Treatment of Gingival Overgrowth in a Child With Bardet‐Biedl Syndrome

Drugowick, Rayen Millanao; Gonçalves, Lorena Da Rós; Barrôso, Áurea Simone; Feres-Filho, Eduardo Jorge; Maia, Lucianne Cople

doi:10.1902/jop.2007.060378

Cited by 12 publications

(16 citation statements)

References 20 publications

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“…Other abnormalities connected to the malformative syndrome are numerous (23), among them gingival overgrowth and dental abnormalities (including hypodontia, microdentia, shorts roots and deep plate) are reported by Drugowick (6).…”

Section: Discussionmentioning

confidence: 99%

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Bardet Biedl Syndrome with Typical Retinitis Pigmentosa and Hypergonadotrophic Hypogonadism

Chentli¹

2011

Acta Endo (Buc)

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Bardet Biedl syndrome (BBS) is a rare autosomal recessive disease, characterized by clinical and genetic heterogeneity. Many genes are involved. BBS seems to be different from Lawrence Moon BBS, although they share some clinical symptoms. The main clinical signs are obesity, pigmentary retinopathy, kidney malformations, and hypogenitalism.Our aim is to report a case with typical retinis pigmentosa, hypergonadotrophic hypogonadism and cerebellum cyst.Case report. A man aged 18 was referred for obesity and blindness. His family history was marked by obesity and diabetes mellitus type II. His medical history began very soon, as he was born with polydactyly, then he became obese and had difficulty to learn and to see. His blindness was progressive, and his puberty was delayed. Clinical and biological exams showed: severe android obesity (BMI = 40kg/m², waist circumference = 130cm), pigmentary retinopathy, small testes with high FSH = 17 mU/mL (1-8), and normal LH = 6.13 mU/mL (0.6-12)], empty sellae, cerebellum cyst, renal malformations, and signs of chronic infections. He did not have any spasticity or ataxia. Genetic study was not done. Conclusion.In this case, all features argued for typical BBS, except for testicular insufficiency which is classically described as hypogonadotrophic. Infections should be treated vigorously to avoid renal insufficiency.

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Section: Discussionmentioning

confidence: 99%

“…The main features or cardinal signs are obesity, severe retinal dystrophy, kidney malformations, and abnormal extremities, plus hypogonadism especially for men (1,3). Other signs are learning disability, strabismus, cataracts, diabetes mellitus, cerebral (4) or bone (5) abnormalities, and dental malformations (6).…”

Section: Introductionmentioning

confidence: 99%

Bardet Biedl Syndrome with Typical Retinitis Pigmentosa and Hypergonadotrophic Hypogonadism

Chentli¹

2011

Acta Endo (Buc)

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“…Intraoral examination revealed bilateral posterior cross-bites, dental spacing, plaque accumulation, and multiple proximal and occlusal caries involving teeth no 2,4,6,7,8,9,10,11,15,18,19,28,29, and 30. OPG examination revealed short dental roots, particularly of the lower anterior teeth, and congenitally missing third molars and lower lateral incisors ( Figure 2 ).…”

Section: Case Repor Tmentioning

confidence: 99%

Oral healthcare management in Bardet Biedl syndrome

Hassona

Kasabreh

Hammoudeh

et al. 2016

Special Care in Dentistry

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“…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 (2) . If spasticity and mental retardation are present, it fulfills the criteria for Laurence-Moon-Bardet-Biedl syndrome (3) .…”

Section: Abstract Introductionmentioning

confidence: 99%

Pigmentary retinopathy due to Bardet-Biedl syndrome: case report and literature review

Andrade¹,

Andrade²,

França³

et al. 2009

Arq. Bras. Oftalmol.

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Bardet-Biedl syndrome (BBS) is a rare autosomal recessive disorder with clinical and genetic heterogeneity. This syndrome was first described by Laurence and Moon in 1866 and additional cases were described by Bardet and Biedl between 1920 and 1922. The main features are obesity, polydactyly, pigmentary retinopathy, learning disabilities, various degrees of intellectual impairment, hypogonadism, and renal abnormalities. Bardet-Biedl syndrome is both phenotypically and genetically heterogeneous. Clinical diagnosis is based on the presence of 4 of the 5 cardinal features. The authors present a typical case of pigmentary retinopathy due to Bardet-Biedl syndrome and made a brief commentary about the disease's cardinal manifestations.

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Treatment of Gingival Overgrowth in a Child With Bardet‐Biedl Syndrome

Abstract: 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.

Cited by 12 publications

References 20 publications

Bardet Biedl Syndrome with Typical Retinitis Pigmentosa and Hypergonadotrophic Hypogonadism

Bardet Biedl Syndrome with Typical Retinitis Pigmentosa and Hypergonadotrophic Hypogonadism

Oral healthcare management in Bardet Biedl syndrome

Pigmentary retinopathy due to Bardet-Biedl syndrome: case report and literature review

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