UNIQUE CASE OF GROWTH-HORMONE (GH) DEFICIENCY ACCOMPANIED BY CLINICALANOPHTHALMIA, HYPOPLASTIC ORBITS, DIGITAL DYSPLASIA, SHORT STATURE, OBESITY, AND DIABETES-MELLITUS

A 43-year-old female was admitted to our hospital for polydipsia and h yperglycemia. She had total blindness and globes were not recognized b y inspection, indicating clinical anophthalmia. Physical examination r evealed short stature, obesity, prematurely gray hair, shortness of fi ngers and toes, syn-dactyly, and multiple dental caries. Laboratory ex amination showed hyperglycemia, increased glycosilated hemoglobin (HbA (1)c) and insulin resistance on euglycemic glucose clamp. Blunted grow th hormone (GH) secretion was shown in response to insulin-induced hyp oglycemia, arginine infusion, and GH-releasing hormone (GHRH) loading test, and in 24 h spontaneous GH profile. Magnetic resonance imaging ( MRI) and computed tomography (CT) showed dysostosis of orbit, defect o f optic nerve, enlarged suprasellar cistern, and prolonged pituitary s talk. This may be the first report of a unique case with GH deficiency accompanied by clinical anophthalmia, hypoplastic orbits, digital dys plasia, short stature, obesity, and diabetes mellitus. (C) 1996 Wiley- Liss, Inc.