Abstract
The P-L-W syndrome is assumed to be cause by hypothalamic dysfunction. Patients have increased appetite,severe obesity, disturbed pubertal development and growth retardation,reduced sensitivity for pain, disturbed body temperatur regulation and muscular hypotonia. This study is performed on prepubertal boys and girls above 4 years of age with retared BA and sufficient energy intake. Diagnosis was based on clinical signs karyotype. Growth hormone therapy was suggested to be beneficial and the objectives were to study the effects of therapy on muscular mass, fat mobilisation and the stimulation of growth. The preliminary data from 6 patients are presented in the table. hGH dosage 0.1 IU/kg/d, max.4IU/d, given 7 d/w. The result is a great effect on growth, expressed as an increase in HV and H-SDS, a reduction in fat expressed in % (Futrex - Infrared refr.of.s.c.fat) and fat/muscle ratio in the thigh (CT scan).
Conclusion: hGH therapy offers a great benefical effect on growth, body weight and composition in P-L-W patients. Families and teachers also experiences a clear improvement in school performance and phychosocial adjusment.
Log in or create a free account to read this content
Gain free access to this article, as well as selected content from this journal and more on nature.com
or
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
Trygstad, O. GROWTH HORMONE TREATMENT IN PRADER-LABARTH-WILLI SYNDROME. Pediatr Res 33 (Suppl 5), S40 (1993). https://doi.org/10.1203/00006450-199305001-00221
Issue date:
DOI: https://doi.org/10.1203/00006450-199305001-00221
This article is cited by
-
Treatment with human growth hormone in patients with Prader-Labhart-Willi syndrome reduces body fat and increases muscle mass and physical performance
European Journal of Pediatrics (1998)
