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 language | English (US) |
|---|---|
| Pages (from-to) | 597-603 |
| Number of pages | 7 |
| Journal | The Journal of pediatrics |
| Volume | 93 |
| Issue number | 4 |
| DOIs | |
| State | Published - 1978 |
| Externally published | Yes |
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ASJC Scopus subject areas
- Pediatrics, Perinatology, and Child Health
Cite this
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 journal › Article
}
TY - JOUR
T1 - Limited effect of erythrocyte and plasma infusions in adenosine deaminase deficiency
AU - Schmalstieg, Frank C.
AU - Mills, Gordon C.
AU - Arly Nelson, J.
AU - May, Linda T.
AU - Goldman, Armond S.
AU - Golblum, Randall M.
PY - 1978
Y1 - 1978
N2 - 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.
AB - 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.
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UR - http://www.scopus.com/inward/citedby.url?scp=0017899040&partnerID=8YFLogxK
U2 - 10.1016/S0022-3476(78)80894-4
DO - 10.1016/S0022-3476(78)80894-4
M3 - Article
VL - 93
SP - 597
EP - 603
JO - Journal of Pediatrics
JF - Journal of Pediatrics
SN - 0022-3476
IS - 4
ER -