[Puberty-specific gonadotropin rhythm in girls with homozygous beta-thalassemia during continuous subcutaneous desferrioxamine infusions and a simultaneous blood transfusion regimen].
The aim of the study was to find endocrinological parameters for the effectiveness of both, s.c. desferrioxamine therapy and a blood transfusion regimen, in 5 girls with homozygous beta-thalassemia treated in this way over 12-18 months. Physiologically, a sleep-dependent rhythm of follicle-stimulating hormone (FSH) and luteinizing hormone (LH) secretion is detectable during early to mid puberty. Girls with homozygous beta-thalassemia develop persistant hypogonadotropic hypogonadism in the course of untreated hypothalamo-pituitary hemosiderosis and do not show this rhythm. All our patients developed a sleep-dependent gonadotropin rhythm, increased estradiol concentrations as well as secondary sex characteristics, including menarche in one girl. The occurence of sleep-dependent gonadotropin rhythms in previously hypogonadotropic girls emphasizes the effectiveness of s.c. desferrioxamine therapy of hypothalamo-pituitary hemosiderosis in combination with a blood transfusion regimen.
D000293:Adolescent; D001803:Blood Transfusion; D003676:Deferoxamine / Q000008:administration & dosage*; D005260:Female; D005640:Follicle Stimulating Hormone / Q000378:metabolism*; D006801:Humans; D007263:Infusions, Parenteral; D007986:Luteinizing Hormone / Q000378:metabolism*; D011627:Puberty; D012890:Sleep; D013789:Thalassemia / Q000503:physiopathology* / Q000628:therapy
D007986:Luteinizing Hormone; D005640:Follicle Stimulating Hormone; D003676:Deferoxamine
Pubertätsspezifische Gonadotropinrhythmik bei Mädchen mit homozygoter beta-Thalassämie während kontinuierlicher subcutaner Desferrioxamin-Infusionen und gleichzeitiger Hochtransfusionsregime.