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PARUL UNIVERSITY IMPORTANT NOTES FOR EXAM
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Immunodeficiency diseases are conditions where the defence mechanisms of the body are impaired, leading to repeated microbial infections of varying severity and sometimes enhanced susceptibility to malignancies.
Involve abnormalities of Elements such as Complement or Phagocytes, which act Nonspecifically in immunity.
Primary Immunodeficiency syndromes are basically Lymphoid cells disorders may affect T- cells, B- cells, or both B- and T-cells.
A. Disorders of specific immunity_._
B. Disorders of complements_._
C. Disorders of phagocytosis.
(e) Immunodeficiencies with Hyper-IgM. (f) Transcobalamin ii deficiency.
(2). Cellular Immunodeficiencies (T cell defects ). (a) Thymic Hypoplasia (Digeorge’s Syndrome). (b) Chronic Mucocutaneous candidiasis (c) Purine Nucleoside phosphorylase (PNP) deficiency
(3). Combined immunodeficiencies (B and T- cell defects). (a) Cellular Immunodeficiency with Abnormal Immunoglobulin synthesis (Nezelof syndrome
(b) Ataxia Telangiectasia. (c) Wiskott-Aldrich Syndrome. (d) Immunodeficiency withThymoma. (e) Immunodeficiency with Short-Limbed Dwarfism.
(a). Complement Component deficiencies. (b). Complement Inhibitor deficiencies.
(d). Leukocyte G-6-pd deficiency. (e). Job’s syndrome. (f). Tuftsin deficiency.
(g). Lazy Leukocyte syndrome.
(h). Hyper- lgE syndrome. (i). Actin-binding Protein deficiency.
Immunodeficiencies, and was discovered in 1952 by Colonel Ogden Bruton.
It is seen only in male infants. The incidence of this condition has one in a hundred thousand population. Such children frequently have a family history of Brothers or Maternal uncles with recurring infections.
Manifestations
The disease presents as recurrent serious infections with pyogenic bacteria, particularly with Pneumococci, Streptococci, Meningococci, Pseudomonas and H. influenzae.
Spontaneous recovery occurs between 18 and 30 months of age.
These ‘Dysgammaglobulinemias’ are common and have been reported in about one per-cent of all patients with recurrent infections.
IgA deficiency is most common, with recurrent respiratory and genitourinary tract infections resulting from lack of secreted IgA on mucosal surfaces.
(ii). Selective IgM deficiency. Selective IgM deficiency is a rare condition and has been found to be associated with Septicemia, may be accompanied by various malignancies or by Autoimmune disease.
