ORAL AND DENTAL FINDINGS OF A CHILD WITH WEILL-MARCHESANI SYNDROME TYPE II: A CASE REPORT WITH 3-YEAR FOLLOW-UP

Author:

GÜÇYETMEZ TOPAL Burcu1ORCID,ELMAS Muhsin2ORCID,TIRAŞ Melike2ORCID

Affiliation:

1. AFYONKARAHİSAR SAĞLIK BİLİMLERİ ÜNİVERSİTESİ

2. Afyonkarahisar Sağlık Bilimleri Üniversitesi

Abstract

Weill-Marchesani syndrome (WMS, OMIM# 227600) is a genetically determined, rare systemic connective tissue disorder. The syndrome is divided into four types according to mutations in related genes. Given the limited number of individuals with WMS in the literature, no genotype-phenotype correlations for ADAMTS10, ADAMTS17, FBN1, or LTBP2 have been identified. In the accessible literature, none of the limited studies were focused on the oral and dental anomalies of WMS. The present case report describes oral and dental findings of a 63 months old female patients with WMS2.

Publisher

Selcuk Dental Journal

Subject

General Medicine

Reference16 articles.

1. 1. Weill G. Ectopie du cristallin et malformations générales. Ann Ocul 1932:169.

2. 2. Marchesani O. Brachydaktylie und angeborene kugellines als systemer-krankung. Klin Monatsbl Augenheilkd 1939;103:392-406.

3. 3. Gorlin RJ, Cohen Jr, MM, Hennekam RC. Syndromes of the head and neck. Oxford university press, 2001.

4. 4. Marzin P, Cormier-Daire V, Tsilou E. Weill-Marchesani Syndrome. GeneReviews®, 2020.

5. 5. Yang GY, Huang, X, Chen BJ, Xu ZP. Weill-Marchesani-like syndrome caused by an FBN1 mutation with low-penetrance. Chin Med J 2021;134(11):1359-1361.

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