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一种罕见关联疾病的挑战性治疗决策：家族性Turcot综合征合并瘘管性克罗恩病的病例报告

A Challenging Treatment Decision for a Rare Association: Case Report of Familial Turcot Syndrome With Fistulizing Crohn's Disease.

作者信息

Corbera-Hincapie Montserrat, Beasley Genie L

机构信息

University of Florida Health, Gainesville, FL, United States.

出版信息

Front Pediatr. 2018 Apr 4;6:83. doi: 10.3389/fped.2018.00083. eCollection 2018.

DOI:10.3389/fped.2018.00083
PMID:29670872
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC5893786/
Abstract

Turcot syndrome and fistulizing Crohn's disease (CD) are two disease entities that are not usually associated with one another, particularly given the rarity of the former. This is a case of a pediatric patient with fistulizing CD treated with biologic therapy, who was later found to have Turcot syndrome. Management of this rare combination of diseases can present several challenges, as surgical options may be limited and chronic immunosuppression to treat CD may lead to accelerated progression of malignancy in Turcot syndrome. This unique case highlights the importance of weighing the risks and benefits involved in treating two disease entities that impact one another.

摘要

Turcot综合征与瘘管性克罗恩病（CD）是两种通常不相关的疾病实体，尤其是考虑到前者的罕见性。这是一例接受生物治疗的瘘管性CD儿科患者，后来被发现患有Turcot综合征。这种罕见的疾病组合的管理可能会带来一些挑战，因为手术选择可能有限，并且用于治疗CD的慢性免疫抑制可能会导致Turcot综合征中恶性肿瘤的加速进展。这个独特的病例凸显了权衡治疗相互影响的两种疾病实体所涉及的风险和益处的重要性。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c2dd/5893786/c43b868aa291/fped-06-00083-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c2dd/5893786/d099efc90332/fped-06-00083-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c2dd/5893786/2f723b440d99/fped-06-00083-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c2dd/5893786/c43b868aa291/fped-06-00083-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c2dd/5893786/d099efc90332/fped-06-00083-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c2dd/5893786/2f723b440d99/fped-06-00083-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c2dd/5893786/c43b868aa291/fped-06-00083-g003.jpg

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J Pediatr Gastroenterol Nutr. 2017 May;64(5):818-835. doi: 10.1097/MPG.0000000000001562.
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Inflammatory bowel disease and cancer: The role of inflammation, immunosuppression, and cancer treatment.炎症性肠病与癌症：炎症、免疫抑制及癌症治疗的作用
World J Gastroenterol. 2016 May 28;22(20):4794-801. doi: 10.3748/wjg.v22.i20.4794.
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加速性癌前息肉病与第二原发性结肠癌：免疫抑制、放疗及全身化疗药物之罪魁祸首
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Fistulizing Crohn's disease: Diagnosis and management.瘘管性克罗恩病：诊断与管理。
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Consensus guidelines of ECCO/ESPGHAN on the medical management of pediatric Crohn's disease.欧洲克罗恩病和结肠炎组织/欧洲儿科胃肠病、肝病和营养学会关于儿童克罗恩病药物治疗的共识指南。
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