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1

ITP blood labs

isolated thrombocytopenia

2

what is bleeding in renal failure patients due to?

dysfunctional platelets

3

chronic granulomatous disease puts you at risk for what infections?

catalase-producing organisms like staph aureus; neutrophils filled with bacteria are classic finding because they can still chemotax and eat the bacteria but just not do intracellular killing

4

chronic granulomatous disease- what diseases are you susceptible to?

pneumonias and adenitis are common because theses are caused by staph

5

adenosine deaminase def classic finding on CBC

lymphopenia because this leads to lack of maturation of B and T cells

6

common organisms that you are at risk for with adenosine deaminase activity

PCP, candida, paraflu, and herpes virus

7

when does complement consumption happen (and lead to immunodef)

diseases like vasculitis, glomerulonephritis, and certain connective tissue diseases. SLE

8

C3 def exposes you to risk of what kinds of infections

encapsulated bacteria, begins shortly after birth

9

most common primary immunodef

B cell maturation arrest; puts you at high risk for infections with encapsulated bacteria;

10

types of infections diGeorge syndrome patiens get

fungi and viral since T cell dysfunction is the hallmark

11

tetrad of HSP

palpable purpura, arthralgias, abdom pain, renal disease

12

platelets in HSP

normal

13

meabolic acidosis

think ischemia

14

causes of orbital cellulitis

staph and streps

15

IUGR, diaphoretic, and high pitched cry. Fetus was exposed to what?

heroin

16

fetal hydrantoin syndrome

phenytoin

17

nail and digit hypoplasia, dysmorphic facies and mental retardation

fetal hydantoin syndrome

18

NT defects, cardiac abnormalities, dysmorphic facies like cleft lip, narrow bifrontal diameter, midface hypoplasia, broad and depressed nasal bridge, long philtrum

valproic acid

19

growth deficiency, long smooth philtrum, small palpebral fissures, thin upper lip, in addition to CNS abnormalities like mental retard.

fetal alcohol syndrome

20

jitteriness, excessive sucking, and hyperactive moro reflex

cocaine in utero

21

neonatal abstinence syndrome

neonate withdrawal from opiates in the first few days of life; high pitched cry, tremors, seizures, sweating, sneezing, vomitting, diarrhea

22

Lesch-Nyan syndrome

self mutilation in the presence of dystonia; X-linked recessive; def in HPRT enzyme, leads to increased uric acid

23

clinical features of Lesch-Nyan syndrome

all due to build up of uric acid; hypotonia, persistent vomitting, progressive mental retard, choreoathetosis, spasticity, dysarthric speech, dystonia and compulsive self-injury; gouty arthritis, tophus formation, and obstructive nephropathy

24

treatemnt for Lesch-nyan

allopurinol to reduce uric acid levels; patients should also drink lots of fluids

25

why are SGA infants at risk for polycythemia

increased EPO in response to hypoxia

26

myotonia

delayed muscle relaxation

27

autoimmune myositis with symmetrical proximal muscle weakness, cutaneous findings, arthralgias, and dysphagia

juvenile dermatomyositis

28

mental retard, global developmental delay, hyperactivity, and long narrow face, large ears and macroorchidism

fragile x syndrome

29

contraindic to breast feeding

active untreated TB, maternal HIV, herpetic breast lesions, varicells infection less than 5 days before; certain meds; chemo or XRT; street drugs or alcohol

30

hand-foot syndrome (aka dactylitis)

earliest manifestation of vaso-occlusion in sickle cell anemia; acute onset of pain and swelling of hands and feet; fever sometimes

31

pathophys of hand-foot syndrome in sickle cell disease

vascular necrosis of the metacarpals and metatarsals, which may be seen on xray as osteolytic lesions

32

when giving IV fluid boluses

only isotonic solutions such as nromal saline or lactated Ringer's should be used

33

hypernatremic hypovolemia bolus?

normal salie; later on you will deliver (non-bolus) hypotonic saline

34

signs of hypernatremia

lethargy, altered mental status, irritability, and seizures

35

organisms that cause septic arthritis in birth to 3 month

staph, GBS, and gram neg bacilli

36

antibiotics for septic arthritis birth to 3 mos

antistaph (nafcillin or vanc) PLUS gent or cef

37

organisms that cause septic arthritis in older than 3 mos

staph, GABHS and strep pneumo

38

antibiotics for septic arthritis in greater than 3 mos

nafcillin, clinda, cef, or vanc

39

causes of anemia of prematurity

transitions in the erythropoeisis site of the neonate; shorter life span of RBCs in neonates; diminished fetoplacental transfusion

40

metoclopramide

for gastric motility

41

4 neonatal rashes

erythema toxicum neonatorium, neonatal HSV, neonatal varicella, staph scalded skin syndrome

42

scttered erythematous macules, papules and pustules throughout the body of a neonate but spares palms and soles

erythema toxicum neonatorium; no treatment

43

vesicular clusters on skin, eyes, mucous membrnaes, CNS infection, fulminent dissem multiorgan disease

neonatal HSV

44

fever, rash ranges from vesicular clusters on skin to fulminant dissem disease

neonatal varicella

45

fever, irritability, and diffuse erythema followed by blishtering and exfoliation, pos Nikolsky sign

staph scalded skin syndrom

46

treatment for SSSS

oxacillin, nafcillin, or vanc

47

treatment for neonatal varicella

acyclovir

48

diagnosis of ALL

bone marrow biopsy (not lymph node biopsy) showing greater than 25% lymphoblasts

49

herpangina

throat infection caused by enteroviruses, like coxsackie A; ulcerative lesions on palate, tonsils, and pharynx

50

when herpangina is accompanied by lesions on the palms and soles

hand-foot-mouth disease

51

GB affects which nerves

primarily peripheral motor but sensory can also be affected

52

initial sx of tingling in the foot, followed by ascening weakness; polyneuropathy; flaccid paralysis in final stage

G-B

53

CSF analysis in G-B

elevated protein levels and mildly elevated cells

54

treatment for G-B

plasmapheresis or human immunoglobulin

55

fanconi anemia

chromosomal breaks on genetic analysis

56

treatment of fanconi anemia

hematopoeitic stem cell transplant

57

aplastic anemia, abnormal thumbs, hypogonadism, hypo or hyperpigmented skin, ear findings

fanconi anemia

58

total anomalous pulm venous connection

cyanotic disease; blood from lungs goes into the RIGHT atrium and there is ALSO an ASD which means this partially oxygenated blood goes from RA to LA

59

decreased pulmonary markings on CXR

pulmonary undercirculation

60

holosystolic murmur loudest at LLSB

VSD

61

complete AV canal defect

assoc with down syndrome; ASD and VSD

62

ebstein's anomaly

assoc with maternal lithium use during pregnancy; displacement of tricuspid valve into the RV; results in severe tricuspid regurg and RA enlargement

63

paucity of vascular markings on CXR

tet of fallot and tricuspid valve atresia

64

boot shaped heart

RV hypertrophy, from tet of fallot

65

truncus is assoc with what syndrome

digeorge

66

treatment of pinworm (enterobius vermicularis)

albendazole or mebendazole is first line treatment; pyrantel pamoate is an alternative; NOT metronidazole, thiabendazole, or ivermectin

67

acute drop in hemoglobin in a sickle cell patient can be caused by what?

hyperhemolytic crisis, splenic sequestration, or an aplastic crisis

68

aplastic crisis in sickle cell disease is not the same as aplastic anemia

aplastic crisis in SS does not have drops in other blood cells

69

splenic sequestraton can lead to

severe hypotensive shock; accompanied by persistent reticulocytosis; rapidly enlarging spleen; high mortality; splenectomy recommended

70

vaso-oclusive crisis

acute onset of pain; severe anemia and low retics are not seen in vasoocclusive crises

71

rash, leukopenia, and thrombocytopenia

measles

72

vitamin A reduces the morbidity and mortality rates or patients with measles through immune enhancement

measles

73

calyceal clubbin (blunting of calices) and focal parenchymal scarring

chronic pyleonephritis

74

HBeAg

protein marker of viral replication and infectivity

75

encapsulated bacteria

Hib, strep pneumo, neisseria meningitidis, GBS, klebsiella, salmonella (HSKNGS)

76

nitroblue tetrazolium (NBT) slide test abnormal

chronic granulomatous disease

77

treatment of chronic granulomatous disease

prevention of infection with daily bactrim and gamma interferon three times a week

78

wiskott-aldrich syndrome

x linked recess; eczema, thrombocytopenia, and recurrent infections with encapsulated germs; presents at birth;

79

blood findings in wiskott-aldrich syndrome

thrombocytopenia, low IgM, high IgA and IgE, moderateley reduced T cells and platelets

80

chediak-higashi syndrome

decreased degranulation, chemotaxis and granilopoeisis; neutropenia and giant lysosomes in neutrophils; treatm with daily bactrim and ascorbic acid

81

mild coagulopathy, peripheral and cranual neuropathy, hepatosplenomegaly, pancytopenia, partial oculocutaneous albinism, freq bacterial infections and progressive lymphoproliferative syndrome

chediak-higashi syndrome

82

Hyper-IgM syndrome

poor response to vaccines; less than 2% CD19+ B cells in the peripheral circulation

83

parinaud's syndrome

paralysis of vertical gaze that may be assoc with pupillary disturbances and eyelid retracton (collier's sign); commonly caused by germinomas and pinealomas; endocrine syndrome due to interruption of the hypothal inhib pways

84

treatment of lyme disease in kids less than 8

oral amoxicillin; it's oral doxy in greater than 8 and not preg

85

hypotonia, flat face, upward slanted palpebral fissures, epicanthal folds, Brushfield spots, dysplasia of the pelvis, cardiac malformations, simian crease, hypoplasia of middle phalynx of 5th finger

downs syndrome

86

cleft lip, flexed fingers with polydactyly, ocular hypotelorism, bulbous nose, low-set malfromed ears, small abnormal skull, cerebral malform, micropthalmia

Patau's syndrome (trisomy 13)

87

microcephaly, protruding metopic suture, cat-like cry, hypotonia, short stature, moonlike face, hypertelorism, bilateral epicanthic folds, mental retard

cri-du-chat syndrome, 5p deletion

88

microcephaly, prominent occiput, micrognathia, closed fists with index finger overlapping the 3rd digir and the 5th digit overlapping the 4th, and rocker bottom feet

edwards syndrome

89

when does chlamydia conjnctivitis present? Chlamydia pneumonia?

conjunctivitis days 5-14 of life; pneumonia age 4-12 weeks

90

treatmen of BOTH chlamydial conjunctivitis and pneumonia

14 day course or oral erythromycin, though there is a risk of hypertrophic pyloric stenosis with oral erythromycin

91

cause of solitary lytic bone lesions

langerhans cell histiocytosis

92

langerhans cell histiocytosis

clonal proliferation of langerhans cells (abnormal cells deriving from bone marrow and capable of migrating from skin to lymph nodes); isolated bone lesions or multisystem disease

93

histiocytoses

abnromal proliferation of histiocytes (activated dendritic cells and macrophages); related to abnormal prolif of WBCs like leukemias and lymphomas

94

most common cause of hypertension in kids

fibromuscular dysplasia

95

polyuria and diaper candidiasis

red flags for DM type 1

96

sideroblastic anemia

body has iron available but cannot incorporate it into hemoglobin. Sideroblasts are atypical erythroblasts that have accum of iron in them

97

dysmorphic anemia

two pops or RBCs; commonly seen in transfusion patients

98

treatment of impetigo

topical mupyricin; oral erythromycin is an alternative (not topical erythro)

99

mupyricin

used for topical skin infections, including MRSA

100

imipramine, amitryptaline, desipramine

TCAs; effective for nocturnal anuresis

101

oxybutinin

anticholinergic that causes urinary retention; it can be used for use in combo with desmopressin to increae bladder capacity in kids with daytme incontinence

102

meningococcus meningitis can cause a petechial or purpruic rash

in 75% of patients

103

when do kids get the meningococcal vaccine

11-12 years old and then booster at age 16; at risk kids (asplenic) can get it as young as 2

104

when do kids get the rotavirus vaccine?

between 2 and 8 mos; first dose between 6 and 14 weeks; final dose not beyond 8 months

105

what kids get vision exam?

all kids 0-5 yo

106

testicular volume in prepubertal boys

less than 3 mL

107

increaed risk of male breast cancer

klinefelters

108

small firm testes

klinefelters

109

p24 antigen testing

detects the presence of the p24 protein of HIV

110

most common primary bone tumor in kids

osteosarcoma

111

second most common bone tumor in kids

ewing's sarcoma

112

spiculated sunburst pattern of bone on xray

osteosarcoma

113

periosteal elvation known as Codman triangle

osteosarcoma

114

alk phos and LDH elevated in osteosarcoma

from turnover of damaged osteocytes

115

treatment of osteosarcoma

tumor excision and chemotherapy

116

osteolytic lesion with a periosteal reaction that produces layers of reactive bone giving onion skin appearance

ewing's sarcoma

117

sclerotic cortical lesion on bone xray with central nidus of lucency

osteoid osteoma

118

how does hydroxylurea work to manage sickle cell crises

causes the bone marrow to make new blood cells with hemoglobin F

119

oligohydramnios can lead to flat facies and limb deformities

interesting

120

excessive consumption f cows milk

can lead to iron def anemia

121

beta thal

microcytic anemia

122

seborrheic dermatitis

cradle cap' cause unknown

123

atopic dermatitis distribution in kids

face, scalp and extensor sufaces (different from distribution seen in adults)

124

first line treatement for pertussis

macrolide antibiotic like erythromicin, azithromycin, or clarithromycin

125

pansystolic murmur over left lower sternal border and diastolic murmur at the apex

VSD; the diastolic murmur is due to increased flow across the mitral valve

126

wide split and fixed S2, systolic ejection murmur in the left upper sternal border, and sometimes if it is large, diastolic murmur at LLSB

ASD

127

elevated 17-oh progesterone

CAH

128

loud P2

pulmonary hypertension

129

parotitis

think mumps

130

orchitis

think mumps

131

sore throat, hyperemic and edematous mucous membranes, chilitis, stomatitis, glossitis, normocytic normochrmic anemia, seborrheic dermatitis, and photophobia

riboflavin (vit B2) def

132

cheilitis

inflamm of the lips

133

stomatitis

inflamm of the mouth

134

impaired collagen synthesis and damaged connective tissue; ecchymosies, petechiae, bleeding gums, hyperkeratosis, Sjogren's, arthralgias, and impaired wound healing

ascorbic acid def (vit C)

135

pellagra

niacin def (vit b3)

136

symmetric reddish rash in exposed areas of skin, red tongue, nonspec symptoms

pellagra

137

infantile and adult beriberi

thiamine (b1) def

138

fulminant cardiac syndrome with cardiomegaly, tachycardia, cyanosis, dyspnea, and vomiting

thiamine def (infantile beriberi)

139

photophobia, drya scaly skin, dry conjunctiva, dry cornea, and a wrinkled cloudy cornea; bitot spots and follicular hyperkeratosis of the shoulders, butt and extensor surfaces are less common findings

vit A def

140

anorexia, pruritis, lack of weight gain, incr irritability, limitation of motion, tender swelling of bones, alopecia, seborrheic cutaneous lesions, incr ICP, hepatomegaly

hypervitaminosis A (ingestion of excessive vit A)

141

crigler-najjar

inherited def of UDP-glucuronyl transferase that results in unconj hyperbilirubinemia; patients die in the first year

142

gilbert's syndrome

inherited def in UDP glucouronyl transferase and leads to unconj hyperbilirubinemia

143

ankylosing spondylitis

inflamm of the spine that can lead to fusion

144

spondylolisthesis

forward displacement of a vertebra, commonly in lumbar spine; results in back pain and neuro disfunction

145

kids must be at least 2 kg to get vaccines

remember in premies

146

sinopulm infections and intermittent episodes of diarrhea since childhood

igA deficiency, since IgA is involved in mucosal barrier protection

147

why are patients with iga def at risk for anaphylactic transfusion reactions?

they form antibodies to iga that they get from the transfused blood

148

hypoparathyroidism, thymic aplasia, hypocalcemia, cleft palat, and congenital heart disease

digeorge syndrome

149

predisposes to fungal infections

digeorge because T cell def

150

sinopulm infections and pseudomonas

x linked agammaglobulinemia (bruton's)

151

x linked combined B and T cell disorder

wiskott-aldrich

152

recurrent otitis media, eczema, and thrombocytopenia

wiskott aldrich

153

recurrent resp infections, sinus disease, pancreatic insuff, and infertility

CF

154

elevated IgM and depressed IgG and IgA

hyper-IgM syndrome

155

susceptible to Giardia and recurrent sinopulm infection, lymphoid hyperplasia common

hyper-igM

156

first line drugs for primary generalized tonic clonic seizures or partial seizures with ot without secondary generalization

phenytoin and carbamazepine

157

first line agent for mixed seizures

lamotrigine

158

drugs for absence seizures

ethosuximide and valproic acid

159

side effects of phenytoin

gingival hypertrophy, lymphadenopathy, hirsutism, and rash

160

side effect of phenytoin and carbamazepine in the first several weeks of therapy

stevens johnson syndrome and toxic epidermal necrolysis

161

aut dom mutation of COL1A1

osteogenesis imperfecta

162

osteopenia, with blue sclerae, recurrent fractures, easy bruisability, hypotonia, and hearing loss

OI

163

dentinogenesis imperfecta

blue gray to yellow brown discoloration caused by dentin shining through the weak enamel in OI

164

aortic root dilation

marfan syndrome

165

retinal hemorrhages and subdural hematomas

child abuse

166

most common affected sites in NEC

terminal ileum and colon

167

feeding intolerance, incr abdominal girth, bloody stools, and vomitting

classic features of NEC

168

pneumatosis intestinalis on abdominal xray

NEC

169

bloody stools, uncommon in the neonate

intussception

170

"target sign" on xray or US of abdomen

intussusception

171

vomitting and abdominal pain n neonates less than 1 month

malrotation with midgut volvulus

172

gasless abdomen on xray

malrotation, due to obstruction in the duodenum

173

toga virus

rubella

174

rubella transmission in the first 4 weeks of pregnancy

risk of CRS is 50%, but drops to % if transmission in the third trimester

175

IUGR, deafness, PDA or ASD, micropthalmia, cataract, retinopathy, hepatosplenomegaly, thrombocytopenia and CNS involvement

congenital rubella syndrome

176

purple skin lesions

blueberry muffin baby rubella

177

port wine stain on the trigeminal nerve distribution, mental retard, seizures and glaucoma

sturge-weber

178

hydrocephalus, cerebral calcification, chorioretinitis, microcephaly, and micropthalmis

CMV and toxo

179

lymphedema, high-arched palate, congenitally bicuspid aortic valves, coarctatin of the aorta

turners syndrome

180

anaphylaxis

ige- mediated type 1hypersensitivity reaction

181

post-exposure prophylaxis for varicella for patients greater than 1 yr who are immunocomp

varicella vaccine

182

post-exposure prophylaxis for immunocomp hosts

varicella zoster immunoglobulin within 10 days of exposure; can reduce disease severity

183

how can H-S lead to intussuscepton

causes a hematoma that acts as a lead point

184

periodic abdominal pain assoc with drawing the legs up toward the abdomen, emesis may follow these episodes

intusussception

185

currant jelly stools

intussception that is not treated and causes ischemia; currant jelly is grossly bloody

186

tubular sausage shaped mass in the RUQ

intussusception

187

what imaging to detect intussusception

ultrasound

188

most common organism in osteomyelitis

staph aureus; also GBS and e coli in infants and strep pyogenes in children

189

osteomyelitis assoc with prosthetic devices. What organism?

staph epidermis

190

osteomyelitis when patient has a history of UTI. What organisms

klebsiella and pseudomonas

191

air xray for bronchiolitis

air trapping or atelectasis may be seen

192

ribavirin

administer to kids at high risk for RSV

193

contraindic to rotavirus vaccine

history of intussusception, history of uncorrected congenital malform of the GI tract like meckel's; severe combined immunodef

194

at what age is the rotavirus vaccine series given

age 2-6 months; there is a risk of intussusception with this vaccine

195

anemia, heat intolerance, steatorrhea, wheezing, hemoptysis, clubbing, rectal prolapse, hx of neonatal jaundice

CF

196

complications of prolonged CF

gallstones, cirrhosis with portal htn, and pancreatic fibrosis

197

pos Coomb's test

autoimmunue hemolytic anemia (warm antibodies directed against RBCs). Typical patient is 2 to 12 yo and the anemia is usually acute and transiet lasting 3-6 mos; freq preceded by infection