NEVO SYNDROME

We report on a patient with Nevo syndrome manifesting intrauterine and postpartum overgrowth, accelerated osseous maturation, dolichocephaly , highly arched palate, large, low-set ears, cryptorchidism, delayed n europsychological development, hypotonia, adema, contractures of the h ands and feet, a single a transverse palmar crease, and tapering digit s. After meningococcal sepsis at age 6 months, he remained decerebrate . Thereafter, overgrowth and especially weight gain were extremely acc elerated until his death at age 18 months, at which time his height wa s 103 cm and his weight was 23 kg. In addition to low plasma concentra tions of growth hormone and insulin-like growth factor, severe insulin resistance was observed. It is presumed that a selective defect in in sulin-stimulated glucose uptake, with preservation of anabolic effect, was one of the causes of his ''over-growth without growth hormone,'' at least in the last 12 months of life after severe brain damage. (C) 1998 Wiley-Liss, Inc.