Background: Bardet-Biedl syndrome (BBS) is a rare, heterogeneous, autosomal recessive condition, primarily characterized by polydactyly, obesity, mental retardation, hypogonadism, retinopathy, and renal failure. Dental anomalies, regarded as secondary manifestations, include hypodontia, microdontia, short roots, and deep palate. Few reports in the literature have described the oral manifestations of BBS. This article reports a case of BBS in a boy who presented some typical oral manifestations added to a generalized gingival overgrowth, an anomaly that had not been reported previously in patients with this syndrome.
Methods: A 12-year-old white male presented with a diagnosis of BBS and chief complaint of gingival enlargement in the anterior segment of both arcades. The treatment plan included surgical removal of the overgrown gingiva followed by orthodontic therapy. The excised tissues were submitted to histologic analysis.
Results: There was no sign of recurrence 1 year after gingivectomy. Histopathology revealed a dense connective tissue with a mild inflammatory infiltrate, irregularly arranged fiber bundles, and epithelial acanthosis, which is characteristic of gingival overgrowth.
Conclusions: The gingival overgrowth was treated successfully by gingivectomy. The periodontal surgery minimized the functional, social, and emotional consequences of the oral manifestation associated with the syndrome.
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http://dx.doi.org/10.1902/jop.2007.060378 | DOI Listing |
Cureus
December 2024
Oral Pathology and Microbiology, Vinayaka Mission's Sankarachariyar Dental College, Vinayaka Mission's Research Foundation (Deemed to be University), Salem, IND.
Gingival enlargements are mostly plaque-induced. Other than plaque, a few genetic conditions also cause enlargements of the gingiva. In recent years, there has been a notable rise in drug-induced gingival overgrowth (DIGO) linked to the increased use of medications for various systemic conditions.
View Article and Find Full Text PDFJ Clin Med
December 2024
Department of Dental Medicine, Faculty of Medicine, University of Novi Sad, 21000 Novi Sad, Serbia.
: Although BRAF inhibitors, such as vemurafenib, produce a marked response in patients with advanced melanoma with a BRAF V600 mutation, they eventually develop resistance to this treatment. To address this issue, vemurafenib is increasingly combined with the MEK inhibitor cobimetinib, leading to improved response rates and enhanced survival. However, this treatment modality is associated with numerous side effects.
View Article and Find Full Text PDFDiseases
December 2024
Department of Biochemistry and Molecular Biology, Nihon University School of Dentistry at Matsudo, Matsudo 271-8587, Chiba, Japan.
Objectives: Cyclosporine A promotes gingival fibrosis by enhancing the proliferation of gingival fibroblasts, leading to gingival overgrowth. The population of gingival fibroblasts is regulated by cell cycle machinery, which balances cell growth and inhibition. Cells that detect DNA damage pause at the G1/S checkpoint to repair the damage instead of progressing to the S phase.
View Article and Find Full Text PDFDent J (Basel)
December 2024
Department of Oral Medicine & Pathology and Hospital Dentistry, School of Dentistry, National and Kapodistrian University of Athens (NKUA), 11527 Athens, Greece.
The etiology of diffuse gingival enlargement is multifactorial, and the definitive diagnosis may be challenging. To highlight the nuances of the differential diagnosis, we present two cases of generalized gingival overgrowth and discuss the diagnostic dilemmas. In the first case, an 82-year-old male with a medical history of hypertension and prostatitis had a chief complaint of symptomatic oral lesions of a 20-day duration, accompanied by fever and loss of appetite.
View Article and Find Full Text PDFbioRxiv
December 2024
Department of Molecular & Cell Biology, Department of Neuroscience, California Institute for Quantitative Biology (QB3), University of California Berkeley, Berkeley, California 94720, USA.
Cannabidiol (CBD) is a prominent non-psychoactive small molecule produced by cannabis plants used clinically as an antiepileptic. Here, we show CBD and other cannabinoids are potent inhibitors of mechanosensitive two-pore domain K (K2P) channels, including TRAAK and TREK-1 that contribute to spike propagation in myelinated axons. Five TRAAK mutations that cause epilepsy or the neurodevelopmental syndrome FHEIG (facial dysmorphism, hypertrichosis, epilepsy, intellectual/developmental delay, and gingival overgrowth) retain sensitivity to cannabinoid inhibition.
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