Human Anatomy & Physiology (9th Edition)

Published by Pearson
ISBN 10: 0321743261
ISBN 13: 978-0-32174-326-8

Chapter 21 - The Immune System: Innate and Adaptive Body Defenses - Review Questions - Critical Thinking and Clinical Application Questions - Page 799: 1a

Answer

death in early childhood ( fatal disease = lethal )

Work Step by Step

Severe Combined Immunodeficiency ( SCID ) - this may be due to failure of differentiation of stem cells into T and B cells however the explanation of this failure is not known. - both humoral and cell mediated immunity are deficient. - these infants suffer from all kinds of opportunistic infections : viral, bacterial, fungal and protozoal. - death occurs early in childhood ( before 1 year ). - the disease may be inherited in an X-linked recessive or autosomal recessive manner, however sporadic forms occur. # Immunologic Findings : 1- Hypoplasia of the Thymus as shown by chest X-ray. 2- Lymphopenia and decreased T cell number and functions: - impaired response to Phytohemagglutinin ( PHA ). - Impaired Delayed Hypersensitivity reactions. 3- Decreased B cell number and function : - Serum Immunogloulins are absent or decreased - No response to antigenic stimuli. # Treatment : patients may be made completely normal by grafting with histocompatible bone marrow from a sibling. incompatible grafts will lead to a fatal " graft versus host " ( GVH ) reactions. - Gammaglobulins may be administered. - Antibiotics are used for treatment of infections.
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