[Prader-Labhart-Willi syndrome].
12 patients (9 male, 3 female) with the Prader-Labhart-Willi-Syndrome (PLWS) were examined, and had medical treatment. Small stature varied in degree; body-height was found below the 25th percentile of normal height in 9 of the patients, 6 of them ranged along or below the 3rd percentile. Growth-hormone (GH) was measured after i.v.-Arginine-provocationtest; in 7 cases GH-peaks did not rise over 10 ng/ml, 4 of the children showed GH-peaks of less than 4 ng/ml. One of the boys was treated with GH for 5 years with good results, no secondary effect on carbohydrate metabolism was observed. In one girl we started with GH-therapy only recently. In 7 of 9 children at the age of puberty we found hypogonadotrophic hypogonadism. In 10 patients, who underwent i.v. GTT, 4 showed a prediabetic metabolic state with elevated plasma insulin levels, a fifth boy suffered from diabetes. The development of type-2-diabetes is caused by abnormal insulin-resistance, which is connected with overfeeding and obesity. Treatment of obesity is the most important problem in the PLWS. Regular hypocaloric food is not accepted by most of the children. Therefore, in cases of ineffective dietetic treatment, we recommend gastric partitioning with proximal gastro-jejunostomy, as we performed in two of our patients with good results regarding weight-loss. In two of six children examined, we found chromosomal abnormalities.
D000293:Adolescent; D001120:Arginine; D002648:Child; D005260:Female; D005951:Glucose Tolerance Test; D013006:Growth Hormone / Q000627:therapeutic use; D006801:Humans; D007006:Hypogonadism / Q000175:diagnosis; D008297:Male; D011218:Prader-Willi Syndrome / Q000175:diagnosis* / Q000188:drug therapy; D011627:Puberty
D013006:Growth Hormone; D001120:Arginine
Untersuchungen beim Prader-Labhart-Willi-Syndrom.