Usmle 1 Immuno & Ct Diseases

Front 1

Dx:

boys afound 6 mo w/ absence of plasma cells in tissue, recurrent bacterial infections w/ strep, staph, h. influenzae and pneumococci, absent or poorly defined germinal centers in LN, no immunoglobulins

genetic problem?

Back 1

Bruton's Agammaglobinemia

(Brutons = Boys = Bacteria infect)

(X-recessive)

Front 2

MCC of B-cell defect

Back 2

Isolated IgA deficiency

Front 3

Dx:

sinus and lung infections, milk allergies, diarrhea

Back 3

Isolated IgA deficiency

(infections of mucosal surfaces)

Front 4

Dx:

recurrent viral and fungal infections, tetany, lymphopenia, cardiac defects, cleft palate

Back 4

Di George syndrome


(CATCH-22; 22q11 deletion)

Front 5

Dx:

severe bacterial, viral, fungal and protozoal infections, failure-to-thrive, high incidence of malignancy, thymic and LN hypoplasia

Back 5

Severe Combined Immunodeficiency Dz
(SCID)

(no/low B or T cells)

Front 6

(3) possible causes of SCIDs

Back 6

Failure to make MHC-2;

defective IL-2 receptors

Adenosine Deaminase Deficiency (ADA)

Front 7

Dx:

Eczema, thrombocytopenic Purpura, recurrent Infections, elevated IgA, low IgM

genetic problem?

Back 7

Wiscott-Aldrich syndrome

(WIPE = Wiscott, Infect, Purpura, Eczema)

(X-linked disorder)

Front 8

what is the dysfunction if patient gets chronic Candida albicans infections?

Back 8

T-cell dysfunction

Front 9

Dx:

cerebellar problems, spider angiomas, IgA deficiency

where is the defect?

Back 9

Ataxia-Telangiectasia

defect:
DNA reapir enzyme

Front 10

what lymphocyte surface protein deficiency would cause an increase in mycobacterial infections?

Back 10

IL-12 receptor deficiency

Front 11

Dx:

baby presents w/ severe pyogenic infections, high IgM, very low IgG, IgA and IgE

where is the defect?

Back 11

Hyper-IgM syndrome

defect:
CD40L on Th1 helper cells
(leads to inability to class-switch)

Front 12

Dx:

recurrent "cold" staph abscesses, eczema, coarse facies, Retained Primary Teeth, increased IgE

where is the defect?

Back 12

Job's syndrome


defect:
Failure of INF-gamma by T-cells (low Macros)

Front 13

What cell surface protein on HIV binds to CD4 T-cells?

what does it inser into host genome?

Back 13

gp120


Proviral DNA
(via reverse transcriptase)

Front 14

what is the test for HIV?

follow-up test?

Back 14

ELISA


follow: Western Blot

Front 15

what type of genome does HIV have?

Back 15

Diploid
(2 molecules of RNA)

Front 16

what is the rectangular nucleocapsid protein (holds the 2 molecules of RNA) in HIV?

Back 16

p24 capsid

Front 17

what is the product of HIV reverse transcriptase?

Back 17

dsDNA

Front 18

(2) AIDS criteria

Back 18

CD4 < 200

or

CD4/CD8 ratio < 1.5

Front 19

what homozygous mutation gives immunity to HIV?

Back 19

CCR5 mutation

Front 20

Dx:

lymphadenopathy, weight loss, arthritis, photosensitivity, raynauds, pulmonary fibrosis, endocarditis, immune-complex vasculitis, glomerular changes

COD?

Back 20

SLE


COD:
Renal failure (and infections)

Front 21

what may be the earliest lab abnormality in some SLE patients?

Back 21

False Positive test for Syphilis

Front 22

Dx:

tightening of facial skin, sclerodactyly (claw-hand), raynauds, dysphagia, pulmonary fibrosis, HTN

Back 22

Scleroderma

(CREST-syndrome also has calcinosis and telangiectasia)

Front 23

Dx:

xerostomia (dry mouth), keratoconjunctivitis (dry eyes), RA or other CT problem, hypergammaglobinemia, parotid enlargement

Back 23

Sjogren syndrome

[when you're JOGin' you sweat and get dry]

Front 24

Dx:

chronic inflammation of proximal extremity muscles due to CD8 injury, possible reddish-purple rash on face and neck, increased CK and Adolase

Back 24

Polymyositis

(w/ skin rash: Dermatomyositis)

Front 25

Dx:

arthralgias, raynauds, myositis, esophageal hypomotility, no renal involvement

antibody?

Back 25

Mixed Connective Tissue Dz
(MCTD)

Ab: anti-nRNP

Front 26

Amyloid associated with:

heart, muscle and tongue; plasma cell disorders

Back 26

AL (Amyloid Light chain)

Front 27

Amyloid associated with:

kidney, liver, adrenals, pancreas, LN and spleen

Back 27

AA protein

Front 28

Amyloid associated with:

chronic inflammatory diseases:
RA, TB, osteomyelitis, syphilis

Back 28

AA protein

Front 29

Amyloid associated with:

Alzheimers
(2)

Back 29

amyloid Beta-protein;

A4 amyloid

Front 30

Amyloid associated with:

Insulin or glucagon (in DM-2)
(2 names)

Back 30

Amylin

Islet Amyloid Polypeptide
(IAPP)

Front 31

Amyloid associated with:

Elderly (called senile Amyloidosis)

Back 31

Transthyretin