Adenosine deaminase and purine nucleoside phosphorylase deficiencies: evaluation of therapeutic interventions in eight patients

ML Markert, MS Hershfield, RI Schiff… - Journal of clinical …, 1987 - Springer
The courses of six patients with adenosine deaminase (ADA) and two with purine
nucleoside phosphorylase (PNP) deficiencies were evaluated before and after therapy. The …

Adenosine deaminase deficiency and immunodeficiencies.

R Hirschhorn - Federation Proceedings, 1977 - europepmc.org
Severe Combined Immunodeficiency (SCID) is a fatal disorder of infancy in which patients
exhibit profound defects of both cellular and humoral immune function. Approximately 50 …

Adenosine deaminase and purine nucleoside phosphorylase deficiency: how they were discovered and what they may mean

ER Giblett - Ciba Foundation Symposium 68‐Enzyme Defects …, 1979 - Wiley Online Library
The serendipitous finding of adenosine deaminase (ADA) deficiency in patients with severe
combined immunodeficiency disease led to the speculation that patients with inherited …

An approach to the restoration of T cell function in a purine nucleoside phosphorylase deficient patient

BJM Zegers, JW Stoop, GEJ Staal… - Enzyme defects and …, 1979 - Wiley Online Library
A patient with a selective impairment of T cell-dependent immunity based on a purine
nucleoside phosphorylase (PNP) deficiency has been treated with transfusions of irradiated …

Immunological aberrations in purine nucleoside phosphorylase deficiencies

AJ Ammann - Enzyme defects and immune dysfunction, 1979 - Wiley Online Library
Purine nucleoside phosphorylase deficiency in association with immunodeficiency is a
distinctive immunological disorder. A total of nine patients, in six different institutions, have …

Purine nucleoside phosphorylase deficiency: improved metabolic and immunologic function with erythrocyte transfusions

KC Rich, E Mejias, IH Fox - New England Journal of Medicine, 1980 - Mass Medical Soc
DEFICIENCY of the purine enzyme nucleoside phosphorylase is associated with a severe T-
lymphocyte immune deficiency. 1 2 3 4 5 6 The abnormality in the immune system results …

Adenosine deaminase deficiency and purine nucleoside phosphorylase deficiency in common variable immunodeficiency

A Fleischman, MS Hershfield, S Toutain… - Clinical Diagnostic …, 1998 - Am Soc Microbiol
The clinical presentations of adenosine deaminase deficiency and purine nucleoside
phosphorylase deficiency are widely variable and include clinical and immunologic findings …

[引用][C] ADA and PNP deficiencies: how it all began

ER Giblett - Annals of the New York Academy of Sciences, 1985 - Wiley Online Library
The fourth edition of Dr. Max Wintrobe's textbook Clinical Hematology,'published in 1956,
contained only three pages describing the lymphocyte and speculating about its function. At …

[引用][C] Inherited enzyme deficiencies and immunodeficiency: adenosine deaminase (ADA) and purine nucleoside phosphorylase (PNP) deficiencies

R Hirschhorn - Clinical immunology and immunopathology, 1986 - Elsevier
The current classification of immunodeficiency disorders can be considered analogous to
the state of classification of pneumonias during the early days of application of …

Enzyme replacement and other biochemical approaches to the therapy of adenosine deaminase deficiency

SH Polmar - Ciba Found. Symp, 1979 - Wiley Online Library
Addition of adenosine deaminase (ADA) restored in vitro responses of lymphocytes from a
patient with ADA deficiency and severe combined immunodeficiency (SCID). Enzyme …