[Intensification of insulin substitution in adolescents and children with type I diabetes mellitus]. / Intensivierung der Insulinsubstitution bei Jugendlichen und Kindern mit Typ-I-Diabetes mellitus.

In order to approach the therapeutic goal of normoglycemia in diabetic children and adolescents, new forms of insulin substitution have been developed and subsequently tested as "intensified conventional insulin therapy" or "multiple daily insulin injections". Typical for this therapy are unmodified insulin injections before each main meal, administration of basal delayed insulin at night and if necessary also during the day, regular daily blood sugar analyses as an aid to decisions regarding therapy, and variation of the unmodified doses according to certain general or individual rules. The article reviews the principles of this therapy, indications for it and the method employed, as well as the authors' own results and those published in the literature. It concludes with a discussion of complications in therapy, of controversial aspects of practical management and of the long-term perspectives.

[Prader-Labhart-Willi syndrome]. / Untersuchungen beim Prader-Labhart-Willi-Syndrom.

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.