Neonatal hemochromatosis

被引:71
作者
Knisely, AS
Mieli-Vergani, G
Whitington, PF
机构
[1] Childrens Mem Hosp, Feinberg Sch Med Northwestern Univ, Dept Pediat, Chicago, IL 60615 USA
[2] Kings Coll Hosp London, Inst Liver Studies, London SE5 8RX, England
[3] Kings Coll Hosp London, Dept Child Hlth, London, England
关键词
D O I
10.1016/S0889-8553(03)00050-5
中图分类号
R57 [消化系及腹部疾病];
学科分类号
摘要
Neonatal hemochromatosis (NH) is a rare gestational condition in which iron accumulates in the liver and extrahepatic sites of the fetus in a distribution similar to that seen in hereditary HFE-associated hemochromatosis [1,2]. Liver damage is, extensive and the dominant clinical feature. Many cases result in late fetal loss, and without vigorous support, live-born children survive only hours to days. NH is the most frequently recognized cause of liver failure in neonates. It recurs within sibships at a rate higher than that predicted for simple Mendelian autosomal-recessive inheritance. Treatment is less than adequate.
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页码:877 / +
页数:15
相关论文
共 70 条
[1]  
ALLEN K, 1998, FETAL NEONATAL PHYSL, P1530
[2]  
ALLEN KJ, 1996, J PEDIAT GASTROENTER, V23, P352
[3]   RENAL PROXIMAL TUBULAR DYSGENESIS ASSOCIATED WITH SEVERE NEONATAL HEMOSIDEROTIC LIVER-DISEASE [J].
BALE, PM ;
KAN, AE ;
DORNEY, SFA .
PEDIATRIC PATHOLOGY, 1994, 14 (03) :479-489
[4]   EVIDENCE FOR A LOW K-M TRANSPORTER FOR NON-TRANSFERRIN-BOUND IRON IN ISOLATED RAT HEPATOCYTES [J].
BARISANI, D ;
BERG, CL ;
WESSLINGRESNICK, M ;
GOLLAN, JL .
AMERICAN JOURNAL OF PHYSIOLOGY-GASTROINTESTINAL AND LIVER PHYSIOLOGY, 1995, 269 (04) :G570-G576
[5]  
BATEY RG, 1977, GASTROENTEROLOGY, V72, P255
[6]   EFFECT OF MASSIVE TRANSPLACENTAL IRON LOADING [J].
BELILES, RP ;
PALMER, AK .
TOXICOLOGY, 1975, 5 (02) :147-158
[7]  
BERNSTEIN SE, 1987, J LAB CLIN MED, V110, P690
[8]   THE APPEARANCE OF TRANSFERRIN RECEPTORS ON CULTURED HUMAN CYTOTROPHOBLAST AND INVITRO-FORMED SYNCYTIOTROPHOBLAST [J].
BIERINGS, MB ;
ADRIAANSEN, HJ ;
VANDIJK, JP .
PLACENTA, 1988, 9 (04) :387-396
[9]   TRANSFERRIN RECEPTOR AFFINITY AND IRON TRANSPORT IN THE HUMAN-PLACENTA [J].
BROWN, PJ ;
MOLLOY, CM ;
JOHNSON, PM .
PLACENTA, 1982, 3 (01) :21-27
[10]   APICAL AND BASOLATERAL TRANSFERRIN RECEPTORS IN POLARIZED BEWO CELLS RECYCLE THROUGH SEPARATE ENDOSOMES [J].
CERNEUS, DP ;
VANDERENDE, A .
JOURNAL OF CELL BIOLOGY, 1991, 114 (06) :1149-1158