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马凡综合征:一例病例报告。

Marfan syndrome: a case report.

作者信息

Ganesh Rajendran, Vijayakumar Rajendran, Selvakumar Haridoss

机构信息

Department of Pedodontics, SRM Dental College, SRM University, Chennai 600078, India.

出版信息

Case Rep Dent. 2012;2012:595343. doi: 10.1155/2012/595343. Epub 2012 Dec 4.

DOI:10.1155/2012/595343
PMID:23304566
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC3529425/
Abstract

Marfan syndrome is an autosomal dominant systemic disorder of the connective tissue. Children affected by the Marfan syndrome carry a mutation in one of their two copies of the gene that encodes the connective tissue protein fibrillin-1. Marfan syndrome affects most organs and tissues, especially the skeleton, lungs, eyes, heart, and the large blood vessel that distributes blood from the heart to the rest of the body. A case report of Marfan syndrome has been reported with oral features. The dental problems of the child were treated under general anesthesia and a one-month review showed intact stainless steel crowns' restorations and no signs of secondary caries.

摘要

马凡综合征是一种常染色体显性遗传性全身结缔组织疾病。患有马凡综合征的儿童,其两个拷贝的编码结缔组织蛋白原纤维蛋白-1的基因中有一个发生了突变。马凡综合征会影响大多数器官和组织,尤其是骨骼、肺、眼睛、心脏以及从心脏向身体其他部位供血的大血管。已有一份关于马凡综合征伴口腔特征的病例报告。该患儿的牙齿问题在全身麻醉下进行了治疗,一个月后的复查显示不锈钢冠修复体完好,无继发龋迹象。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/81b4/3529425/e686b8cca9b7/CRIM.DENTISTRY2012-595343.009.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/81b4/3529425/f28a183cbb46/CRIM.DENTISTRY2012-595343.001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/81b4/3529425/e8057261c2a3/CRIM.DENTISTRY2012-595343.002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/81b4/3529425/0af9cb7de6e3/CRIM.DENTISTRY2012-595343.003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/81b4/3529425/0a96cb71980d/CRIM.DENTISTRY2012-595343.004.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/81b4/3529425/fc87cfee5493/CRIM.DENTISTRY2012-595343.005.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/81b4/3529425/c6e1991d992d/CRIM.DENTISTRY2012-595343.006.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/81b4/3529425/09b92f222663/CRIM.DENTISTRY2012-595343.007.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/81b4/3529425/694f73fc4051/CRIM.DENTISTRY2012-595343.008.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/81b4/3529425/e686b8cca9b7/CRIM.DENTISTRY2012-595343.009.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/81b4/3529425/f28a183cbb46/CRIM.DENTISTRY2012-595343.001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/81b4/3529425/e8057261c2a3/CRIM.DENTISTRY2012-595343.002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/81b4/3529425/0af9cb7de6e3/CRIM.DENTISTRY2012-595343.003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/81b4/3529425/0a96cb71980d/CRIM.DENTISTRY2012-595343.004.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/81b4/3529425/fc87cfee5493/CRIM.DENTISTRY2012-595343.005.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/81b4/3529425/c6e1991d992d/CRIM.DENTISTRY2012-595343.006.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/81b4/3529425/09b92f222663/CRIM.DENTISTRY2012-595343.007.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/81b4/3529425/694f73fc4051/CRIM.DENTISTRY2012-595343.008.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/81b4/3529425/e686b8cca9b7/CRIM.DENTISTRY2012-595343.009.jpg

相似文献

1
Marfan syndrome: a case report.马凡综合征:一例病例报告。
Case Rep Dent. 2012;2012:595343. doi: 10.1155/2012/595343. Epub 2012 Dec 4.
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The molecular basis of Marfan syndrome.马凡综合征的分子基础。
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A Novel Fibrillin-1 Gene Mutation Leading to Marfan Syndrome in a Korean Girl.导致一名韩国女孩患马凡综合征的一种新型原纤蛋白-1基因突变
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[Marfan syndrome in childhood and adolescence].[儿童及青少年期马方综合征]
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Marfan syndrome: what internists and pediatric or adult cardiologists need to know.马凡综合征:内科医生以及儿科或成人心脏病专家需要了解的内容。
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Identification of defects in the fibrillin gene and protein in individuals with the Marfan syndrome and related disorders.马凡综合征及相关疾病患者中纤连蛋白基因和蛋白缺陷的鉴定。
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Autosomal dominant Marfan-like connective-tissue disorder with aortic dilation and skeletal anomalies not linked to the fibrillin genes.常染色体显性遗传性马方综合征样结缔组织病,伴有主动脉扩张和骨骼异常,与原纤维蛋白基因无关。
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Fibrillin-1 mutations in Marfan syndrome and other type-1 fibrillinopathies.马凡综合征及其他1型原纤维蛋白病中的原纤维蛋白-1突变
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引用本文的文献

1
Dental findings in marfan syndrome: a case report.马凡综合征的口腔检查结果:一例病例报告。
J Istanb Univ Fac Dent. 2017 Apr 3;51(2):61-67. doi: 10.17096/jiufd.78944. eCollection 2017.

本文引用的文献

1
Diagnosis and management of Marfan syndrome.马凡综合征的诊断与管理
Future Cardiol. 2008 Jan;4(1):85-96. doi: 10.2217/14796678.4.1.85.
2
Novel dental anomalies associated with congenital contractural arachnodactyly: a case report.与先天性挛缩性蜘蛛指(趾)症相关的新型牙齿异常:一例报告。
Pediatr Dent. 2003 Sep-Oct;25(5):501-4.
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Severe periodontitis in Marfan's syndrome: a case report.马凡综合征中的重度牙周炎:一例报告
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Therapeutic management of patients with Marfan syndrome: focus on cardiovascular involvement.马凡综合征患者的治疗管理:关注心血管受累情况。
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Systemic management of Marfan's syndrome during dental treatment: a case report.
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Aortic root dilatation and mitral valve prolapse in Marfan's syndrome: an ECHOCARDIOgraphic study.马凡综合征中的主动脉根部扩张和二尖瓣脱垂:一项超声心动图研究。
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