FETAL ADRENAL TRANSPLANTATION ATTEMPT IN JUVENILE ADDISON'S DISEASE

Abstract

The clinical history and management are presented of a boy who, at age 8 years, had onset of adrenal insufficiency of undetermined cause. He was controlled well with corticosteroid therapy and had trouble only during infections and hot weather. At age 12 years adrenal cortex tissue from a premature infant was transplanted into his abdominal wall. ACTH was given daily for 3 months to stimulate the transplant. No measurable 17-OHCS were present in his blood during this time. Seven and 9 months after the transplantation intravenous ACTH tests revealed low but definite concentrations of 17-OHCS in his blood. Ten months after the transplantation he was "stressed" by stopping his exogenous steroid therapy for 17 days. He developed progressive signs of adrenal insufficiency, but he also responded with measurable concentrations of 17-OHCS in his blood. Chromatographic analysis of his urine during the "stress" period revealed a steroid profile that suggested adrenal cortex activity. His corticosteroid production at best was only 10% of normal. The transplant was not examined directly.