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44 Cards in this Set
- Front
- Back
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present at birth
often x-linked in males |
congenital
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Primary Humoral (B cell) Immunodeficiencies (5)
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transient hypogammaglobulinemia
x-linked hypogammaglobulinemia common variable hypogammaglobulinemia selective IgA deficiency selective IgG deficiency |
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Inadequate production of IgG after 6 months of life
B cells have delayed plasma cell maturation normal in number in function fail to communicate with T cells causes lots of URI and ear infections usually resolves by toddlerhood "B cells and T cells don't talk" |
transient hypogammaglobulinemia
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Bruton's Disease
(X-linked hypogammaglobulinemia) |
males only
low or no IgA, IgG, or IgM Normal T levels Symptoms appear after 6 months of age Leaves vulnerable to many diseases/ infections |
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similar to Bruton's disease
B cells don't mature into plasma cells B lymphocytes are normal Absolutely no plasma cells Begins later in life around 15-35 both genders equally |
common variable hypogammaglobulinemia
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Most common immunodeficiency
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selective IgA deficiency
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B cells fail to convert to IgA
therefore reduced secretory and serum IgA unaware of it except lots of allergies Must "wash" blood of IgA before transfusing IgA has a short lifespan |
selective IgA deficiency
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Primary Combined Immunodeficiency Disorders (3)
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Wiskott- Aldrich Syndrome (WAS)
Severe Combined Immunodeficiency Disorder (SCID) Reticular Dysgenesis (RD) |
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x linked hereditary syndrome
altered movement IgM antibody production defective platelet production defective susceptible to polysaccharide antigens |
Wiscott Aldrich Syndrome
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kids dont live long with this
signs at birth... |
bleeding
bloody diarrhea petichiae purpura thrombocytopenia |
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stem cell deficiency
small thymus deficient in NK, B and T cells and all immunoglobulins rib cage and skeletal deformities |
SCID
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similar S and S to HIV
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SCID
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Must have a "germ free" enviornment
need stem cells transplant or gene transfer therapy |
SCID
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rarest most severe form of combined immunodeficiencies
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Reticular Dysgenesis
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Thymus, tonsils, Peyers patches, and adenoids all absent
bilateral deafness ALL WBC's fail to develop |
Reticular Dysgenesis
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DiGeorge's Syndrome S and S
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stiffness d/t no parathyroid therefore no calcium production
decreases T cells, Helper Ts and Lymphocytes low ears wide set eyes small jaw split uvula |
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Absent Thyms and or parathyroid gladn
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DiGeorge's Syndrome
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occurs in utero before 12th week due to chromosomal abnormalities
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DiGeorge's Syndrome
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what is secondary humoral deficiency?
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aquired later in life due to loss of immunoglobulins through GI or GU tract
malnutrition cancer chemotherapy immunosuppressives corticosteroids common drugs ionizing radiation surgery anesthesia lose IgG and IgA but not IgM b/c it is so big |
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Protein energy malnutrition/ Marasmus/ Kwashiorkor
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decreases:
T cell production T cell function complement activity chemotaxis bactericide |
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which HIV is more slow progressing, usually in western africa?
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HIV 2
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8 steps of HIV infection
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Binding
Envelopes Release of reverese transcriptase Integration of DNA into nucleus of T cell Transcription (mRNA) Translation (tRNA) Cleavage (cut-more HIV produced) Assembly package, coat, etc. and release (T cell dies) |
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block reverse transcriptase of HIV
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Nucleoside Reverse transcriptase inhibitors "nukes"
non nucleoside reverse transcriptase inhibitors nucleoside analog reverse transcriptase inhibitors |
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prevents cleavagge of HIV
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protease inhibitors
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prevents HIV from attaching to CD4+
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fusion inhibitors
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Diagnostic tests of HIV
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OraQuick test- needs confirmatory test
Polymerase Chain reaction- Detects HIV virus but must be careful b/c newborns would have their mom's antibodies anyways OraSure- saliva, send it in and results are sent to you ELISA- enzyme linked immunosorbent assay- has rare false negatives but has large false positives Western Blot- confirmation test used with ELISA |
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How to monitor HIV?
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CD4+ levels
CD8+ levels |
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Stage 1 of HIV
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Acute infection
virus replicates early-no detectable antibodies towards end you can detect Ab may last up to 6 months |
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Stage 2 of HIV
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flu like symptoms
mono like symptoms fever night sweats may last 1- 2 weeks normal CD4+ count |
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Stage 3 of HIV
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Asymptomatic
CD4+ strats to declien antibodies present may last up to 20 years |
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Stage 4 of HIV
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Symptomatic
persistent fever night sweats profuse diarrhea persistant lymphadenopathy rapid weight loss oral lesions cognitive dysfunction neuro changes |
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wasting syndrome
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10 % weight loss, diarrhea, fever, glucose intolerance
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Stage 5 of HIV
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AIDS
CD4+ count < 200 opportunistic infections last 1-3 years |
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Mycobacterium Avium Intracellular Complex (MAC)
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opportunistic infection
acquired orally or by inhalation tissues affected fever shakes diarrhea abd. cramping severe wt. loss treat with antibiotics |
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Mycobacterium tuberculare (TB)
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communicable opportunistic disease
fever wt. loss night sweats dyspnea fatigeu cough hemoptysis (coughing up blood) Multiple drug regimen |
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number one killer of HIV patients
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TB
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Pneumocystis carinii Pneumonia (PCP)
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ok in healthy people
fever dyspnea cough (productive or non productive) + lung biopsy/ culture |
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Candida albicans
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lives in healthy people
pain diff. swallowing mucosa white patches itching thrush esophagitis vaginitis |
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cryptosporidium parvae
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parasite in farm animals, undercooked foods, contaminated water, pets
profuse diarrhea exp. cow bovine treatment (like anti venom) |
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very painful opportunistic infection that follows pathways, lies in ganglian root
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shingles or herpes zoster
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cytomegalovirus
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transmitted like an STD
direct tissue destruction and tumor formation hemolytic anemia thrombocytopenia |
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opportunistic malignancies (3)
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kaposi's sarcoma (KS)
non hodgkins lymphoma cervical cancer |
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purple or brown nodules that dont blanche to the touch, move to lymph nodes and organs
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kaposi's sarcoma
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how often to HIV women need a pap smear?
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every 6 months
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