225 resultados para Sindrome de Gorlin


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We report on four Brazilian patients with, among other signs, cleft lip and palate, dental anomalies, ectropion of the lower eyelids, euryblepharon, and lagophthalmia, Two were sporadic cases and two were familial cases, a mother and her equally affected son, Recently, the reports with different combination of these signs were reviewed by Gorlin et al, [1996; Am J Med Genet 65:109-112] and named blepharo-cheilo-dontic (BCD) syndrome, Variable expressivity and autosomal dominant inheritance were observed. (C) 1998 Wiley-Liss, Inc.

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Investigamos a imunidade humoral e celular em 18 pacientes com sindrome de West, 12 com sindrome de Lennox-Gastaut e 19 controles. Os exames realizados foram os seguintes: percentagem de linfócitos Ô e  no sangue periférico, niveis séricos de IgG, IgA e IgM, sensibilização cutânea com o DNCB, PHA intra dérmica, teste de inibição de migração de leucócitos e transformação blástica de linfócitos em presença de PHA. Detectamos deficiência de imunidade celular em 28 crianças, (18 com sindrome de West e 10 com sindrome de Lennox-Gastaut) e baixos níveis de imunoglobulinas em apenas 6. A depressão imunitária mostrou-se mais intensa nas crianças que apresentam sindrome de West.

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Background: Odontogenic tumors are lesions that are derived from remnants of the components of the developing tooth germ. The calcifying cystic odontogenic tumor or calcifying odontogenic cyst is a benign cystic neoplasm of odontogenic origin that is characterized by an ameloblastoma-like epithelium and ghost cells. Calcifying cystic odontogenic tumor may be centrally or peripherally located, and its ghost cells may exhibit calcification, as first described by Gorlin in 1962. Most peripheral calcifying cystic odontogenic tumors are located in the anterior gingiva of the mandible or maxilla. Case presentation. Authors report a rare case of a peripheral calcifying cystic odontogenic tumor of the maxillary gingiva. A 39-year-old male patient presented with a fibrous mass on the attached buccal gingiva of the upper left cuspid teeth. It was 0.7-cm-diameter, painless and it was clinically diagnosed as a peripheral ossifying fibroma. After an excisional biopsy, the diagnosis was peripheric calcifying cystic odontogenic tumor. The patient was monitored for five years following the excision, and no recurrence was detected. Conclusions: All biopsy material must be sent for histological examination. If the histological examination of gingival lesions with innocuous appearance is not performed, the frequency of peripheral calcifying cystic odontogenic tumor and other peripheral odontogenic tumors may be underestimated. © 2012 Lima et al.; licensee BioMed Central Ltd.

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Pós-graduação em Alimentos e Nutrição - FCFAR

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Pós-graduação em Fonoaudiologia - FFC

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Conselho Nacional de Desenvolvimento Científico e Tecnológico (CNPq)

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Coordenação de Aperfeiçoamento de Pessoal de Nível Superior (CAPES)

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Pós-graduação em Pediatria - FMB