title: Noonan syndrome presenting growth hormone neurosecretory dysfunction.
abstract: Noonan syndrome has been diagnosed by the characteristic physical stigmata for more than two decades. Recent studies of growth hormone secretory pattern provide a new category of growth hormone neurosecretory dysfunction to characterize short stature. We describe herein a case of growth hormone neurosecretory dysfunction in a 16-year-old boy with Noonan syndrome. Growth hormone neurosecretory dysfunction was diagnosed primarily based on the low amplitude and small numbers of the spontaneous bursts of growth hormone secretion during 12-hour nocturnal growth hormone sampling. Treatment with synthetic human growth hormone has markedly accelerated the growth velocity for one year and a half. This case notes the wide spectrum of short stature in Noonan syndrome and the effectiveness of treatment with human growth hormone.
authors.affiliation: Department of Internal Medicine, Koshigaya Hospital, Dokkyo University School of Medicine, Saitama, Japan.
authors.forename: K
authors.initials: K
authors.lastname: Tanaka
authors.forename: A
authors.initials: A
authors.lastname: Sato
authors.forename: T
authors.initials: T
authors.lastname: Naito
authors.forename: K
authors.initials: K
authors.lastname: Kuramochi
authors.forename: H
authors.initials: H
authors.lastname: Itabashi
authors.forename: Y
authors.initials: Y
authors.lastname: Takemura
mesh_terms: D000293:Adolescent; D001120:Arginine / Q000494:pharmacology; D002940:Circadian Rhythm; D004393:Dwarfism, Pituitary / Q000188:drug therapy / Q000209:etiology* / Q000503:physiopathology; D007987:Gonadotropin-Releasing Hormone / Q000494:pharmacology; D013006:Growth Hormone / Q000172:deficiency* / Q000378:metabolism; D013007:Growth Hormone-Releasing Hormone / Q000494:pharmacology; D006801:Humans; D008297:Male; D009634:Noonan Syndrome / Q000503:physiopathology*; D010903:Pituitary Gland, Anterior / Q000187:drug effects / Q000378:metabolism*; D011993:Recombinant Fusion Proteins / Q000627:therapeutic use; D012636:Secretory Rate / Q000187:drug effects
chemical_list: D011993:Recombinant Fusion Proteins; D007987:Gonadotropin-Releasing Hormone; D013006:Growth Hormone; D013007:Growth Hormone-Releasing Hormone; D001120:Arginine