Abstract
A 13 year old boy with Blackfan-Diamond anemia treated with frequent transfusions was investigated for endocrine abnormalities. Prepubertal plasma LH and FSH values, lack of sleep-related hormone rhythms of the gonadotropins, as well as prepubertal responses of LH and FSH to acute stimulation with LHRH strongly suggests that a hypothalamic-pituitary abnormality is the cause of the hypogonadotropic hypogonadism observed in this patient. As a result of impaired stimulation of the gonads plasma testosterone was prepubertal. A three-to fourfold increase of basal plasma PRL values was found without any signs of a typical sleep-dependent increase. Values obtained ranged between 21 ng/ml and 24 ng/ml (normal range 5–8 ng/ml). A normal response to TRH stimulation was found.
These results suggest that hemosiderosis may responsible for the hyperprolactinemia as a result of hypothalamic-pituitary dysfunction. Furthermore, dysfunction is demonstrated by prepubertal responses of LH and FSH to LHRH stimulation.
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Abbreviations
- LH:
-
luteinizing hormone
- FSH:
-
follicle-stimulating hormone
- PRL:
-
prolactin
- LHRH:
-
luteinizing-hormone-releasing hormone
- TRH:
-
thyrotropin releasing hormone
- PIF:
-
prolactin inhibiting factor
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Beck, W., Stubbe, P. & Tillmann, W. Endocrine studies in Blackfan-diamond anemia: Evidence for hypothalamic-pituitary dysfunction under frequent transfusion therapy. Eur J Pediatr 135, 103–105 (1980). https://doi.org/10.1007/BF00445904
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DOI: https://doi.org/10.1007/BF00445904