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44 Cards in this Set

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present at birth

often x-linked in males
congenital
Primary Humoral (B cell) Immunodeficiencies (5)
transient hypogammaglobulinemia
x-linked hypogammaglobulinemia
common variable hypogammaglobulinemia
selective IgA deficiency
selective IgG deficiency
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
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
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
Most common immunodeficiency
selective IgA deficiency
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
Primary Combined Immunodeficiency Disorders (3)
Wiskott- Aldrich Syndrome (WAS)
Severe Combined Immunodeficiency Disorder (SCID)
Reticular Dysgenesis (RD)
x linked hereditary syndrome
altered movement
IgM antibody production defective
platelet production defective
susceptible to polysaccharide antigens
Wiscott Aldrich Syndrome
kids dont live long with this

signs at birth...
bleeding
bloody diarrhea
petichiae
purpura
thrombocytopenia
stem cell deficiency
small thymus
deficient in NK, B and T cells and all immunoglobulins
rib cage and skeletal deformities
SCID
similar S and S to HIV
SCID
Must have a "germ free" enviornment
need stem cells transplant
or gene transfer therapy
SCID
rarest most severe form of combined immunodeficiencies
Reticular Dysgenesis
Thymus, tonsils, Peyers patches, and adenoids all absent

bilateral deafness

ALL WBC's fail to develop
Reticular Dysgenesis
DiGeorge's Syndrome S and S
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
Absent Thyms and or parathyroid gladn
DiGeorge's Syndrome
occurs in utero before 12th week due to chromosomal abnormalities
DiGeorge's Syndrome
what is secondary humoral deficiency?
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
Protein energy malnutrition/ Marasmus/ Kwashiorkor
decreases:
T cell production
T cell function
complement activity
chemotaxis
bactericide
which HIV is more slow progressing, usually in western africa?
HIV 2
8 steps of HIV infection
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)
block reverse transcriptase of HIV
Nucleoside Reverse transcriptase inhibitors "nukes"
non nucleoside reverse transcriptase inhibitors
nucleoside analog reverse transcriptase inhibitors
prevents cleavagge of HIV
protease inhibitors
prevents HIV from attaching to CD4+
fusion inhibitors
Diagnostic tests of HIV
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
How to monitor HIV?
CD4+ levels
CD8+ levels
Stage 1 of HIV
Acute infection
virus replicates
early-no detectable antibodies
towards end you can detect Ab
may last up to 6 months
Stage 2 of HIV
flu like symptoms
mono like symptoms
fever
night sweats
may last 1- 2 weeks
normal CD4+ count
Stage 3 of HIV
Asymptomatic
CD4+ strats to declien
antibodies present
may last up to 20 years
Stage 4 of HIV
Symptomatic
persistent fever
night sweats
profuse diarrhea
persistant lymphadenopathy
rapid weight loss
oral lesions
cognitive dysfunction
neuro changes
wasting syndrome
10 % weight loss, diarrhea, fever, glucose intolerance
Stage 5 of HIV
AIDS
CD4+ count < 200
opportunistic infections
last 1-3 years
Mycobacterium Avium Intracellular Complex (MAC)
opportunistic infection
acquired orally or by inhalation
tissues affected

fever
shakes
diarrhea
abd. cramping
severe wt. loss

treat with antibiotics
Mycobacterium tuberculare (TB)
communicable opportunistic disease

fever
wt. loss
night sweats
dyspnea
fatigeu
cough
hemoptysis (coughing up blood)

Multiple drug regimen
number one killer of HIV patients
TB
Pneumocystis carinii Pneumonia (PCP)
ok in healthy people
fever
dyspnea
cough (productive or non productive)
+ lung biopsy/ culture
Candida albicans
lives in healthy people

pain
diff. swallowing
mucosa white patches
itching

thrush
esophagitis
vaginitis
cryptosporidium parvae
parasite in farm animals, undercooked foods, contaminated water, pets

profuse diarrhea

exp. cow bovine treatment (like anti venom)
very painful opportunistic infection that follows pathways, lies in ganglian root
shingles or herpes zoster
cytomegalovirus
transmitted like an STD

direct tissue destruction and tumor formation
hemolytic anemia
thrombocytopenia
opportunistic malignancies (3)
kaposi's sarcoma (KS)
non hodgkins lymphoma
cervical cancer
purple or brown nodules that dont blanche to the touch, move to lymph nodes and organs
kaposi's sarcoma
how often to HIV women need a pap smear?
every 6 months