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费尔蒂综合征合并结节性再生性增生时的食管静脉曲张出血

Oesophageal variceal bleeding in Felty's syndrome associated with nodular regenerative hyperplasia.

作者信息

Blendis L M, Lovell D, Barnes C G, Ritland S, Cattan D, Vesin P

出版信息

Ann Rheum Dis. 1978 Apr;37(2):183-6. doi: 10.1136/ard.37.2.183.

DOI:10.1136/ard.37.2.183
PMID:306226
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC1001187/
Abstract

Four patients with Felty's syndrome developed massive upper gastrointestinal bleeding due to oesophageal varices. The underlying hepatic pathology in all 4 was nodular regenerative hyperplasia. This appears to be a difficult histological diagnosis to make, having been initially reported as normal on percutaneous biopsy or as fibrosis or cirrhosis on wedge biopsy. This series brings the total number of cases reported in the English literature of this association to 12, suggesting a definite symptom complex. The portal hypertension seems to be due to a combination of increased splenic blood flow and postsinusoidal resistance. The clinical importance of this syndrome is that the appropriate therapy for bleeding oesophageal varices appears to be shunt procedure such as a splenorenal shunt with splenectomy, which should be well tolerated.

摘要

4例费尔蒂综合征患者因食管静脉曲张发生大量上消化道出血。所有4例患者潜在的肝脏病理改变均为结节性再生性增生。这似乎是一种难以做出的组织学诊断,最初经皮活检报告为正常,楔形活检报告为纤维化或肝硬化。该系列病例使英文文献中报道的这种关联的病例总数达到12例,提示存在一种明确的症状复合体。门静脉高压似乎是脾血流量增加和窦后阻力增加共同作用的结果。该综合征的临床意义在于,对于出血性食管静脉曲张,合适的治疗方法似乎是分流手术,如脾肾分流加脾切除术,患者对此耐受性良好。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/bbde/1001187/3c75f2a4bc46/annrheumd00084-0079-b.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/bbde/1001187/0077f859d2f0/annrheumd00084-0079-a.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/bbde/1001187/3c75f2a4bc46/annrheumd00084-0079-b.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/bbde/1001187/0077f859d2f0/annrheumd00084-0079-a.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/bbde/1001187/3c75f2a4bc46/annrheumd00084-0079-b.jpg

相似文献

1
Oesophageal variceal bleeding in Felty's syndrome associated with nodular regenerative hyperplasia.费尔蒂综合征合并结节性再生性增生时的食管静脉曲张出血
Ann Rheum Dis. 1978 Apr;37(2):183-6. doi: 10.1136/ard.37.2.183.
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Noncirrhotic portal hypertension in Felty's syndrome.费尔蒂综合征中的非肝硬化性门静脉高压症。
Am J Dig Dis. 1977 Feb;22(2):145-8. doi: 10.1007/BF01072959.
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Porto-Sinusoidal Vascular Disease as the Cause of Portal Hypertension in Felty's Syndrome: A Case Report and Literature Review.特发性门脉高压伴脾大症(Felty's syndrome)并发窦前性门静脉高压的病因:病例报告及文献复习。
Biomed Res Int. 2020 Jul 1;2020:2618260. doi: 10.1155/2020/2618260. eCollection 2020.
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Nodular regenerative hyperplasia of the liver and esophageal varices in Felty's syndrome: a clinicopathologic case report with review of the literature.费尔蒂综合征中的肝脏结节性再生性增生和食管静脉曲张:一例临床病理病例报告并文献复习
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Esophageal varices in Felty's syndrome: A case report and review of the literature.费尔蒂综合征中的食管静脉曲张:一例病例报告及文献综述
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Liver disease in Felty's syndrome.费尔蒂综合征中的肝脏疾病。
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Nodular regenerative hyperplasia of the liver and bleeding esophageal varices in Felty's syndrome: a case report and literature review.费尔蒂综合征中的肝脏结节性再生性增生和食管静脉曲张出血:一例报告及文献综述
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Sinusoidal lymphocytosis of the liver in Felty's syndrome with a review of the liver involvement in Felty's syndrome.
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Nodular regenerative hyperplasia of the liver in Felty's syndrome.
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[Anatomo-clinical study of a case of regenerative nodular hyperplasia of the liver with Felty's syndrome and portal hypertension].
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Idiopathic Non-Cirrhotic Intrahepatic Portal Hypertension (NCIPH)-Newer Insights into Pathogenesis and Emerging Newer Treatment Options.特发性非肝硬化性肝内门静脉高压症(NCIPH)——发病机制的新见解及新出现的治疗选择
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[Inflammatory diseases with liver and joint involvement. A differential diagnostic challenge].

本文引用的文献

1
Nodular regenerative hyperplasia of the liver.肝脏结节状再生性增生
Am J Pathol. 1959 Sep-Oct;35(5):943-53.
2
Liver in Felty's syndrome.费尔蒂综合征中的肝脏
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Spleen blood flow and splanchnic haemodynamics in blood dyscrasia and other splenomegalies.血液系统疾病及其他脾肿大时的脾血流量和内脏血流动力学
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Clinical associations between arthritis and liver disease.关节炎与肝脏疾病之间的临床关联。
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Systemic lupus erythematosus and nodular regenerative hyperplasia of the liver.系统性红斑狼疮与肝脏结节性再生性增生
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"Idiopathic" portal hypertension due to occlusion of intrahepatic portal veins by organized thrombi. A study based on postmortem vinylite-injection corrosion and dissection of the intrahepatic vasculature in 4 cases.由机化血栓阻塞肝内门静脉引起的“特发性”门静脉高压症。一项基于4例尸体死后用乙烯基树脂注射腐蚀及肝内血管系统解剖的研究。
Medicine (Baltimore). 1974 Jan;53(1):77-91. doi: 10.1097/00005792-197401000-00003.
5
Cirrhosis of the liver in Felty's syndrome.
Scand J Rheumatol. 1973;2(1):29-32.
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Nodular regenerative hyperplasia of the liver in Felty's syndrome.
Q J Med. 1974 Jan;43(169):25-32.
7
Nodular, non-cirrhotic liver associated with portal hypertension in a patient with rheumatoid arthritis.类风湿关节炎患者中与门静脉高压相关的结节性、非肝硬化肝脏。
J Clin Pathol. 1974 Dec;27(12):963-6. doi: 10.1136/jcp.27.12.963.
8
Felty's syndrome. A clinical and pathological survey of 21 patients and their response to treatment.费尔蒂综合征。21例患者的临床与病理研究及其对治疗的反应
Ann Rheum Dis. 1971 Jul;30(4):359-74. doi: 10.1136/ard.30.4.359.
9
Focal nodular hyperplasia of the liver and intrahepatic hemorrhage in young women on oral contraceptives.
Ann Intern Med. 1975 Sep;83(3):301-6. doi: 10.7326/0003-4819-83-3-301.
10
Nodular regenerative hyperplasia of the liver.
Gastroenterology. 1975 Sep;69(3):746-51.