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马凡氏综合征

Marfan Syndrome.

作者信息

Sivasankari T, Mathew Philips, Austin Ravi David, Devi Sakthi

机构信息

Department of Oral Medicine and Radiology, Indira Gandhi Institute of Dental Sciences, Sri Balaji Vidyapeeth University, Puducherry, India.

Department of Oral Medicine and Radiology, Government Dental College, Kottayam, Kerala, India.

出版信息

J Pharm Bioallied Sci. 2017 Jan-Mar;9(1):73-77. doi: 10.4103/jpbs.JPBS_326_16.

DOI:10.4103/jpbs.JPBS_326_16
PMID:28584496
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC5450473/
Abstract

Marfan syndrome (MFS) is the autosomal dominant-inherited multisystem connective-tissue disorder, with a reported incidence of 1 in 10,000 individuals and equal distribution in both genders. The main clinical manifestation of this disorder consists of an exaggerated length of the upper and lower limbs, hyperlaxity, scoliosis, alterations in the cardiovascular and pulmonary systems, and atypical bone overgrowth. Orofacial manifestations such as high-arched palate, hypodontia, long narrow teeth, bifid uvula, mandibular prognathism, and temporomandibular disorders are also common. Early diagnosis of MFS is essential to prevent the cardiovascular complications and treatment of orofacial manifestations, thus to increase the quality of life of the patient.

摘要

马凡综合征（MFS）是一种常染色体显性遗传的多系统结缔组织疾病，据报道发病率为万分之一，男女发病率相等。该疾病的主要临床表现包括上肢和下肢过长、关节过度松弛、脊柱侧弯、心血管和肺部系统改变以及非典型骨过度生长。口面部表现如高拱腭、牙列缺损、长而窄的牙齿、悬雍垂裂、下颌前突和颞下颌关节紊乱也很常见。马凡综合征的早期诊断对于预防心血管并发症和治疗口面部表现至关重要，从而提高患者的生活质量。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0ef2/5450473/09ace6fa664f/JPBS-9-73-g007.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0ef2/5450473/25c92410c03b/JPBS-9-73-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0ef2/5450473/2197de085ce1/JPBS-9-73-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0ef2/5450473/c02ca839ed18/JPBS-9-73-g004.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0ef2/5450473/bb7b59089d25/JPBS-9-73-g005.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0ef2/5450473/ff60d42882ff/JPBS-9-73-g006.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0ef2/5450473/09ace6fa664f/JPBS-9-73-g007.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0ef2/5450473/25c92410c03b/JPBS-9-73-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0ef2/5450473/2197de085ce1/JPBS-9-73-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0ef2/5450473/c02ca839ed18/JPBS-9-73-g004.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0ef2/5450473/bb7b59089d25/JPBS-9-73-g005.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0ef2/5450473/ff60d42882ff/JPBS-9-73-g006.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0ef2/5450473/09ace6fa664f/JPBS-9-73-g007.jpg

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2
Craniofacial disorders and dysplasias: Molecular, clinical, and management perspectives.颅面疾病与发育异常：分子、临床及管理视角
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本文引用的文献

1
Dental treatment of Marfan syndrome. With regard to a case.马凡综合征的牙科治疗。附病例报告。
Med Oral Patol Oral Cir Bucal. 2010 Nov 1;15(6):e859-62. doi: 10.4317/medoral.15.e859.
2
Marfan syndrome: clinical diagnosis and management.马凡综合征：临床诊断与管理
Eur J Hum Genet. 2007 Jul;15(7):724-33. doi: 10.1038/sj.ejhg.5201851. Epub 2007 May 9.
3
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
Mol Genet Genomic Med. 2021 Oct;9(10):e1775. doi: 10.1002/mgg3.1775. Epub 2021 Jul 29.
J Periodontol. 2002 Jul;73(7):823-6. doi: 10.1902/jop.2002.73.7.823.
4
Oral manifestations of patients with Marfan syndrome: a case-control study.马凡综合征患者的口腔表现：一项病例对照研究。
Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 2002 May;93(5):564-72. doi: 10.1067/moe.2002.121430.
5
The Marfan syndrome.马凡综合征。
Annu Rev Med. 2000;51:481-510. doi: 10.1146/annurev.med.51.1.481.
6
Anaesthesia and Marfan's syndrome: case report.麻醉与马凡综合征：病例报告
Can J Anaesth. 1987 May;34(3 ( Pt 1)):311-4. doi: 10.1007/BF03015173.