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新生儿血色素沉着症:去铁胺治疗失败

Neonatal hemochromatosis: failure of deferoxamine therapy.

作者信息

Jonas M M, Kaweblum Y A, Fojaco R

机构信息

Department of Pediatrics, University of Miami School of Medicine, FL 33101.

出版信息

J Pediatr Gastroenterol Nutr. 1987 Nov-Dec;6(6):984-8.

PMID:3681586
Abstract

We describe a premature neonate with severe hepatic dysfunction from birth which progressed to fatal hepatic failure at 3 months of age. The diagnosis of a familial disorder, neonatal hemochromatosis, was made, and therapy was attempted with deferoxamine. Features of iron metabolism are presented, and this infant's illness is contrasted with other liver diseases of infancy associated with hepatic iron overload.

摘要

我们描述了一名早产新生儿,自出生起即患有严重肝功能障碍,在3个月大时进展为致命性肝衰竭。诊断为一种家族性疾病——新生儿血色素沉着症,并尝试用去铁胺进行治疗。本文介绍了铁代谢的特征,并将该婴儿的疾病与其他与肝铁过载相关的婴儿肝病进行了对比。

相似文献

1
Neonatal hemochromatosis: failure of deferoxamine therapy.新生儿血色素沉着症:去铁胺治疗失败
J Pediatr Gastroenterol Nutr. 1987 Nov-Dec;6(6):984-8.
2
Type 3 hemochromatosis and beta-thalassemia trait.3型血色素沉着症和β地中海贫血特质。
Eur J Haematol. 2004 May;72(5):370-4. doi: 10.1111/j.1600-0609.2004.00230.x.
3
Long-term intraperitoneal deferoxamine for hemochromatosis.长期腹腔内注射去铁胺治疗血色素沉着症。
Am J Med. 1996 Mar;100(3):308-12. doi: 10.1016/S0002-9343(97)89489-2.
4
Is there a threshold of hepatic iron concentration that leads to cirrhosis in C282Y hemochromatosis?在C282Y型血色素沉着症中,是否存在导致肝硬化的肝铁浓度阈值?
Am J Gastroenterol. 2001 Feb;96(2):567-9. doi: 10.1111/j.1572-0241.2001.03472.x.
5
Iron homeostasis, hepatocellular injury, and fibrogenesis in hemochromatosis: the role of inflammation in a noninflammatory liver disease.铁稳态、肝细胞损伤和血色病中的肝纤维化:炎症在非炎症性肝病中的作用。
Semin Liver Dis. 2010 Aug;30(3):271-87. doi: 10.1055/s-0030-1255356. Epub 2010 Jul 21.
6
Juvenile hemochromatosis associated with B-thalassemia treated by phlebotomy and recombinant human erythropoietin.
Haematologica. 2000 Aug;85(8):865-7.
7
[Disorders of iron metabolism in liver patients].[肝病患者的铁代谢紊乱]
Internist (Berl). 1966 Jan;7(1):33-42.
8
Primary hemochromatosis: easily missed cause of cirrhosis, diabetes and heart failure.
Geriatrics. 1973 Apr;28(4):131-4.
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[Neonatal hemochromatosis. Report of 3 autopsy cases].[新生儿血色沉着症。3例尸检病例报告]
Rev Invest Clin. 1999 Mar-Apr;51(2):81-7.
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Hepatic cirrhosis and hemochromatosis in three horses.三匹马的肝硬化和血色素沉着症
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引用本文的文献

1
Recurrence of neonatal haemochromatosis in half sibs born of unaffected mothers.未受影响母亲所生半同胞中新生儿血色病的复发。
J Med Genet. 1996 Jun;33(6):444-9. doi: 10.1136/jmg.33.6.444.
2
Hepatic morphology and iron quantitation in perinatal hemochromatosis. Comparison with a large perinatal control population, including cases with chronic liver disease.围产期血色病的肝脏形态学与铁定量分析。与大型围产期对照人群的比较,包括慢性肝病病例。
Am J Pathol. 1993 Nov;143(5):1312-25.
3
Neonatal hemochromatosis. The regulation of transferrin-receptor and ferritin synthesis by iron in cultured fibroblastic-line cells.
新生儿血色沉着症。铁对培养的成纤维细胞系细胞中转铁蛋白受体和铁蛋白合成的调节作用。
Am J Pathol. 1989 Feb;134(2):439-45.