Ellis-van Creveld Syndrome: Systemic And Oral Findings

Aims: To present a case of Ellis-van Creveld syndrome highlighting the systemic and oral manifestations and expand the phenotypic spectrum of the disorder. Case description: A 4-year-old female, with an initial diagnosis of Ellis-van Creveld syndrome, was referred for dental treatment. The patient had hexadactyly of the hands, thorax disproportionate to the shortened limbs, hypopigmented and dry skin, short stature, a wide gap between the 1st and 2nd toes of the right foot and markedly dystrophic toenails. The oral manifestations were upper labial frenulum causing large vestibule and absence of diastema, labiogingival frenulum, enamel hypoplasia, conical teeth, claw-like cusp, oligodontia, microdontia and malocclusion. Conclusions: Ellis-van Creveld syndrome is one among several syndromes with oral manifestations that demands the participation of a multidisciplinary team to better patient assessing, treatment and monitoring. Dentists have the leading role in the control and treatment of the oral manifestations.222109112Alves-Pereira, D., Berini-Aytés, L., Gay-Escoda, C., Ellis-Van Creveld Syndrome Case report and literature review (2009) Med Oral Patol Oral Cir Bucal, 14, pp. E340-E343da Silva, E.O., Janovitz, D., de Albuquerque, S.C., Ellis-van Creveld syndrome: report of 15 cases in an inbred kindred (1980) J Med Genet, 17, pp. 349-356Kurian, K., Shanmugan, S., Harsh Vardah, T., Chondroectodermal dysplasia (Ellis-van Creveld syndrome) A report of three cases with review of literature (2007) Indian J Dent Res, 18, pp. 31-34Hills, C.B., Kochilas, L., Schimmenti, L.A., Ellis-van Creveld syndrome and congenital heart defects: Presentation of an additional 32 cases (2011) Pediatr Cardiol, 32, pp. 977-982Tompson, S.W., Ruiz-Perez, V.L., Blair, H.J., Sequencing EVC and EVC2 identifies mutations in two-thirds of Ellis-van Creveld syndrome patients (2007) Hum Genet, 120, pp. 663-670Ruiz-Perez, V.L., Ide, S.E., Strom, T.M., Mutations in a new gene in Ellis-van Creveld syndrome and Weyers acrodental dysostosis (2000) Nat Genet, 24, pp. 283-286Galdzicka, M., Patnala, S., Hirshman, M.G., A new gene, EVC2, is mutated in Ellis-van Creveld syndrome (2002) Mol Genet Metab, 77, pp. 291-295Ruiz-Perez, V.L., Tompson, S.W., Blair, H.J., Mutations in two nonhomologous genes in a head-to-head configuration cause Ellis-van Creveld syndrome (2003) Am J Hum Genet, 72, pp. 728-732Ruiz-Perez, V.L., Goodship, J.A., Ellis-van Creveld syndrome and Weyers acrodental dysostosis are caused by cilia-mediated diminished response to hedgehog ligands (2009) Am J Med Genet C Semin Med Genet, 151 C, pp. 341-351Biggerstaff, R.H., Mazaheri, M., Oral manifestations of the Ellis-van Creveld syndrome (1968) J Am Dent Assoc, 77, pp. 1090-1095Aminabadi, N.A., Ebrahimi, A., Oskouei, S.G., Chondroectodermal dysplasia (Ellis-van Creveld syndrome): a case report (2010) J Oral Sci, 52, pp. 333-336Hunter, M.L., Roberts, G.J., Oral and dental anomalies in Ellis van Creveld syndrome (chondroectodermal dysplasia): report of a case (1998) Intern J Paed Dent, 8, pp. 153-157Fukuda, A., Kato, K., Hasegawa, M., Recurrent knee valgus deformity in Ellis-van Creveld syndrome (2012) J Pediatr Orthop B, 21, pp. 352-355Cahuana, A., Palma, C., Oral manifestations in Ellis-van Creveld syndrome (2004) Report of five cases. Pediatr Dent., 26, pp. 277-282Souza, R.C., Martins, R.B., Okida, Y., Giovani, M., Ellis-van Creveld syndrome: oral manifestations and treatment (2010) J Health Sci Inst, 28, pp. 241-243Kalaskar, R., Kalaskar, R.A., Oral manifestations of Ellis-van Creveld syndrome (2012) Contemp Clin Dent, 3, pp. S55-S5

Consórcio sorgo-feijão: efeito de arranjos de fileiras no rendimento de grãos.

Com o objetivo de estudar os efeitos de arranjos de fileiras no consorcio sorgo-feijao, instalou-se um experimento (marco de 1992) no CNPMS/EMBRAPA, Sete Lagoas, MG, Brasil. usaram-se duas linhagens de sorgo granifero (BR 007B e CMSXS 210B) em monocultura e em tres arranjos de fileiras (plantio na mesma fileira, plantio em fileiras alternadas e duas fileiras de feijao nas entrelinhas do sorgo), mais um tratamento adicional (feijao em monocultura), no esquema fatorial (2 x 4) + 1 e delineamento de blocos inteiramente casualizados, com quatro repeticoes. Foi utilizado cultivar de feijao Ouro, de habito de crescimento indeterminado (tipo II). Os rendimentos individuais das culturas foram maiores no arranjo em fileiras alternadas, mas suas medias nao diferiram das obtidas nos seus respectivos monocultivos. Independentemente da linhagem, os maiores rendimentos totais foram obtidos nos arranjos de fileiras alternadas e de duas fileiras de feijao nas entrelinhas do sorgo. Quando o consorcio foi feito usando a CMSXS 210B no arranjo de duas fileiras de feijao nas entrelinhas do sorgo, houve reducao do rendimento de feijao e dos indices de equivalencia de area (IEAs), devido a reducao do numero de vagens por planta. Concluiu-se que os arranjos em fileira alternadas e de duas fileiras de feijao nas entrelinhas do sorgo podem ser usados no consorcio sorgo-feijao, todavia este ultimo arranjo seria adequado apenas com cultivares de sorgo menos competitivos.Made available in DSpace on 2011-10-18T00:01:12Z (GMT). No. of bitstreams: 1 Consorciosorgo.pdf: 6733876 bytes, checksum: 4d0c4d340f4c78f8655d4791cde3e562 (MD5) Previous issue date: 1998-01-29199

Symptomatic Swelling On The Lingual Surface Of The Mandible

[No abstract available]1175546550Ramesh, R.S., Manjunath, S., Ustad, T.H., Pais, S., Shivakumar, K., Unicystic ameloblastoma of the mandible - An unusual case report and review of literature (2010) Head Neck Oncol, 2, p. 1Bisinelli, J.C., Ioshii, S., Retamoso, L.B., Moyses, S.T., Moyses, S.J., Tanaka, O.M., Conservative treatment of unicystic ameloblastoma (2010) A J Orthod Dentofacial Orthop, 137, pp. 396-400Covani, U., Barone, A., Piezosurgical treatment of unicystic ameloblastoma (2007) J Periodontol, 78, pp. 1342-1347Karandikar, S., Nilesh, K., Dadhich, A., Large odontogenic keratocyst of mandible - A case report (2011) JIDA, 5, pp. 822-824Venkateshwarlu, M., Geetha, P., Radhika, B., Central giant cell granuloma (2010) IJDA, 2, pp. 15-20Stavropoulos, F., Katz, J., Central giant cell granulomas: A systematic review of the radiographic characteristics with the addition of 20 new cases (2002) Dentomaxillofacial Radiology, 31 (4), pp. 213-217. , DOI 10.1038/sj.dmfr.4600700Araujo De Morais, H.H., Jose De Holanda Vasconcellos, R., De Santana Santos, T., Guedes Queiroz, L.M., Dantas Da Silveira, E.J., Glandular odontogenic cyst: Case report and review of diagnostic criteria (2012) J Craniomaxillofac Surg, 40, pp. e46-e50Krishnamurthy, A., Sherlin, H.J., Ramalingam, K., Glandular odontogenic cyst: Report of two cases and review of literature (2009) Head Neck Pathol, 3, pp. 153-158Sai, H.K., Hsieh, M.C., Yang, L.H., Tu, S.T., Maxillary brown tumor as initial presentation of parathyroid adenoma: A case report (2012) J Med Sci, 28, pp. 400-403Guimaraes, A.L.S., Marques-Silva, L., Gomes, C.C., Castro, W.H., Mesquita, R.A., Gomez, R.S., Peripheral brown tumour of hyperparathyroidism in the oral cavity (2006) Oral Oncology Extra, 42 (3), pp. 91-93. , DOI 10.1016/j.ooe.2005.09.007, PII S1741940905000865Guldfred, L.A., Daugaard, S., Buchwald, C., Brown tumor mimicking maxillary sinus mucocele as the first manifestation of primary hyperparathyroidism (2012) Auris Nasus Larynx, pp. 418-421Reddy, S.P., Naag, A., Kashyap, B., Odontogenic myxoma: Report of two cases (2010) Natl J Maxillofac Surg, 1, pp. 183-186Lin, Y.L., Basile, J.R., A case of odontogenic myxoma with unusual histological features mimicking a fibro-osseous process (2010) Head Neck Pathol, 4, pp. 253-256Manne, R.K., Kumar, V.S., Venkata Sarath, P., Anumula, L., Mundlapudi, S., Tanikonda, R., Odontogenic myxoma of the mandible (2012) Case Rep Denti, 1, pp. 1-4Singaraju, S., Wanjari, S.P., Parwani, R.N., Odontogenic myxoma of the maxilla: A report of a rare case literature (2010) J Oral Maxillofac Pathol, 14, pp. 19-23Daniels, J.S.M., Primary hyperparathyroidism presenting as a palatal brown tumor (2004) Oral Surgery, Oral Medicine, Oral Pathology, Oral Radiology and Endodontology, 98 (4), pp. 409-413. , DOI 10.1016/j.tripleo.2004.01.015, PII S1079210404000824Kar, D.K., Gupta, S.K., Agarwal, A., Mishra, S.K., Brown tumor of the palate and mandible in association with primary hyperparathyroidism (2001) Journal of Oral and Maxillofacial Surgery, 59 (11), pp. 1352-1354. , DOI 10.1053/joms.2001.2753