One-year metreleptin improves insulin secretion in patients with diabetes linked to genetic lipodystrophic syndromes.

Recombinant methionyl human leptin (metreleptin) therapy was shown to improve hyperglycaemia, dyslipidaemia and insulin sensitivity in patients with lipodystrophic syndromes, but its effects on insulin secretion remain controversial. We used dynamic intravenous (i.v.

Near normalization of final height with adapted doses of growth hormone in Turner's syndrome.

An adapted GH dose regimen was evaluated in 14 untreated patients with Turner's syndrome. The initial GH dose (0.7 U/kg.

[Cushing disease in children and in adolescents. Therapeutic results].

Background: Trans-sphenoidal surgery is currently the treatment of choice for Cushing's disease in children.

Patients And Methods: The results obtained in 20 consecutive patients referred to the Pediatric Endocrinology Department of hôpital Saint-Vincent-de-Paul are reported.

Results: A remission of Cushing's disease was observed in 12/16 (75%) patients in whom surgery was the first treatment.

[Primary hypothyroidism revealing pseudohypoparathyroidism without hypocalcemia and hyperphosphoremia].

Background: Type la pseudohypoparathyroidism is due to a molecular defect causing Gs protein deficiency. It is responsible for multi-hormonal resistance and skeletal abnormalities. Parathyroid hormone resistance can be subtle so that the diagnosis can be difficult in patients with atypical manifestations.

Growth hormone therapy for Turner syndrome: evidence for benefit.

Growth hormone (GH) is registered for children with Turner syndrome (TS) in several countries. Improving the final heights (FH) is certainly the most worthy goal of therapy, but evaluation of treatment effect is complicated by methodological difficulties. Several series of FH results have now been published, with estimated benefits ranging from 0-9.

Genetic homogeneity of autoimmune polyglandular disease type I.

Autoimmune polyglandular disease type I (APECED) is an autosomal recessive autoimmune disease (MIM 240300) characterized by hypoparathyroidism, primary adrenocortical failure, and chronic mucocutaneous candidiasis. The disease is highly prevalent in two isolated populations, the Finnish population and the Iranian Jewish one. Sporadic cases have been identified in many other countries, including almost all European countries.