Disorder of Immunity II Flashcards
(30 cards)
HIGH YEILD
Mikulicz Disease vs. Mikulicz Syndrome
anything involving the lacrimal/salivary glands. Whatever causes problem of the glands leads to enlargement of the gland.
The syndrome means the glands are enlarged.
Describe the symptoms dealing with Sjogren Syndrome
Involvement of the lacrimal and salivary glands. Patients present with xerostomia (dry mouth), keratoconjunctivitis (dry eyes), rheumatoid factor may be seen in 75% of patients. Patients will have chipmunk appearance.
What is the most common cause of death for Lupus patients
renal failure and intercurrent infections
What is essential for the diagnosis of Sjogren Syndrome
biopsy of the lip
What cell is causing the problem in fibrosis?
Fibroblast are the bad cells because they are the ones that makes collagen
Scleroderma is also known as? Is it an acute or chronic disease? What causes this disease? What is it characterized by?
Systemic Sclerosis
Chronic
Etiology unknown
Characterized by abnormal accumulation of fibrosis in skin and multiple organs
What are the two major categories of Systemic Sclerosis? How do you tell them apart?
Diffuse & Limited which are associated with the CREST syndrome
The prognosis is different between the two, and 2 separate antibodies are associated with them
Involvement of the heart, kidney, and lungs with scar tissue is associated with which form of Scleroderma?
Diffuse bka SCL 70
Local - is limited to where it is in the body and has a better prognosis than diffuse
CREST stands for
C = Calcinosis - calcium deposits in the body R = Raynaud's phenomenon - fingers and toes get cold, ischemic, brought on by cold waterr, gets vasospasms of fingers and toes E = Esophageal dismotility - problems swallowing S = Sclerodactyly - claw hand deformity T = Telangiectasia - small dilated little blood vessels
An older women around the age of 56 complains skin is tight and shiny with no hair follicles. The patient has malignant hypertension, respiratory difficulties, arrhythmias and reflux in the GI tract. What does this patient have
Diffuse form of Scleroderma
Primary Immunodeficiencies is commonly seen in?
Infants and children. Normally congenital or genetic
The infant will have recurrent infections because they do not have the defense to fight that particular infection
Secondary will be acquired and commonly seen in?
Adults for ex: HIV
An 8th mouth baby boy has recurrent bacterial infections such as gastritis, pneumonia etc.. Test results showed no antibodies present in the blood. What is the name of this disorder? Is it primary or secondary immunodeficiency?
It is primary.
X-Linked Bruton Agammaglobulinemia
What is the underling problem in X-Linked Bruton Agammaglobulinemia?
Failure of pre B-cells to differentiate into B-cells
B-cell problem only
T-cells are okay so the boy can fight viral infections
ONLY seen in boys
What is the most common of ALL the primary immunodeficiency disorders
IgA deficiency - European descent, rare in blacks and asians
due to the failure of selective IgA B-cells to differentiate into plasma cells.
Most patients are asymptomatic
CVI (Common Variable Immunodeficiency) is due to? Onset occurs? The patients will have an increase in which type of disorders? Will antibodies be present in the patients blood? How is it different from X-Linked Bruton?
B-cells fail to mature to plasma cells
Onset occurs in childhood or adolescence
Increase in autoimmune disorders
Antibodies will be present, but at low amount
CVI is not an X-linked so it can occur in male and female. Some antibodies are present unlike in Bruton
In DiGeorge Syndrome which pharyngeal pouches are undeveloped?
3rd and 4th
Thymic hypoplasia is the same thing as
DiGeorge
A patient with tetany has hypocalcemis, recurrent viral infections and fungal infections, and abnormal facial features. What is the immunodeficiency
DiGeorge
deficiency on T-cells
no thymus
A male patient has poor response to polysaccharide antigen due to antibody problem. T-cells deficient, male with viral infections, bleeding, and skin rash. What disorder is associated with these symptoms?
Wiscott Aldrich Syndrome
Accumulation of toxic products in T and B cells –> defective lymphocytic function. What is the name of the defective enzyme.
ADA deficiency
Adenosine DeAminase
Defect in humoral and T-cell mediated immunity is associated with? What is the treatment
SCID
Sever Combined Immunodeficiency Disease
bone marrow transplant
What is the infective portion of the AIDs virus and which cells do it mainly affects?
gp120
CD4+ Tcells
normal ratio CD4 : CD8 = 2:1
abnormal ratio = 1:8
CD8 will out number the CD4 cells due to AIDS
Cryptococcal is the most common fungal infection. What is its vector?
Pigeon droppings
