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Immunodeficiency
Patho
| Question | Answer |
|---|---|
| abnormality in one or more branches of the immune system and renders the person susceptible to disease normally prevented | immunodeficiency disorders |
| what are the 2 classifications of immunodeficiency disorders? | primary: congenital or inherited (most are x-linked) and secondary: more common |
| what are the 4 immunodef disorders that are a result of a deficiency in humoral immunity as a result of selective loss of immunoglobulin thru the GI or GU | x-linked agammaglobulinemia, common variable immunodef, selective immunoglobulin A def, immunoglobulin G subclass def |
| any disease that prevents the B cell maturation of stem cells in infants. this leads to recurrent respiratory and ear infections, resolves by 2-4 years of age, > r/f strep, haemophilus influenzea, staph, and pseudomonas | immunodef disorders that are a result of a deficiency in humoral immunity |
| recessive trait affecting only males and they essentially no immunogloublins...they are susceptible to everything from staph to meningitis, but T cells are normal | x-linked agammaglobulinemia |
| s/s of x-linked agammaglobulinemia | being around months when the mother's immunity has run out |
| a clue of ______ is that infections do not respond well to ABT | x-linked agammaglobulinemia |
| what is god to look at to test for x-linked agammaglobulinemia? | look at immunoglobulin levels |
| what is the TX for x-linked agammaglobulinemia? | immunoglobulin infusions and early ABT |
| what are some complications ofx-linked agammaglobulinemia? | early pulmonary disease |
| differentiation of B cells in to plasma is blocked-probably in utero and usually also have problems with t cells | common variable immunodef |
| complete absence of IgG and IgA production, an > rate of spontaneous apoptosis, impaired DNA repair, and has the presence somatic mutations affecting B cells regulation | common varibale immunodef |
| what are the s/s of common variable immunodef disorder? | similar to x-linked agamma. s/s, but appears later like 15-35 years of age....equal with genders...>r/f chronic lung dx, hepatitis, gastric carcinoma, & chronic diarrhea w/ malabsorption |
| what is the treatment for common variable immunodef disorder? | similar to x-linked agamma (immunogloblin infusions and early ABT |
| most common immunoglobulin definiceny | selective immunoglobulin A deficiency |
| reduction in levels of serum and secretory IgA r/t a block in the pathwy of B cells differenetation into IgA | selective immunoglobulin A deficiency |
| most are usually asymptomatic r/t IgM and IgG compensate and half of the cases reslove before age 14 | selective immunoglobulin A deficiency |
| what are the s/s for selective immunoglobulin A deficiency? | repiratory and GI infections, allergies, asthma |
| there is no tx for ______ | selective immunoglobulin A deficiency |
| can develop antibodies against IgA that can lead to anaphylaxis w/ the adm of blood componets that contain IgA | selective immunoglobulin A deficiency |
| is a deficiency in any of the 4 subclasses of IgG | Immunoglobulin G Subclass deficiency |
| B cells _____ immunity | humoral |
| T cells ______immunity | cell-mediated |
| what are the 4 subclasses of the immunoglobulin G deficiency? | B cells, T cells, phagocytes, and complement |
| what is the treatment for Immunoglobulin G Subclass deficiency? | ABT may be used to prevent infection, IV immunoglobulins and polysaccharide vaccines conjugated to protein carriers |
| rare immunodef. disorders that have a low survival rate | T cell deficiency |
| what is the cause of the T cell def? (3) | defect in the expression of t cells, cytokinin production, and t cell activation |
| What are 2 of the T cell def immunodef disorders? | DiGeorge Syndrome and -linked immunidef with hyper IgM |
| Caused by a large deletion from chromosome 22, produced by an error in recombination at meiosis | DiGeorge Syndrome |
| Defect occurring before the 12th week gestation when the thymus, parathyroid, and parts of the head, neck and heart are developing | DiGeorge Syndrome |
| Caused by teratogens and affects both genders | DiGeorge Syndrome |
| What are the S/S of the DiGeorge Syndrome? | cleft palate, autism, learning disabilities, heart defects, recurrent infections |
| Commonly see hypercalcemia and tetany within 24 hours r/t an absent parathyroid and will have recurrent infections | DiGeroge Syndrome |
| what is the TX for DiGeroge Syndrome? | Tx-thymus and bone marrow transplantation |
| Low IgG and IgA with normal or high levels of IgM | X-linked immunodef with hyper Igm |
| Inability of the T cells to signal B cells to switch to IgG and IgA, leaving IgM and is in MALES ONLY | X-linked immunodef. with Hyper IgM |
| What are the S/S of X-linked immunodef. with Hyper IgM? | appear 1-2 years of age including pyrogenic infections, increased r/f Pneumocystis carinii infections, hemolytic anemia, thrombocytopenia, neutropenia, and increased occurrence of other autoimmune disorders |
| The Combined Immunodef disorder is _______ and affects all aspects of the ______ function. | life-threatening and immune |
| requires bone transplants and may be mild to fatal | combined |
| Combined immunodeficiency syndromes and a variety of inherited conditions and Disruption of the T and B cell system and deregulation of the immune response | combined |
| In a severe combined immunodef there is an absence of ALL ______ finctions and 50% of cases have _____ | immune...fam history |
| Both autosomal and X linked andAppears like AIDS in infants w/ chronic diarrhea and opportunistic infections usually leading to death by 2 | severe combined immunodef |
| If caught before 3 months of age, can be treated with human leukocyte antigens or T cell depleted bone marrow stem cell transplants | severe combined immunodef |
| 50% of cases have a decrease in adenosine deaminase leading to toxic metabolite accumulation | severe combined immunodef |
| Enzyme replacement in conjunction with usually tx may help | severe combined immunodef |
Created by:
TayBay15
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