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Anomalies in the permanent dentition and other oral findings in 29 individuals with Laurence‐Moon‐Bardet‐Biedl syndrome
Author(s) -
Borgström M. Krasse,
Riise R.,
Tornqvist K.,
Granath L.
Publication year - 1996
Publication title -
journal of oral pathology and medicine
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.887
H-Index - 83
eISSN - 1600-0714
pISSN - 0904-2512
DOI - 10.1111/j.1600-0714.1996.tb00198.x
Subject(s) - bardet–biedl syndrome , polydactyly , medicine , permanent dentition , ectodermal dysplasia , hypodontia , dentition , dystrophy , dentistry , anatomy , dermatology , biology , pathology , genetics , gene , phenotype
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.