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先天性胆道闭锁:一种始于宫内的疾病及其对治疗、诊断和发病机制的影响。

Biliary Atresia as a Disease Starting In Utero: Implications for Treatment, Diagnosis, and Pathogenesis.

机构信息

Department of Pediatrics, Division of Gastroenterology, Hepatology, and Nutrition, Baylor College of Medicine and Texas Children's Hospital, Houston, TX.

出版信息

J Pediatr Gastroenterol Nutr. 2019 Oct;69(4):396-403. doi: 10.1097/MPG.0000000000002450.

DOI:10.1097/MPG.0000000000002450
PMID:31335837
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC6942669/
Abstract

Biliary atresia (BA) is the most common reason for pediatric liver transplant. BA's varied presentation, natural history, and treatment with the Kasai portoenterostomy have been well described; however, when BA starts relative to birth has not been clearly defined. In this review, we discuss laboratory, imaging, and clinical data which suggest that most if not all forms of BA may start before birth. This early onset has implications in terms of delivering treatments earlier and identifying possible factors underlying BA's etiology.

摘要

先天性胆道闭锁(BA)是小儿肝移植最常见的原因。BA 的多种表现、自然病史以及Kasai 门腔分流术的治疗已得到充分描述;然而,BA 相对于出生的起始时间尚未明确界定。在这篇综述中,我们讨论了实验室、影像学和临床数据,这些数据表明,即使不是所有形式的 BA 都可能在出生前开始。这种早期发病提示我们更早地提供治疗,并识别 BA 病因的可能因素。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1b01/6942669/3a0b89516d8f/nihms-1534205-f0003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1b01/6942669/3146bae45303/nihms-1534205-f0001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1b01/6942669/553823bbf516/nihms-1534205-f0002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1b01/6942669/3a0b89516d8f/nihms-1534205-f0003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1b01/6942669/3146bae45303/nihms-1534205-f0001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1b01/6942669/553823bbf516/nihms-1534205-f0002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1b01/6942669/3a0b89516d8f/nihms-1534205-f0003.jpg

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本文引用的文献

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Universal screening of newborns for biliary atresia: Cost-effectiveness of alternative strategies.新生儿胆道闭锁的普遍筛查:替代策略的成本效益
J Med Screen. 2019 Sep;26(3):113-119. doi: 10.1177/0969141319832039. Epub 2019 Mar 14.
2
Quantification of Serum Matrix Metallopeptide 7 Levels May Assist in the Diagnosis and Predict the Outcome for Patients with Biliary Atresia.血清基质金属蛋白酶 7 水平的定量检测可能有助于胆道闭锁患者的诊断和预后预测。
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Recent advances in understanding biliary atresia.胆管闭锁认识方面的最新进展。
F1000Res. 2019 Feb 25;8. doi: 10.12688/f1000research.16732.1. eCollection 2019.
4
Adjuvant therapy of cytomegalovirus IgM + ve associated biliary atresia: Prima facie evidence of effect.巨细胞病毒 IgM 阳性相关胆道闭锁的辅助治疗:初步的疗效证据。
J Pediatr Surg. 2019 Sep;54(9):1941-1945. doi: 10.1016/j.jpedsurg.2018.12.014. Epub 2019 Jan 22.
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A Phase I/IIa Trial of Intravenous Immunoglobulin Following Portoenterostomy in Biliary Atresia.经 Porta 肠吻合术后静脉注射免疫球蛋白治疗胆道闭锁的 I/IIa 期临床试验。
J Pediatr Gastroenterol Nutr. 2019 Apr;68(4):495-501. doi: 10.1097/MPG.0000000000002256.
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Identification of Polycystic Kidney Disease 1 Like 1 Gene Variants in Children With Biliary Atresia Splenic Malformation Syndrome.鉴定胆汁淤积性肝外胆管闭锁伴脾畸形综合征患儿多囊肾病 1 样 1 基因变异。
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