New form of hidrotic ectodermal dysplasia in a Lebanese family

Mégarbané, André; Noujeim, Ziad; Fabre, M; Kaloustian, Vazken M. Der

doi:10.1002/(sici)1096-8628(19980113)75:2<196::aid-ajmg15>3.0.co;2-k

Cited by 15 publications

(2 citation statements)

References 5 publications

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“…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. The pattern of missing teeth, including deciduous teeth, can also be similar in incontinentia pigmenti28 and in Kabuki syndrome 29…”

Section: Discussionmentioning

confidence: 96%

Recessively inherited lower incisor hypodontia

Pirinen

2001

Journal of Medical Genetics

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Section: Discussionmentioning

confidence: 96%

Recessively inherited lower incisor hypodontia

Pirinen

2001

Journal of Medical Genetics

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“…The pedigree revealed the extent of consanguinity, which may explain the presence of the same mutation and phenotype among members of different families. These families subscribe to the same religion and originated from the same region as the families described by Megarbane et al 7 8 …”

Section: Discussionmentioning

confidence: 85%

Impacted Lower Second Permanent Molars at the Ramus and Coronoid Process: A New Clinical Symptom of the WNT10A Mutation in Ectodermal Dysplasia

Sfeir

2017

International Journal of Clinical Pediatric Dentistry

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Aim: Hidrotic ectodermal dysplasia (ED) with the WNT10A mutation produces variable dentofacial symptoms. The aim of this study was to describe a new clinical symptom, i.e., specific to the WNT10A mutation in hidrotic ED. The study investigated the migratory trend of the lower second permanent molars to the ramus or coronoid process. To the best of authors' knowledge, no data in the literature describe this trend in cases of hidrotic ED. Materials and methods:A three-generation family pedigree was established for seven families after the diagnosis of hidrotic ED in a 10-year-old boy. Thereafter, a genetic and clinical study was conducted on three families with at least one individual affected by hidrotic ED (20 individuals). We selected the children with molar germs 37 and 47. The eruption axes of these germs were then traced on the panoramic images at the initial time (T 0 ) and 1 year later (T 0 + 1 year), and the deviations between these axes were measured.Results: A significant familial consanguinity was shown. Eight subjects presented with the hidrotic ED phenotype. Among them, three individuals carried germs 37 and 47. Over time, the measured deviations between the eruption axes of the latter displayed, in the majority of the cases, a distal inclination toward the ramus.Limitations: A larger sample size is mandatory to assess the frequencies and treatment modalities. Conclusion:The presence of germs in the lower second permanent molars in patients with hidrotic ED is an important clinical symptom that should be monitored to detect and prevent ectopic migration of these teeth. Clinical significance:In hidrotic ED cases, the study of the presence of the second lower permanent germs must include clinical and radiological examinations. Establishing an interceptive treatment is necessary to prevent the migration of the molars in question.

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On the inheritance of ?new? syndromes

Zlotogora¹

1998

Am. J. Med. Genet.

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New form of hidrotic ectodermal dysplasia in a Lebanese family

Cited by 15 publications

References 5 publications

Recessively inherited lower incisor hypodontia

Recessively inherited lower incisor hypodontia

Impacted Lower Second Permanent Molars at the Ramus and Coronoid Process: A New Clinical Symptom of the WNT10A Mutation in Ectodermal Dysplasia

On the inheritance of ?new? syndromes

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