Hepcidin is the key regulator of systemic iron homeostasis. We describe the modulation of hepcidin production induced by plasma transfusions in a patient with congenital hypotransferrinemia that offers a unique model in which to study the mechanism of hepcidin regulation by iron and erythropoiesis. Urinary hepcidin increased from zero at baseline, when hemoglobin and serum transferrin was low, to a maximum of 98 ng/mg creatinine on day 60, and subsequently decreased. Time-course of urinary hepcidin and serum transferrin concentration suggests that hepcidin production is regulated by the combination of marrow iron requirements and iron supply by transferrin.

Trombini, P., Coliva, T., Nemeth, E., Mariani, R., Ganz, T., Biondi, A., et al. (2007). Effects of plasma transfusion on hepcidin production in human congenital hypotransferrinemia. HAEMATOLOGICA, 92(10), 1407-1410 [10.3324/haematol.11377].

Effects of plasma transfusion on hepcidin production in human congenital hypotransferrinemia

COLIVA, TIZIANA ANGELA;MARIANI, RAFFAELLA;BIONDI, ANDREA;PIPERNO, ALBERTO
2007

Abstract

Hepcidin is the key regulator of systemic iron homeostasis. We describe the modulation of hepcidin production induced by plasma transfusions in a patient with congenital hypotransferrinemia that offers a unique model in which to study the mechanism of hepcidin regulation by iron and erythropoiesis. Urinary hepcidin increased from zero at baseline, when hemoglobin and serum transferrin was low, to a maximum of 98 ng/mg creatinine on day 60, and subsequently decreased. Time-course of urinary hepcidin and serum transferrin concentration suggests that hepcidin production is regulated by the combination of marrow iron requirements and iron supply by transferrin.
Si
Articolo in rivista - Articolo scientifico
Scientifica
hypotransferrinemia, hepcidin, transferrin saturation, transfusion, erytrhopoiesis
English
1407
1410
4
Trombini, P., Coliva, T., Nemeth, E., Mariani, R., Ganz, T., Biondi, A., et al. (2007). Effects of plasma transfusion on hepcidin production in human congenital hypotransferrinemia. HAEMATOLOGICA, 92(10), 1407-1410 [10.3324/haematol.11377].
Trombini, P; Coliva, T; Nemeth, E; Mariani, R; Ganz, T; Biondi, A; Piperno, A
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Utilizza questo identificativo per citare o creare un link a questo documento: http://hdl.handle.net/10281/4082
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