1996
DOI: 10.1136/jmg.33.2.137
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On the genetics of hypodontia and microdontia: synergism or allelism of major genes in a family with six affected members.

Abstract: Familial severe hypodontia of the permanent dentition is a rare condition. The genetics ofthis entity remains unclear and several modes of inheritance have been suggested. We report here an increase in the number of congenitally missing teeth after the mating of affected subjects from two unrelated Norwegian families. This condition may be the result of allelic mutations at a single gene locus. Alternatively, incompletely penetrant non-allelic genes may show a synergistic effect as expected for a multifactoria… Show more

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Cited by 33 publications
(29 citation statements)
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“…A similar patient from Turkey, a child of first cousins, has also been described 25. The Norwegian sibs reported26 could also well have the autosomal recessive condition described here. A brother and sister from Lebanon with only a few permanent teeth and thin nails were recently reported27 with the suggestion that they may have the condition described by Fried 24.…”
Section: Discussionmentioning
confidence: 51%
“…A similar patient from Turkey, a child of first cousins, has also been described 25. The Norwegian sibs reported26 could also well have the autosomal recessive condition described here. A brother and sister from Lebanon with only a few permanent teeth and thin nails were recently reported27 with the suggestion that they may have the condition described by Fried 24.…”
Section: Discussionmentioning
confidence: 51%
“…Oligodontias têm sido frequentemente relatadas em humanos (Lyngstadaas et al, 1996); registros em animais domés-ticos, como ratos e cães também são comuns na literatura (Butkovic et al, 2001). Hoff e Hoff (1996) …”
Section: Agênese Múltipla (= Oligodontia)unclassified
“…Hypodontia is commonly reported in coexistence with other dental clinical features (2,3,6) such as taurodontism, peg-shaped incisors, teeth rotations and generalized spacing, canine-lateral incisor transposition, retained primary teeth, or abnormal dental morphologies (1). Microdontia is also highly reported in hypodontia case reports (3,24,25).…”
Section: Introductionmentioning
confidence: 99%