PMID: 6403832Jan 1, 1983Paper

Puberty-specific gonadotropin rhythm in girls with homozygous beta-thalassemia during continuous subcutaneous desferrioxamine infusions and a simultaneous blood transfusion regimen

Monatsschrift Kinderheilkunde : Organ der Deutschen Gesellschaft für Kinderheilkunde
W BeckW Tillmann

Abstract

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.

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