Limited effect of erythrocyte and plasma infusions in adenosine deaminase deficiency

Frank C. Schmalstieg, Gordon C. Mills, J. Arly Nelson, Linda T. May, Armond S. Goldman, Randall M. Golblum

Research output: Contribution to journalArticle

28 Citations (Scopus)

Abstract

A 10-month-old child with a profound deficiency of adenosine deaminase and severe combined immunodeficiency was treated for a period of 17 months with red cell and plasma transfusions containing normal amounts of the deficient enzyme. Following each transfusion, the plasma adenosine, red cell and lymphocyte ATP, urinary adenine, and urinary deoxyadenosine decreased transiently. During this period, the absolute blood lymphocyte count rose and a limited increased in the response of the lymphocytes to PHA-P was observed. Delayed hypersensitivity skin tests remained negative during the transfusion periods. A quantitative elevation of serum immunoglobulins occurred, but specific antibody formation was not elicited. In contrast to a previous report of successful therapy of ADA deficiency with red cell and plasma infusions, this patient responded poorly to enzyme replacement therapy. The difference may be related to a more profound enzyme deficiency in our patient.

Original languageEnglish (US)
Pages (from-to)597-603
Number of pages7
JournalThe Journal of pediatrics
Volume93
Issue number4
DOIs
StatePublished - 1978
Externally publishedYes

Fingerprint

Plasma Cells
Erythrocytes
Lymphocytes
Enzyme Replacement Therapy
Severe Combined Immunodeficiency
Delayed Hypersensitivity
Lymphocyte Count
Adenine
Enzymes
Skin Tests
Adenosine
Antibody Formation
Immunoglobulins
Adenosine Triphosphate
Serum
Severe combined immunodeficiency due to adenosine deaminase deficiency
Therapeutics
2'-deoxyadenosine
phytohemagglutinin-P

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health

Cite this

Schmalstieg, F. C., Mills, G. C., Arly Nelson, J., May, L. T., Goldman, A. S., & Golblum, R. M. (1978). Limited effect of erythrocyte and plasma infusions in adenosine deaminase deficiency. The Journal of pediatrics, 93(4), 597-603. https://doi.org/10.1016/S0022-3476(78)80894-4

Limited effect of erythrocyte and plasma infusions in adenosine deaminase deficiency. / Schmalstieg, Frank C.; Mills, Gordon C.; Arly Nelson, J.; May, Linda T.; Goldman, Armond S.; Golblum, Randall M.

In: The Journal of pediatrics, Vol. 93, No. 4, 1978, p. 597-603.

Research output: Contribution to journalArticle

Schmalstieg, FC, Mills, GC, Arly Nelson, J, May, LT, Goldman, AS & Golblum, RM 1978, 'Limited effect of erythrocyte and plasma infusions in adenosine deaminase deficiency', The Journal of pediatrics, vol. 93, no. 4, pp. 597-603. https://doi.org/10.1016/S0022-3476(78)80894-4
Schmalstieg, Frank C. ; Mills, Gordon C. ; Arly Nelson, J. ; May, Linda T. ; Goldman, Armond S. ; Golblum, Randall M. / Limited effect of erythrocyte and plasma infusions in adenosine deaminase deficiency. In: The Journal of pediatrics. 1978 ; Vol. 93, No. 4. pp. 597-603.
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