3 resultados para OSTEOPETROSIS

em Repositório Institucional UNESP - Universidade Estadual Paulista "Julio de Mesquita Filho"


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Osteopetrosis is a rare hereditary condition characterized by increased bone density. The jaws, bones, and teeth invariably are affected and the osteopetrosis is directly proportional with the severity of the disease. This article describes a clinical case of osteopetrosis and reviews the clinicopathologic, radiographic, and treatment features.

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The clinical, radiological, pathological and laboratory findings of two brothers with autosomal recessive malignant osteopetrosis are presented. Our findings are similar to characteristics previously reported in the literature about patients with osteopetrosis. The 6-year-old male patient was pale and had petechiae on his arms and legs. He also had macrocephalia, splenomegaly, severe pancytopenia, genu valgus, hypocalcemia, amaurosis, cessation of physical development, generalized bone sclerosis and recurrent infections with a history of multiple incidences of acute otitis media. Generalized bone sclerosis and irregular sclerosis of the maxilla and mandible were seen on radiographs. The oral mucosa was apparently normal but permanent tooth eruption was delayed although there was early loss of deciduous teeth. The recommended treatment was blood transfusion and therapy with antibiotics when necessary; a bone marrow transplant was not possible due to lack of a compatible donor.

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Osteopetrosis (OP) is a rare hereditary disorder characterized by a dysfunction of the osteoclasts that impairs bone resorption, which together with the normal osteoblastic activity forms intense bone sclerosis with reduction of marrow. A common complication that arises, most frequently, as a result of tooth extraction is mandibular osteomyelitis. There is no consensus on the literature about the treatment of this infection in an osteopetrotic patient, therefore, the purpose of this paper is to report a case of marginal resection for treatment of mandibular osteomyelitis in an osteopetrotic patient and discuss relevant features of this procedure. © 2010 European Association for Cranio-Maxillo-Facial Surgery.