.2 Flashcards
digeorge syndrome
will not have items from 3rd and 4th pouch
What deficiency’s would be found in digeorge syndrome
T cell deficiency (thyroid)
hypocalcemia (parathyroid)
abnormalities of heart, great vessels and face
SCID are effected by
defected humoral and cell mediated immunology
Etologies of SCID
defects cytokine receptor adenosine deaminase deficenty MHC class 2 deficency
Adenosine deaminase deficiency
build up of adenosine and oxydenosine can cause a build up in neutrophils causing immunodeficiency
CD4+ Tcells
help b cells mature and help CD8 fight infections
Cytokine receptors deficiency can cause
immunodeficeny
MHC class 2 are able to activate
CD+4 causeing a deficency in t and b cells
Patients with SCID
don’t have t cells and will get fungal and viral infections
They also don’t have b cells and will get protezoal and bacterial infection
also have problems with live vaccines and are worse than aids patients
SCID treatment include
sterile isolation and stem cell transplant
Why are they given stem cell transplants
the stem cell transplant can differentiate into b and t cells.
X linked agammaglobulinemia
Complete lack of immunoglobulin
disordered b cell mutation problem hence not allowing them to mature into plasma cells
Tyrosine kinase
To help b cells mature into plasma cells
the lack of tyrosine kinase will cause
bacterial, enterovirus and giardia infections.
after 6 months of life. because of the lack of IgG IgA, IgA in that order. No live vaccines given
the x linked aggamagobulinemeia is due to a mutation in what
Bruton tyrosine kinase
Common variable Immune disease
Low immunoglobulin due to B-cell or helper T-cell defects
What are the common infections with CVID
autoimmune disease and lymphoma in late adulthood.
when younger there will be bacterial infections, enterovirus, and giardia.
IgA deficiency
Increase mucosal viral infections
celiac disease
cause by IgA deficeincy
Hyper IgM syndrome is cause by a mutation in the
CD40 2nd way of being activated not working so 2nd pathway for IGA and IgG and IgE, prevents class switching.
2 ways b cells are activated
- antigen binds IgM making a IgM secreting plasma cell
- B cell can internalize the antigen and present MAclass 2 and a CD 40 stimulus is expressed on the b cell for T cell and will activate CD4 and produce IL4 and IL 5 and will go back to the b cell and allow it to mature and class switch to produce IGA IgG IgE
IgA and IgG and IgE are created from the b cell class switching cause by IL4 and IL5 what will not be activated with someone with Hyper IgM syndrome
The CD40 will not be expressed for the t cell so IL4 and IL 5 will not be produced preventing the b cell from class switching. meaning there will be high IgM but low IgA IgG IgE
what are common problems with Hyper IgM syndrome
mucosal infections
puss forming recurrent infection
Wiskott Aldrich Syndrome
a triad of
Thrombocytopenia
Excema
recurrent infection
wiskott aldrich syndrom is caused by a mutation in the
wiskot aldrich syndrome X LINKED!!!
compliment deficiency
C5-‘C9 deficiency can cause a risk for Neisseria infection
if there is a deficiency of C1 inhibitor can cause
over activation of c1 causing over activation of complement
causes include vasodilation increased vascular permeability. Periorbital edema
Hereditary angio edema is and example of
compliment deficiency with a periorbital edema caused by over activation of complement because of a c1 inhibitor deficiency
autoimmune desease, we constantly creating lymphocytes buy they are
usually apoptotic, or become anergic (go to sleep) generally effect woman more than men at child bearing age.
Etiology of autoimmune disorders
Environmental trigger in a genetically susceptible individuals, increased incidence in twins and associated with certain HLA subtypes
first autoimmune disease
SLE
SLE Systeic autoimmune disease involves
multiple organs have antibodies by binding and causing damage type 2 cytotoxic
or Type 3 antigen antibody complex Hyper sensitivity reaction deposited in the tissue.
Long list of lupus
fever wight loss, Mallar butterfly rash Arthritis Pleuritis pericarditis CNS cycosis ANY HEART LAYER LEADMANSACKS endocarditis vegetation of both sides of the valve. ANEMIA THROMBOCYTOPENIA LEUKOPENIA
Lupus common cause of death is from
Renal damage - diffuse proliferative glomeruloephritis
INFECTION
lupus is Characterized by a ANA
Anti nutruphil body and a anti DS DNA
Lupus can also be drug induced by what drugs
ANTI HISTONE ANTIBODY Hydralazine procainamide and isoniazid
removal of drug will get rid of disease
Antiphosphlipid syndrome also accounted with lupus
antibody against phosophlipds specifically
anticardiolipin, (false postive syphilis test)
lupus anticoagulant, interfering a ppt (false elevation)
Antiphospholipid syndrome in lupus actually causes
arterial and venous thrombosis
Deep venous thrombosis and hepatic vein thrombosis, placental thrombosis and stroke
what is required life long when diagnosed with antiphsopholipid syndrome.
Anticoagulation medication
Bud keori syndrome is caused by and most commonly found with
Deep venous thrombosis
polly cithemia verra
Sjorgren syndrome
lacrimal and salivary glands are filled with fibrosis due type 4 hypersensitivity
common symptoms,
dirt in eye and multiple carries
Sjorgren syndrome is characterized by
Antiribonucleoprotein antibodies
antibody has the SS-A SS-B
What is the target of SS-A and SS-B
Ribonucleoproteins
Most auto immune diseases is associated wit h
rhematoid arthritis
A increased risk of what with sjorgens and swollen partied gland, and unilateral growth the partied gland
B cell lymphoma is what they are looking for.
Scleroderma
autoimmune both diffuse and localized
Tough skin from excess amounts of collagen in the skin
Scleroderma diffuse type is what
exhibits skin and early visceral involvement
Esophogus is commonly affected causing disorder motility (dysphagia for solids and liquids)
Scleroderma diffuse is Characterized by
Anti DNA topoisomerase 1 (Scl-70 antibody)
Localized scleroderma is CREST syndrome which is
Calcinosis and Anti Centromere antibodies Raynaud phenomenon Esophageal dysmptility Sclerodactyly (hands) Telangiectasia's of skin
Mixed connective tissue disease
Tissue damage with features of SLE, 100% have antibodies against U1 ribonucleoprotein
common damage with mixed features
of SLE, systemic sclerosis and polymyotsitis
Wound healing
regeneration and repair, replace tissue with collagen and fibrosis
3 types of tissues based of regenerative capacity
labile tissues
Small and large bowel,(stem cells in mucosal crypts)
skin (stem cells basal cells)
bone marrow (hematopoietic stem cells)
where are the stem cells of the bowel
crypts
where are the stem cells for bone marrow
hematopoietic stem cells CD34+
what is the stem cell of the lung
type 2 phnemocytes
Stable tissues is the 2nd tissue
quiscent (sit out side of cell cycle and can reenter) Liver example will produce additional cells and then reenters quiescence also kidney
the third is permeant tissues
lacks regenerative abilities
cardiac and skeletal muscle and neurons.
REPAIR not REGENERATION
if the stem cell layer are damaged what will happen instead of regeneration fro a cut
Repair will accrue not regeneration
repair cycle
Granulation tissue will become a scar
fibroblasts
capillaries
myofibroblasts
granuloma,
epithelhoid histiocyte
formed by by IL 12 from macrophage hits the the CD4 t cell turns it into the t herper cell
name collagen 1 through 4 and what they are used for
- used in bone
- used in collagen
- used in blood vesssels
- basement membrane
What is the cofactor for collegase in wound repair (scar)
Zinc!!!!!!!
What is the mechanism of regeneration and repair
paracrine signalling via growth factors by macrophages
TGF-alpaha
epithelial and fibroblast growth factors
TGF-Beta
important fibroblast growth factor inhibits inflammation also works with IL 10
PDGF-
endothelium smooth muscle fibroblast growth factor
FGF, very important
angiogenesis( helps to grow blood vessels) , skeletal development
VEGF
angiogenesis
Cutaneous healing can occur by two mechanisms
Primary wound edges brought together for minimal scar formation
secondary- wound edges remain open and granulation tissue fills in the defects. causes contraction of the the area. (myofibroblasts)
delayed wound healing
infection is the most common cause with continued inflammation which delay healing.
WE need VIT C!!!
What are important assisting substances in healing
VIT C-( used to hydroxolate or add a OH to proline or Lysine on collagen) for strengthening collagen
Copper- (lysyl oxidase) used to crosslink collagen making it stronger
Zinc- used to convert type 3 to type 1 collagen for collagenase.
Other causes of delayed wound healing
ischemia, foreign body, dehiscence, diabetes colitis, hypertrophic scar, malnutrition.j
hypertrophic scar
excess deposition of type 1 collagen
Keloid is what
Excess production of scar, excess type 3, genetic predisposition on african americans,
classically seen on the ear lobe
