MYH9 disease is a rare genetic disorder in which there is a mutation in the gene for the non-muscle myosin heavy chain IIA. It initially causes macrothrombocytopenia followed by other clinical manifestations. When the patient reaches adulthood, he can develop chronic kidney failure. Thus, the risk of suffering a hemorrhage, difficulty in repairing and, infections increases in individuals with this disease. In addition, the use of drugs in these patients should be carefully evaluated. An adult patient sought dental care with a complaint associated with a tooth with advanced dental caries. He had severe thrombocytopenia (7000 platelets/mm3 ), hearing loss, and chronic kidney failure. The diagnosis of MYH9 disease was confirmed through genotyping. After clinical examination, extraction was planned. Local and systemic procedures were used to prevent hemorrhage, especially postoperatively. Although the patient had an infection at the surgical wound site and no episode of postoperative bleeding, the repair process occurred normally. The purpose of this article is to report the surgical management of a patient with MYH9 disease.
Keywords: Fechtner syndrome; Myh9-related disorder; macrothrombocytopenia with leukocyte inclusions; surgical procedure.
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