GROWTH HORMONE TREATMENT IN PRADER-LABARTH-WILLI SYNDROME

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).