title: [Prophylactic prenatal therapy with intravenous immunoglobulins for patients at risk for neonatal haemochromatosis].
abstract: Neonatal haemochromatosis (NH) is a connatal hepatopathy that is lethal in 32% and necessitates liver transplantation in 63% of the survivors. The classical diagnostic criteria of extrahepatic siderosis do not apply in all patients who are suspected to have NH. The hypothesis of NH as an alloimmune disease is supported by the quantitative immunohistochemical proof of C5b-9 complement complexes on the hepatocytes of liver biopsy material. This has opened a new perspective in the therapy and prophylaxis for this severe disease. Prophylactic therapy with intravenous immunoglobulins (IVIG) for mothers at risk can prevent a relevant NH in most cases.
authors.affiliation: Abteilung für Allgemeinpädiatrie und Neonatologie, Zentrum für Kinderheilkunde und Jugendmedizin, Justus Liebig Universität, Giessen, Germany. Dirk-Faas@paediat.med.uni-giessen.de
authors.forename: D
authors.initials: D
authors.lastname: Faas
authors.forename: R
authors.initials: R
authors.lastname: Axt-Fliedner
authors.forename: K-P
authors.initials: KP
authors.lastname: Zimmer
authors.forename: M
authors.initials: M
authors.lastname: Heckmann
mesh_terms: D006432:Hemochromatosis / Q000517:prevention & control*; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000008:administration & dosage*; D007155:Immunologic Factors / Q000008:administration & dosage; D007231:Infant, Newborn; D007275:Injections, Intravenous; D008297:Male; D012307:Risk Factors; D055502:Secondary Prevention / Q000379:methods; D016896:Treatment Outcome
chemical_list: D016756:Immunoglobulins, Intravenous; D007155:Immunologic Factors