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Query: UMLS:C0025362 (mental retardation)
 15,878 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Cherubism is an inherited, autosomal dominant disorder that characteristically affects the jaws of children. The disease typically manifest as a bilateral swelling with associated submandibular lymph node enlargements and usually regresses as age advances. The disease is microscopically indistinguishable from other giant cell lesions and is essentially a clinical diagnosis. The association of cherubism with gingival fibromatosis, epilepsy, mental retardation, stunted growth, and hypertrichosis is referred as Ramon syndrome. We report a case of Ramon syndrome in an 8 year old girl.
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PMID:Cherubism combined with epilepsy, mental retardation and gingival fibromatosis (Ramon syndrome): a case report. 2051 37

Cherubism is an inherited, autosomal dominant disorder that affects the jaws of children. The disease is usually obvious as a painless bilateral swelling in which bone is replaced with fibrous tissue. Affected children appear normal at birth. Swelling of the jaws usually occurs between 2 and 7 years of age and relapses as age progresses leaving a few facial deformities and malocclusion. The disease is microscopically indistinguishable from other giant cell lesions. The association of cherubism with gingival fibromatosis, epilepsy, mental retardation, stunted growth, and hypertrichosis is referred to as a rare case of possible Ramon syndrome with extraordinary tissue enlargement over the teeth. Here, we present a case of Ramon syndrome in a 6-year-old girl describing the clinical and radiographic features successfully treated with a brief review of literature.
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PMID:Ramon Syndrome- A Rare Form of Cherubism. 3190 27