• 文献检索
  • 文档翻译
  • 深度研究
  • 学术资讯
  • Suppr Zotero 插件Zotero 插件
  • 邀请有礼
  • 套餐&价格
  • 历史记录
应用&插件
Suppr Zotero 插件Zotero 插件浏览器插件Mac 客户端Windows 客户端微信小程序
定价
高级版会员购买积分包购买API积分包
服务
文献检索文档翻译深度研究API 文档MCP 服务
关于我们
关于 Suppr公司介绍联系我们用户协议隐私条款
关注我们

Suppr 超能文献

核心技术专利:CN118964589B侵权必究
粤ICP备2023148730 号-1Suppr @ 2026

文献检索

告别复杂PubMed语法,用中文像聊天一样搜索,搜遍4000万医学文献。AI智能推荐,让科研检索更轻松。

立即免费搜索

文件翻译

保留排版,准确专业,支持PDF/Word/PPT等文件格式,支持 12+语言互译。

免费翻译文档

深度研究

AI帮你快速写综述,25分钟生成高质量综述,智能提取关键信息,辅助科研写作。

立即免费体验

一个中国家庭中具有一些不寻常特征的杜宾-约翰逊综合征。

Dublin-Johnson syndrome with some unusual features in a Chinese family.

作者信息

Lo N S, Chan C W, Hutchison J H

出版信息

Arch Dis Child. 1979 Jul;54(7):529-33. doi: 10.1136/adc.54.7.529.

DOI:10.1136/adc.54.7.529
PMID:485195
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC1545475/
Abstract

Three cases of chronic nonhaemolytic jaundice with conjugated bilirubin in the serum are described in a Chinese family. Bromsulphthalein excretion tests gave results typical of the Dubin-Johnson syndrome. Liver histology in the proband showed cytoplasmic pigment of the lipofuscinmelanin variety, and intravenous cholecystography failed to show visualisation of the gallbladder. Unusual findings included onset during the neonatal period in the proband and the presence of some iron pigment in the hepatic cells with a little canalicular cholestasis. It is suggested that the infant may have had a concomitant nonspecific hepatitis. These cases are regarded as belonging to a disease group in which the Dubin-Johnson syndrome is at one end of a spectrum. The mode of inheritance is discussed.

摘要

在中国一个家族中描述了3例血清中结合胆红素升高的慢性非溶血性黄疸病例。磺溴酞钠排泄试验结果符合杜宾-约翰逊综合征的典型表现。先证者的肝脏组织学检查显示有脂褐素-黑色素样的细胞质色素,静脉胆囊造影未能显示胆囊显影。异常表现包括先证者在新生儿期发病,肝细胞内存在一些铁色素并有轻度胆小管胆汁淤积。提示该婴儿可能同时患有非特异性肝炎。这些病例被认为属于一个疾病组,其中杜宾-约翰逊综合征处于该疾病谱的一端。文中还讨论了遗传方式。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ed46/1545475/4aac61e612a7/archdisch00791-0046-a.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ed46/1545475/4aac61e612a7/archdisch00791-0046-a.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ed46/1545475/4aac61e612a7/archdisch00791-0046-a.jpg

相似文献

1
Dublin-Johnson syndrome with some unusual features in a Chinese family.一个中国家庭中具有一些不寻常特征的杜宾-约翰逊综合征。
Arch Dis Child. 1979 Jul;54(7):529-33. doi: 10.1136/adc.54.7.529.
2
Dubin-Johnson syndrome presenting with neonatal cholestasis.以新生儿胆汁淤积为表现的杜宾-约翰逊综合征。
Arch Dis Child. 1990 Aug;65(8):898-9. doi: 10.1136/adc.65.8.898.
3
Dubin-Johnson syndrome.杜宾-约翰逊综合征
J Coll Physicians Surg Pak. 2008 Mar;18(3):188-9.
4
[Familial benign conjugated hyperbilirubinemia with pigment deposition in the liver (Dubin-Johnson syndrome). Report of 4 cases in siblings].
Z Gesamte Inn Med. 1971 Sep 15;26(18):596-9.
5
A case of the Dubin-Johnson syndrome complicated by acute hepatitis.一例合并急性肝炎的杜宾-约翰逊综合征病例。
Gut. 1970 Oct;11(10):817-21. doi: 10.1136/gut.11.10.817.
6
Dubin-Johnson syndrome and intrahepatic cholestasis of pregnancy in a Sri Lankan family: a case report.斯里兰卡一个家族中的杜宾-约翰逊综合征与妊娠期肝内胆汁淤积症:一例报告
BMC Res Notes. 2017 Sep 18;10(1):487. doi: 10.1186/s13104-017-2811-6.
7
Neonatal cholestasis in two siblings: a variant of Dubin-Johnson syndrome?
J Paediatr Child Health. 1995 Dec;31(6):557-60. doi: 10.1111/j.1440-1754.1995.tb00884.x.
8
Clinical, Pathologic, and Genetic Features of Neonatal Dubin-Johnson Syndrome: A Multicenter Study in Japan.新生儿 Dubin-Johnson 综合征的临床、病理和遗传特征:日本多中心研究。
J Pediatr. 2018 May;196:161-167.e1. doi: 10.1016/j.jpeds.2017.12.058. Epub 2018 Feb 28.
9
Dubin-Johnson syndrome in a neonate.
Eur J Pediatr. 1979;132(4):299-301. doi: 10.1007/BF00496853.
10
Neonatal Dubin-Johnson syndrome with severe cholestasis: effective phenobarbital therapy.新生儿杜宾-约翰逊综合征伴严重胆汁淤积:苯巴比妥治疗有效
Acta Paediatr Scand. 1991 Mar;80(3):381-5. doi: 10.1111/j.1651-2227.1991.tb11867.x.

引用本文的文献

1
Case Report: A case of Dubin-Johnson syndrome in a newborn.病例报告:一例新生儿杜宾-约翰逊综合征病例。
Front Pediatr. 2024 Jul 19;12:1417649. doi: 10.3389/fped.2024.1417649. eCollection 2024.

本文引用的文献

1
Familial chronic idiopathic jaundice (Dubin-Sprinz disease), with a note on bromsulphalein metabolism in this disease.家族性慢性特发性黄疸(杜宾-斯普林兹病),附该病中溴磺酞钠代谢的说明
Am J Med. 1960 Jan;28:42-50. doi: 10.1016/0002-9343(60)90221-7.
2
Studies of chronic familial non-hemolytic jaundice with conjugated bilirubin in the serum with and without an unidentified pigment in the liver cells.关于慢性家族性非溶血性黄疸的研究,该病症血清中存在结合胆红素,肝细胞中有无一种不明色素。
Am J Med. 1961 Oct;31:510-8. doi: 10.1016/0002-9343(61)90135-8.
3
Studies of chronic idiopathic jaundice (Dubin-Johnson syndrome). I. Demonstration of hepatic excretory defect.
慢性特发性黄疸(杜宾-约翰逊综合征)的研究。I. 肝排泄缺陷的证明。
Gastroenterology. 1963 Feb;44:101-11.
4
Chronic idiopathic jaundice. A study of two afflicted families.慢性特发性黄疸。对两个患病家族的研究。
Am J Med. 1960 Jan;28:32-41. doi: 10.1016/0002-9343(60)90220-5.
5
Biliary transport and hepatic storage of sulfobromophthalein sodium in the unanesthetized dog, in normal man, and in patients with hepatic disease.未麻醉犬、正常人及肝病患者中磺溴酞钠的胆汁转运和肝脏储存
J Clin Invest. 1960 Jul;39(7):1131-44. doi: 10.1172/JCI104128.
6
Familial nonhemolytic jaundice with conjugated bilirubin in the serum; a case study.血清中结合胆红素升高的家族性非溶血性黄疸;病例报告
N Engl J Med. 1959 Jun 25;260(26):1315-8. doi: 10.1056/NEJM195906252602604.
7
Familial DubinJohnson syndrome.家族性杜宾-约翰逊综合征
Gastroenterology. 1958 Oct;35(4):387-9.
8
Chronic idiopathic jaundice; a review of fifty cases.慢性特发性黄疸;50例病例回顾
Am J Med. 1958 Feb;24(2):268-92. doi: 10.1016/0002-9343(58)90315-2.
9
Chronic idiopathic jaundice; two cases occurring in siblings, with histochemical studies.慢性特发性黄疸;两例发生于兄弟姐妹,并进行了组织化学研究。
Am J Med. 1956 Jul;21(1):138-42. doi: 10.1016/0002-9343(56)90030-4.
10
Persistent non-hemolytic hyperbilirubinemia associated with lipochrome-like pigment in liver cells: report of four cases.持续性非溶血性高胆红素血症伴肝细胞内脂褐素样色素沉着:4例报告
Ann Intern Med. 1954 Nov;41(5):952-62. doi: 10.7326/0003-4819-41-5-952.