Enzyme replacement and other biochemical approaches to the therapy of adenosine deaminase deficiency.
Ciba Found Symp
; (68): 213-30, 1978.
Article
em En
| MEDLINE
| ID: mdl-227649
ABSTRACT
Addition of adenosine deaminase (ADA) restored in vitro responses of lymphocytes from a patient with ADA deficiency and severe combined immunodeficiency (SCID). Enzyme replacement therapy, using red blood cells as a source of encapsulated human ADA, restored both T and B cell function in this patient. Ten other ADA--SCID patients have been treated with this form of enzyme replacement and five have responded to therapy. Lymphocytes from ADA--SCID patients treated with enzyme replacement become immunocompetent but remain enzyme deficient. Studies of these cells provide evidence supporting both cyclic AMP- and dATP-mediated immunosuppressive mechanisms in ADA--SCID. These observations suggest that inhibition of cyclic AMP synthesis and/or deoxycytidine (and possibly thymidine) supplementation may be useful new biochemical approaches to the therapy of ADA--SCID.
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Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Adenosina Desaminase
/
Síndromes de Imunodeficiência
/
Nucleosídeo Desaminases
Limite:
Humans
/
Infant
/
Male
Idioma:
En
Revista:
Ciba Found Symp
Ano de publicação:
1978
Tipo de documento:
Article