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1

clinical features of absence epilepsy of childhoo

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

2

absence seizures on ee

generalized spike and wave discharge arising from both hemispheres

3

management of sbsence seizures

ethosuximide (first line) or valproic acid

4

prognosis for absence seizures

very good; seizures usually resolve by adolescence

5

benign rolandic epilepsy

aka benign centrotemporal epilepsy; nocturnal partial seizures with secondary generalization

6

most common partial epilepsy durign childhood

benign rolandic epilepsy

7

what age for benign rolandic seizures?

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

8

inheritance of benign rolandic seizures

aut dom, with variable penetrance

9

clinical features of benign rolandic epilepsy

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

10

benign rolandic epilepsy on EEG

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

11

treatment fo rbenign rolandic seizures

valproic acid of carbamazepine

12

prognosis for benign rolandic seizures

excellent; seizures remit spontaneously during adolescence

13

throbbing or pounding headache

suggests migraine

14

aching feeling of pressure in the headache

suggests tension

15

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

migraine

16

generalized or bitemporal headaches

tension headaches

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headaches in the morning

from incr ICP

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headaches at night

tension headaches

19

most common cause of headaches in kids

migraines

20

inheritance of migaines

aut dom

21

what is the pathophys of migraines?

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

22

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

without

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migraine equivalent

no headache but transient vomitting, abdom pain, or vertigo

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ophthalmoplegic migraine

unilateral ptosis or CN III palsy accompanies headache

25

basilar artery migraine

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

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abortive treatment for migraines

sumatriptan (selective seratonin agonist)

27

prophylactic treatment for migraines

propranolol

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prognosis for migraines

waxing and waning but often lifelong disorder

29

tension headaches age

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

30

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

tension headache

31

treatment for tension headaches

reassurance and pain control, stress and anxiety reduction

32

cluster headaches

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

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cluster headaches in childhood?

rare

34

treatment for cluster headaches

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

35

ataxia

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

36

acute cerebellar ataxia

unsteady gait 2/2 a presumed autoimmune or postinfectious cause

37

most common cause of ataxia in kids

acute cerebellar ataxia

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age for acute cerebellar ataxia

18 month to 7 years; rarely after age 10

39

common preceding infections for acute cerebellar ataxia are?

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

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cause of acute cerebellar ataxia

immune complex deposition in the cerebellum

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clinical features of acute cerebellar ataxia

truncal ataxia, slurred speech, nystagmus, fever is absent

42

treatment of acute cerebellar ataxia

supportive; complete resolution in 2-3 months

43

guillain-barre syndrome

acute inflamm demyelinating polyneuropathy

44

most common associated infectiuous agent in guillain barre

campylobacter jejuni (prodromal gastroenteritis)

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principle sites of demyelination in G-B

ventral spinal roots and peripheral myelinated nerves

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pathophys of G-B

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

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clinical features of G-B

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

48

miller-fisher syndrome

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

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dx of G-B

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

50

albuminocytologic dissoc

elevated CSF protein in the absence of elevated cell count

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EMG for G-B

decr nerve conduction velocity or conduction block

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dx G-B in kids less than 3

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

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treatment of G-B

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

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prognosis for G_B

complete recovery is the rule in kids

55

sydenham chorea

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

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is syndenham chorea common?

well it occurs in a quarter of people with rheumatic fever

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onset age for syndenham chorea

5 and 13 yo

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pathophys of syndenham chorea

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

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clinical features of syndenham chorea

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

60

chameleion tongue

in syndenham chorea; unable to sustain protrusion of the tongue

61

choreic hand

hand flexed and hyperextended at metacarpal joints

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milkmaid's grip

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

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diagnosis of sydnenham chorea

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

64

treatment of sydenham chorea

haloperidol, valproic acid, or phenobarbitol

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prognosis of syndenham chorea

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

66

tourette syndrome

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

67

tics in tourettes

must be present for at least 1 year

68

coprolalia

uttering obscene words

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associated findings in tourettes

learning disabilities; ADHD; ocd

70

management of tourettes

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

71

prognosis of tourettes

tics decr in adulthood; drugs generally successful

72

duchenne and becker muscular dystrophies

both are x-linked myopathies characterized by motor degeneration

73

clonidine

alpha 2 ag; used to treat htn

74

which is more severe- duchenne or becker MD?

duchenne

75

onset of musc dystrophy sx?

between 2 and 5 yo

76

genetics of DMD and BMD

x linked; deletion in the dystrophin gene

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pathophys if muscular dystrophy

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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pathology of DMD and BMD

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

79

clinical features of DMD and BMD

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

80

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

dmd by ten years; bmd by 20 yo or laer

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gower's sign

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

82

cognitive impairment in musc dystrophy?

mild in DMD but normal intelligence in BMD

83

diagnosis of muscular dystrophy

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

84

management of muscular dystrophy

no cure but oral steroids can help improve strength a little

85

life expectancy for DMD? For BMD?

DMD- late teens; BMD- into their 50

86

myasthenia gravis

autoimmune that presents w progressive weakness

87

pathophys of myasthenia gravis

antibodies to Ach receptor at NMJ

88

neonatal vs juvenile MG

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

MG in girls vs boys

juvenile MG is 6x more common in girls than boys

90

most common presenting sign in juvenile MG

bilateral ptosis

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clinical features of juvenile MG

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

92

dx of MG

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

treatment for MG

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

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cholinesterase inhib used for MG

pyridostigmine bromide

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immunotherapy for MG

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

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prognosis of MG

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

97

hgb and age

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

fetal hemoglobin disappears by when?

by 6-9 month

99

usual percentage of RBCs that are retics

1%

100

two most common types of microcytic hypochromic anemia durign childhood

iron def anemia and beta thal trait

101

nutritional iron def most common in what two age groups?

9-24 mos and adolescent girls

102

spoon shaped nails

sign of anemia

103

increased free erythrocyte protoporphyrin

sign of anemia

104

iron is given with what to enhance absorption?

vit C

105

severe anemia can lead to

CHF, cardiac dilaton, shortness of breath, hepatosplenomegaly

106

characteristics of the thalasemmias

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

107

alpha thal in what ethnicity

southeast asians

108

4 types of alpha thal

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

2 types of beta thal

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

110

race for beta thal major

mediterranean

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cooley's anemai

aka beta thal major

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clinical features of beta thal mjor

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

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lab findings in beta thal major

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

114

treatment of beta thal major

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

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complications of beta thal major

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

116

beta thal minor- sx?

asympt; no treatment

117

sideroblastic anemia

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

118

how do you get sideroblastic anemia?

may be inherited or acquired by drugs/toxins

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other causes of microcytic hypochromic anemia

lead poisoning and chronic disease like malignancy or infection

120

two causes of macrocytic anemias

folic acid and vit b 12 def

121

exclusive feedings with goat milk can cause what?

folic acid def

122

how is vit B12 absorbed?

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

123

clinical features of b12 def

signs of anemia, smooth red tongue, and neuro manifestations

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treatment of b12 def

monthyl IM vit B12 injections

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causes of normocytic normochromic anemias

hemolytic anemias, red cell aplasias, and sickle cell anemia

126

most common inherited abnormality of the RBC membrane

hereditary spherocytosis (northern european most common)

127

etiology for hereditary spherocytosis

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

128

clinical features of hereditary spheroctyosis

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

129

lab finding in hereditary spherocytosis

abnormal RBC fragility with osmotic fragility studies

130

management of hereditary spherocytosis

blood transfusion; splenectomy delayed until 5 years of age

131

why is splenectomy delayed until 5 yo?

to decr infections with encapsulated bacteria

132

hereditary elliptocytosis

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

133

two examples of glycolytic enzymatic defects of RBCs

pyruvate kinase def and G6PD def

134

pyruvate kinase def

aut recessive; leads to ATP depletion and decr RBC survival

135

blood smear for pyruvate kinase def

polychromatic RBCs

136

management of PK def

transfusion and splenectomy

137

most common RBC enzymatic def

G6PD def;

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G6PD def

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

139

pathophys of G6PD def

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;

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lab findings in G6PD def

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

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treatment for G6PD def

transfusions needed; splenectomy not beneficial

142

autoimmune hemolytic anemia

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

143

prognosis for acute and prolonged AIHA?

acute is good prognosis and prolonged is high mortality

144

coomb's test

detects coating of antibodies on the surface of RBCs or complement

145

direct coombs result for AIHA

pos

146

management of AIHA

corticoseroids, transfusion

147

alloimmune hemolytic anemia

newborn Rh and ABO hemolytic anemia

148

ABO alloimmune hemolytic anemia

can occur in the first pregnancy, unlike Rh

149

coombs for ABO and Rh alloimmune anemia

coombs strongly pos in Rh, weakly pos in ABO

150

microangiopathic hemolytic anemia

caused by passage through injured vessels; RBC fragments

151

causes of microgiopathic hemolytic anemia

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

152

lab findings for microangiopathic hemolytic anemia

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

153

cause of sickle cell disease

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

154

Sickle cell trait symptoms?

usually asymp without anemia, unless exposed to severe hypoxemia

155

leading cause of death from sickle cell disease

infection as a result of decr splenic function;

156

encapsulated bacteria

H. flu; Strep pneumonia; salmonella; neisseria meningitidis

157

osteomyelitis in sickle cell disease

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

158

target cells

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

159

howell-jolly bodies

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

160

preventative care for sickle cell disease

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

161

life expectancy for sickle cell patient

40s

162

sickle cell hemoglobin C disease

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

163

RBC aplasias

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

164

three examples of RBC aplasias

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

165

pancytopenia

bone marrow failure with decreased RBCs, leukocytes, and platelets

166

fanconi anemia

congenital aplastic anemia

167

genetic of fanconi anemia

aut recessive

168

clinical features of fanconi anemia

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

169

skeletal abnormalities in pancytopenia

short stature, absence or hypoplasia of the thumb and radius

170

Lab findings in congenital aplastic anemia

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

171

treatment of congenital aplastic anemia

transfusions and BM transplant

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acquired aplastic anemia causes

drugs, infections, chemicals, radiation, idiopathic

173

clinical features of acquired aplastic anemia

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

174

polycythemia

hematocrit over 60%

175

cause of primary polycythemia

polycythemia vera- a malignancy

176

secondary polycythemia cause

increased EPO

177

causes of approp increase in EPO

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

178

inapprop causes of incr EPO

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

179

clinical features of polycythemia

ruddy facial complexion with normal size liver and spleen

180

most common cause of relative polycythemia

dehydration

181

complicaitons of polycythemia

thromboiss and bleeding

182

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

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

183

anemia due to parvovirus b19

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

184

hemarthroses

bleeding into joint spaces

185

two factor VIII disorders

vWF and hemophilia A

186

hemophophilia

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

187

vWD

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

188

von willebrands factor

substance necessary for platelet adhesion to blood vessel walls

189

hemophilia A inheritance

x-linked

190

clinical features of hemophilia A

hemarthroses, deep soft tissue bleeds;

191

treatment for hemophilia

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

192

most common hereditary bleeding disorder

von willebrand's disease

193

3 types of von willebrand disease

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

hemarthroses in vwf?

unusual

195

ristocetin cofactor assay

quanititative assay for vWF activity; diagnositc ofvWD

196

management of vwD

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

197

hemophilia B

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

198

vit K def is essential for synthesis of what factors?

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

199

etiology of vit K def

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

200

medications that interfere with vit K metabolism

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