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Open AccessCase Report

Zimmermann-Laband-1 Syndrome: Clinical, Histological, and Proteomic Findings of a 3-Year-Old Patient with Hereditary Gingival Fibromatosis

1
Istituto di Odontoiatria, Fondazione Policlinico Universitario A. Gemelli IRCCS, Università Cattolica del Sacro Cuore, 00168 Rome, Italy
2
Istituto di Biochimica e Biochimica Clinica, Università Cattolica del Sacro Cuore, Fondazione Policlinico Universitario A. Gemelli, IRCCS, 00168 Rome, Italy
*
Author to whom correspondence should be addressed.
Biomedicines 2019, 7(3), 48; https://doi.org/10.3390/biomedicines7030048
Received: 29 May 2019 / Revised: 27 June 2019 / Accepted: 27 June 2019 / Published: 29 June 2019
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Abstract

Background: Zimmermann-Laband-1 syndrome (ZLS-1; OMIM# 135500) is a rare genetic disorder whose oral pathognomonic sign is the development of progressive, diffuse, and severe gingival hypertrophy. Most children with abnormally gingival hyperplasia may also present multiple unerupted teeth and skeletal deformities of maxillary arches (i.e., skeletal anterior open bite). Despite phenotypic variability of the clinical spectrum, gingival fibromatosis is the hallmark of ZLS-1. Method: In this study, we report a 3-year-old male patient with a ZLS-1-related gingival overgrowth and failure of eruption of the deciduous teeth in the molar area. Surgical excision was performed under general anesthesia. Results: At three weeks follow-up, esthetics was significantly improved in terms of gingival appearance, and teeth eruption allowed an adequate masticatory function. Conclusion: In severe cases, surgical removal of the hyperplasic fibrous tissue may be required to expose unerupted teeth and establish a proper gingival contour. Surgical excision under general anesthesia is an elective procedure for patients with special needs, mental disability, as well as young and adult patients with dental anxiety type II and IV associated with poor oral health. View Full-Text
Keywords: gingival fibromatosis; Zimmermann-Laband syndrome; periodontal disease; oral microbiome gingival fibromatosis; Zimmermann-Laband syndrome; periodontal disease; oral microbiome
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This is an open access article distributed under the Creative Commons Attribution License which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited (CC BY 4.0).
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Guglielmi, F.; Staderini, E.; Iavarone, F.; Di Tonno, L.; Gallenzi, P. Zimmermann-Laband-1 Syndrome: Clinical, Histological, and Proteomic Findings of a 3-Year-Old Patient with Hereditary Gingival Fibromatosis. Biomedicines 2019, 7, 48.

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