Clinical evaluation of recombinant human growth hormone in Noonan syndrome.
The objective of this study was to investigate the effect of administration of recombinant human growth hormone (hGH) in patients with Noonan syndrome. hGH was administered (0.5 IU/kg/week) to 15 patients with Noonan syndrome over a 2 year period. Average patient age prior to therapy was 7.5 +/- 2.5 (mean +/- SD) yr, the height SD score was -2.8 +/- 0.7, and the pretreatment height velocity and bone age were 4.8 +/- 1.0 cm/yr and 5.8 +/- 2.1 yr, respectively. The height velocity in the year prior to treatment, and 0-12 and 12-24 months after commencing treatment was 4.8 +/- 1.0 cm/yr, 7.0 +/- 1.2 cm/yr, and 5.5 +/- 0.6 cm/yr, respectively. The height velocity in the first year of treatment was significantly greater (P = 0.0001, n = 14) than the pretreatment value, but there was no significant difference in the second year. The height SD scores at the commencement of treatment, and after 12 and 24 months of treatment were -2.8 +/- 0.7, -2.4 +/- 0.7, and -2.2 +/- 0.5, respectively. Bone age advanced by 1.1 +/- 0.5 yr in the 12 months after commencing treatment. We conclude that the use of hGH may be beneficial in the treatment of Noonan syndrome, although further research is required.
Department of Pediatrics, Nagoya University, School of Medicine, Nagoya, Japan.
Masamichi
M
Ogawa
Naoki
N
Moriya
Hiroyuki
H
Ikeda
Ayako
A
Tanae
Toshiaki
T
Tanaka
Kenji
K
Ohyama
Osamu
O
Mori
Takeshi
T
Yazawa
Keinosuke
K
Fujita
Yoshiki
Y
Seino
Toshihide
T
Kubo
Hiroyuki
H
Tanaka
Yoshikazu
Y
Nishi
Masaaki
M
Yoshimoto
D000365:Age Determination by Skeleton; D001827:Body Height / Q000187:drug effects; D002648:Child; D002675:Child, Preschool; D005260:Female; D006128:Growth / Q000187:drug effects; D019382:Human Growth Hormone / Q000009:adverse effects / Q000627:therapeutic use*; D006801:Humans; D008297:Male; D009634:Noonan Syndrome / Q000000981:diagnostic imaging / Q000188:drug therapy* / Q000503:physiopathology; D011994:Recombinant Proteins / Q000009:adverse effects / Q000627:therapeutic use; D016896:Treatment Outcome
D011994:Recombinant Proteins; D019382:Human Growth Hormone