Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0002986 (Fabry)
 5,646 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The presence of supernumerary teeth, also known as hyperdontia, is the condition of having teeth in addition to the regular number of teeth. The occurrence of multiple supernumerary teeth is often found in association with syndromes such as Gardner syndrome, Anderson-Fabry disease, Ellis-van Creveld syndrome, Ehlers-Danlos syndrome, incontinentia pigmenti, and Tricho-rhino-phalangeal syndrome. Only a few examples of nonsyndromic multiple supernumerary teeth have been reported in literature. In this report, we present the unusual case and 9-year follow up of a non-syndrome female patient with bilateral supernumerary teeth that occurred with an interval of several years.
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PMID:Non-syndrome patient with bilateral supernumerary teeth: Case report and 9-year follow-up. 2340 7

Supernumerary teeth are common in the general population, with a prevalence that varies between 0.1 percent to 3.8 percent. Multiple supernumerary teeth are associated with Gardner's syndrome, Fabry-Anderson syndrome, Ehlers-Danlos syndrome, labial palatal cleft, and cleidocranial dysplasia. Multiple hyperdontia not associated with syndromes is rare and ranges between 0.04 percent to 0.1 percent. The purpose of this report was to describe the occurrence of nonsyndromic multiple hyperdontia in monozygotic twin sisters.
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PMID:Non-syndromic multiple hyperdontia in monozygotic twin sisters: a report of two cases. 2470 35

Supernumerary teeth (ST) are odontostomatologic anomaly characterized by as the existence excessive number of teeth in relation to the normal dental formula. This condition is commonly seen with several congenital genetic disorders such as Gardner's syndrome, cleidocranial dysostosis and cleft lip and palate. Less common syndromes that are associated with ST are; Fabry Disease, Ellis-van Creveld syndrome, Nance-Horan syndrome, Rubinstein-Taybi Syndrome and Trico-Rhino-Phalangeal syndrome. ST can be an important component of a distinctive disorder and an important clue for early diagnosis. Certainly early detecting the abnormalities gives us to make correct management of the patient and also it is important for making well-informed decisions about long-term medical care and treatment. In this review, the genetic syndromes that are related with ST were discussed.
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PMID:Genetic background of supernumerary teeth. 2571