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Guglielmi F, Staderini E, Iavarone F, et al. Zimmermann-Laband-1 Syndrome: Clinical, Histological, and Proteomic Findings of a 3-Year-Old Patient with Hereditary Gingival Fibromatosis. Biomedicines. 2019;7(3)doi: 10.3390/biomedicines7030048.
Guglielmi, F., Staderini, E., Iavarone, F., Di Tonno, L., & Gallenzi, P. (2019). Zimmermann-Laband-1 Syndrome: Clinical, Histological, and Proteomic Findings of a 3-Year-Old Patient with Hereditary Gingival Fibromatosis. Biomedicines, 7(3), . https://doi.org/10.3390/biomedicines7030048
Guglielmi, Federica, et al. "Zimmermann-Laband-1 Syndrome: Clinical, Histological, and Proteomic Findings of a 3-Year-Old Patient with Hereditary Gingival Fibromatosis." Biomedicines vol. 7,3 (2019). doi: https://doi.org/10.3390/biomedicines7030048
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 Jun 29;7(3). doi: 10.3390/biomedicines7030048. PMID: 31261938; PMCID: PMC6783959.
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Biomedicines. 2019 Jun 29;7(3). doi: 10.3390/biomedicines7030048.

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

Biomedicines

Federica Guglielmi, Edoardo Staderini, Federica Iavarone, Laura Di Tonno, Patrizia Gallenzi

Affiliations

  1. Istituto di Odontoiatria, Fondazione Policlinico Universitario A. Gemelli IRCCS, Università Cattolica del Sacro Cuore, 00168 Rome, Italy.
  2. Istituto di Odontoiatria, Fondazione Policlinico Universitario A. Gemelli IRCCS, Università Cattolica del Sacro Cuore, 00168 Rome, Italy. [email protected].
  3. Istituto di Biochimica e Biochimica Clinica, Università Cattolica del Sacro Cuore, Fondazione Policlinico Universitario A. Gemelli, IRCCS, 00168 Rome, Italy.

PMID: 31261938 PMCID: PMC6783959 DOI: 10.3390/biomedicines7030048

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.

Keywords: Zimmermann-Laband syndrome; gingival fibromatosis; oral microbiome; periodontal disease

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