@article{59e5c89efb5647e1bd9923b25cf1d98e,
title = "Limited effect of erythrocyte and plasma infusions in adenosine deaminase deficiency",
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.",
author = "Schmalstieg, {Frank C.} and Mills, {Gordon C.} and {Arly Nelson}, J. and May, {Linda T.} and Goldman, {Armond S.} and Golblum, {Randall M.}",
note = "Funding Information: From the Departments of Pediatrics, Pharmacology and Human Biological Chemistry and Genetics, The University of Texas Medical Branch. Supported in part by the following: National Institutes of llealth, Grant No. DHEW RR-00073-14, General Clinical Research Centers Branch, {"}Division of Research Facilities and Resources; Grant No. RO1-CA-17057; Grant No. DHEW 06-S-000170-09,~ DHEW Research Grant 5T32-GM-02204; a grant from Multidisciplinary Research Program in Mental llealth, UTMB, Galveston; and a grant from The National Foundatr of Dhnes No. 6-130. Dr. Schmalstieg was the recipient of Th.e Jeanne B. Kempner Scholarship A ward; Drs. May and Schmalstieg qre recipients. of The James IV. .McLaughlin Post-DoctoralF ellowship, The University of Texas Medical Branch, Galveston. *Reprint address: Division of lmmunology and Allerg),;. Department of Pediatrics, The University of Texas Medical Branch. Gah'eston, TX 77JJO.",
year = "1978",
month = oct,
doi = "10.1016/S0022-3476(78)80894-4",
language = "English (US)",
volume = "93",
pages = "597--603",
journal = "Journal of Pediatrics",
issn = "0022-3476",
publisher = "Mosby Inc.",
number = "4",
}