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本文引用的文献

1
Intestinal lymphangiectasia and reversible high liver stiffness.肠淋巴管扩张症和可逆性肝硬度增高。
Hepatology. 2014 Aug;60(2):759-61. doi: 10.1002/hep.27025. Epub 2014 Jun 18.
2
The architecture of diagnostic research: from bench to bedside--research guidelines using liver stiffness as an example.诊断研究的架构:从基础到临床——以肝硬度检测为例的研究指南。
Hepatology. 2014 Jul;60(1):408-18. doi: 10.1002/hep.26948.
3
Evidence for medium chain triglycerides in the treatment of primary intestinal lymphangiectasia.中链甘油三酯治疗原发性肠淋巴管扩张症的证据。
Eur J Pediatr Surg. 2009 Aug;19(4):241-5. doi: 10.1055/s-0029-1216389. Epub 2009 May 15.
4
Primary intestinal lymphangiectasia (Waldmann's disease).原发性肠淋巴管扩张症(瓦尔德曼病)。
Orphanet J Rare Dis. 2008 Feb 22;3:5. doi: 10.1186/1750-1172-3-5.
5
[Congenital hepatic fibrosis, multiple renal cysts and primary intestinal lymphangiectasia (author's transl)].先天性肝纤维化、多发性肾囊肿和原发性肠淋巴管扩张症(作者译)
Gastroenterol Clin Biol. 1982 Apr;6(4):326-32.

原发性肠淋巴管扩张症中的肝纤维化:一个被低估的话题。

Liver fibrosis in primary intestinal lymphangiectasia: An undervalued topic.

作者信息

Licinio Raffaele, Principi Mariabeatrice, Ierardi Enzo, Di Leo Alfredo

机构信息

Raffaele Licinio, Mariabeatrice Principi, Enzo Ierardi, Alfredo Di Leo, Gastroenterology Unit, Department of Emergency and Organ Transplantation, University of Bari, Cesare, 70124 Bari, Italy.

出版信息

World J Hepatol. 2014 Sep 27;6(9):685-7. doi: 10.4254/wjh.v6.i9.685.

DOI:10.4254/wjh.v6.i9.685
PMID:25276285
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC4179148/
Abstract

The relationship between primary intestinal lymphangiectasia (PIL) and liver fibrosis is an emerging topic with many obscure aspects due to the rarity of the disorder. A recent paper reported that a six-month low-fat diet improved liver fibrosis. We report the case of a 17-year-old girl affected by PIL whose hepatic fibrosis progressively worsened within one year, despite dietetic support. This and the previous case report describe extraordinary events, which do not allow clear-cut clinical aspects to be established. Nevertheless, both cases suggest that in patients with PIL, it is necessary to closely monitor liver morphology with in-depth investigations including not only ultrasonography, but also elastography.

摘要

由于原发性肠淋巴管扩张症(PIL)较为罕见,其与肝纤维化之间的关系是一个新出现且诸多方面尚不明朗的课题。最近一篇论文报道,为期六个月的低脂饮食可改善肝纤维化。我们报告一例17岁患PIL的女孩,尽管给予饮食支持,但其肝纤维化在一年内仍逐渐加重。本病例及之前的病例报告描述了一些特殊情况,无法明确确立清晰的临床特征。然而,两例病例均提示,对于PIL患者,有必要通过包括超声检查及弹性成像等深入检查密切监测肝脏形态。