-12 years in a wheel chair

258
Q

Syndeham chorea triggered by?

A

An infection with Group A beta hemolytic strep

259
Q

unilateral facial lesion and blindness suggests

A

Sturge-Webber syndrome or PHACE syndrome

260
Q

What does PHACE syndrome involve?

A

posterior fossa malform, hemangioma, arterial anomalies, coarctation of the aorta, cardiac defects, eye abnormality

261
Q

large, irregular unilateral cafe-au lait spots and fibrous dysplasia of bones in precocious puberty in girls, goiter

A

McCune Albright syndrome

262
Q

coryza, conjunctivitis, widespread maculopapular rash with white pinpoint lesions on mucosa

A

measles

263
Q

maculopapular rash for 3 days, enlargement of posterior cervical and occipital lymph nodes

A

german measles or rubella

264
Q

sore throat that progresses to adherent membrane covering tonsils, uvula, palate, posterior oropharynx etc.

A

Diphtheria

265
Q

What do you do in infant born to HIV mother before confirm HIV status

A

PCP prophylaxis

266
Q

aspirin + viral infection =

A

Reye syndrome an acquired mitochondrial hepatopathy

267
Q

Common cause of death in Reye’s syndrome

A

cerebral edema

268
Q

Seizures and bloody diarrhea

A

Shigella

269
Q

Kawasaki disease treatment?

A

apsirin gama globulin

270
Q

What cause of tinea capitis fluoresces under wood lamp

A

microsporum canis

271
Q

Exposure to animals fever, rash, meningismus

A

leptospirosis - most are mild

272
Q

Age range for kawasaki’s disease

A

under 5 years

273
Q

Any general infection like otitis media with a new change in AMS in young infant

A

LP to check for meningitis

274
Q

Newborn with sibling who developed pertussis

A

Give newborn erythromycin

275
Q

CBC findings in EBV mononucleosis

A

predominance of lymphocytes with at least 10% being atypical

276
Q

How long does it take to get finding on Xray for osteomyelitis

A

Can take days to get soft tissue changes and up to 12 days to see actual changes in bone

277
Q

16 day old infant with meningitis from gram positive rod

A

Late onset Listeria monocytogenes

278
Q

well appearing infant with sudden elevated fever and and left shift in WBC and markedly elevated WBC

A

pneumococcal bacteremia - can also consider H flu and meningococcus

279
Q

When does rubella really harm the fetus?

A

at the first 2 months

280
Q

What is dacrocystitis

A

infection of nasolacrimal sac

281
Q

posterior and occipital lymphadenopathy with 3 days of fever

A

rubella

282
Q

What is a possible worrisome complication of mumps

A

CNS meningitis that can occur during mumps or 10 days after

283
Q

prophylaxis for bat bite?

A

wound cleaning + rabies immunoglobulin + five injection rabies vaccine

284
Q

RMSF treatment options

A

have to use doxy. Amox doesn’t work like it does in lyme

285
Q

thrombocytopenia + eczema + increased susceptibility to infection

A

Wiskott-Aldrich syndrome = X linked recessive combined immunodeficiency

286
Q

2 weeks of diarrhea in kid who is at daycare with people getting diarrhea

A

Crypto - even though immunocompetent can get it. Can pick it up on microscopy

287
Q

erythema multiforme + vesicular, ulcerated lesions of mucous membranes

A

stevens-johnson syndrome

288
Q

DRESS stands for? and associated with?

A

Drug rash, eosinophilia, systemic symptoms

associated with anticonvulsants

289
Q

weakness, facial swelling, muscle pain, fever eos on CBC. GI symptoms earlier. from rural central america

A

ate improperly cooked T spiralis

290
Q

How do you treat trichanella spiralis

A

mebendazole or albendazole

291
Q

contraindications to LP

A

severe ICP without an open fontanelle
skin infection at puncture site
uncontrolled bleeding disorder
too unstable

292
Q

Most common cause of croup

A

parainfluenza virus

293
Q

Digeorge syndrome has hypoplasia of what?

A

Thymus and parathyroid glands bc defective embryologic development of third and fourth pharyngeal pouches

294
Q

Immunoglobulins in Wiskott-Aldrich syndrome

A

diminished IgM. Elevated IgA and IgE

Also have thrombocytopenia

295
Q

hypergammaglobulin E, eczema like rash, recurrent severe staph infections

A

Job-Buckley syndrome - disorder of phagocytic chemotaxis

296
Q

How is wound through a tennis shoe vs on bare foot different?

A

Through tennis shoe can get pseudomonas bc warm wet conditions
Through bare foot get outdoor stuff like staph

297
Q

Bartonella Henselae causes

A

catch-scratch disease

298
Q

young child with few days of high fever that quickly goes away and followed by an evanescent maculopapular rash

A

Roseola - mostly caused by HHV-6

299
Q

infant with interstitial infiltrates, eosinophilia. No fever. Mom had?

A

Chlamydia trachomatis infection

300
Q

Under 1 year old with URI for a few days that suddenly becomes bad fever with severe respiratory symptoms?

A

Staph pneumonia

301
Q

Learn the fungal causes of pneumonia

A

learn fungal causes of pneumonia

302
Q

What is the concern with prednisone for treating suspected ITP

A

Would want to do a bone marrow biopsy first to rule out leukemia.

303
Q

When are levels of erythrocyte protoporphyrin elevated?

A

lead poisoning or when iron levels are severely limited

304
Q

Sickle cell patient with stroke. What is first thing you do

A

blood transfusion to dilute out sickled cells

305
Q

sickle cell daily treatment?

A

folate and penicillin

306
Q

What is kasabach-Meritt phenomenon

A

large vascular anomalies cause sequestration at birth getting thrombocytopenia, coagulopathy and MAHA

307
Q

infantile fibrosarcoma in an infant often mistaken for a

A

infantile hemangioma - think this when the hemangioma is not resolving

308
Q

rash, fever, lymphadenopathy wtih associated pneumonia or sepsis every 3 weeks. Treat with?

A

cyclic neutropenia - treat with recom human granulocyte colony-stimulating factor

309
Q

When does hgb reach physiologic nadir

A

6-8 weeks

310
Q

pale baby who is otherwise normal with good retic coung and no ABO incompatability what happened?

A

fetomaternal transfusion

311
Q

Why do you get a reading of atypical lymphocytes on smear in leukemia

A

Actually blasts but read as atypical lymphocytes

312
Q

aniridia in an infant, check for?

A

Wilm’s tumor aka nephroblastoma

Both associated with chromosome 11 mutation

313
Q

Eosinophilia in a healthy newborn likely caused by?

A

helminth infestation

314
Q

What can cause spherocytosis other than hereditary spherocytosis

A

ABO incompatibility (not Rh), G6PD, hyperthermia,

315
Q

PT in hemophilia A and B?

A

Not prolonged

316
Q

thrombin time test for

A

conversion of fibrinogen to fibrin

317
Q

VWF type 1, with some acute bleeding

A

Give desmopressin

318
Q

Ewing sarcoma rarely occurs in?

A

Blacks

319
Q

Bone pain that occurs in the middle of the night is concerning or not concerning?

A

very concerning

320
Q

lymphadenopathy of cervical and inguinal areas with cough and nightly fevers what should you do first?

A

Get a CXR. suspecting hodgkins. Get CXR before lymph node biopsy

321
Q

Child starts having jaundice, hepatomegaly, seizures after starting to drink fruit juice.

A

hereditary fructose intolerance

Will have reducing substance on urine test

322
Q

Gaucher disease characterized by? What do you see in Xray

A

Beta glucocerebrosidase deficiency

- get erlenmyer flask appearing long bones

323
Q

Child born to mother with hyperthyroidism gets

A

heart failure, thrombocytopenia, microcephaly etc.

All low things because the body is overdoing it

324
Q

What penile length and testicular volume indicates that some pubertal development has occured

A

penis longer than 2.5cm, Testicular volume greater than 3 cubic centimeters

325
Q

unilateral or bilateral deafness, heterochromia irides, white forelock

A

Waardenburg syndrome

326
Q

Most common cause of goiter in western child

A

lymphocytic thyroiditis

327
Q

10 year old very overweight, short, has osteopenia on xray, hypertension has?
If this was an infant?

A

congenital adrenal hyperplasia

if infant its adrenal carcinoma

328
Q

patients with galactosemia at increased risk of?

A

E. Coli sepsis

329
Q

elevated blood or urine succinylacetone

A

tyrosinemia

330
Q

elevated leucine, isoleucine, valine, depression of alanine

A

maple syrup urine disease

331
Q

In achondroplasia what ratios are off

A

the limbs are short and head is large

332
Q

prolonged jaundice and constipation

A

hypothyroidism

333
Q

macrosomic w/ macroglossia, abdominal wall defects, organomegaly, linear ear creases. Hypoglycemia as newborn

A

Beckwith- Wiedemann syndrome

have increased incidence of cancer

334
Q

constitutional short stature have what bone age and height age

A

bone age and height age that are younger than chronologic age. So although shorter will have time to grow and reach a good height

335
Q

neuro complication of achondroplasia

A

hydrocephalus bc boney overgrowth at foramen magnum

336
Q

first step in evaluating solitary thyroid nodule found on exam

A

serum thyroid function tests

337
Q

long term complication of patients with bilateral retinoblastoma

A

Some other primary tumor - most commonly an osteosarcoma

338
Q

short, delayed bone age, mental retardation, increased bone density, brachydactyly, obesity, subcapsular cataracts, lots of calcifications

A

pseudohypoparathyroidism or Albright hereditary osteodystrophy
body doesn’t respond to PTH so look hypoparathyroid but have high PTH

339
Q

Immobilized following fracture of weight bearing bone can get

A

hypercalcemia

340
Q

In 21 hydroxylase deficiency what do you find on labs

A

elevated 17 hydroxyprogesterone deficiency beyond 3 days of life

341
Q

urine turns black on exposure to oxygen

A

alkaptonuria - deficiency of homogenistic acid oxidase

342
Q

What do you get in the kidneys with wilson syndrome

A

Fanconi syndrome - renal tubular dysfunction. RTA type 2 with lots of other absorption problems

343
Q

Menkes syndrome vs wilsons disease

A

In menkes have low ceruloplasmin and low copper

344
Q

hypothermia, hypotonia, myoclonic seizures in first months of life

A

Menkes syndrome

345
Q

What are the renal findings in vitamin D deficiency

A

phosphaturia and hypocalciuria bc low calcium and phosphate stimulates PTH which triggers release of phosphate and reabsorprtion of calcium

346
Q

tetany and convulsions in first 72 hours of life likely

A

idiopathic hypocalecmia - from maternal problems, resp distress, low birth weight, hypomagnesemia

347
Q

lab findings in patients with vitamin-D resistant ricketts

A

genetic abnormality in reabsorption of phosphate in kidneys

get hypophosphatemia with usually normal calcium

348
Q

blue sclera, easily broken bones what is it and what are lab ca and phos

A

osteogenisis imperfecta - have normal Ca and Phos

349
Q

obesity, mental retardation, hypogonadism, polydactyly, retinitis pigmentosa with night blindness

A

Laurence - Moon- Biedl syndrome also called Bardet-Biedl

Think moon is fat and at night.

350
Q

what abnormality do you see on lab values of G6PD deficiency

A

patients are hyperlipidemic. Increased TG levels cause factitiously low electrolyte values

351
Q

newborn with low sloping shoulders, hand attached at elbow, agenesis of forearm, missing chest wall musculature, birid thumb

A

Holt-Oram syndrome

352
Q

Mitochondrial disease mainly involves what organs?

A

Neuro and muscle

353
Q

hypopigmented whorls on upper extremities

A

hypomelanosis of Ito

354
Q

isoretinoin problems?

A

teratogenic
liver abnormalities. Messes with your lipids
can cause muscle pain

355
Q

treatment of trunk acne

A

benzoyl peroxide with some added retinoic acid if you want

356
Q

first hint of hypertrophic cardiomyopathy can be?

A

passes out during practice or at a game

357
Q

What defines a grade 1 concussion?

A

no loss of consciousness and gets back to normal under 15 minutes

358
Q

small papules on genitals that become pustular, erode, ulcerate with concomitant painful inguinal lymphadenopathy

A

chancroid

359
Q

painless papule on genital that resolved. now has unilateral draining inguinal lymphadenitis

A

lymphogranuloma venereum - serotype of C trachomatis

360
Q

How to differentiate chancroid and lymphogranuloma venereum

A

chancroid has lymphadenopathy at same time as ulcer or ulcers.

361
Q

Mostly shoulder injuries including rotator cuff tendinitis think

A

swimming

362
Q

osgood schlatter seen in what sports

A

basketball and volleyball

363
Q

Most common injury in skiiers

A

skiers thumb - abductiona nd hyperextension of thumb spraining of ulnar collateral ligament

364
Q

Diseases that involve triplet repeats

A

Fragile X, DMD, Huntington chorea, Friedreich ataxia