Effects of plasma transfusion on hepcidin production in human congenital hypotransferrinemia.
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
Paola
P
Department of Clinical Medicine and Centre for Diagnosis and Therapy of Hemochromatosis, University of Milano-Bicocca, San Gerardo Hospital, Monza, Milan, Italy.
Coliva
Tiziana
T
Nemeth
Elizabeta
E
Mariani
Raffaella
R
Ganz
Tomas
T
Biondi
Andrea
A
Piperno
Alberto
A
D023181:Antimicrobial Cationic Peptides / Q000096:biosynthesis*; D001803:Blood Transfusion; D002648:Child; D064451:Hepcidins; D006801:Humans; D007501:Iron / Q000097:blood; D008297:Male; D008661:Metabolism, Inborn Errors / Q000097:blood* / Q000628:therapy*; D013997:Time Factors; D014168:Transferrin / Q000378:metabolism*
D023181:Antimicrobial Cationic Peptides; C578702:HAMP protein, human; D064451:Hepcidins; D014168:Transferrin; D007501:Iron