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Immune Deficiency Disorder in Children
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PRIMARY IMMUNE DEFICIENCY DISORDER IN CHILDREN
genetic defects (inborn defects) that can lead to dysfunction in B cells or T cells or both or neutrophils and can cause immune deficiency.
These conditions are known as Primary Immunodeficiency Disease and over 70 primary immune deficiency disorders have been identified in last few decades.
The various primary immunodeficiency disorders seen are:
Severe Combined Immunodeficiency (SCID): SCID is characterized by profoundly abnormal T cell function and antibody production. Patients usually present with chronic diarrhea, recurrent pneumonias and inability to put on weight. There are various types of SCID described, however the most common form is X-linked SCID seen in males. Children usually survive upto 1-2 years of age unless intervened. It is commonly called as "bubble boy disease". Treatment consists of bone marrow transplant. Patients should avoid live viral vaccines and monthly injections of intravenous immunoglobulin (IVIG) may be required. IVIG are antibody injections
Hyper IgM syndrome: Again this defect is more common in males and infants present with recurrent pneumonia, diarrhea and inability to gain weight. Here there is a defect in antibody conversion due to various genetic defects. Treatment again is bone marrow transplant and monthly injections of IVIG to decrease frequency of infections.
Common Variable Immunodeficiency (CVID): Here, there is absent or ineffective antibody production. Patients present in 1st decade or 2nd decade of life with recurrent pneumonias. These patients require longer courses of antibiotics for their treatment. They have poor response to vaccines. Other associated features may be rheumatoid arthritis. The treatment consists of monthly IVIG therapy.
IgA deficiency: This is the commonest type of primary immunodeficiency and occurs in 1 in 300 to 1 in 1,000 adults. It usually is diagnosed when screening family members of a patient with primary immunodeficiency. Most people with IgA deficiency are healthy. An exception to that are individuals who also lack IgG2, who are prone to severe bacterial infections. For patients with isolated IgA deficiency and no serious infections, no treatment is required. For patients with combined IgG2 deficiency, monthly IVIG is treatment of choice.
Chronic mucocutaneous candidiasis: This is characterized by recurrent candidial (fungal) infection in mouth, fingernails, toe-nails and in females in the vagina. Patients may have associated thyroid problem or diabetes mellitus. Treatment consists of anti fungals.
Hypogammaglobulinemia: This is characterized by low antibody production and recurrent bacterial infections. It is commonly seen after the age of 6 months and treatment consists of monthly IVIg.
Other primary immune deficiencies include Wiskott-Aldrich syndrome, defects in neutrophils and complement defects.
Conclusion: A child with recurrent and serious infections should be screened for an underlying immune defect be it an acquired (HIV) or an inborn defect (Primary Immunodeficiencies).
Last updated on 01-09-2005
The various primary immunodeficiency disorders seen are:
Severe Combined Immunodeficiency (SCID): SCID is characterized by profoundly abnormal T cell function and antibody production. Patients usually present with chronic diarrhea, recurrent pneumonias and inability to put on weight. There are various types of SCID described, however the most common form is X-linked SCID seen in males. Children usually survive upto 1-2 years of age unless intervened. It is commonly called as "bubble boy disease". Treatment consists of bone marrow transplant. Patients should avoid live viral vaccines and monthly injections of intravenous immunoglobulin (IVIG) may be required. IVIG are antibody injections
Hyper IgM syndrome: Again this defect is more common in males and infants present with recurrent pneumonia, diarrhea and inability to gain weight. Here there is a defect in antibody conversion due to various genetic defects. Treatment again is bone marrow transplant and monthly injections of IVIG to decrease frequency of infections.
Common Variable Immunodeficiency (CVID): Here, there is absent or ineffective antibody production. Patients present in 1st decade or 2nd decade of life with recurrent pneumonias. These patients require longer courses of antibiotics for their treatment. They have poor response to vaccines. Other associated features may be rheumatoid arthritis. The treatment consists of monthly IVIG therapy.
IgA deficiency: This is the commonest type of primary immunodeficiency and occurs in 1 in 300 to 1 in 1,000 adults. It usually is diagnosed when screening family members of a patient with primary immunodeficiency. Most people with IgA deficiency are healthy. An exception to that are individuals who also lack IgG2, who are prone to severe bacterial infections. For patients with isolated IgA deficiency and no serious infections, no treatment is required. For patients with combined IgG2 deficiency, monthly IVIG is treatment of choice.
Chronic mucocutaneous candidiasis: This is characterized by recurrent candidial (fungal) infection in mouth, fingernails, toe-nails and in females in the vagina. Patients may have associated thyroid problem or diabetes mellitus. Treatment consists of anti fungals.
Hypogammaglobulinemia: This is characterized by low antibody production and recurrent bacterial infections. It is commonly seen after the age of 6 months and treatment consists of monthly IVIg.
Other primary immune deficiencies include Wiskott-Aldrich syndrome, defects in neutrophils and complement defects.
Conclusion: A child with recurrent and serious infections should be screened for an underlying immune defect be it an acquired (HIV) or an inborn defect (Primary Immunodeficiencies).
Last updated on 01-09-2005
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