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PATHOLOGY AND MICROBIOLOGY, Slides of Pathology

PARUL UNIVERSITY IMPORTANT NOTES FOR EXAM

Typology: Slides

2022/2023

Available from 07/30/2023

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**********Immunodeficiency
diseases.*******
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.
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**********Immunodeficiency

diseases.*******

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.

(A) Specific Immunodeficiency diseases-----

Involve abnormalities of T or B- cells of the Adaptive

Immune system.

(B) Non-specific Immunodeficiency diseases----

Involve abnormalities of Elements such as Complement or Phagocytes, which act Nonspecifically in immunity.

(A) Primary immunodeficiencies.

Primary Immunodeficiency syndromes are basically Lymphoid cells disorders may affect T- cells, B- cells, or both B- and T-cells.

Deficiencies involving components of nonspecific

mediators of innate immunity, such as Phagocytes or

Complement, are impaired.

A. Disorders of specific immunity_._

B. Disorders of complements_._

C. Disorders of phagocytosis.

Classification----

(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.

(B). Disorders of Complement.------

(a). Complement Component deficiencies. (b). Complement Inhibitor deficiencies.

(C). Disorders of Phagocytosis.---

a. Chronic Granulomatous diseases.

b. Myeloperoxidase Deficiency.

c. Chediak-Higashi syndrome.

(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

Manifestations are not apparent till about

six months of age due to the passive protection

afforded by maternal antibodies.

The disease presents as recurrent serious infections with pyogenic bacteria, particularly with Pneumococci, Streptococci, Meningococci, Pseudomonas and H. influenzae.

When there is a delay, Immunodeficiency occurs,

with recurrent Otitis media and Respiratory infections

are the common diseases found in these conditions

Spontaneous recovery occurs between 18 and 30 months of age.

c). Common Variable Immunodeficiency (CVID).

CVID is characterized by a profound

decrease in numbers of Antibody-producing Plasma

cells,

low levels of most Immunoglobulin isotypes,

(Hypogammaglobulinemia), and recurrent infections.

These ‘Dysgammaglobulinemias’ are common and have been reported in about one per-cent of all patients with recurrent infections.

(1)Selective IgA deficiency.

IgA deficiency is most common, with recurrent respiratory and genitourinary tract infections resulting from lack of secreted IgA on mucosal surfaces.

Along with such as Intestinal malabsorption,

Allergic disease, and Autoimmune disorders may

also be associated with low IgA levels.

(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.