title: Effects of plasma transfusion on hepcidin production in human congenital hypotransferrinemia.
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.
authors.affiliation: Department of Clinical Medicine and Centre for Diagnosis and Therapy of Hemochromatosis, University of Milano-Bicocca, San Gerardo Hospital, Monza, Milan, Italy.
authors.forename: Paola
authors.initials: P
authors.lastname: Trombini
authors.forename: Tiziana
authors.initials: T
authors.lastname: Coliva
authors.forename: Elizabeta
authors.initials: E
authors.lastname: Nemeth
authors.forename: Raffaella
authors.initials: R
authors.lastname: Mariani
authors.forename: Tomas
authors.initials: T
authors.lastname: Ganz
authors.forename: Andrea
authors.initials: A
authors.lastname: Biondi
authors.forename: Alberto
authors.initials: A
authors.lastname: Piperno
mesh_terms: 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*
chemical_list: D023181:Antimicrobial Cationic Peptides; C578702:HAMP protein, human; D064451:Hepcidins; D014168:Transferrin; D007501:Iron