Intraparenchymal cerebral hemorrhage in patient with Christmas disease

• Autor(a) principal: Bernardo, Thiago Tavares
• Data de Publicação: 2017
• Outros Autores: Fadlallah, Abner David, de Carvalho, Gabriela Medeiros, Borges, Camila de Faria, Fadlallah, Jussara David
• Tipo de documento: Artigo
• Idioma: por
• Título da fonte: Revista Científica da Faculdade de Medicina de Campos
• Texto Completo: https://www.fmc.br/ojs/index.php/RCFMC/article/view/101
• topic: Acidente Vascular Cerebral; Hematologia; Hemofilia B; Transtornos Hemorrágicos; Medicina Clínica; Clinical Medicine; Hematology; Hemophilia B; Hemorrhagic Disorders; Stroke

Intraparenchymal cerebral hemorrhage (ICH) or hemorrhagic stroke (HS) is a clinical situation in which a vascular injury followed by cerebral hemorrhage, which usually has high morbidity and mortality, occurs. One of the predisposing factors to ICH is hemorrhagic diathesis, such as hemophilia. Christmas disease or hemophilia B is a X-linked recessive genetic disease caused by mutations in the F9 gene, which encodes the coagulation factor IX. The severity of the disease is closely related to the percentage concentration of factor in the patient's blood.  The objective is to report the occurrence of intraparenchymal cerebral hemorrhage in a patient with Christmas disease. An evaluation of the patient's chart was made, based on anamnesis, laboratory and imaging tests (computed tomography). Male, 63 years old, caucasian, smoker and chronic alcohol user. Diagnosed with moderate B hemophilia, in Factor IX on-demand home use. He reports a fall of one's height 1 day after alcoholic libation, evolving with inability to walk, motor incoordination mainly to the right, temporo-spatial disorientation and pressure deregulation (180x100mmHg). Computed tomography (CT) examination of the skull at the time of admission was evidenced hyperdense left core-capsular area, compatible with bleeding. The treatment was based on blood pressure control with antihypertensive and vasoactive amines. Factor IX administration was elevated to 100% for 7 days, shortly thereafter reduced to 50% as maintenance dose. Although the comorbidity presented by the patient is an aggravating factor, the conduct recommended by the Ministry of Health (Ministério da Saúde) for the stabilization of the hemophiliac patient, as well as the standard conduct for the patients affected by the hemorrhagic stroke were highly effective in the treatment and recovery of the patient , which nevertheless had a satisfactory recovery. ICH has a well-defined clinical course, but its management is highly complex, especially in patients with other comorbidities. Rapid diagnosis as well as adequate treatment are crucial in patient recovery, thus reducing the mortality rates of people affected by it.