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32 Cards in this Set

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  • Back
what is caused by deficiency of C1 esterase inhibitor, with low serum C4 and consumption of complement components like C3?
hereditary angioedema
what is the hypersensitivity reaction: target cells are killed by Fc receptor bound cytotoxic leukocytes, no complement involvement, and major NK cell involvement.
Antibody-dependent cell-mediated cytotoxicity (ADCC)

subtype of type II
which disease?

type III HS rxn; deposit antigen-antibody complexes in many sites like heart, joints, kidneys
serum sickness
what HS type is SLE?
type III
what HS type is arthus reaction?
type III
what HS type is polyarteritis nodosa?
type III, involves small and medium sized arteries
name the disease:
- X linked, present in male infants, found at 6 mos
- no Ab synthesis due to failure of B cells to mature
- no plasma cells or immunoglobulins
- recurrent bacterial infections
Bruton's agammaglobulinemia
disease?
- occasional anaphylactic rxn to transfused blood and infections, especially in mucosal surfaces
isolated IgA deficiency
disease?
- congenital T-cell deficiency
- abnormalities of mandible, ear, aortic arch
- recurrent viral and fungal infections
- tetany
DiGeorge syndrome

CATCH 22:
Cardiac defects
Abnormal facies
Thymic hypoplasia
Cleft palate
Hypocalcemia

microdeletion of chromosome 22
disease?
- low B and T cell, severe defects in humoral and cell-mediated immunity
- adenosine deaminase deficiency (ADA), leading to toxins against lymphocytes
- failure to thrive
SCID: severe combined immunodeficiency disease

(aka Swiss-type agammaglobulinemia)
immunodeficiency disease?
- eczema, thrombocytopenia and recurrent infection
- x-linked disorder with normal total immunoglobulins
- poor Ab response to polysaccharide antigens
Wiskott Aldrich syndrome

TIE:
Thrombocytopenia
Infections recur
Eczema
how does HIV infection begin?`
HIV virion expresses a cell surface protein, gp120, with binding sites for the CD4 molecule on those T cells
which collagen disease?
- Raynaud phenomenon
- serosal inflammation (pericarditis and pleuritis)
- diffuse interstitial pulmonary fibrosis
- atypical non bacterial endocarditis
- immune complex vasculitis
SLE
which collagen disease?
- ANA reacts to Sm (Smith) antigen
- biologic false positive for syphilis
SLE
disease?
- ANA anti-Scl-70
- anti centromere
- widespread fibrosis and degenerative change
CREST syndrome (scleroderma)

Calcinosis
Raynaud phenomenon
Esophageal dysfunction
Sclerodactyly
Telangectasia
disease?
- hypertrophy of collagen fibers of subcutaneous tissue -> fixed facial appearance
- sclerodactyly
- dysphagia
- interstitial pulmonary fibrosis
- HTN
scleroderma
which collage disease?

triad:
1. dry mouth (xerostomia)
2. keratoconjunctivitis
3. occurs with other connective tissue or autoimmune disease
- involves salivary glands
Sjogren syndrome

anti-SS-B antibodies are found
which collagen disease?
- xerostomia (dry mouth) + keratoconjunctivitis
Sicca syndrome (Sjogren syndrome)
which collagen disease?
- chronic inflammation of proximal muscles of extremities
- increased serum creatinine kinase
- biopsy shows necrotic muscle cells and lymphcytes
polymyositis
disease?
- women 35-40 yrs
- arthralgia, Raynaud phenomenon, esophageal hypomotility
- ANA anti-nRNP, with speckled nuclear appearance
MCTD: mixed connective tissue disease
disease?
- found in men
- abdominal pain, HTN, uremia, polyneuritis, splenomegaly, fever etc.
- immune complex vasculitis with segmental fibrinoid necrosis in walls of small and medium sized vessels
polyarteritis nodosa
what type of amyloidosis is involved in multiple myeloma and in Waldenstroms macroglobulinemia?
primary amyloidosis, caused by deposition of AL (amyloid light chain) protein
what amyloid disease is a complication of chronic inflammatory disease, including RA, TB, osteomyelitis, syphilis or leprosy?
secondary (or reactive systemic) amyloidosis
disease?
- amyloid derived from transthyretin
- severe peripheral nerve involvement due to amyloid deposits
Portuguese type of polyneuropathy
disease?
- A4 or amyloid-beta protein deposits
- the code for this protein is on chromosome 21
Alzheimer disease
disease?
- episodic fever and polyserositis
- similar distribution and type to that of secondary (AA) amyloidosis
- characteristic demographic
familial Mediterranean fever
disease?
- prominent amyloid deposits in the tumor derived from calcitonin
medullary carcinoma of the thyroid
disease?
- amyloid deposit in islet cells
- amyloid is known as islet amyloid polypeptide (IAPP)
diabetes mellitus (type 2, insulin resistant)
disease?
- minor deposits of amyloid found at autopsy in the elderly
- when occurring in the heart, the amyloid is derived from transthyretin
senile amyloidosis
disease?
- amyloid deposits in joints of pts who have undergone years of dialysis
- amyloid derived from beta-microglobulin
dialysis-associated amyloidosis
what is the CREST syndrome?
a scleroderma subtype:

Calcinosis
Raynaud's phenomenon
Esophageal dysfunction
Sclerodactyly
Telangectasia
what is the CATCH 22 in DiGeorge syndrome (aka thymic hypoplasia)?
CATCH 22:
Cardiac defects
Abnormal facies
Thymic hypoplasia
Cleft palate
Hypocalcemia

microdeletion of chromosome 22