title: Nevo syndrome.
abstract: 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 neuropsychological development, hypotonia, adema, contractures of the hands and feet, a single a transverse palmar crease, and tapering digits. After meningococcal sepsis at age 6 months, he remained decerebrate. Thereafter, overgrowth and especially weight gain were extremely accelerated until his death at age 18 months, at which time his height was 103 cm and his weight was 23 kg. In addition to low plasma concentrations of growth hormone and insulin-like growth factor, severe insulin resistance was observed. It is presumed that a selective defect in insulin-stimulated glucose uptake, with preservation of anabolic effect, was one of the causes of his "overgrowth without growth hormone," at least in the last 12 months of life after severe brain damage.
authors.affiliation: Department of Pediatrics, University Hospital, Zagreb, Croatia.
authors.forename: M
authors.initials: M
authors.lastname: Dumić
authors.forename: D
authors.initials: D
authors.lastname: Vukelić
authors.forename: V
authors.initials: V
authors.lastname: Plavsić
authors.forename: A
authors.initials: A
authors.lastname: Cviko
authors.forename: L
authors.initials: L
authors.lastname: Sokolić
authors.forename: B
authors.initials: B
authors.lastname: Filipović-Grcić
mesh_terms: D001786:Blood Glucose / Q000378:metabolism; D004487:Edema / Q000235:genetics; D006130:Growth Disorders / Q000235:genetics* / Q000378:metabolism / Q000473:pathology; D019382:Human Growth Hormone / Q000097:blood; D006801:Humans; D007223:Infant; D007333:Insulin Resistance; D007515:Islets of Langerhans / Q000473:pathology; D008297:Male; D009123:Muscle Hypotonia / Q000235:genetics; D013002:Somatomedins / Q000378:metabolism; D013577:Syndrome
chemical_list: D001786:Blood Glucose; D013002:Somatomedins; D019382:Human Growth Hormone