Venza Nicolò, Danesi Carlotta, Contò Diego, Fabi Francesco, Mampieri Gianluca, Sangiuolo Federica, Laganà Giuseppina
Department of Clinical Sciences and Translational Medicine, University of Roma "Tor Vergata", Roma, Italia.
Laboratory of Medical Genetics, Department of Biomedicine and Prevention, University of Rome "Tor Vergata", Rome, Italy.
PeerJ. 2019 Apr 23;7:e6606. doi: 10.7717/peerj.6606. eCollection 2019.
Marfan's syndrome (MFS) is a systemic disorder of connective tissue caused by mutations in the extracellular matrix protein fibrillin-1. Orofacial characteristics may be useful in identification of the syndrome. Severe periodontitis is sometimes observed in MFS patients, but no in-depth information has been reported in Italian groups of growing subjects with MFS. The aim of this study was to analyze the periodontal condition on a group of growing subjects affected by MFS, in comparison with a typically developed control group.
A group of 16 subjects with diagnosed MFS were recruited from the Centre for Rare Diseases for Marfan Syndrome and Related Disorders of Tor Vergata University Hospital. The Marfan Group (MG) was compared with a Control Group (CG) composed by 20 nonsyndromic subjects. The periodontal clinical parameters like Marginal Gingival Thickness (GT), Plaque Index (PI), Bleeding On Probing (BOP) and Modified Periodontal Screening and Recording (PSR) were assessed.
The mean value of PI in MG was 59%, instead in CG it reached 21%. Analysis showed a significant difference between MG and CG also for the BOP. In MG the mean value of BOP attained 36% and in CG it reached 16%. A statistical significant difference of distribution of PSR index between the two groups was found for all sextant examined.
Patients with Marfan syndrome reveal a higher presence of plaque and consequently a generalized inflammation in the oral cavity when compared with a control group.
马凡综合征（MFS）是一种由细胞外基质蛋白原纤蛋白-1突变引起的结缔组织系统性疾病。口腔面部特征可能有助于该综合征的诊断。MFS患者有时会出现重度牙周炎，但意大利关于MFS生长发育期患者群体的深入信息尚未见报道。本研究的目的是分析一组患有MFS的生长发育期患者的牙周状况，并与典型发育的对照组进行比较。
从托雷维加塔大学医院马凡综合征及相关疾病罕见病中心招募了16名确诊为MFS的患者。将马凡组（MG）与由20名非综合征患者组成的对照组（CG）进行比较。评估了牙周临床参数，如边缘龈厚度（GT）、菌斑指数（PI）、探诊出血（BOP）和改良牙周筛查与记录（PSR）。
MG组PI的平均值为59%，而CG组达到21%。分析显示，MG组和CG组在BOP方面也存在显著差异。MG组BOP的平均值为36%，CG组为16%。在所有检查的牙象限中，两组PSR指数分布存在统计学显著差异。
与对照组相比，马凡综合征患者口腔内菌斑更多，因此存在全身性炎症。
