Idiopathic adrenal hypoplasia in children.

Abstract

The cases of 10 patients with adrenal hypoplasia are reported, including two occurring in siblings; one in a monozygous twin with adrenal hypoplasia whose twin had adrenal glands of normal size; one in a trizygous triplet with adrenal hypoplasia, whose two siblings had no evidence of adrenal hypoplasia; and two in infants who were small for their gestational ages. Although all 10 infants had clinical signs consistent with adrenal insufficiency, this diagnosis was not made clinically in any case, and postmortem studies failed to provide anatomic evidence of adrenal insufficiency. Morphologically normal, but miniature, adrenal glands were found in nine of the ten patients.