Show:
Pathophyzish
Choices
Fix Me?
families are defective
ewww! bugs!
100
What causes hereditary Angioedema
Lack of C1 inhibitor
100
A 2-year old boy has eczema and thrombocytopenia. There is also a history of recurrent infection. HIs  brother has similar abnormalities, but none of his three sisters are affected. Patients with this disorder are known to have impaired antibody response to which of the following types of antigen? a. Deoxyribonucleic acid b. phospholipid c. polysaccharide d. ribonucleic acid e. steroid
c. - wiskott-Aldrich
100
Should children with SCID get live attenuated virus vaccines?
no
100
Which inheritance pattern occurs most commonly in SCID?
x-linked
100
Which unusual organism is the most common cause of pneumonia in kids with SCID
PCP
200
What is CVID
Deficiency of all immunoglobulin subclasses with apparently normal B-cell development
200
A 7 month old boy has had multiple bouts of OM, sinusitis, bronchitis, oral candidiasis, and multiple viral infections. Cessation of the recurrent infections follows successful engraftment of bone marrow transplant. The  basis of the clinical improvement is a. direct transfusion of antibody-producing B cells b. direct transfusion of donor CD4+ and CD8+ lymphocytes c. donor supprssion of recipient cytotoxic T cells d. infusion o fdonor-derived cytokines e. maturation of donor lymphoid proginator cells
What is E - severe combined immunodeficiency
200
What lymphocyte count finding is nearly universal in infants with SCID?
lymphopenia
200
Severe combined immunodeficiencies tend to manifest first as _________
T-cell defects
200
Deficiencies in what complement components result in recurrent meningococcal and gonococcal infections?
C5, c6, c7, or c8
300
What is characteristic about the lymph nodes and tonsils in X-linked aggamaglobulinemia?
They are small
300
Leukocyte adhesion deficiency has an absence of: a. CD7 b. CD27 c. CD18 d. CD56 e. CD19
c
300
What are the immunoglobulin findings in Hyper-IgM syndrome?
Low IgA and IgG, normal to hig IgM
300
A child is severely immunosuppressed, can his sister receive OPV or MMR?
OPV: No MMR: yes
300
Deficiencies in what result in increased Neisseria infections
Factor B, properdin
400
What is the classic triad of Wiskott-Aldrich Syndrome?
Thrombocytopenia, eczema, immunodeficiency
400
A deficiency of tyrosine kinase that stops B-cell maturation in the bone marrow at the pre-B stage is known as a. Transient hypogammaglobunlinemia of infancy b. Bruton X-linked hypogammaglobulinemia c. Severe combined immunodeficiency d. Common variable hypogammaglobulinemia e. Chediak-Higashi syndrome
b
400
What is distinctive about platelets in WAS?
small platelets
400
What is the mutation responsible for X-linked agammaglobulinemia?
Btk (at Xq22)
400
For what type of infections is a child with X-linked agammaglobulinemia at risk?
Encapsulated bacterial infections of the respiratory tract (pneumonia, otitis, sinusitis)
500
On which cells would you find the Class II HLA antigens
Monocytes/macrophages, Langerhan's cells, dendritic cells, B cells
500
____ defects predispose to infections with intracellular pathogens and ____ predispose to infections with extracellular pathogens? a) B-cell, T-cell, phagocyte (intra) and Complement (extra) b)Complement (intra) and B-cell, T-cell, phagocyte (extra) c) B-cell, phagocyte, complement (intra) and T-cell (extra) d) T-cell (intra) and B-cell, T-cell, phagocyte (extra)
d
500
What type of blood transfusions should SCID kids get?
Irradiated (non-irradiated contains T-cells leading to GVHD)
500
What is the inheritance pattern for Wiskott-Aldrich syndrome?
X-linked recessive
500
This defect results in increased infections with pyogenic bacteria
Deficiencies in classical complement pathway (c1q, c1r, c1s, c4, c2)