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1
Q

multiple bacterial infections, MC lung and skin infection, Absence of MCH-1, Bare lymphocyte

A

Peptide transporter (TAP)

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2
Q

Galactorrhea, no mass, no pain, neg hCG, Amenorrhea, infertility

A

inc prolactin, dec GnRH–>LH & FSH Low

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3
Q

Basal ganglia atrophy, involuntary limb movement

A

Repeats: AD: HD(CAG), AR: Friedreich Ataxia
(GAA)

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4
Q

polygonal tumor cell, amyloid: amorphous protein, scanty colloid, few normal cell

A

Medullary thyroid carcinoma

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5
Q

Medullary Thyroid carcinoma association

A

MEN 2a (. ), MEN 2b ()

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6
Q

low energy state, vomiting, viral infection, jaundice and encephalopathy. damaged organelle in liver or brain?

A

Mitochondrial

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7
Q

AML(acute myelogenous Leukemia) pt presents with?

A

Adult: pancytopenia, gingival hypertrophy, 42% blasts,

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8
Q

CML 9:22 Activating ABL1 tyrosine kinase treatment

A

Imatinib: Lab: diverse cell in stages of maturity of granulocytic

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9
Q

Mature, differentiated B lymphocytes, lymphocytosis large blue nuclei, and scant cytoplasm, No blast

A

Ibrutinib CLL

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10
Q

Fe 3+ methemoglobinemia treatment?
Cause?

A

Methylene blue converts ferric to ferrous,
Exposure to nitrates… lactic acidosis and cyanosis

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11
Q

MTB infects ___________ and results in granuloma formation.
What activates the 2 primary cells in granuloma formation?

A

Macrophages

Interleukin-12 and intereron-gamma

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12
Q

M-spike, RBC- Rouleaux, Bone marrow infiltration
associated with

A

Multiple myeloma
Waldenstrom macroglobulinemia
monoclonal gammopathy

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13
Q

congenital CMV

A

intracranial calcification

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14
Q

mumps virus: orchitis

A

paramyxovirus…parotid gland swelling

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15
Q

Measles virus triad

A

cough, coryza, conjunctivitis
rash begins on face and spread cephalocaudally

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16
Q

Microangiopathic hemolysis (MAHA) seen in

A

infection/ inflammatory damage to endothelia

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17
Q

subacute sclerosing panencephalitis, servere Vit A defficient,

A

Measles: gaint Cell pneumonia, no rash, HIV

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18
Q

first-line treatment for muscle spasticity

A

Baclofen

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19
Q

Guanin and hypoxanthine

A

HGRPT: Lesh-Nyhan-X-lined

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20
Q

Menopause

A

Low estrogen, high LH,FSH,GnRH

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21
Q

RA synovial aspiration

A

lymphocyte and plasma cells
perivascular inflammation

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22
Q

alcohol withdrawal treatment

A

Benzo: Binds GABA & increase CL- channel opening Freq.

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23
Q

TPP(Thrombotic Thrombocytopenia purpura) cause

A

Dec ADMTS 13 metalloprotease

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24
Q

Dec ADMTS 13 metalloprotease signs

A

Renal dysfunction, confusion, syncope, thrombocytopenia, Anemia, fever

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25
Q

G(+), motile, facultative anaerobes, beta-hemolytic, transmission: raw milk, soil

A

Listeria monocytogenes

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26
Q

Pregnant/immunocompromised with abrupt fever: serum: Motile, facultative anaerobes B-hemolytic: treatment

A

Ampicillin(pregnant)
Gentamicin

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27
Q

ceftriaxone

A

3rd gen cephalosporin: Neisseria: g(-) bug

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28
Q

Doxycycline avoided in pregnancy

A

permanent teeth discoloration and enamel hypoplasia

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29
Q

Blurry vision and amenorrhea (prolactinoma), high calcium, bone fracture

A

AD MEN I: Pituitary adenoma, parathyroid, pancreas

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30
Q

MEN IIa seen with

A

Pheochromocytoma, parathyroid adenoma, medullary thyroid carcinoma

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31
Q

MEN IIb seen with

A

Pheo, Medullary thyroid cancer, Marfanoind and mucosal neuromas

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32
Q

what drug blocks to CCR5 in HIV pt

A

Maraviroc

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33
Q

Enfuvirtide blocks

A

HIV gp41 inhibits fusion

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34
Q

non-nucleoside reverse transcriptase target viral DNA synthesis

A

Efavirenz

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35
Q

LoSartan and ARBs

A

block angiotensin II, dec plasma aldosterone

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36
Q

3rd 4th pouches defect

A

Thymic hypoplasia, facial defects, congenital heart dz, 22q11.2 deletion, T-cell defect

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37
Q

shallow and sharply-demarcated oral mucosal ulcerations, Mono-like symptoms Neg Monospot

A

Acute HIV infection

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38
Q

sore throat lymphadenopathy and hepatosplenomegaly, mono neg

A

CMV

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39
Q

with G6PDH def which pathway is intact?
what is the Cofactor?

A

Malic –>Pyruvate
NADPH

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40
Q

M-spike in protein in MM

A

light chains from IgG

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41
Q

cause Acute promyelocytic leukemia (APL)

A

mutation in the retinoid acid receptor vein

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42
Q

Balance translocation of PML/RARA 17:15

A

functional pancytopenia

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43
Q

ALL-trans retinoid acid treats APL, increases

A

histone acetyltransferases and relax DNA

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44
Q

Immunocompromise, oral hair leukoplakia, can’t remove

A

EBV

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45
Q

Increase Absolute lymphocyte count
massive spleen and lymphadenopathy

A

CLL
Burton’s tyrosine kinase

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46
Q

Skin necrosis shortly after starting this drug

A

Inhibit epoxy reductase: warfarin

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47
Q

cause of hyperosmolar non-ketotic coma in diabetic 2

A

high serum glucose being eliminated by kidney leading to dehydration and hypotension.

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48
Q

Kids: Measles (rubeola)
Paramyxovirus

A

SS neg-sense RNA virus

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49
Q

Kids: Major cause of bronchiolitis:
SS neg-sense RNA virus

A

Resp. syncytial virus

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50
Q

Kids: SS neg-sense RNA virus: infectious croup

A

parainfluenza

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51
Q

Kids: Mumps… Orchitis, pancreatitis, Encephalitis…family?

A

SS neg-sense RNA virus

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52
Q

Kids: congenital Rubella presentation

A

teratogenic

Sensorineural deafness
Cataracts
PDA

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53
Q

Kids: Slapped Check..central clearing-laces

A

Aplastic anemia
Fifth DZ
PV B-19

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54
Q

Strawberry tongue Exudative pharynx

A

scarlet fever
Acute rheumatic fever
glomerulonephritis

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55
Q

Puritis following exposure to hot water, gouty arthritis and peptic ulcer Disease

A

Polycythemia vera

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56
Q

chronic myeloproliferative disorder with low ESR increase RBC

A

Polycythemia vera

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57
Q

Bar body formation: x-chrom inactivation occurs in

A

Normal female and Klinefelter syndrome (47 XXY)

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58
Q

Major depression refractory to antidepressant indicates

A

ECT treatment

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59
Q

ECT treatment adverse effect

A

memory loss
cardiovascular events: asystole or arrhythmia

60
Q

Erythema infectiosum (5th DZ)

A

Parvo B-19

P antigen (globoside) on erythroid progenitor cells

cessation of erythropoiesis

61
Q

Congenital TORCH infections findings

A

fetal demise
Hepatosplenomegaly
Growth retardation
Thrombocytopenia

62
Q

Congenital TORCH infections

A

Toxoplasmosis
Others (e.g., syphilis, varicella, parvovirus B19 infection, listeriosis)
Rubella
Cytomegaly (CMV)
Herpes simplex virus (HSV) infection

63
Q

TACO: transfusion-associated circulatory overload treatment

A

Stop the transfusion
Diuretics

64
Q

TRALI: Transfusion-related acute lung injury

A

Immune-mediated reaction
supportive care

65
Q

Interferon-gamma

A

activate MACROPHAGES

66
Q

cells in Granuloma

A

Th1 lymphocytes
epithelioid macrophages
multinucleate paint cells

67
Q

cytokine that activates macrophages, Incre Th1 differentiation, increases MCH expression, and isotope switching to IgG

A

IFN-Y

68
Q

Findings: abundant mononuclear cell infiltration and basophilic nuclear and cytoplasm inclusions

Treatment

A

CMV

Ganciclovir: inhibits viral polymerase

69
Q

CMV reactivation in immunocompromised

MC location
Presentation

A

GI
viral syndrome
tissue-invasion dz(CMV in organ)

70
Q

Pt presents with high T4, low TSH level, low thyroglobulin and no radioiodine uptake, no thyroid mass

A

Struma ovarii (ovarian goiter)

ovarian teratoma secreting thyroxine

71
Q

Nitrosamines, polycyclic hydrocarbon (food preservative)

A

Gastric caner in east and central Asian

72
Q

low activity of ___________ in alcoholics lead to hypoglycemia. Why

A

oxaloacetate to phosphoEnoPV

NADH/NAD+ ratio increased due to
decrease glucose

73
Q

Antidepressant (mood disorder) that causes priapism?
MOA

A

Trazodone
Inhibits serotonin reuptake and 5HT3 receptor antagonist

74
Q

Female taking OCP or Hormone replacement therapy with sudden shortness of breath, and leg swelling. Pathophysio

A

Increase in (aPC)activated protein C resistance (natural anticoagulant)
aPC resistance leads to hyper coagulable state

75
Q

clostridia perfringens infection causes?

virulence factor?

A

myonecrosis, foul-smelling dishwater-colored drainage

alpha-toxin: lecithinase-mediated destruction of host cell membranes

76
Q

TCAs:Clomipramim and amitriptyline used in obsessive-compulsive disorder:MOA

A

inhibits norepinephrine and 5HT reuptake.

Blocks muscarinic, histamine H1 and alpha-1

causing mydriasis, visual disturbance, dry mouth, and palpitations

77
Q

SD 1,2 & 3

A

68%
95.5%
99.7%

78
Q

What complication is seen with this condition?

A

disseminated intravascular coagulation: AML
Auer rods with azurophilic granules

79
Q

ALL treatment with a drug that causes peripheral neuropathy would prevent?

A

Microtubule formation

Vincristine,
prednisone
anthracycline (danunorubicin or doxorubicin)
cyclophosphamide or L-asparaginase

80
Q

prevent common complication of burn

A

Heparin for prophylaxis of thromboembolism
antibiotics

81
Q

Roth spots seen on fundoscopy: small white spots surrounded by hemorrhage.

painful nodes on fingers palm toes and feet.

A

Bacterial endocarditis causing small fibrin clots

82
Q

Repeated passive immunotherapy (snake venom) may lead to

A

immune complex deposition (in tissue eg kidney)
type III HS

83
Q

Budd-chiari syndrome
occlusion of venous outflow

A

ascites
hepatomegaly
abdominal pain

84
Q

Cause of Budd-Chiari syndrome leading to liver transplant

A

antiphospolipid syndrome

85
Q

Pt with ITP caused by

present with

treatment abciximab

A

autoantibody to GP 2b/3a

low platelets, increase bleeding time, platelet count improved with steroid

86
Q

acid-fast staining that shows large oocyst in the stool

A

cryptosporidium parvum

87
Q

Hereditary spherocytosis

A

spectrin and ankyrin defect

88
Q

trisomy13 gene mutation

polydactyly, rocker-bottom feet
cardiac also with

A

sonic hedgehog gene

cyclopia, holoprosencephaly, proboscis (extra long nose)

89
Q

determine paternity with

A

allotype

90
Q

How do unfractionated heparin work

monitor using

A

binds to antithrombin III and factor II(thrombin)

inactivate thrombin and prevent clot

activated PTT

91
Q

manifestation of B-19 in adult

A

self-limiting small joint arthritis

92
Q

intermediate-sized lymphocyte (large), interspersed with macrophages surrounded by clear spaces

A

MYC
t(8:14)

93
Q

T-cell inhibitor not associated with renal toxicity

A

Sirolimus
Inhibit mTOR serine-threonine kinase

94
Q

Rocky Mountain spotted fever is transmitted by wood tick dermacentor which also transmits

A

Francisella tularensis

95
Q

risk factors: immune compromise (medication or illness) Bat cave, pancytopenia

treatment

A

Disseminated histoplasmosis

amphotericin B or itraconazole

96
Q

Improper breakdown of vWF aggregates with platelets to form micro thrombi

A

thrombotic thrombocytopenia purpura (def ADAMTS13)

HUS

elevated: LDH, indirect bilirubin

97
Q

low extremity pain, swelling, thigh varicosities, and femoral vein thrombosis are consistent with

A

Anatomic variation (may-turner syndrome)

Compression of the left common iliac vein

98
Q

Nasopharyngeal carcinoma associated with ? pt symptom, viral infection?

A

cervical lymphadenopathy, splenomegaly, sore throat

EBV

99
Q

pos antiphospholipid antibody (high Lupus anticoagulant antibody), repeated DVT, Miscarriage
proper treatment?

A

warfarin

100
Q

the greatest risk for erythrocyte spectrin or ankyrin defect (hereditary spherocytosis

A

Gallstones due to splenic RBC destruction

101
Q

chronic myelogeneous leukemia suspect in patient with systemic complain of

translocation

A

early satiety, hepatosplenomegaly, and elevated WBC with immature neutrophil(Band cell)

9:22

102
Q

DVT, pos D-dimer, risk of venous thromboembolism in pregnant treat with

A

LMWH (Dalteparin)

103
Q

clinical pentad of TTP

A

fever,
micorangio anemia
thrombocytopenia
renal (high creatinine)
confusion (neurologic)

104
Q

Necrotic skin after starting warfarin due to rapid depletion of

A

Protein C

105
Q

Describe the organism: blue-green pigment and fruity oder in CGD pt

exotoxin A

similar pathogenesis as

A

Pseudomonas aeruginosa
ecthyma gangrenous
G(-) oxidase-pos bacillus aerobic rod

inactivates elongation factor-2 stops protein synthesis

Diphtheria toxin

106
Q

why 45,X0 has increase FSH

A

ovarian dysgenesis & low estrogen and inhibin = no anterior Pituitary feedback raising LH and FSH levels

107
Q

Retroviruses and hepadnaviruses express

A

Reverse transcriptase (RNA-dependent, DNA-polymerase)

Drugs that affect these viruses may affect Human telomerase

108
Q

autosomal codominant disease, mutation in SERPINA1 gene

seen on Liver Biopsy

A

chromosome 14 (AATD)

PSA+ globules (miss folded AAT molecules in hepatocyte

109
Q
A

Large mature B cell
Hairy cell leukemia
BRAF 15:17
TRAP pos

110
Q
A

CLL smudged cell
Mature B-Cell

111
Q
A

APL
PML-RARA gene (15:17)
DIC
All-trans retinoid acid

112
Q
A

activated neutrophils
Bacterial infection

113
Q
A

Eosinophilia
parasite
allergy

114
Q

MC trisomy seen with spontaneous abortion

A

47XY,+16

115
Q

extrapyramidal effect of antipsychotic

A

dystonia and torticollis
twisting muscle spasm

116
Q

X-Linked, MC urea cycle disorder

lab plasma high glutamine and urine orotic acid

spontaneous pneumothorax, poor/no feeding

A

OTC defect

increase ammonia

117
Q

prospective cohort study assessing risk of have a dz will report

A

relative risk

118
Q

KRAS and APC gene

A

gene deletion

119
Q

1st pregnancy, jaundice, pos Coombs several microspherocytes, high total Bilirubin and direct bilirubin

A

ABO compatibility

120
Q

The brickshaped complex virus that replicates in the cytoplasm?

A

Poxviruses
use virion-associated transcriptase

121
Q

Porphobilinogen deaminase function

A

porphobilinogen–>hydroxymethylbilane

122
Q

acute intermittent porphyria enzyme

A

porphobilinogen deaminase

123
Q

urinalysis showing aminolevulinic acid dehydrates and aminolevulinic acid (ALA)

A

porphobilinogen deaminase

124
Q

severe abdominal pain, neurologic problems and psychiatric disturbance no cutaneous rash or blistering

A

porphobilinogen deaminase

125
Q

porphyria cutanea tarda (vampire)

A

uroporphyrinogen III decarboxylase

126
Q

inhibited by lead

A

ferrochelatase
ALA dehydratase

127
Q

edema, frothy urine and proteinuria hypercoagulable state

A

Antithrombin II loss

128
Q

conditions associated with target cells

A

HALT
HbC dz
Asplenia
Liver dz
Thalassemia

129
Q

Porphobilinogen deaminase

A

sudden abdominal pain, neuronal and psychologic disturbances, ALA and porphobilinogen high

130
Q

pt with aplastic anemia with pancytopenia and bone marrow hypocellularity

A

paroxysmal noturnal hematuria clone

131
Q

sudden abdominal pain, neuronal and psychologic disturbances, ALA and porphobilinogen high

A

Porphobilinogen deaminase

132
Q

pt in coma, serum osmo 398mOs… high serum glucose 1,200 mg/dl, no ketone, low BP. cause of low BP

A

hyperosmolar nonketotic coma is a major complication of DM2

133
Q

Kawasaki disease treatment

A

IVIG

134
Q

4M+ idiopathic vasculitis of medium-sized old vessels, fever rash, high platelet, and coronary artery aneurysms

A

Kawasaki Dz

135
Q

CRASH and burn: conjunctiva injection, rash(desquamative on hands and feet), Adenopathy(neck), Strawberry tongue, Hand/foot edema, Fever(burn)

A

Kawasaki DZ

136
Q

mucocutaneous lymph node syndrome

A

kawasaki

137
Q

decreased differentiation of mature B-cell to plasma cells
delay HS response to candida antigens
recurrent bacterial infection
Low levels of all IG

A

common variable immunodeficiency

138
Q

locus heterogeneity?

A

similar phenotype from mutation of two or more diff gene or loci

139
Q

probability of affecting a child with locus heterogeneity parents

A

0%

140
Q

progressive multifocal leukoencephalopathy (PML) cause by

A

JC virus

141
Q

JC virus is

A

non-envelope, circular, ds DNA
seen with immune compromise pt (transplant, HIV/Aids)

142
Q

congenital ornithine transcarbamylse def

A

carbamoyl phosphate synthetase 2

143
Q

carbaymoyl phosphate synthetase 1 found where?
What is the substrate

A

Mitochondrial enzyme
free ammonia as substrate

144
Q

carbamoyl phosphate synthetase 2

A

cytoplasmic enzyme
uses glutamate as substrate

145
Q

OTC mutation

A

increase carbamoyl phosphate
result in orotic acuduria in babies

146
Q

impaired speech, vision, coordination, mentation: transplant Pt symptoms consistent with

A

JC virus DNA in CSF

147
Q

DAF(CD55)and CD59 absent resulting int dark morning urine

A

Paroxysmal nocturnal hemoglobinuria

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