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December 1976

Partial DiGeorge Syndrome or Branchial Dysembryogenesis?-Reply

Author Affiliations

Department of Pediatrics University of California Los Angeles, CA 90024; Department of Pediatrics University of Alberta Edmonton, Alberta Canada T6G 2G3

Am J Dis Child. 1976;130(12):1377. doi:10.1001/archpedi.1976.02120130082024

In Reply.—The point made by Miller underlines our plea for early immunologic assessment and periodic monitoring of the immune response in infants suspected of or diagnosed as having the anomalad of branchial dysembryogenesis. The simplest and quickest screening test to be applied is E-rosetting of the patient's peripheral lymphocytes with sheep red blood cells,1.2 a test not available when we studied our two patients. Reconstitution with fetal thymus is still the preferred mode of therapy if T-cell function is found to be diminished. However, gestational age3 of the thymic graft is critical to minimize graft-vs-host reactions.3 Thus, full immunologic evaluation is essential before reconstitution is considered4 in babies with T-cell deficiency in whom even transfusions have been known to precipitate severe graft-vs-host disease.5

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