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Pediatrics > Jeopardy 3- Neuro Heme Onc > Flashcards

Jeopardy 3- Neuro Heme Onc Flashcards

1
Q

Central Hypotonia involves

A

UMNs and the cortical pyramidal neurons/corticospinal tract

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2
Q

Peripheral hypotonia involves

A

LMNs and Spinal Motor Neurons

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3
Q

Symptoms of central hypotonia

A

Altered mental status, increased DTRs and clonus

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4
Q

Symptoms of peripheral hypotonia

A

Decreased muscle bulk and DTRs

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5
Q

Hypotonia + weakness + tongue fasciculations

A

Spinal Muscular Atrophy

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6
Q

2nd most common hereditary neuromuscular disorder

A

SMA

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7
Q

Area affected by SMA

A

Anterior Horn Cell Degeneration

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8
Q

Type of SMA occurring before 6 months

A

Werding-Hoffman Disease

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9
Q

Inheritance of SMA

A

AR, SMN1 gene on Chromosome 5

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10
Q

Function of Botulinum Toxin

A

Prevents presynaptic release of ACh

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11
Q

Botulism symptoms occur when after ingestion

A

12-48 hrs

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12
Q

FIRST SYMPTOM of Botulism

A

Constipation

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13
Q

Symmetric and descending paralysis

A

Botulism

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14
Q

EMG of botulism shows

A

Small amplitude potentials + incremental response during high frequency stimulation

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15
Q

Treatment of botulism

A

Botulism Immune Globulin

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16
Q

Genetics of Congenital Myotonic Dystrophy

A

AD trinucleotide repeat on Chromosome 19

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17
Q

Bilateral weakness + Hypotonia + Areflexia + Arthrogryposis (joint contractures)

A

Congenital Myotonic Dystrophy

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18
Q

Adults w/ Congenital Myotonic Dystrophy have what complications

A

Mental Retardation + Cataracts + Arrhythmias + Infertility

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19
Q

Enlarged ventricles caused by obstruction

A

Noncommunicating Hydrocephalus

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20
Q

Enlarged ventricles caused by decreased absorption

A

Communicating Hydrocephalus

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21
Q

Type of hydrocephalus seen w/ meningitis

A

Communicating

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22
Q

Ventricular enlargement 2/2 brain atrophy

A

Hydrocephalus ex Vacuo

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23
Q

Downward displacement of the Cerebellum and Medulla through the foramen magnum

A

Chiari Type II Malformation

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24
Q

Chiari Type II malformations are associated w/

A

Lumbosacral myelomeningocele

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25
Q

Absent/Hypoplastic Cerebellar Vermis –> cystic enlargement of the 4th Ventricle –> blocks CSF

A

Dandy-Walker Malformation

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26
Q

Inheritance of Congenital Aqueductal Stenosis

A

X-linked Recessive

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27
Q

MOST COMMON cause of hydrocephalus in preterm infants

A

Intraventricular Hemorrhage

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28
Q

Sunset Sign (downward deviation of both eyes) is caused by

A

Hydrocephalus (enlargement of 3rd Ventricle)

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29
Q

Failure of bone fusion of the vertebral column

A

Spina Bifida

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30
Q

Herniation of the spinal cord tissue and meninges through bony cleft

A

Myelomeningocele

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31
Q

Herniation of only meninges through bony cleft

A

Meningocele

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32
Q

Teratogens that cause Spina Bifida

A

Valproate, Phenytoin, Colchicine, Vincristine, Azathioprine, MTX

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33
Q

Lumbosacral myelomeningoceles (90%) are associated w/

A

Chiari Type II Malformation

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34
Q

Accumulation of fluid within the spinal cord

A

Cervical Hydrosyringomyelia

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35
Q

Serum protein elevated w/ spinal defects

A

Alpha-Fetoprotein

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36
Q

MOST SENSITIVE test for diagnosing spinal defects

A

Fetal Sonogram

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37
Q

MOST COMMON causes of coma in younger than 5

A

Non-accidental trauma and near-drowning

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38
Q

MOST COMMON cause of coma in older children

A

Drug OD and Accidental Head Injury

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39
Q

CSF or blood draining from the nose or auditory canal

A

Basilar Skull Fracture

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40
Q

Decerabrate posturing is seen w/

A

sub-cortical injury

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41
Q

Decorticate posturing is seen w/

A

Bilateral cortical injury

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42
Q

Cheyne-Stokes breathing is associated w/

A

Bilateral cortical injury

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43
Q

Apneustic breathing (pausing at inspiration) is seen w/

A

Pontine damage

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44
Q

Uncal herniation has what physical sign

A

Unilateral dilated non-reactive pupil

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45
Q

Normal caloric response

A

Eye deviation to the irrigated side

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46
Q

Criteria for epilepsy

A

> 2 spontaneous unprovoked seizures

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47
Q

Time for status epilepticus

A

> 30 mins

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48
Q

% who have an afebrile seizure before 16

A

4-6%

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49
Q

Incidence of epilepsy

A

.5-.8%

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50
Q

MOST COMMON type of generalized seizure

A

Tonic-Clonic

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51
Q

Treatment of status epilepticus

A

IV Diazepam/Lorazepam… repeat… Phenobarbital or Phenytoin

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52
Q

Best treatment for generalized epilepsy

A

Valproate or Phenobarbital

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53
Q

Best treatment for absence epilspsy

A

Ethosuximide

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54
Q

Best treatment for partial epilepsy

A

Carbamazepine or phenytoin

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55
Q

COMMON SIDE EFFECT of vagal nerve stimulators

A

Hoarseness

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56
Q

% Children who have febrile seizures

A

3%

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57
Q

What makes a febrile seizure complicated

A

Focal signs, recurs within 24hrs, >15 mins

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58
Q

Percent of patients w/ febrile seizures who have a recurrence

A

30%

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59
Q

MOST COMMON cause of Infantile Spasm (West Syndrome)

A

Tuberous Sclerosis

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60
Q

Brief myoclonic jerks (arm extension, head/trunk flexion) lasting 1-2seconds which occur 5-10 x over about 3minutes

A

Infantile Spasm (West Syndrome)

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61
Q

Age range for Infantile Spasm (West Syndrome)

A

3-8 months

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62
Q

EEG of Hypsarrhythmia

A

Infantile Spasm (West Syndrome)

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63
Q

Tx of infantile spasm

A

ACTH, Valproate or Vigabatrin

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64
Q

Best treatment for Infantile Spasm 2/2 Tuberous Scleosis

A

Vigabatrin

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65
Q

Inheritance of Absence Epilepsy

A

AD

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66
Q

EEG of 3-Hz Spike and Wave Discharges

A

Absence Epilepsy

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67
Q

MOST COMMON partial epilepsy of childhood

A

Benign Rolandic Epilepsy (Benign Centrotemporal Epilepsy)

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68
Q

Inheritance of Benign Rolandic Epilepsy (Benign Centrotemporal Epilepsy)

A

AD

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69
Q

Early morning oral-buccal seizures (moan, grunt, saliva) –> generalize to Tonic Clonic

A

Benign Rolandic Epilepsy (Benign Centrotemporal Epilepsy)

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70
Q

First line drug for Benign Rolandic Epilepsy (Benign Centrotemporal Epilepsy)

A

Valproic Acid

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71
Q

MOST COMMON cause of HA in children

A

Migraine

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72
Q

Path of Migraines

A

Changes to cerebral blood flow

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73
Q

MOST COMMON form of migraine in children

A

Migraine without aura

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74
Q

Ptosis/ CN III palsy w/ HA

A

Ophthalmoplegic Migraine

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75
Q

Vertigo, tinnitus, ataxia or dysarthria prior to HA

A

Basilar Artery Migraine

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76
Q

Migraine prophylactic drug of choice

A

Propranolol

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77
Q

MOST COMMON cause of ataxia in children

A

Acute Cerebellar Ataxia

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78
Q

Common infections –> acute cerebellar ataxia

A

Varicella, influenza, EBV and mycoplasma

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79
Q

Ascending weakness + areflexia + normal sensation

A

Guillain Barré Syndrome

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80
Q

MOST COMMON cause of Guillain Barré

A

Campylobacter Jejuni

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81
Q

Path of Guillain Barré

A

Demyelniation of ventral spinal roots and peripheral nerves via cell mediated response to infection

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82
Q

Ophthalmoplegia + Ataxia + Areflexia

A

Miller-Fischer Syndrome

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83
Q

LP in Guillain Barré shows

A

Albuminocytologic Dissociation (increased protein, norm cell count)

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84
Q

Unable to maintain protrusion of tongue (Charmeleon Sign) and unable to maintain grip (Milkmaid’s Grip)

A

Sydenham Chorea

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85
Q

Treatment of Sydenham Chorea

A

Haloperidol, Valproic Acid or Phenobarbital

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86
Q

Tics occur in

A

3% children

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87
Q

Incidence of Tourretes

A

1/1000

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88
Q

Corprolalia occurs in what % of Tourretes

A

15%

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89
Q

Tx of Tourretes

A

Pimozide, Clonidine, Haldoperidol (risk Tardive Dyskinesia)

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90
Q

DMD and BMD are

A

X-linked deletion in Dystrophin Genes

91
Q

First sign of DMD/BMD

A

Weakness of the legs

92
Q

Cause of pseydohypertrophy of calves

A

lipids replacing degenerating muscle

93
Q

Cause of death in DMD

A

respiratory failure in teens

94
Q

Path of Myasthenia Gravis

A

Antibodies against ACh receptors at NMJ

95
Q

Bilateral ptosis + weakness at end of day + diplopia

A

MG

96
Q

Diagnosis of MG

A

Tensilon Test (IV Edrophonium –> transient improvement of ptosis)

AChR Antibody Titers

97
Q

Surgery done in pts w/ MG

A

Thymectomy

98
Q

Treatment of MG

A

Pyridostigmine

99
Q

HgB nadir occurs at

A

2-3 months (1-2 in preterm)

100
Q

Fetal Hg disappears by

A

6-9 months

101
Q

MOST COMMON blood disease during childhood

A

Iron Deficiency Anemia

102
Q

Spoon shaped nails are seen w/

A

Iron Deficiency Anemia

103
Q

Iron Deficiency lab findings

A

Low Iron, Ferritin and %Sat; High Transferrin and Erythrocyte protoporphyrin

104
Q

Substance given w/ iron to increase intestinal absorption

A

Vitamin C

105
Q

Disease seen w/ Southwest Asians

A

Alpha thal

106
Q

Hgb Bart is seen w/

A

Hemoglobin H Disease (3 alpha globins gone)

107
Q

Beta Thal is seen in

A

Mediterraneans

108
Q

Microcytosis, Target Cells and Poikilocytes

A

Beta Thal

109
Q

Complication of Beta Thal

A

Hemochromatosis (increased iron from intestines and from transfusions)

110
Q

Cause of sideroblastic anemia

A

Iron in mitochondria of RBC precursors

111
Q

Drugs that cause Sideroblastic Anemia

A

Isoniazid, Alcohol, Lead Poisoning, Chloramphenicol

112
Q

Macrocytic =

A

MCV > 95

113
Q

B12 is absorbed at the

A

Terminal Ileum after binding w/ Intrinsic Factor from the Parietal Cells

114
Q

Smooth Red Tongue + Ataxia + Hyporeflexia + Babinski

A

B12 Deficiency

115
Q

MOST COMMON inherited abnormality of RBC membrane

A

Hereditary Spherocytosis

116
Q

Genetics of Hereditary Spherocytosis

A

AD mutation in Spectrin

117
Q

Clinical Sx of Hereditary Spherocytosis

A

Pallor, weakness, pigmented gallstones and aplastic crisis

118
Q

Diagnostic Test for Hereditary Spherocytosis

A

Osmotic Fragility Test and Abnormal RBC Fragility

119
Q

Genetics of Hereditary Elliptocytosis

A

AD mutation in Spectrin

120
Q

Inheritance of Pyruvate Kinase Deficiency

A

AR

121
Q

Polychromic RBCs suggest

A

Pyruvate Kinase Deficiency

122
Q

MOST COMMON RBC enzymatic defect

A

Glucose-6-Phosphate Dehydrogenase Deficiency

123
Q

Bite Cells and Heinz Bodies

A

G6PD

124
Q

Drugs that can trigger hemolysis in G6PD

A

Salicylates, Sulfa an Anti-Malarials

125
Q

Diagnostic test for Autoimmune Hemolytic Anemias

A

Direct Coombs Test Positive

126
Q

Condition that can cause hemolytic disease in first child

A

ABO Hemolytic Disease

127
Q

Incidence of SS Disease

A

1/800 (8% have trait)

128
Q

Path of SS

A

Valine –> Glutamic Acid at #6 of B-globin chain

129
Q

MOST COMMON crisis in SS

A

Painful Bone Crisis

130
Q

Leading cause of death in SS patients

A

INFECTION (sepsis or meningitis)

131
Q

Function of Hydroxyuria in SS

A

Increases Hgb F

132
Q

Vaccines to give in SS

A

23-Valent Polysaccharide Pneumococcal at 2yrs and Meningococcal

133
Q

Evaluation of stroke in SS

A

Begin at 2yrs w/ serial transcranial doppler or MRA

134
Q

3 Types of Childhood Red Blood Cell Aplasias

A

1) Diamond Blackfan Anemia
2) Transient Erythroblastopenia of Childhood (TEC)
3) Parvovirus B19

135
Q

Anemia + cranial/facial anomalies + cardiac and renal anomalies + short stature + triphalangeal thumbs

A

Diamond Blackfan Anemia

136
Q

Pancytopenia + Short Stature + Absence or hypo plastic thumb + skin hyper pigmentation + renal problems

A

Fanconi Anemia

137
Q

Inheritance pattern of Fanconi Anemia

A

AR

138
Q

Drugs that can cause aplastic anemia

A

Sulfonamides, AEDs, Chloramphenicol

139
Q

MOST COMMON cause of polycythemia in childhood

A

Congenital Heart Disease

140
Q

Hemophilia A is a deficiency in

A

Factor VIII

141
Q

Inheritance of Hemophilia A

A

XL

142
Q

Treatment of Hemophilia A

A

DDAVP (Desmopressin Acetate)

143
Q

MOST COMMON hereditary bleeding disorder

A

VWD

144
Q

Assay for VWF activity

A

Ristocetin Cofactor assay

145
Q

Vitamin K dependent factors

A

1972 (10, 9, 7, 2, Protein C and S)

146
Q

Disease causing large hemangiomas

A

Kasabach-Merritt Syndrome

147
Q

Path of HSP

A

IgA mediated vasculitis

148
Q

Symptoms of HSP

A

Palpable purpura, renal issues, abdominal pain and arthritis

149
Q

Inheritance of Hereditary Hemorrhagic Telangiectasia

A

AD

150
Q

Small Platelets, thrombocytopenia, eczema and defects in T and B cells

A

Wiskott-Aldrich Syndrome

151
Q

Inheritance of Wiskott Aldrich Syndrome

A

XL

152
Q

AR disease w/ thrombocytopenia + absent radius + cardiac/renal problems

A

Thrombocytopenia-Absent Radius Syndrome

153
Q

MOST COMMON acquired platelet abnormality in childhood

A

Immune Thrombocytopenic Purpura

154
Q

Cause of ITP

A

Cross reaction 1-4 wks after Viral Infection where antibodies target platelets

155
Q

Treatment of ITP

A

IVIG

156
Q

Cause of Passive Autoimmune Thrombocytopenia

A

Mom with ITP and antibodies to platelets cross placenta

157
Q

Cause of Isoimmune Thrombocytopenia

A

Mom makes antibodies to baby’s platelets

158
Q

AR disease of GIIb/IIIa on platelet membrane

A

Glanzmann’s Thrombasthenia

159
Q

AR disease of GPIb on platelet

A

Bernard-Soulier Syndrome

160
Q

MOST POTENT anticoagulant

A

Protein C

161
Q

Shock + fever + skin bleeding + intravascular thrombosis

A

Purpura Fulminans (presentation of Protein C def)

162
Q

MOST COMMON cause of neutropenia

A

Infection

163
Q

AR severe agranulocytosis w/ infections beginning in infancy

A

Kostmann Syndrome

164
Q

Albinism + Blue-Gray Granules + Neutropenia + Silver Hair

A

Chediak-Higashi

165
Q

Pancreatic Insufficiency + Short Stature + Metaphyseal chondrodysplasia + neutropenia

A

Schwachman-Diamond Syndrome

166
Q

Leading cause of death from disease in kids

A

Cancer

167
Q

MOST COMMON childhood cancer

A

Acute Lymphocytic Leukemia

168
Q

Wiscott Aldrich predisposes to

A

Lymphoma and Leukemia

169
Q

Leukocoria is associated w/

A

Retinoblastoma

170
Q

Anemia + Thrombocytopenia + WBC changes (norm, high or low)

A

ALL

171
Q

Factors making for a favorable ALL prognosis

A

Female, 1-9, CALLA+, no t(9,22)

172
Q

Induction tx for ALL is

A

Intrathecal MTX

173
Q

Causes of neutropenic fever

A

Staph aureus, Staph epidermidis, Pseudomonas, E. coli

174
Q

Cancer associated w/ Down Syndrome

A

Acute Myelogenous Leukemia (specifically M7- megakaryocytic)

175
Q

Fever + HSM + gingival hypertrophy and bone pain

A

AML

176
Q

Leukemic Myeloblasts and Auer Rods

A

AML

177
Q

Cancer associated w/ t(9,22)

A

CML (Philadelphia Chromosome- BCR/ABL)

178
Q

Fever + eczema-like facial rash + petechiae + lymphadenopathy

A

Juvenile Chronic Myelogenous Leukemia

179
Q

Cancer of APCs

A

Hodgkin Disease

180
Q

Infection associated w/ Hodgkin

A

EBV

181
Q

Reed-Sternberg Cell

A

Hodgkin Lymphoma

182
Q

Complications of treatment of Hodgkin Lymphoma

A

Growth retardation, secondary malignancy, hypothyroidism and male sterility

183
Q

MOST COMMON presenting symptom of Non-Hodgkin Lymphoma

A

Painless Lymphadenopathy

184
Q

Cancer presenting as an anterior mediastinal mass and possible SVC syndrome

A

Lymphoblastic Lymphoma

185
Q

What condition must be considered w/ intussusecption

A

Lymphoma

186
Q

Jaw Mass in an African

A

Burkitt Lymphoma

187
Q

MOST COMMON solid tumor of childhood

A

Brain Cancer

188
Q

MOST COMMON type of brain cancer in kids

A

Glial Cell Tumors

189
Q

Most common location of brain tumors

A

Infratentorial

190
Q

MOST COMMON infratentorial tumor

A

Medulloblastoma

191
Q

MOST COMMON supratentorial tumor

A

Astrocytoma

192
Q

CSF findings for medulloblastoma

A

Homovanillic Acid, (HVA) VMA and Polyamines

193
Q

Brain cancer w/ poorest prognosis

A

Brainstem Glioma

194
Q

Urine VMA, HVA and catecholamines

A

Neuroblastoma

195
Q

MOST COMMON childhood renal tumor

A

Wilms Tumor

196
Q

Genetic Conditions associated w/ Wilms Tumor

A

Beckwith-Wiedemann Syndrome (hemihypertrophy, macroglossia, visceromegaly), deletion of q11 and WAGR Syndrome (Wilms + Aniridia +GU problems + Retardation)

197
Q

Tumor that does not cross abdominal midline

A

Wilms

198
Q

% Wilms tumors that are bilateral

A

5%

199
Q

MOST COMMON site of rhabdomyosarcoma

A

Head and Neck

200
Q

MOST COMMON bone tumor

A

Osteosarcoma

201
Q

Site of Osteosarcoma

A

Metaphysis of long bones

202
Q

Site of Ewings

A

Flat bones and diaphysis

203
Q

Systemic symptoms are more common with which bone tumor

A

Ewings

204
Q

Sunburst Pattern

A

Osteosarcome

205
Q

Onion skin appearance and periosteal retractions

A

Ewings

206
Q

Translocation w/ Ewings

A

11 and 21

207
Q

Hepatoblastoma is associated w/

A

Beckwith-Widemann Syndrome

208
Q

Bony skull lesions + chronic draining ears + seborrheic dermatitis + pituitary involvement

A

Langerhans Cell Histiocytosis

209
Q

Neurofibromatosis II

A

Acoustic Neuroma

210
Q

Neurofibromatosis I

A

Brain tumors, lymphoma, leukemia and schwannoma

211
Q

Beckwith-Wiedemann Syndrome

A

Wilms, Hepatoblastoma, Rhabdomyosarcoma, Adrenal

212
Q

Bloom Syndrome

A

Leukemia/Lymphoma and GI tumors

213
Q

Ataxia Telangiectasia

A

Hodgkin and non-Hodgkin

214
Q

Fanconi Anemia

A

Leukemia

215
Q

Klinefelter

A

Leukemia, Germ Cell and Breast

216
Q

Trisomy 18

A

Wilms and neurogenic tumors

217
Q

Trisomy 13

A

Leukemia and teratoma

218
Q

Turner

A

Gonadoblastoma

219
Q

Downs

A

ALL or AML

220
Q

Prolonged PTT only

A

Factor VIII, IX deficiency,

221
Q

Prolonged PTT and Bleeding Time

A

VWF

222
Q

Prolonged bleeding time only

A

Platelet dysfunction OR thrombocytopenia

223
Q

Prolonged PT and PTT

A

Vitamin K Deficiency

224
Q

Prolonged PT, PTT and Bleeding Time

A

DIC

Pediatrics (4 decks)

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