[New insights into perinatal hemochromatosis].
Perinatal hemochromatosis (PH) includes neonatal acute liver failure (ALF) with cirrhosis and extrahepatic iron overload sparing the reticuloendothelial system. This is the main cause of neonatal ALF. Prognosis is very poor, usually with neonatal death or neonatal orthotopic liver transplantation occurring in more than 70%. The recurrence rate is more than 90%. Diagnosis is hard to make and is proved after exclusion of other neonatal ALF causes. A recent physiopathological hypothesis proposed HP as a maternofetal alloimmune disease against the fetal liver. A maternal antibody may activate the terminal complement cascade, responsible for the membrane attack complex directed against fetal hepatocytes. Maternal prenatal treatment after a pregnancy complicated by PH modifies the course and the prognosis of this disease. In France, approval of prenatal IVIG treatment is required after analysis of clinical and pathological data by a national multidisciplinary committee.
Service d'hépatologie pédiatrique et maladies métaboliques, CHU de Toulouse, 31000 Toulouse, France. jbaruteau@yahoo.fr
J
J
Baruteau
S
S
Heissat
S
S
Collardeau-Frachon
D
D
Debray
P
P
Broué
V
V
Guigonis
D006432:Hemochromatosis / Q000175:diagnosis / Q000503:physiopathology / Q000628:therapy; D006801:Humans; D007231:Infant, Newborn