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DEFICIENCY
FAT I M A A L - A W A D H
OBJECTIVES
Define the selective IgA deficiency.
Recognize the structure and function of IgA.
Mention the causes, pathophysiology, signs &
symptoms, complications, diagnosis, Treatment,
Prognosis and Prevention.
State the effect of vaccination on this disorder.
STRUCTURE
FUNCTION OF IGA
CAUSES
The defect causing these
deficiencies is not known in a
majority of cases; rarely, the
deficiencies may be caused
by mutations of Ig heavy
chain constant region genes.
PATHOPHYSIOLOGY
inherited
maturation
defect in B
cells
Alterations in
trans-membrane
activator, calcium
modulator &
cyclophilin ligand
interactor gene
inability to
produce Ig
A
bacteria
are more
able to
cause
disease
PATHOPHYSIOLOGY
B LY M P H O C Y T E S A R E U N A B L E T O P R O D U C E I G A
COMPLICATIONS
An autoimmune disorder such as rheumatoid
arthritis.
severe, even life-threatening, reactions to
transfusions of blood and blood products because
they develop anti-IgA antibody.
DIAGNOSIS
Patient & family
history.
Quantitative
immunoglobulins.
Serum
Immunoelectrophore
sis.
QUANTITATIVE IMMUNOGLOBULINS
rapid and accurate measurement of the
amounts of the immunoglobulins M, G, and
A.
tests of blood serum demonstrate
absence of IgA with normal levels of
the other major classes of
immunoglobulins (IgG and IgM).
NORMAL results of Ig M, G and A
IgG
IgM
45 to 250 mg/dL
IgA
SERUM
IMMUNOELECTROPHORESIS
MANAGEMENT
patients who do not have any symptoms
do not need any treatment.
prevent anaphylactic reaction
secondary to blood transfusion
treatment of associated diseases
PROGNOSIS
The prognosis is good in patients with IgA
deficiency if it is not associated with a significant
disease.
PREVENTION
There is no means of prevention of Selective
IgA Deficiency.
Because IgA Deficiency does not become
detectable until approximately six months of age,
prenatal and neonatal detection of this disorder is
currently not possible.
Contraindicated
Vaccines
Risk-specific
recommended
Vaccines
Effective Vaccines
OPV2
BCG
Yellow fever
Other live vaccines
appear to be safe
Pneumococcal
REFERANCES
Basic Immunology, Abbas 3th ed.
http://
www.nlm.nih.gov/medlineplus/ency/article/00147
6.htm
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC28
21513
/
http://
primaryimmune.org/wp-content/uploads/2011/04/Se
lective-IgA-Deficiency.pdf
http://
www.cdc.gov/vaccines/pubs/pinkbook/downloads/
appendices/A/immuno-table.pdf
THANK YOU