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Partial DiGeorge Syndrome With Substantial Cell-Mediated Immunity
Henry F. Pabst, MD, FRCP(C);
William C. Wright, MD;
Jean LeRiche, MD, FRCP(C);
E. Richard Stiehm, MD
Am J Dis Child. 1976;130(3):316-319.
Abstract
• Results of studies on two male infants with incomplete expression of the DiGeorge syndrome are analyzed. Both infants demonstrated neonatal tetany with hypoparathyroidism, cardiovascular anomalies, and absence of a thymus shadow on roentgenographic examination. Some degree of cellular immunity was present in both infants, however, including normal in vitro responses to phytohemagglutinin, thus postponing attempts at thymus transplantation. Both infants died suddenly at home, one at age 7 weeks and the other at age 44 weeks. At autopsy, no thymus was found in one, and a 2 x 2-mm thymus was detected after extensive search in the other. These cases emphasize the need for repeated monitoring of all immunologic measurements in the partial DiGeorge syndrome, so that early therapeutic intervention can be undertaken.
(Am J Dis Child 130:316-319, 1976)
Author Affiliations
From the departments of pediatrics (Dr Pabst) and pathology (Dr LeRiche), University of Alberta, Edmonton, Canada, and the Department of Pediatrics, University of California, Los Angeles (Drs Wright and Stiehm).
Footnotes
Received for publication Nov 4, 1974; accepted March 25, 1975.
Reprint requests to Department of Pediatrics, Clinical Sciences Building, University of Alberta, Edmonton, Alberta, Canada (Dr Pabst).
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