3112818
Frage 1
Frage
All of the following are correctly matched except?
Antworten
-
APCED - AIRE gene is affected - T cells react to self antigens (Central tolarence)
-
IPEX - FOX P3 is affected - Lack of Tregs - autoimmune - Peripheral tolarence
-
Chronic granulomatous Disease - gp91 gene - NADPH oxidase deficiency
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Leucocyte adhesion deficiency - CD20 deficiency
Frage 2
Frage
All of the following lead to suspicion of an immunodeficiency in infants or children except
Antworten
-
Needs for IV antibiotics and or hospitalization
-
2 or more serious sinus infections or pneumonias within a year
-
Recurrent or resistant candidasis
-
Infection with opportunistic organism
-
Resistant to viral infections
Frage 3
Frage
All of the following are defects in phagocytic cells except?
Antworten
-
LAD (Leukocyte Adhesion Deficiency)
-
CGD (chronic granulomatous disease)
-
G6PD deficiency
-
Myeloperoxidase deficiency
-
Chediak-Higashi syndrome
-
None of the above
Frage 4
Frage
All of the following are seen in LAD except?
Antworten
-
Recurrent viral infections
-
Elevated white blood count
-
autosomal recessive
-
Rebuck Skin window test and flow cytometric analyses show absence of CD18 are diagnostic
-
Absence of pus and delayed wound healing
Frage 5
Frage
For CGD dihydrorhodamine test (DHR) is more accurate than NBT
Antworten
- True
- False
Frage 6
Frage
All of the following are correctly matched except?
Antworten
-
Chediak Higashi - no oxidative burst
-
Chronic granulomatous disease - no oxidative burst
-
Both of the above
-
None of the above
Frage 7
Frage
all of the following are correctly matched except?
Antworten
-
Paroxysmal Nocturnal Hemoglobinuria - DAF deficiency (CD55/CD59)
-
Hereditary Angioedema - C1 esterase deficiency
-
CH50 tests - alternate pathway and A50 tests - classic pathway
Frage 8
Frage
All of the following about Bruton's agammaglobulinemia are correct except?
Antworten
-
X linked recessive transmission
-
Serum titers of IgM is not detectable, Iga and IgG are low
-
cannot handle measles infection
-
none of the above
-
Caused by mutation in btk gene (Bruton's tyrosine kinase)
Frage 9
Frage
X linked hyper IgM (XHIGM) - all of the following are true except?
Antworten
-
a B cell defect
-
CD 40 L is deficient
-
None of the above
Frage 10
Frage
All of the following for selective IgA deficiency are true except?
Antworten
-
Most common immunodeficiency in European descent
-
Only mucosal IgA is absent and serum is normal
-
none of the above
-
Some patients when transfused with blood containing normal IgA develop severe even fatal anaphylactic reaction because they have made IgE anti-IgA antibodies
Frage 11
Frage
IN CVID the roles in B and plasma cell survival and isotype switch is common due to a defect in TACI
Antworten
- True
- False
Frage 12
Frage
all of the following are true about Di George syndrome except
Antworten
-
Autosomal dominant inheritance
-
Deletion of 22q11
-
Hypercalcemia
-
Low set ears, underdeveloped jaw, small mouth, abnormally increased distance between the eyes
Frage 13
Frage
All of the following are true about Bare Lymphocytes syndrome (BLS) except?
Antworten
-
BLS type I - MHCII deficiency, BLS type II - MHC I deficiency
-
Mutation in TAP 1 or TAP 2 genes
-
All of the above
-
None of the above
Frage 14
Frage
All the following statements are true except?
Antworten
-
ADA deficiency - has NO T,B, NK cell
-
PNP deficiency - No T cells
-
X linked SCID - No T cells
-
Autosomal SCID - NO T or B cells
Frage 15
Frage
All of the following about X- linked SCID are true except?
Antworten
-
LEAST common form of SCID
-
Mutation of the gamma-c chain of IL-2 receptor (CD132)
-
Normal B cell levels
-
lack of delayed hypersensitivity, atrophy of thymus, susceptible to viral fungal and bacterial infections, lymphocytopenia
Frage 16
Frage
All of the following are true except?
Antworten
-
JAK3 deficiency - Autosomal Dominant SCID
-
Omenn Syndrome - Cannot undergo somatic recombination
Frage 17
Frage
The following are correctly matched except?
Antworten
-
X linked SCID - absent T cells, B cells present - Jak 3 deficiency and CD45 deficiency
-
IL-7R alpha chain deficiency - CD3 delta chain deficiency - absent T cell, B cell absent
-
ADA deficiency - absent T and B cells
-
RAG1 or RAG2 deficiency - absent T and B cells
Frage 18
Frage
All of the following are true about Wiskott-ALdrich Syndrome except ?
Antworten
-
X-linked recessive
-
Thrombocytosis (elevated platelet levels)
-
Severe eczema by one year of age - elevated IgE
-
recurrent pyogenic infections with encapsulated bacteria, bloody diarrhea
Frage 19
Frage
All of the following about Ataxia-Telangiectasia are true except?
Antworten
-
Autosomal recessive
-
Selective IgM deficiency
Frage 20
Frage
X-linked lymphoproliferative syndrome - highly fatal infectious mononucleosis
Antworten
- True
- False
Frage 21
Frage
In both multiple mylemoa and Waldenstrom's Macrogobulinemia have punched out lesions in the skull
Antworten
- True
- False
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