title: Clinical spectrum of the syndrome of growth hormone insensitivity. Advisory Committee on Growth Hormone Insensitivity.
abstract: A survey of 46 patients with suspected growth hormone (GH) insensitivity is presented. Clinical details and plasma samples from these patients were analysed centrally. A diagnostic score based on basal levels of GH and insulin-like growth factor I (IGF-I), IGF-I response to GH (0.1 U/kg daily for 4 days) and height SDS was determined for all patients to identify those with GH insensitivity. A total of 29 such patients was identified (14 males and 15 females; age range, 3.2-22.6 years). Their mean height SDS was -6.0 (range, -8.9 to -2.6), mean weight SDS was -2.9 (range, -5.2 to 1.2). Pubertal development was delayed in three out of three boys and two out of six girls of pubertal age. Their basal GH level was 32.4 mU/l (range, 0.8-158.2 mU/l) and mean basal IGF-I level was 33.6 ng/ml (range, 20-97 ng/ml). None of the 29 patients showed a significant serum IGF-I response to injections of GH.
authors.affiliation: Division of Paediatric Endocrinology, St Bartholomew's Hospital, London, UK.
authors.forename: M O
authors.initials: MO
authors.lastname: Savage
authors.forename: P G
authors.initials: PG
authors.lastname: Chatelain
authors.forename: M A
authors.initials: MA
authors.lastname: Preece
authors.forename: M B
authors.initials: MB
authors.lastname: Ranke
authors.forename: A
authors.initials: A
authors.lastname: Sietnieks
authors.forename: P
authors.initials: P
authors.lastname: Wilton
mesh_terms: D000293:Adolescent; D000328:Adult; D001827:Body Height; D002352:Carrier Proteins / Q000097:blood; D002648:Child; D002675:Child, Preschool; D004392:Dwarfism / Q000097:blood; D005260:Female; D013006:Growth Hormone / Q000097:blood; D006801:Humans; D018969:Insulin-Like Growth Factor Binding Proteins; D007334:Insulin-Like Growth Factor I / Q000032:analysis; D008297:Male; D008875:Middle Aged; D011986:Receptors, Somatotropin / Q000032:analysis; D013002:Somatomedins / Q000032:analysis; D013577:Syndrome
chemical_list: D002352:Carrier Proteins; D018969:Insulin-Like Growth Factor Binding Proteins; D011986:Receptors, Somatotropin; D013002:Somatomedins; D007334:Insulin-Like Growth Factor I; D013006:Growth Hormone; C051815:somatotropin-binding protein