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Pediatrics > BRS question notes > Flashcards

BRS question notes Flashcards

1
Q

First sign of puberty in males

A

testicular enlargement

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2
Q

First sign of puberty in females

A

breast development

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3
Q

Girl delayed puberty and older than 14 with high FSH and LH and normal genitalia think?

A

Think primary amenorrhea = turner syndrome or ovarian failure
turner more likely in short stature girl

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4
Q

Gradual testicular pain with blue coloration what do you do

A

rest and analgesics. Its a testicular appendage

Doppler would just show normal or increased uptake

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5
Q

Treatment options for uncomplicated cervicitis

A

Cover gonorrhea and chlamydia
Gonorrhea = IM ceftriaxone, oral ofloxacin, ciprofloxacin, cefixime
Chlamy = azithro, erythro, doxy

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6
Q

Teenager girl with irregular bleeding and anemia what do you do?

A

Perform complete pelvic exam and then put her on progestin:only pill since is anemic so the bleeding is causing symptoms

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7
Q

Teen pregnancy problem for mother health bc?

A

Increased risk of hypertension, anemia, and preterm labor
Don’t need parents for prenatal care
Infants are also at higher risk for having problems.

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8
Q

Homeless child and needs outpatient medication what do you do?

A

Warrant inpatient because of poor follow:up with outpatient therapy

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9
Q

Painful genital ulcers wtih irregular borders and purulent base =

A

chancaroid from Haemophilus ducreyi

Can also have lymphadenopathy

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10
Q

HSV vs chancroid

A

HSV have many shallow painful ulcers. Bases are nonpurulent

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11
Q

Newborn with increased oral secretions and respiratory difficulty =

A

esophageal atresia bc accumulated saliva in proximal esophageal pouch

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12
Q

Respiratory distress, scaphoid abdomen, bowel sounds in chest =

A

congenital diaphragmatic hernia

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13
Q

Most likely cause of abdominal newborn mass =

A

renal origin : hydronephrosis

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14
Q

What is hydrometrocolpos

A

retention of vaginal secretions presents as small cyst between laiba and during childhood as lower midline abdominal mass

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15
Q

Indirect hyperbilirubinemia in the first week of life and otherwise normal think

A

Breastfeeding jaundice due to inadequate fluid intake. Increase Feeds, monitor bili

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16
Q

Hyperbilirubinemia after first week of life in otherwise healthy infant think

A

breast milk jaundice from high lipase and beta:glucuronidase in breast milk

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17
Q

Where does omphalocele occur vs gastroschisis

A

Omphalocele is central through umbilical ring, has sack
Gastroschesis is lateral abdominal wall defect. : get inflammation of bowel and risk of bowel obstruction bc exposure to amniotic fluid

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18
Q

Omphalocele vs gastroschisis associated problems

A

Omphalocele associated with congenital defects heart, trisomy 13 and 18 NOT 21
Gastro has more damage to actual bowel bc it is free in amniotic fluid

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19
Q

By defintion persistent pulmonary hypertension excludes?

A

Anything caused by congenital heart disease

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20
Q

PPHN occurs most commonly in

A

near:term, full:ter and post term infants

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21
Q

What is most effectiv vasodilator for PPHN

A

oxygen therapy

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22
Q

Premature infant with diffuse ground glass opacities and air bronchograms =

A

respiratory distress syndrome

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23
Q

Two common causes of PPHN

A

perinatal asphyxia and meconium aspiration syndrome

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24
Q

Excess fluid in newborn appears on CXR as

A

normal or increased lung volumes with increased vascular markings

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25
Q

Breastfeeding vs formula and jaundice

A

breastfeeding have higher peak serum bilirubin values

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26
Q

When does benign physiologic jaundice peak in term and preterm infants

A

Term : 3:4 days, Preterm : 5:7 days

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27
Q

What is a derm lesion over face in trigeminal region and why is it important?

A

port wine stain or nevus flammeus : could indicate Sturge:webber sydrome which has intracranial vascular malformations and calcifications

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28
Q

small dry rash over dark acular base more commonly seen in african americans =

A

pustular melanosis

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29
Q

What is nevus simplex and does it have malginancy risk

A 
vascular lesion (salmon colored) rash often on back of neck
benign and often transient
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30
Q

whitish papules over nose, cheeks, forehead, chin=

A

milia which are bengin small cysts around pilosebaceous follices

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31
Q

What is erythema toxicum neonatorum

A

benign rash usually present at 72 hours of life in 50% of infants. Is maculopapular rash that are erythematous

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32
Q

CXR showing abdominal distension, air fluid levels, thickened bowel walls, and pneumatosis intestinalis =

A

Necrotizing enterocolitis esp if have bloody stools and abdominal pain

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33
Q

Double bubble on CXR think

A

duodenal atresia

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34
Q

CXR with soap bubble appearance =

A

meconium ileus, could be caused by cystic fibrosis

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35
Q

Down syndrome associated with what congenital GI problem

A

duodenal atresia

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36
Q

Choledochal cyst causes?

A

An elevated bilirubin with large direct component

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37
Q

5 areas of apgar score

A

appearance, pulse, grimace or reflex irritabiity, activity (flexion of extremities), repiratory effort

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38
Q

Tetralogy of fallot response to 100% oxygen

A

will not increase PaO2 by much because there is not much pulmonary flow in the first place

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39
Q

Truncus arteriosis response to 100% oxygen

A

Because there is a decent amount of pulmonary flow, they will have some response but not as much as in pulmonary diseases

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40
Q

VACTERL association group

A

Vertebral defects, anal atresia, cardiac anomalies, TracheoEsophageal fistula, renal and genital defects, limb defects

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41
Q

Newborn with vomiting after feeding w/ breast milk or cow’s milk, hypoglycemia and hepatomegaly think

A

galactosemia

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42
Q

Newborns with galactosemia susceptible to?

A

E coli sepsis

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43
Q

Neurodegeneration + splenomegaly + bony changes:often Erlenmyer flask distal femur =

A

Gaucher’s disease : sphingolipids accumulate in cells and organs. Deficiency of glucocerebrosidase
Most common lysosomal storage disease

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44
Q

Developmental delay, coarse facies, corneal clouding, dysostosis multiplex think

A

Hurler syndrome also called mucopolysaccharidosis : buildup of glycosaminoglycans

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45
Q

personality changes, ataxia, seizures in infant think

A

wilson’s disease

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46
Q

decreased height, coarctation of aorta, webbed neck think

A

turner syndrome

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47
Q

Noonan syndrome characteristic features…. vs turners?

A

Kind of like turner syndrome with webbed neck, pectus excavatum, short stature. Also have right sided heart lesions whereas turners have left sided heart lesions

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48
Q

Short stature with skeletal asymmetry, triangle shaped head,

A

Silver:Russel syndrome

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49
Q

Hyperactivity, developmental delay, mousy or musty odor think

A

phenylketonuria

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50
Q

Peripheral neuropathy, renal and liver disease, odor of rotten fish or cabbage =

A

tyrosinemia type 1

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51
Q

Hyperactive startle, loses eye contact, cherry red macula, enlarging head, neurodeneration/developmental delay, progressive blidness and seizures =

A

Tay:Sach’s disease a disorder of hexosaminidase A deficiency

Death by 4 years of age

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52
Q

Dysostosis multiplex found in

A

Hunter and hurler syndrome = when patients have mucopolysaccharides

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53
Q

Sodium benzoate useful for?

A

Helping to remove hyperammonemia

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54
Q

In maple syrup disease what should you avoid and go for in terms of feeding

A

give IV glucose as a basic energy source
don’t give parenteral nutrition with protein bc in disease can’t break down branched:chain amino acids. Also avoid enteral feeds until you confirm the diagnosis

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55
Q

large ears, macrocephaly, blue irides, and large testes, intellectual disability=

A

fragile X syndrome

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56
Q

Tall stature, gynecomastia, small penis and testes =

A

Klinefelter’s syndrome

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57
Q

infantile hypotonia, hypogonadism, short stature, obesity and hyperphagia =

A

Prader:willisyndrome

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58
Q

loquacious personality, supraclavicular aortic stenosis, hypercalcemia, elfin facial appearance=

A

Williams syndrome

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59
Q

atlantoaxial instability is a complication of what common syndrome

A

Down’s syndrome

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60
Q

ADHD symptoms, small head, short palpebral fissure, long smooth philtrum w/ a thin upper lip

A

fetal alcohol syndrome

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61
Q

upslanting palpebral fissure, epicanthal skin folds, protruding tongue =

A

Down syndrome

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62
Q

mental retardation, finger and nail abnormalities, cardiac defect =

A

fetal phenytoin syndrome

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63
Q

hypogonadism, almond shaped eyes, short stature, hyperphagia

A

prader:willi syndrome

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64
Q

mental retardation, small head, puppetlike gait =

A

angleman syndrome : problem with mothers 15

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65
Q

homocystinuria vs marfan syndrome

A

marfanoid habitus but without arachnodactyly aka long fingers = homocystinuria
homocystinuria has downward lens subluxation, marfan has upward
homocyst has mitral and aortic regurg but no aortic dilation like in marfarn
mental retardation in homocystinuria
Both have hypogonadism

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66
Q

scissoring lower extremity, clenched hands w/ overlapping digits, rocker bottom feet, delicate small facial features

A

trisomy 18

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67
Q

trisomy 13 associated w/

A

midline defects of brain and forebrain, microphthalmia, holopresencephaly, cleft lip and palate

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68
Q

Deletion on chromosome 7 causes?

A

william syndrome = elfin face and cocktail personality

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69
Q

corneal clouding, stiff joints, coarsened facial features =

A

hurler syndrome : mucopolysaccharidosis

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70
Q

catlike cry with slow growth, microcephaly, mental retardation, hypertelorism, =

A

Cri du chat syndrome : partial deletion on chromosome 5

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71
Q

defect in type 5 collagen in what syndrome

A

Ehler’s:Danlos syndrome

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72
Q

hyperextensible joints, fragile blood vessels, easily bruised skin, tissue:paper think scars, mitral valve prolapse, aortic root dilation =

A

Ehler’s danlos syndrome

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73
Q

decreased growth rate + early morning emesis think

A

mass lesion in CNS. craniopharyngioma

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74
Q

Which growth conditions have normal bone growth rate

A

genetic short stature and constitutional growth delay

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75
Q

Most common form of female pseudohermaphrodism is?

A

congenital adrenal hyperplasia from 21 hydroxylase deficiency

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76
Q

Stronger hereditary component in which type of DM

A

DM Type 2

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77
Q

What genetic markers do type 1 DM have

A

HLA haplotypes DR3 or DR4

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78
Q

What happens to bone age in growth hormone deficiency?

A

growth hormone deficiency causes slow growth velocity

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79
Q

Bone growth with hypothyroidism

A

delayed bone age aka bone age less than chrono age

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80
Q

bone growth in hypercortisolism

A

delayed bone age

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81
Q

Turner syndrome bone age?

A

normal bone age

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82
Q

skeletal dysplasia bone age

A

normal

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83
Q

intrauterine growth retardation bone age?

A

normal

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84
Q

When should dextrose be added while treating ketoacidosis

A

When sugar drops below 250 mg/dl

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85
Q

premature thelarce means

A

transient isolated early breast development in girls under 7

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86
Q

premature adrenarche

A

early onset pubic or axillary hair

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87
Q

If you have pubic hair and apocrine odor with no breast development or advanced bone age you have?

A

premature adrenarche

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88
Q

androgen and cortisol levels in peripheral precocious puberty

A

will have high androgens but low cortisol. Some

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89
Q

Differences in etiology of girls and boys with central precicious puberty

A

In girls its often idiopathic. In boys its almost always organic : get MRI of head

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90
Q

How is GnRH used in precocious puberty

A

administer and if dramatic increase in LH its central

If flat response its peripheral or any HPGA not activated

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91
Q

In peripheral precocious puberty how do boys present

A

feminization or premature pubic hair. NO testicular enlargement bc no increased FSH

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92
Q

In peripheral precoc puberty how do girls present?

A

Girls get virilization and breast development

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93
Q

Midline defect like cleft palate, hypoglycemia, and microphallus think

A

congenital hypopituitarism

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94
Q

What happens in nesidioblastosis

A

beta cell hyperplasia so you get hyperinsulinemia

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95
Q

large for gestational age, macroglossia, umbilical hernia, hyperinsulin think

A

Beckwith Wiedemann syndrome

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96
Q

Common bacterial infections in under 3 months caused by?

A

Group B strep, E. Coli, Listeria Monocytogenes

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97
Q

When does heterophile antibody measurement not work in mono

A

when kid is under 4

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98
Q

Most common cause of viral meningitis

A

Enteroviruses

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99
Q

What does CSF fluid cell count look like early in viral meningits

A

More poolymorphos that eventually shift to lymph predominance

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100
Q

Initial abx for bacterial meningitis

A

3rd gen cephalosporin and vanc

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101
Q

When are steroids useful for mengitis

A

Can reduce hearing loss in Haemophilus influenzae type b meningitis

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102
Q

What increases risk fo HIV transmission from mother to infant

A

high viral load, chorioamnionitis or genital tract infection, premature birth, prolonged rupture of membranes

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103
Q

HIV and breastmilk?

A

yes transmission can occur

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104
Q

Features of scarlet fever?

A

sandpaper rash, pharyngitis, fever

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105
Q

scarlet fever caused by

A

erythrogenic toxin producing strains of group A beta hemolytic strep

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106
Q

which complications of strep infection can be reduced by treatment

A

PANDAS and rheumatic fever

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107
Q

Therapy that is helpful with measles infection

A

supportive care and vitamin A

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108
Q

When do koplic spots occur? and what are they related to?

A

they are transient and occur before the rash happens

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109
Q

Fever, headache, conjunctivitis, diffuse macular rash on trunk and face think of

A

measles

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110
Q

What is common cause of death with measles

A

bacterial pneumonia

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111
Q

intracranial calcification, hydrocephalus, chorioretinitis consistent with?

A

Toxoplasmosis neonatal infection

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112
Q

wheezing, eosinophilia, pulmonary infiltrates think

A

noninvasive allergic disease secondary to aspergillus

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113
Q

When is PPD >10 positive?

A

when patient younger than 4yrs

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114
Q

What age are children not really contageous with TB and why?

A

When under 12 don’t have strong cough and significant pulmonary manifestation

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115
Q

Bloody stool sfrom? (6)

A

salmonella, shigella, campylobacter, EHEC, Cdiff

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116
Q

Shigella infection associated with?

A

seizures bc release of neurotoxin

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117
Q

salmonella associated with?

A

ingestion of contaminated poultry or exposure to turtles and lizards

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118
Q

Yersinia can unusually cause?

A

mesenteric adenities causing pain mimicking acute appendicitis

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119
Q

Acute appendicitis mimicker

A

Yersinia infection

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120
Q

Buccal cellulitis caused by

A

HiB

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121
Q

Impetigo caused by?

A

Staph aureus and group A Beta hemolytic strep

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122
Q

What is nikolsky sign

A

extension of bullae with lateral pressure applied to skin

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123
Q

Nikolsky sign indicates

A

staph scalded skin syndrome

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124
Q

Patient in Tet spell what do you do?

A

place patient in knee:chest position to increase SVR

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125
Q

What drugs are contraindicated in tetratolagy of fallot spell

A

dopamine and epinephrine

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126
Q

Preferred treatment after restenosis of coarctation?

A

Balloon angioplasty

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127
Q

What accompanies coarctation of aorta in half of patients

A

bicuspid aortic valve

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128
Q

How are VSD size and murmur related?

A

Smaller size VSD leads to higher pitched murmur

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129
Q

Newborn cyanosis and left axis deviation and LVH

A

Tricuspid atresia

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130
Q

What patients require prophylaxis against bacterial endocarditis

A

any patient with structural heart disease including patent ductus arteriosus. Structural but repaired in last 6 months. EXCEPT ostium secundum

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131
Q

Grade 1:3 systolic murmur heard best at mid:left systolic border that decreases on standing?

A

Stills murmur

132
Q

grade 1:2 high pitched systolic murmur heard best at upper left sternal border

A

pulmonic systolic murmur

133
Q

heart disease of infants born to lupus mothers?

A

congenital third degree AV block

134
Q

What are two inherited forms of long QT?

A

Jervell:Lnage:Nielsen syndrome : when there is also congenital deafness and
Romano:Ward syndrome when there is no congenital deafness

135
Q

What is inherited long QT sometimes associated with?

A

congenital deafness in Jervell:Lange:Nielsen syndrome

136
Q

What causes snowman appearance on CXR?

A

unrepaired total anomalous pulmonary venous connection with supracardiac drainage

137
Q

Most common cause of croup

A

parainfluenza

138
Q

CXR thumbprint sign means

A

epiglottitis

139
Q

Most effective management for bronchiolitis

A

supportive care

140
Q

Most common cause of bronchiolitis

A

RSV

141
Q

At what age does chlamydia trachomatis pneumonia generally occur

A

in young infants 1:3 months of age

142
Q

Most common cause of pneumonia older children and adolscents

A

Mycoplasma pneumoniae and chlamydia pneumoniae

143
Q

When does group B strep cause pneumonia

A

neonatal period

144
Q

What colonizes patients with CF over time

A

first staph aureus and then pseudomonas

145
Q

Class electrolytes of CF

A

hyponatremic, hypochloremic, hypokalemic, metabolic alkasosi

146
Q

How do PFTs change in CF over time

A

first obstructive. Eventually become restrictive

147
Q

GI symptoms of CF

A

Get pancreatic insufficiency. Replace with fat soluble vitamins

148
Q

fever, muffled speech, tripoding, drooling, and neck hyperextension think

A

epiglottis. Used to be from Hflu but can also be from strep or staph

149
Q

What should you avoid in epiglotittis

A

any stimulation of glottis like examining with tongue depressor

150
Q

afebrile, staccato cough, hx of conjunctivitis, elevated eos on cbc, and neg blood cultures think

A

Chlamydia trachomatis : treat with azithro or erythro

151
Q

Daily asthma symptoms with nighttime wheezing more than once per week =

A

moderate persistent asthma

152
Q

What occurs in heterotaxy

A

intestines begin rotation from abnormal position and so end in one where they can get volvulus

153
Q

Down’s syndrome patients often have what GI symptoms

A

Duodenal atresia

154
Q

persistent vomiting with hypochloremic metabolic alkalsosi, may have hypokalemia =

A

hypertrophic pyloric stenosis

155
Q

polyhydramnios, vomiting, epigastric sitension, scaphoid abdomen think

A

duodenal atresia

156
Q

scaphoid abdomen can be what two things, differences?

A

congenital diaphragmatic hernia : severe resp problems

duodenal atresia : polyhydramnios

157
Q

leading and second leading cause of pancreatitis in kids

A

trauma and then idiopathic

158
Q

colicky abdominal pain, vomiting, lethargy, blood in stools think

A

intussuception

159
Q

study to do when suspecting intussuception =

A

contrast enema that involves intestinal segment and often reduces

160
Q

What can cause functional constipation in neonates?

A

Nay truama like diaper rash, painful diarrhea, abuse : baby doesn’t want to go

161
Q

What do you do when child shows signs of protein intolerance

A

change to different protein. Don’t do studies

162
Q

Rice and celiac disease?

A

Rice is ok

163
Q

Conjugated bilirubinemia in newborn rule out

A

biliary atresia and choledochal cyst

164
Q

When are kids with Hep A contagious

A

2:3 weeks before onset and 1 week after onset

165
Q

Serology in Hep A

A

IgM and IgG rise early in infection

166
Q

Infants with UTI have increased risk for?

A

underlying structural abnorm including vesicoureteral reflux

167
Q

UTIs more common in boys or girls and when

A

before 6 months more in boys but after more common in women

168
Q

How do you get urine in infant and neonate?

A

Must do cath or suprapubic aspiration

169
Q

When can you get false negative on dipstick for blood?

A

Vitamin C ingestion

170
Q

When is oral rehydration therapy acceptable?

A

Secretory diarrhea like in cholera, bc still maintain ability to absorb fluid and electrolytes

171
Q

What is appropriate fluid tyep and amount for emergency phase of rehydration therapy?

A

20ml/kg of NS or LR

172
Q

Where does strep infection have to occur for PSGN

A

skin or pharynx

173
Q

When does nephrotic syndrome in kids occur?

A

Kids under 5

174
Q

What are the two forms of HUS

A

shiga toxi:associated form and atypical form from medication or genetics

175
Q

What are poor prognostic signs in HUS

A

elevated WBC and prolonged oliguria

176
Q

When do you give antibiotics in HUS

A

Don’t give, actually could increase likelihood of developing HUS

177
Q

Asian with recurrent gross hematuria after respiratory infections =

A

IgA nephropathy or Berger’s disease

178
Q

Abnormalities ureterovescial junction inherited as?

A

Autosomal dominant

179
Q

Treatment for VUR of any grade? When do you see a urologist?

A

All need low dose prophylactic abx. See urologist if grade 4 or 5

180
Q

How much do you increase maintenance fluids for every degree over 38?

A

Increase by 12 %

181
Q

Maintenance fluids using surface area method follow requirement of?

A

1500ml/m2/day

182
Q

Infant with gross hematuria and unilateraly or bilateral flank mass, mother may have diabetes?

A

Renal vein thrombosis

183
Q

Classic electrolyte presentation of RTA

A

hyperchloremic metabolic acidosis, with normal anion gap

184
Q

Patient with acidosis, glucosuria, aminoaciduria, hyperphophaturia =

A

fanconi syndrome

185
Q

severe htn, liver disease, enlarged cystic kidneys in kid think?

A

Infantile polycystic disease : Autosomal recessive

186
Q

Most common inheritance of Alport syndrome

A

X linked dominant form

187
Q

HTN, hematuria, renal failure in boys, hearing loss, ocular abnromalities think

A

alport syndrome

188
Q

Patient has mother with difficulty releasing handgrip think

A

Congenital myotonic dystrophy : AD inheritance

189
Q

infant with hypotonia, feeding problems, respiratory problems, facial weakness, hyporeflexia

A

congenital mytonic dystrophy

190
Q

constipation+hypotonia, problems suck/swallow, progressive weakness w/ some paralysis =

A

Infant with botulism

191
Q

Weakness and hypotonia but normal DTRs in infant think

A

Neonatal myasthenia from mother

192
Q

Hypotonia and tongue fasciculations think

A

spinal muscular atrophy

193
Q

Ascending symmetric paralaysi with absent DTRs and normal sensory exam think

A

Guillain:Barre syndrome

194
Q

Most common agent in GBS is?

A

campylobacter jejuni

195
Q

Ate honey, symmetric descending paralysis usually first have constipation. Hypotonia and hyporeflexia

A

Infantile botulism

196
Q

generalised 3Hz spike? What is it and how is it inherited

A

absence seizure inherited as AD

197
Q

treatment for absence seizure

A

ethosuximide

198
Q

brief myclonic jerks lasting 1:2 seconds with a few happening over 3:5 minutes
extension of arm and flexion of neck think?

A

Infantile spasms or West syndrome.

199
Q

Infantile spasm most commonly caused by?

A

Tuberous sclerosis. Can also be perinatal asphyxia, intravascular hemorrhage, and meningitis

200
Q

Duchenne muscular dystrophy inherited as?

A

X linked

201
Q

How do you make diagnosis of duchenne muscular dystrophy? and what would an EMG show?

A

DNA testing of dystrophin gene. EMG shows small polyphasic muscle potentials with normal nerve conduction veolocities

202
Q

First thing you evaluate when suspecting acute cerebellar ataxia?

A

There is no actual physical evidence but do neuroimaging to rule out anything worse

203
Q

bulging fontanelles and downward deviation of eyes =

A

hydrocephalus. Increased third ventricle pressure injures upward gaze center.

204
Q

How do HgB levels change at birth?

A

High at birth and decrease until 2:3 months of age. (full:term)
In preterm its at lowest at 1:2 months

205
Q

When does fetal hemaglobin disappear

A

Declines and disappears by 6:9 months

206
Q

Normal growth and appearance, recurrent infxns, ANC and WBC low, below 4 =

A

chronic benign neutropenia

207
Q

Oculocutaneous albinism and severe infections think

A

Chediak:Higashi syndrome

208
Q

Frequent severe infections and extrememly low ANC think

A

Kostmann syndrome (severe agranulocytosis)

209
Q

Poor growth, pancreatic insufficiency, metaphyseal chondrodysplasia =

A

Schwachman:Diamond syndrome

210
Q

Treatments for ITP

A

supportive care, intravenous immune globulin, steroids, anti:D immunoglobulin

211
Q

When is ITP more likely to become chronic?

A

Chronic ITP more likely if older than 10

212
Q

What is earliest lab finding in iron deficiency anemia

A

low serum ferritin

213
Q

Hemaglobin in sickle cell disease vs trait

A

Disease cannot have any Hgb A

214
Q

Common problems in sickle cell trait

A

Inability to concentrate urine and hematuria

215
Q

bruising, epistaxis, menorrhagia think

A

Von willebrand’s disease

216
Q

Missing thumb and radius with pancytopenia, short stature

A

Fanconi Anemia

Can also have skin findings and kidney anomalies

217
Q

What are some of the less obvious complications of B thalassemia

A

bone marrow hyperplasia esp in bones of face, delays in growth and puberty, hepatosplenomegaly. Hemochromatosis

218
Q

Goats milk feeding leads to

A

folic acid deficiency.

219
Q

Spoon shaped nails indicates

A

iron deficiency anemia

220
Q

Thrombocytopenia and large hepatic hemangioma

A

Kasabach:Merritt Syndrome

221
Q

Eczema, T and B cell defects, low platelet count =

A

Wiskott:Aldrich syndrome

222
Q

Thrombocytopenia, absent radius, cardiac and renal disease

A

Thrombocytopenia:absent radius syndrome

223
Q

TAR syndrome vs fanconi anemia

A

Thumb is present in TAR syndrome. Thumb AND radius missing in fanconi anemia

224
Q

Diarrhea then prolonged PT, aPTT, normal bleeding time =

A

Vitamin K deficiency

225
Q

Prolonged aPTT, normal PT, prolonged bleeding time, he bleeds after a procedure

A

von Willebrand’s disease

226
Q

is common acute lymphocytic leukemia antigen negative a good predictive factor or positive?

A

negative is an unfavorable prognostic indicator

227
Q

Is it better to be female or male with ALL

A

better to be a female and between 1:9yrs

228
Q

Why do they give intrathecal methotrexate during induction treatment for ALL

A

give it to all patients regardless of prognosis as prophylaxis against CNS inovlement

229
Q

Avoid brain radiation in which kids

A

Younger than 5 years

230
Q

Hepatosplenomegaly, bursing, bone pain, gingival hypertrophy

A

AML

231
Q

AML associated with what developmental disorder? And what does this mean about prognosis?

A

AML is associated with Down Syndrome. Has good prognosis

232
Q

How is hodgkins staging done with A and B?

A

A means no systemic symptoms, B is many systemic symptoms like fever and weight loss

233
Q

Male sterility common in what type of cancer?

A

Hodgkins disease

234
Q

Most common tumors between 1:12 yrs

A

Infratentorial brain tumors

235
Q

What is the most common infratentorial tumor? 2nd, 3rd

A

Medulloblastoma, astrocytoma, brainstem glioma

236
Q

What imaging is good for looking in the infratentorial region?

A

MRI is preferred

237
Q

How can you detect medulloblastoma

A

Homovanillic acid and vanillymandelic acid in the CSF

238
Q

Most testicular tumors are benign or malignant?

A

2/3 are malignant

239
Q

What are the most common testicular tumor?

A

Yolk sac tumors

240
Q

What serum marker elevated in hepatocellular carcinoma

A

alpha fetoprotein

241
Q

Down syndrome predisposes to what cancers?

A

AML and ALL

242
Q

Beckwith:Wiedemann syndrome predisposes to what cancers?

A

Wilms tumor, rhabdomyoscaroma, hepatoblastoma

243
Q

Neurofibromatosis type 1 vs 2 in terms of cancers

A

Type 1 to brain tumors and lymphomas

Type 2 to acoustic neuromas

244
Q

Cancer: painless soft tissue mass

A

rhabdomyosarcoma

245
Q

Osteogenic vs Ewing’s sarcoma in terms of area of bone where it affects

A

Osteogenic : metaphysis of tubular long bones, distal femur, proximal tibia
Ewing does flat bones and diaphysis like axial skeleton (pelvis)

246
Q

sunburt =

A

osteogenic sarcoma

247
Q

Onion skin =

A

Ewing’s sarcoma

248
Q

How does langerhans cell histiocytosis normally present?

A

As a bony lesion.

249
Q

Mass behind ear and chronic ear drainage

A

Langerhans cell histiocytosis

250
Q

What is most common teratoma in infancy

A

Sacrococcygeal teratoma : soft tissue mass in area of cocyx. Mostly FEMALES

251
Q

Anaphylaxis mediated by what cells?

A

IgE mediated

252
Q

Most effective therapy for allergic rhinitis

A

intranasal steroids

253
Q

Best method for testing food allergies are?

A

provocative food challenge : double blinded placebo controlled

254
Q

Hypogammaglobulinemia, Tcell dysfunction with normal B and T cell numbers : later in childhood

A

cmmon variable immunodeficiency

255
Q

chronic granulomatous disease due to what problem in neutrophils

A

defect in oxidative metabolism

256
Q

Patient with repeat meningococcal sepsis

A

deficiencies in late complement C5,6,8

257
Q

cerebellar ataxia, red spots on conjunctiva and skin, chronic sinopulmonary infections

A

ataxia telangiectasia

258
Q

hallmark of immunoglobulin deficiency is?

A

increased susceptibility to sinopulmonary infections caused by encapsulated bacteria

259
Q

What is the nitroblue tetrazolium test used for?

A

For diagnosing oxidative metabolism = chronic granulomatous disease

260
Q

Infantile vs childhood eczema

A

Infantile involves extensor surfaces and cheeks

Early childhood involves flexural surfaces

261
Q

Failure to thrive + recurrent soft tissue infections from neutropenia =

A

Schwachman:Diamond syndrome : because they have neutropenia and pancreatic exocrine insufficiency

262
Q

Immunodeficiency with lots of abscess formation think about

A

Combined granulomatous disease : defect in neutro oxidative metabolism so cant do intracellular killing of catalase positive bacteria and fungal pathogens

263
Q

Eczema + congenital thrombocytopenia + combined immunodeficiency (encapsulated organism infections) + small platelets =

A

Wiskott:Aldrich syndrome

264
Q

adenosine deaminase deficiency is a subtype of?

A

severe combined immunodeficiency disease

265
Q

adenosine deficiency given away by infection with?

A

both common and opportunistic stuff like candida or PCP

266
Q

fatigue, muscle weakness, heliotrope rash

A

dermatomyositis

267
Q

What happens to muscles in dermatomyositis

A

calcinosis = calcium deposition in muscle, fascia, and subcutaneous tissue

268
Q

Difference between childhood and adult dermatomyositis

A

In childhood no increased risk of malignancy

269
Q

IgA mediated vasculitis involving skin, joints, gi tract, kidneys

A

HSP

270
Q

Why do Kawasaki disease patients get abdominal pain?

A

Hydrops of the gallbladder

271
Q

Salmon:colored maculopapular, on trunk and proximal extremities

A

systemic onset JRA rash

272
Q

What happens to systemic onset JRA joints?

A

Have 50% chage of severe erosive arthritis

273
Q

Difference between systemic:onset JRA and early:onset pauciarticular JRA in terms of serology

A

ANA is negative in systemic onset

ANA is positive in early:onset and in 50% of patients with polyarticular JRA

274
Q

How many hours for tic to give lyme

A

at least 36 hours

275
Q

When does chorea occur in acute rheumatic fever?

A

Usually several months after

276
Q

Why is late onset pauciarticular JRA different?

A

Male predominant, children older than 8, HLA:B27 positive

277
Q

List different types of Juvenile Rheumatoid Arthritis

A

Early onset pauciarticular :
Late onset pauciarticular :
polyarticular :
systemic:onset

278
Q

What occurs during subacute phase of Kawasaki disease

A

decreased ESR, marked thrombocytosis, coronary artery aneurysms

279
Q

IBD can also cause?

A

HLA:B27 positive axial skeleton arthritis

280
Q

violaceous rash around eyes, erythematous hypertrophic papules =

A

Dermatomyositis

281
Q

Sexually active and arthritis think?

A

Reiter’s disease : can’t see, can’t pee, can’t climb a tree

282
Q

Rash on buttocks or thigh with abd pain, arthritis, glomerulonephritis think

A

HSP : IgA mediated vasculitis

283
Q

Spiral fracture of tibia with nothing on fibula

A

Toddler’s fracture : only need casting if its non:displaced

284
Q

swelling and tenderness over tibial tuberosity

A

Osgood:Schlatter disease : overuse injury w/ inflammation or microfracture of tibial tuberosity : rest and stretch

285
Q

When do you intervene in metatarsus adductus

A

When foot cannot be manipulated beyond straight

286
Q

With septic joint important fact about treatment

A

Don’t start abx until cultures are obtained

287
Q

When do you treat scoliosis

A

After growth spurt if cobb angle >50

brace during growth period to stop progression

288
Q

When does growth stop in females

A

6 months after onset of menstrual cycle

289
Q

supracondylar fracture is a?

A

orthopedic emergency bc risk of compartment syndrome and neurovascular injury

290
Q

What should you move to check for compartment syndrome in supracondylar fracture

A

passive extensino of digits. Don’t move elbow bc can cause neurovascular injury

291
Q

Who is at risk for slipped capital femoral epiphysis

A

male+obesity. Hypothyroidism makes you at risk for bilateral

292
Q

complication of congenital torticollis

A

plagiocephaly

293
Q

3 consecutive wedged vertebrae =

A

Scheuermann’s kyphosis : a painful hunched back

294
Q

Down syndrome patient wants to do gymnastics what do you check?

A

Do lateral flexion:extension cervical spine to rule out instability bc predisposed to atlantoaxial instability

295
Q

allergic conjunctivitis is what type of hypersensitivity reaction

A

type 1 allergic reaction

296
Q

Hallmark of retinoblastoma

A

calcification within the tumor

297
Q

conjunctivitis in first 24 hours

A

chemical irrigation

298
Q

serous or purulent conjunctivitis with some lid swelling in first 4:10 days

A

chlamydia trachomatis

299
Q

serous or purulent conjunctivitis with some lid swelling in 2:4 days

A

Gonorrhea

300
Q

diagnosis of HSV

A

giant cells on tzanck prep

301
Q

well:demarcated hair loss without scalp inflamm =

A

alopecia areata

302
Q

cause of alopecia areata

A

autoimmune lymphocyte:mediated injuryt to hair follicle

303
Q

alopecia with hairs borken off at scalp w/ lymphadenopathy think

A

tinea capitis

304
Q

thickened white borken hairs, flouresce under woods light

A

M. Canis infection from dogs and cats

305
Q

What is Koebner phenomenon in psoriasis

A

new lesions develop at sites of trauma

306
Q

psoriasis inheritance

A

AD

307
Q

Treatment for pityriasis rosea?

A

don’t need anything. can give antihistamines for itching

308
Q

Why do you use only low dose corticosteroids in the groin

A

thinness of epidermis means absorption of steroid greater. Get growth suppression, cushing syndrome, adrenal suppression, pigmentation changes

309
Q

What is the most effective method of moisturizing skin

A

Use ointments>creams>lotions

310
Q

severe cystic or nodular acne treat with

A

isoretinoic acid

311
Q

gianotti crosti syndrome rash =

A

red or flesh colored papules

312
Q

HSV rash =

A

grouped vesicles on erythematous base

313
Q

flesh colored papules w/ umbilication =

A

molluscum contagiosum

314
Q

Lisch nodules or iris hamartomas =

A

Neurofibromatosis type 1

315
Q

ash:leaf spots, angiofibromas =

A

Tuberous sclerosis

316
Q

First sign of cerebral herniation in under 4 kids =

A

bradycardia

317
Q

When does cushing’s triad actually happen in edema

A

late in the course of ICP

318
Q

Modified GCS

A

Modified GCS

319
Q

Why do children do better than adults in drowning?

A

They have primitive dive reflex shunting blood to necessary organs

320
Q

What happens to PFTs after drowning

A

PFTs deteriorate during 12:24 hours after submersion injury

321
Q

Why can’t you use charcoal, ipecac, NG tube in alkali ingestion

A

Burns esophagus so can’t use NG
charcoal messes up endoscopy
No Ipecac in caustic ingestion??

322
Q

When do you get infection with pasteurella multocida

A

cat and dog bites

323
Q

Brown recluse spider vs black widow spider bite?

A

Black widow has few local symptoms but then get muscle cramps and HTN
brown recluse get lots of local signs and symptoms

324
Q

Iron ingestion toxicity management

A

gastric lavage and whole bowel irrigation. Also iron chelator with deferozamine. Don’t do activated charcoal, ipecac.

325
Q

When do you see basophilic stippling?

A

lead intoxication

Pediatrics (4 decks)

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