Literature DB >> 8667262

Anomalies in the permanent dentition and other oral findings in 29 individuals with Laurence-Moon-Bardet-Biedl syndrome.

M K Borgström1, R Riise, K Tornqvist, L Granath.   

Abstract

This paper reports a clinical and roentgenological examination of the teeth, jaws and saliva of 29 Scandinavian individuals with Laurence-Moon-Bardet-Biedl (LMBB) syndrome, whose cardinal signs are retinal dystrophy, polydactyly, obesity, hypogenitalism and mental retardation. All subjects had at least three of these signs, including retinal dystrophy. Compared with normal subjects, the group had statistically significantly higher frequencies of hypodontia, small teeth and short roots. In addition, the saliva showed a buffering capacity higher than normal. In conclusion, there seem to exist disturbances of both dental and skeletal formation in the LMBB syndrome.

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Year:  1996        PMID: 8667262     DOI: 10.1111/j.1600-0714.1996.tb00198.x

Source DB:  PubMed          Journal:  J Oral Pathol Med        ISSN: 0904-2512            Impact factor:   4.253


  5 in total

1.  Ciliopathies with skeletal anomalies and renal insufficiency due to mutations in the IFT-A gene WDR19.

Authors:  Cecilie Bredrup; Sophie Saunier; Machteld M Oud; Torunn Fiskerstrand; Alexander Hoischen; Damien Brackman; Sabine M Leh; Marit Midtbø; Emilie Filhol; Christine Bole-Feysot; Patrick Nitschké; Christian Gilissen; Olav H Haugen; Jan-Stephan F Sanders; Irene Stolte-Dijkstra; Dorus A Mans; Eric J Steenbergen; Ben C J Hamel; Marie Matignon; Rolph Pfundt; Cécile Jeanpierre; Helge Boman; Eyvind Rødahl; Joris A Veltman; Per M Knappskog; Nine V A M Knoers; Ronald Roepman; Heleen H Arts
Journal:  Am J Hum Genet       Date:  2011-10-20       Impact factor: 11.025

2.  Intrafamilial variation of the phenotype in Bardet-Biedl syndrome.

Authors:  R Riise; S Andréasson; M K Borgaström; A F Wright; N Tommerup; T Rosenberg; K Tornqvist
Journal:  Br J Ophthalmol       Date:  1997-05       Impact factor: 4.638

Review 3.  Role of Primary Cilia in Odontogenesis.

Authors:  M Hampl; P Cela; H L Szabo-Rogers; M Kunova Bosakova; H Dosedelova; P Krejci; M Buchtova
Journal:  J Dent Res       Date:  2017-06-12       Impact factor: 6.116

Review 4.  Oral and Craniofacial Anomalies of Bardet-Biedl Syndrome: Dental Management in the Context of a Rare Disease.

Authors:  A Panny; I Glurich; R M Haws; A Acharya
Journal:  J Dent Res       Date:  2017-06-29       Impact factor: 6.116

5.  New criteria for improved diagnosis of Bardet-Biedl syndrome: results of a population survey.

Authors:  P L Beales; N Elcioglu; A S Woolf; D Parker; F A Flinter
Journal:  J Med Genet       Date:  1999-06       Impact factor: 6.318

  5 in total

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