title: Studies of very severe short stature with severe GH deficiency: from the data registered with the foundation for growth science.
abstract: The ratio, clinical characteristics, and therapeutic efficacy of hGH treatment in patients with severe short stature (HtSDS below -4SD) with severe GHD (all peak GH values to provocation tests: below 2 mug/L) were studied. From March 1986 to January 1998, 23,110 patients with idiopathic GH deficiency (IGHD) were registered with the Foundation for Growth Science, Japan. These subjects were divided into 5 groups as follows: Group 1 (G1), all subjects; Group 2 (G2), at least one GH peak to provocative test > or = 5 microg/L; Group 3 (G3), 2 microg/L < or = GH peak<5 microg/L; Group 4 (G4), all GH peaks<2 microg/L and HtSDS>-4; Group 5 (G5), all GH peaks<2 microg/L and HtSDS< or = -4. The ratio of G5 was 139 patients (0.6%) out of 23,110 patients with IGHD. In G5, there were no significant differences in birth weight, birth length, gestational age and parental height between G2, G3 and G4. However, asphyxia at delivery was more frequent in G5 and G4 than G2 and G3. Chronological age (CA), bone age (BA) and BA/CA ratio at registration were significantly lower in G5 than G2, G3 and G4. Further, the IGF-I SD score in G5 was significantly lower than those in G2 and G3. After hGH treatment, the final height and final height SDS in G5 remained the lowest, while the DeltaHtSDS value in G5 was the greatest among G2 to G5 groups. In conclusion, the ratio of severe short stature with severe GH deficiency (G5) is only 0.6% of all IGHD cases. Growth failure in G5 seems to occur after birth, and its etiology in G5 seems to be different from that of patients with other forms of IGHD. Early diagnosis and hGH treatment are needed to attain better final height.
authors.affiliation: Hanew Endocrine Clinic, Sendai, Japan.
authors.forename: Kunihiko
authors.initials: K
authors.lastname: Hanew
authors.forename: Katsuhiko
authors.initials: K
authors.lastname: Tachibana
authors.forename: Susumu
authors.initials: S
authors.lastname: Yokoya
authors.forename: Kenji
authors.initials: K
authors.lastname: Fujieda
authors.forename: Toshiaki
authors.initials: T
authors.lastname: Tanaka
authors.forename: Yutaka
authors.initials: Y
authors.lastname: Igarashi
authors.forename: Akira
authors.initials: A
authors.lastname: Shimatsu
authors.forename: Hiroyuki
authors.initials: H
authors.lastname: Tanaka
authors.forename: Takakuni
authors.initials: T
authors.lastname: Tanizawa
authors.forename: Akira
authors.initials: A
authors.lastname: Teramoto
authors.forename: Yoshikazu
authors.initials: Y
authors.lastname: Nishi
authors.forename: Yukihiro
authors.initials: Y
authors.lastname: Hasegawa
authors.forename: Naomi
authors.initials: N
authors.lastname: Hizuka
authors.forename: Takeki
authors.initials: T
authors.lastname: Hirano
authors.forename: Keinosuke
authors.initials: K
authors.lastname: Fujita
mesh_terms: D000365:Age Determination by Skeleton; D001238:Asphyxia Neonatorum / Q000453:epidemiology; D001724:Birth Weight; D001827:Body Height / Q000187:drug effects; D002648:Child; D005260:Female; D005865:Gestational Age; D006130:Growth Disorders / Q000097:blood / Q000473:pathology* / Q000503:physiopathology*; D019382:Human Growth Hormone / Q000172:deficiency* / Q000627:therapeutic use*; D006801:Humans; D015994:Incidence; D007231:Infant, Newborn; D007334:Insulin-Like Growth Factor I / Q000378:metabolism; D008297:Male; D012042:Registries; D012720:Severity of Illness Index
chemical_list: D019382:Human Growth Hormone; D007334:Insulin-Like Growth Factor I