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peds32 Flashcards

1
Q

clinical features of absence epilepsy of childhoo

A

absence seizures lasting 5-10 sec; occur hundreds of times per day; important: loss of posture, urinary incont, and postictal state do not occur

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2
Q

absence seizures on ee

A

generalized spike and wave discharge arising from both hemispheres

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3
Q

management of sbsence seizures

A

ethosuximide (first line) or valproic acid

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4
Q

prognosis for absence seizures

A

very good; seizures usually resolve by adolescence

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5
Q

benign rolandic epilepsy

A

aka benign centrotemporal epilepsy; nocturnal partial seizures with secondary generalization

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6
Q

most common partial epilepsy durign childhood

A

benign rolandic epilepsy

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7
Q

what age for benign rolandic seizures?

A

3-13 yo; boys are more likely to be affected

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8
Q

inheritance of benign rolandic seizures

A

aut dom, with variable penetrance

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9
Q

clinical features of benign rolandic epilepsy

A

early morning seizures with oral-buccal manifestations; seizures spread and become tonic-clonic

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10
Q

benign rolandic epilepsy on EEG

A

biphasic spike and sharp wave disturibance in the midtemporal and central regions

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11
Q

treatment fo rbenign rolandic seizures

A

valproic acid of carbamazepine

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12
Q

prognosis for benign rolandic seizures

A

excellent; seizures remit spontaneously during adolescence

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13
Q

throbbing or pounding headache

A

suggests migraine

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14
Q

aching feeling of pressure in the headache

A

suggests tension

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15
Q

unilateral headache that starts in the periorbital area and spreads to the forehead and occiput

A

migraine

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16
Q

generalized or bitemporal headaches

A

tension headaches

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17
Q

headaches in the morning

A

from incr ICP

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18
Q

headaches at night

A

tension headaches

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19
Q

most common cause of headaches in kids

A

migraines

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20
Q

inheritance of migaines

A

aut dom

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21
Q

what is the pathophys of migraines?

A

changes in cerebral blood flow secondary to release of serotonin, substance P, and vasoactive intestinal peptide from changes in neuronal activity

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22
Q

most common form of migraine in kids? (w or wo aura?)

A

without

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23
Q

migraine equivalent

A

no headache but transient vomitting, abdom pain, or vertigo

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24
Q

ophthalmoplegic migraine

A

unilateral ptosis or CN III palsy accompanies headache

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25
Q

basilar artery migraine

A

vertigo, tinnitus, ataxia, or dysarthria preceding the onset of headache

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26
Q

abortive treatment for migraines

A

sumatriptan (selective seratonin agonist)

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27
Q

prophylactic treatment for migraines

A

propranolol

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28
Q

prognosis for migraines

A

waxing and waning but often lifelong disorder

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29
Q

tension headaches age

A

extremely rare in kids less than 7; uncommon in childhood altogether

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30
Q

isometric contraction of the temporalis, masseter, or trapezius muscle often accompanies the headache

A

tension headache

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31
Q

treatment for tension headaches

A

reassurance and pain control, stress and anxiety reduction

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32
Q

cluster headaches

A

unilateral frontal or facial pain, accompanied by conjunctival erythema, lacrimation, and nasal congestion

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33
Q

cluster headaches in childhood?

A

rare

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34
Q

treatment for cluster headaches

A

abortive therapy with oxygen or sumatriptan. Prophylactic treatment includes CCBs and valproic acid

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35
Q

ataxia

A

inability to coordinate muscle activity during voluntary movement; causesd by cerebellar or proprioceptive dysfunction

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36
Q

acute cerebellar ataxia

A

unsteady gait 2/2 a presumed autoimmune or postinfectious cause

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37
Q

most common cause of ataxia in kids

A

acute cerebellar ataxia

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38
Q

age for acute cerebellar ataxia

A

18 month to 7 years; rarely after age 10

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39
Q

common preceding infections for acute cerebellar ataxia are?

A

varicella, influenza, EBV, and mycoplasma; ataxia usually follows 2-3 weeks later

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40
Q

cause of acute cerebellar ataxia

A

immune complex deposition in the cerebellum

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41
Q

clinical features of acute cerebellar ataxia

A

truncal ataxia, slurred speech, nystagmus, fever is absent

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42
Q

treatment of acute cerebellar ataxia

A

supportive; complete resolution in 2-3 months

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43
Q

guillain-barre syndrome

A

acute inflamm demyelinating polyneuropathy

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44
Q

most common associated infectiuous agent in guillain barre

A

campylobacter jejuni (prodromal gastroenteritis)

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45
Q

principle sites of demyelination in G-B

A

ventral spinal roots and peripheral myelinated nerves

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46
Q

pathophys of G-B

A

cell mediated immune response to an infectious agent that cross reacts to Schwann cell membrane

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47
Q

clinical features of G-B

A

asceniding symmetric paralysis; no sensory loss; CN involvement (facial weakness)

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48
Q

miller-fisher syndrome

A

variant of G-B; characterized by ophthalmoplegia, ataxia, and areflexia

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49
Q

dx of G-B

A

LP shows albuminocytologic dissociation, which is evident 1 week after sx onset

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50
Q

albuminocytologic dissoc

A

elevated CSF protein in the absence of elevated cell count

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51
Q

EMG for G-B

A

decr nerve conduction velocity or conduction block

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52
Q

dx G-B in kids less than 3

A

do spinal MRI, since their sensory exam is difficult to do

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53
Q

treatment of G-B

A

IVIG for 2-4 days; plasmapharesis over a 4-5 day period

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54
Q

prognosis for G_B

A

complete recovery is the rule in kids

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55
Q

sydenham chorea

A

St. Vitus’ dance; autoimmune disorder associated with rheumatic feverthat presents with chorea and emotional lability

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56
Q

is syndenham chorea common?

A

well it occurs in a quarter of people with rheumatic fever

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57
Q

onset age for syndenham chorea

A

5 and 13 yo

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58
Q

pathophys of syndenham chorea

A

antibodies cross react with membrane antigens on both group A strep and basal ganglia cells

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59
Q

clinical features of syndenham chorea

A

2-7 mos after strep pharyngitis; restless; speech affected; no sustained protrusion of the tongue; choreic hand; milkmaid’s grip; gait and cognition NOT affected

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60
Q

chameleion tongue

A

in syndenham chorea; unable to sustain protrusion of the tongue

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61
Q

choreic hand

A

hand flexed and hyperextended at metacarpal joints

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62
Q

milkmaid’s grip

A

on gripping the examiner’s hand, the patient cannot maintain the grip

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63
Q

diagnosis of sydnenham chorea

A

e;evated ASO or ADB titer; neuroimaging may show incr signal in caudat and puamen; CT shows incr perfusion to the thalamus and striatum

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64
Q

treatment of sydenham chorea

A

haloperidol, valproic acid, or phenobarbitol

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65
Q

prognosis of syndenham chorea

A

sx last for several months to 2 year; generally all patients recover

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66
Q

tourette syndrome

A

chronic, lifelong disorder with motor and phonic tics; presents before 18 yo

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67
Q

tics in tourettes

A

must be present for at least 1 year

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68
Q

coprolalia

A

uttering obscene words

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69
Q

associated findings in tourettes

A

learning disabilities; ADHD; ocd

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70
Q

management of tourettes

A

pimozide (drug of choice), clonidine (side effect is sedation), haloperidol (risk of tardive dyskinesia), hypnotherapy

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71
Q

prognosis of tourettes

A

tics decr in adulthood; drugs generally successful

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72
Q

duchenne and becker muscular dystrophies

A

both are x-linked myopathies characterized by motor degeneration

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73
Q

clonidine

A

alpha 2 ag; used to treat htn

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74
Q

which is more severe- duchenne or becker MD?

A

duchenne

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75
Q

onset of musc dystrophy sx?

A

between 2 and 5 yo

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76
Q

genetics of DMD and BMD

A

x linked; deletion in the dystrophin gene

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77
Q

pathophys if muscular dystrophy

A

dystrophin is a high MW protein that assoc with actin; absence of dystophin causes weakness and eventually rupture of the plasma membrane, leading to degen of musle fibers

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78
Q

pathology of DMD and BMD

A

degen and regen of muscle fibers; replacement of muscle with fibroblasts and lipid depositis; infiltration of lymphocytes

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79
Q

clinical features of DMD and BMD

A

slow progressive weakness affecting legs first; pseudohypertrophy of calves; gower’s sign; cardiac involvement

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80
Q

when do kids lose ability to walk in DMD? In bmd?

A

dmd by ten years; bmd by 20 yo or laer

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81
Q

gower’s sign

A

weakness of pelvic muscles, so when kids get up from the floor, they extend each leg and climb up each thigh

82
Q

cognitive impairment in musc dystrophy?

A

mild in DMD but normal intelligence in BMD

83
Q

diagnosis of muscular dystrophy

A

large calf muscles with weakness; CK levels are very high; muscle biopsy

84
Q

management of muscular dystrophy

A

no cure but oral steroids can help improve strength a little

85
Q

life expectancy for DMD? For BMD?

A

DMD- late teens; BMD- into their 50

86
Q

myasthenia gravis

A

autoimmune that presents w progressive weakness

87
Q

pathophys of myasthenia gravis

A

antibodies to Ach receptor at NMJ

88
Q

neonatal vs juvenile MG

A

neonatal is transient weakness in the newborn pd 2/2 transplacental antibodies from mom with MG; juvenile MG present in childhood 2/2 AchR antibody formation

89
Q

MG in girls vs boys

A

juvenile MG is 6x more common in girls than boys

90
Q

most common presenting sign in juvenile MG

A

bilateral ptosis

91
Q

clinical features of juvenile MG

A

increasing weakness occurs later in the day and w repetitive or sustained muscle activity; diplopia; DTRs preserved; other coexisting autoimm disorders

92
Q

dx of MG

A

tensilon test (IV injection of edrophonium chloride, a cholinesterase inhib, produces transient improvement); decremental response to low frequency repetitive nerve stimulation; presence of AchR antibody titers

93
Q

treatment for MG

A

cholinesterase inhib or IVIG in neonatal; in juvenile, cholinesterase inhib and immunotherapy

94
Q

cholinesterase inhib used for MG

A

pyridostigmine bromide

95
Q

immunotherapy for MG

A

corticosteroids used with cholinesterase inhib fail; plasmapheresis; IVIG; thymectomy

96
Q

prognosis of MG

A

neonatal resolves in 1-3 weeks; juvenile- remission of sx can be as high as 60% after thymectomy

97
Q

hgb and age

A

hgb is high at birth and normally declines, reaching nadir between 2 and 3 mos of age in the term infant and between 1 and 2 mos in the preterm infant

98
Q

fetal hemoglobin disappears by when?

A

by 6-9 month

99
Q

usual percentage of RBCs that are retics

A

1%

100
Q

two most common types of microcytic hypochromic anemia durign childhood

A

iron def anemia and beta thal trait

101
Q

nutritional iron def most common in what two age groups?

A

9-24 mos and adolescent girls

102
Q

spoon shaped nails

A

sign of anemia

103
Q

increased free erythrocyte protoporphyrin

A

sign of anemia

104
Q

iron is given with what to enhance absorption?

A

vit C

105
Q

severe anemia can lead to

A

CHF, cardiac dilaton, shortness of breath, hepatosplenomegaly

106
Q

characteristics of the thalasemmias

A

hemolysis that leads to incr bone marrow activity (and incr size of bones in the face, skull, and elsewhere)

107
Q

alpha thal in what ethnicity

A

southeast asians

108
Q

4 types of alpha thal

A

silent carrier (one deletion- asymp); a-thal mino (2 del- mild anemia); HbH dz (3 del- severe anemia at birth, w elevated Hgb Bart); hydrops (4 del- death)

109
Q

2 types of beta thal

A

beta thal minor (1 del- mild asymp anemia); beta thal major (profound hemolytic anemia in infancy)

110
Q

race for beta thal major

A

mediterranean

111
Q

cooley’s anemai

A

aka beta thal major

112
Q

clinical features of beta thal mjor

A

hematosplenomegaly, bone marrow hyperplasia, thalassemia facies (frontal bossing, skull deformities), delayed growth

113
Q

lab findings in beta thal major

A

elevated serum iron, LDH; electrophoresis shows low or absent Hgb A and elevated Hgb F

114
Q

treatment of beta thal major

A

lifelong transfusions and often splenectomy; bone marrow transplant is a potential option

115
Q

complications of beta thal major

A

hemochromatosis, which is iron accum in various organs due to incr iron absorption in the intestines and infused RBCs

116
Q

beta thal minor- sx?

A

asympt; no treatment

117
Q

sideroblastic anemia

A

ring sideroblasts in the bone marrow; result from accum of iron in mitochondria of RBC precursors

118
Q

how do you get sideroblastic anemia?

A

may be inherited or acquired by drugs/toxins

119
Q

other causes of microcytic hypochromic anemia

A

lead poisoning and chronic disease like malignancy or infection

120
Q

two causes of macrocytic anemias

A

folic acid and vit b 12 def

121
Q

exclusive feedings with goat milk can cause what?

A

folic acid def

122
Q

how is vit B12 absorbed?

A

must combine with intrinsic factor secreted by the gastric parietal cells; absorption then occurs in the terminal ileum

123
Q

clinical features of b12 def

A

signs of anemia, smooth red tongue, and neuro manifestations

124
Q

treatment of b12 def

A

monthyl IM vit B12 injections

125
Q

causes of normocytic normochromic anemias

A

hemolytic anemias, red cell aplasias, and sickle cell anemia

126
Q

most common inherited abnormality of the RBC membrane

A

hereditary spherocytosis (northern european most common)

127
Q

etiology for hereditary spherocytosis

A

aut dom; defect in spectrin that causes rbc to be spherical

128
Q

clinical features of hereditary spheroctyosis

A

hemolytic anemia, so splenomegaly, pigmentary gallstones, and aplastic crises (esp associated with parvovirus)

129
Q

lab finding in hereditary spherocytosis

A

abnormal RBC fragility with osmotic fragility studies

130
Q

management of hereditary spherocytosis

A

blood transfusion; splenectomy delayed until 5 years of age

131
Q

why is splenectomy delayed until 5 yo?

A

to decr infections with encapsulated bacteria

132
Q

hereditary elliptocytosis

A

aut dom; defect in spectrin also; may or may not result in hemolysis; most patients are asympy

133
Q

two examples of glycolytic enzymatic defects of RBCs

A

pyruvate kinase def and G6PD def

134
Q

pyruvate kinase def

A

aut recessive; leads to ATP depletion and decr RBC survival

135
Q

blood smear for pyruvate kinase def

A

polychromatic RBCs

136
Q

management of PK def

A

transfusion and splenectomy

137
Q

most common RBC enzymatic def

A

G6PD def;

138
Q

G6PD def

A

may occur in acute hemolytic disease or induced by infection or meds;

139
Q

pathophys of G6PD def

A

RBC is not protected from oxidative stress; so anything from infection to fava beans to drugs can cause hemolysis; sx occur 24-48 hrs after exposure;

140
Q

lab findings in G6PD def

A

hemoglobinuria, incr retics, smear shows bite cells and hemighosts; heniz bodies

141
Q

treatment for G6PD def

A

transfusions needed; splenectomy not beneficial

142
Q

autoimmune hemolytic anemia

A

fulminant acute type can be preceded by a resp infection; prolonged type AHA is prolonged and prob underlying disease

143
Q

prognosis for acute and prolonged AIHA?

A

acute is good prognosis and prolonged is high mortality

144
Q

coomb’s test

A

detects coating of antibodies on the surface of RBCs or complement

145
Q

direct coombs result for AIHA

A

pos

146
Q

management of AIHA

A

corticoseroids, transfusion

147
Q

alloimmune hemolytic anemia

A

newborn Rh and ABO hemolytic anemia

148
Q

ABO alloimmune hemolytic anemia

A

can occur in the first pregnancy, unlike Rh

149
Q

coombs for ABO and Rh alloimmune anemia

A

coombs strongly pos in Rh, weakly pos in ABO

150
Q

microangiopathic hemolytic anemia

A

caused by passage through injured vessels; RBC fragments

151
Q

causes of microgiopathic hemolytic anemia

A

severe hypertension, hemolytic uremic syndrome; artificial heart valves; DIC

152
Q

lab findings for microangiopathic hemolytic anemia

A

RBC fragments (burr cells), target cells, irreg shaped cells, and thrombocytopenia

153
Q

cause of sickle cell disease

A

glutamic acid changes to valine on beta globin chain of HgB; results in stacking of HgB when RBC is exposed to low ox

154
Q

Sickle cell trait symptoms?

A

usually asymp without anemia, unless exposed to severe hypoxemia

155
Q

leading cause of death from sickle cell disease

A

infection as a result of decr splenic function;

156
Q

encapsulated bacteria

A

H. flu; Strep pneumonia; salmonella; neisseria meningitidis

157
Q

osteomyelitis in sickle cell disease

A

painful bone crisis; infection caused by salmonella acquired through the GI traact, though staph aureus can also cause it

158
Q

target cells

A

decr hemoglobin content means the volume is smaller, so there is a greater SA to volume ratio that causes the target cell shape

159
Q

howell-jolly bodies

A

indicates a damaged spleen, since these inclusions (DNA) are normally removed by the spleen

160
Q

preventative care for sickle cell disease

A

hydroxyurea increases Hgb F and decr vasooclusive crises; daily oral penicillin prophylaxis; daily folic acid; immunizations; serial transcranial or angiography to detect stroke

161
Q

life expectancy for sickle cell patient

A

40s

162
Q

sickle cell hemoglobin C disease

A

one hgb c and one hgb S; less severe than sickle cell disease

163
Q

RBC aplasias

A

anemia, reticulocytopenia, and few RBC precursors in the bone marrow

164
Q

three examples of RBC aplasias

A

Diamond-Blackfan anemia (congenital hypoplastic anemia); transient erythroblastopenia of childhood; and parvovirus B19- associateed red cell aplasia

165
Q

pancytopenia

A

bone marrow failure with decreased RBCs, leukocytes, and platelets

166
Q

fanconi anemia

A

congenital aplastic anemia

167
Q

genetic of fanconi anemia

A

aut recessive

168
Q

clinical features of fanconi anemia

A

onset of BM failure at mean age 7 yo; ecchymosis and petechiae; skeletal abnormalities; skin hyperpigmentation, renal abnormalities

169
Q

skeletal abnormalities in pancytopenia

A

short stature, absence or hypoplasia of the thumb and radius

170
Q

Lab findings in congenital aplastic anemia

A

pancytopenia, RBC macrocytosis, low retics, elevated Hgb F, and bone marrow hypocellularity

171
Q

treatment of congenital aplastic anemia

A

transfusions and BM transplant

172
Q

acquired aplastic anemia causes

A

drugs, infections, chemicals, radiation, idiopathic

173
Q

clinical features of acquired aplastic anemia

A

bruising, petechiae, pallor, or serious infection as a result of neutropenia

174
Q

polycythemia

A

hematocrit over 60%

175
Q

cause of primary polycythemia

A

polycythemia vera- a malignancy

176
Q

secondary polycythemia cause

A

increased EPO

177
Q

causes of approp increase in EPO

A

hypoxemia as a result of congenital heart disease (this is the most common cause of polycythemia in kids!), pulm disease, or high altitude

178
Q

inapprop causes of incr EPO

A

tumors of kidney, cerebellum, ovary, liver, and adrenal gland; excess hormone production (steroids, GH, androgens), and kidney abnormalities like hydronephrosis

179
Q

clinical features of polycythemia

A

ruddy facial complexion with normal size liver and spleen

180
Q

most common cause of relative polycythemia

A

dehydration

181
Q

complicaitons of polycythemia

A

thromboiss and bleeding

182
Q

difference between congenital hypoplastic anemia (diamond-blackfan) and transient erythroblastopenia of childhood

A

diamond-blackfan is aut recess and TEC is post-viral autoimmune; D-B needs transfusion/BMT and TEC has spontaneous recovery

183
Q

anemia due to parvovirus b19

A

associated URI sx; anemia generally not symptomatic; spontaneous recovery in a few weeks

184
Q

hemarthroses

A

bleeding into joint spaces

185
Q

two factor VIII disorders

A

vWF and hemophilia A

186
Q

hemophophilia

A

defect in factor VIII procoagulant activity; prolongs aPTT but not PT

187
Q

vWD

A

factor VIII variable but platelet function is defective; prolonged aPTT, normal PT; prolonged bleeding time; platelet COUNT normal

188
Q

von willebrands factor

A

substance necessary for platelet adhesion to blood vessel walls

189
Q

hemophilia A inheritance

A

x-linked

190
Q

clinical features of hemophilia A

A

hemarthroses, deep soft tissue bleeds;

191
Q

treatment for hemophilia

A

replacement of factor VIII; DDAVP may cause the release of stored factor VIII from patient’s own cells

192
Q

most common hereditary bleeding disorder

A

von willebrand’s disease

193
Q

3 types of von willebrand disease

A

type 1 (classic)- mild quantitative deficiencies in factor VIII and vWF; type 2- qualitative abnormality in vWF; type 3- absence of vWF (most severe)

194
Q

hemarthroses in vwf?

A

unusual

195
Q

ristocetin cofactor assay

A

quanititative assay for vWF activity; diagnositc ofvWD

196
Q

management of vwD

A

DDAVP induces vWF release from endothelial cells (can be used for prophylaxis too); cryoprecipitate, which contains intact vWF

197
Q

hemophilia B

A

factor IX def; aka christmas disease; x-linked disorder

198
Q

vit K def is essential for synthesis of what factors?

A

2, 7, 9, and 10, and proteins C and S

199
Q

etiology of vit K def

A

pancreatic insuff, biliary obstruction, diarrhea; dietary def is rare; medications; hemorrhagic disease of the newborn

200
Q

medications that interfere with vit K metabolism

A

cephalasporins, rifampin, isoniazid, and warfarin; these cause vit K def

Peds (99 decks)

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