Term
| In B cell deficiency, the infections are caused by which bacteria? |
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Definition
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|
Term
| what are the types of infections in B cell deficiency? |
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Definition
otitis media
sinusitis
pneumonia
meningitis
osteomyelitis
septic arthriti |
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Term
| what lab study is for B cell immunity? |
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Definition
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Term
| what lab study is for T cell immunity? |
|
Definition
| ALC, HIV and thymic shadow on chest x ray |
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Term
| what laboratory study is for phagocyte immunity |
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Definition
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Term
| What laboratory study is for the complement system? |
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Definition
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Term
| Laboratory study in Wiskott-Aldrich syndrome |
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Definition
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Term
| This test measures T cell, B cell and NK cell numbers and rhodamine |
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Definition
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|
Term
| What tests T lymphocyte function |
|
Definition
| mitogen and antigen assay |
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Term
| What is neutrophil oxidative burst measured by? |
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Definition
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Term
| How do you calculate the ALC? |
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Definition
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Term
| What are the treatment options for B cell disorders? |
|
Definition
Ab's
Immunoglobulin replacement |
|
|
Term
| what is the treatment for T cell disorders |
|
Definition
| transplantation and gene therapy |
|
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Term
| what is the treatment for phagocytic disorders |
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Definition
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Term
| What is the treatment option for complement? |
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Definition
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Term
| Effect of XLA on lymph development |
|
Definition
failure of immature B cells to mature
NO B cells absent = No IgG, M or A |
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|
Term
| Clinical presentation of XLA |
|
Definition
sinusitis, otitis media, pneumonia, enteroviral meningitis.
NO tonsils or lymph nodes |
|
|
Term
| Hyper IgM syndrome Effect |
|
Definition
X linked
defect in isotype switching |
|
|
Term
| clinical presentation of Hyper IgM syndrome |
|
Definition
neutropenia
autoimmunity
recurrent infections |
|
|
Term
|
Definition
low B cells, normal T cells function
CANNOT mount specific Ab responses to immunization |
|
|
Term
| What is the genetic mutation in XLA |
|
Definition
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Term
|
Definition
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Term
| Clinical presentation of CVID |
|
Definition
same as XLA or Hyper IgM
sinopulmonary bacterial infections
COPD
gastric carcinoma
splenomegaly
follicular hyperplasia |
|
|
Term
| This disease is co expressed in families w/CVID |
|
Definition
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Term
|
Definition
| defective migration of 3rd and 4th branchial pouches during development |
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|
Term
| How does classical DGS present? |
|
Definition
| lymphopenia, thymic aplasia, T cell deficiency, and Hypothyroidism and abnormal facial features |
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Term
|
Definition
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|
Term
| clinical presentation of DGS |
|
Definition
hearing loss
cranial nerve palsies
cleft lip and palate
choanal atresia |
|
|
Term
| These two diseases involve a deficient T cell response to Candida albicans |
|
Definition
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|
Term
| this disease results from mutations in the transcription factor Foxp3 |
|
Definition
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Term
|
Definition
| profound lymphopenia (LOW ALC) |
|
|
Term
| genetic cause of X linked SCID |
|
Definition
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|
Term
| This causes autosomal recessive SCID |
|
Definition
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|
Term
| What is still present in RAG deficient SCID |
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Definition
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|
Term
| ANP and PNP enzyme defects result in |
|
Definition
elevated levels of dATP and dGTP
PNP only affects T cells |
|
|
Term
| Clinical presentation of WAS |
|
Definition
| eczema, thrombocytopenia, and immune deficiency |
|
|
Term
|
Definition
T cell decrease
B cell expansion
elevated IgA and E |
|
|
Term
|
Definition
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|
Term
|
Definition
Normal B cells
hypogammaglobulinemia due to lack of T cells |
|
|
Term
|
Definition
dilated eye capillaries
abnormal gait
neurologic problems
IgA deficiency |
|
|
Term
|
Definition
|
|
Term
|
Definition
sensitive to radiation.
IgA deficient |
|
|
Term
|
Definition
neutrophil disorder
defective oxidative burst killing |
|
|
Term
| CGD are susceptible to which infections? |
|
Definition
| Staph aureus and Gram - organisms. |
|
|
Term
| CGD clinical presentation |
|
Definition
vomiting
urinary retention |
|
|
Term
|
Definition
| defective migration of neutrophils |
|
|
Term
|
Definition
elevated neutrophils
absent pus and neutrophils in tissues |
|
|
Term
| Clinical presentation of LAD |
|
Definition
poor wound healing
gingivitis
recurrent fungal infections |
|
|
Term
|
Definition
NK impaired
normal cytotoxic T cell killing |
|
|
Term
| clinical presentation of Chediak |
|
Definition
partial albinism
bleeding disorders |
|
|
Term
| IFN gamma and IL-12 deficiency result in susceptibility to |
|
Definition
|
|
Term
| Hereditary Angioedema etiology |
|
Definition
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