Immune deficient children

UNINTENDED CDNSEQUENCES OF GENE THERAPY Immune Deficient Children... 

Young child born with immune deficiency syndrome plays in the enclosed, germ-free, plastic environment in which he must live to survive. 

Citak A, Garratty G, Ucsel R, Karabocuoglu M, Uzel N. Ceftriaxone-induced haemolytic anaemia in a child with no immune deficiency or haematological disease. J Paediatr Child Health 2002 38(2) 209-10. 

Evidence that purine metabolism is important in the immune response has been obtained from the observation that markedly reduced or absent adenosine deaminase (ADA) activity in man has been casually associated with an autosomal recessive form of severe combined immunodeficiency disease (3). Recently, ADA levels in lymphocytes from patients with untreated chronic lymphatic leukemia have been found to be consistently lower than in lymphocytes from normal subjects (4). Children with ADA deficiency and immunodeficiency have been shown to have increased levels in plasma, urine, lymphocytes and erythrocytes of adenosine, adenine, deoxy-adenosine, adenine nucleotides, and deoxyadenine (5, 6). Although the exact biochemical mechanism(s) is unknown, elevated levels of adenosine, and/or deoxyadenosine and their metabolites are thought to be selective inhibitors of both differentiation and effector function of lymphocytes (7, 8). Adenosine was known to inhibit the PHA-induced blastogenesis of human peripheral blood lymphocytes (9) even before the discovery of the first ADA-deficient child. In addition, elevated levels of cyclic AMP (cAMP) were known to be inhibitory for lymphocyte-mediated cytotoxicity (7). Since... 

AIDS (acquired immune deficiency syndrome) is a breakdown in the body s immune system that can be suffered by both sexes. It can be transmitted from person to person in body fluids during sexual intercourse and infection can occur in transfusion of blood products, donated organs, by mother to child during childbirth or breast feeding and through the use of infected needles used for injections. 

G4. Giblett, E. R., Ammann, A. J., Wara, D. W., Sandman, R., and Diamond, L. K., Nucleoside phos-phorylase deficiency in a child with severely defective T cell immunity and normal B cell immunity. Lancet 1, 1010-1013 (1975). 

Vitamin A deficiency is a major problem of children under five in developing countries, being the single most common preventable cause of blindness. Table 2.1 shows the prevalence of vitamin A deficiency in different regions of the world. The increased susceptibility to infection and impairment of immune responses in vitamin A deficiency causes significant childhood mortality. A number of trials of vitamin A supplementation in areas of endemic deficiency show a 20% to 35% reduction in child mortality. 

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