Final adult height in children with Prader-Willi syndrome with and without human growth hormone treatment.
Short stature is characteristic of children with Prader-Willi syndrome (PWS). While previous studies have demonstrated acceleration of linear height velocity with growth hormone (GH) treatment, the long-term benefit on final adult height (AH) has not been reported. The objective of this study was to compare AH attained in PWS subjects with and without GH treatment. We reviewed the records of 21 children (aged 8.3 +/- 2.7 years) with PWS and confirmed GH deficiency that attained AH after receiving human GH treatment (0.25 +/- 0.06 mg/kg/week) for a period of 7.9 +/- 1.7 years. A group of 39 non-GH-treated adults with matched initial height standard deviation score (SDS) at age 6.8 +/- 1.3 years was used as control. In the GH-treated group the mean initial height and AH-SDS was -1.9 +/- 1.7 and -0.3 +/- 1.2 respectively (P < 0.0001), whereas the mean initial and AH-SDS in the control group was -1.9 +/- 1.3 and -3.1 +/- 1 respectively (P < 0.0001). Scoliosis was seen in 43% and 39% in the GH-treated and control group respectively. Premature adrenarche (PA) was noticed in 57% of GH-treated group. Six subjects in the control group but none of the GH-treated subjects developed type 2 diabetes mellitus. Our data show that administration of GH to children with PWS restores linear growth and final AH without significant adverse effects other than PA. Further studies will be necessary to determine related morbidity and mortality in individuals with PWS that reached final AH with or without GH treatment.
Angulo
Moris A
MA
Division of Pediatrics and Medical Genetics, Department of Pediatrics, Winthrop-University Hospital, 120 Mineola Boulevard, Mineola, New York, USA. mangulo@winthrop.org
Castro-Magana
Mariano
M
Lamerson
Michele
M
Arguello
Raul
R
Accacha
Siham
S
Khan
Asjad
A
D000293:Adolescent; D000328:Adult; D001827:Body Height / Q000187:drug effects*; D016022:Case-Control Studies; D005260:Female; D019382:Human Growth Hormone / Q000008:administration & dosage*; D006801:Humans; D008297:Male; D011218:Prader-Willi Syndrome / Q000175:diagnosis / Q000188:drug therapy*
D019382:Human Growth Hormone