Intraparenchymal cerebral hemorrhage in patient with Christmas disease
Autor(a) principal: | |
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Data de Publicação: | 2017 |
Outros Autores: | , , , |
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 |
Resumo: | 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. |
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Intraparenchymal cerebral hemorrhage in patient with Christmas diseaseHemorragia cerebral intraparenquimatosa em paciente com doença de ChristmasAcidente Vascular CerebralHematologiaHemofilia BTranstornos HemorrágicosMedicina ClínicaClinical MedicineHematologyHemophilia BHemorrhagic DisordersStrokeIntraparenchymal 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.A hemorragia cerebral intraparenquimatosa (HIP) ou acidente vascular cerebral hemorrágico (AVCH) é uma situação clínica na qual ocorre uma lesão vascular seguida de hemorragia cerebral, que normalmente possui elevada morbimortalidade. Um dos fatores que predispõe à HIP são as diáteses hemorrágicas, como hemofilia. A Doença de Christmas ou hemofilia B é uma doença genética recessiva ligada ao cromossomo X causada por mutações no gene F9, que codifica o fator de coagulação IX. A gravidade da doença está intimamente relacionada com a concentração em percentual do fator no sangue do paciente. O objetivo é relatar a ocorrência de hemorragia cerebral intraparenquimatosa em paciente com doença de Christmas. Foi feita uma avaliação do prontuário do paciente, que foi baseado em anamnese e exames laboratoriais e de imagem (tomografia computadorizada). Sexo masculino, 63 anos, caucasiano, tabagista e etilista crônico. Diagnosticado com hemofilia B moderada, em uso domiciliar de Fator IX sob demanda. Relata queda de própria altura há 1 dia após libação alcóolica, evoluindo com incapacidade de marcha, incoordenação motora principalmente à direita, desorientação temporoespacial e descontrole pressórico (180x100mmHg). Solicitada tomografia computadorizada (TC) de crânio no ato da internação, evidenciou-se área núcleo-capsular esquerda hiperdensa, compatível com sangramento. O tratamento baseou-se em controle da pressão arterial com anti-hipertensivos e aminas vasoativas. A administração do fator IX foi elevada a 100% durante 7 dias, logo após foi reduzida a 50% como dose de manutenção. Apesar da comorbidade apresentada pelo paciente ser um fator agravante, a conduta preconizada pelo Ministério da Saúde para a estabilização do paciente hemofílico, bem como a conduta padrão para os pacientes acometidos pelo acidente vascular encefálico hemorrágico foram altamente eficazes no tratamento e recuperação do paciente, que apesar disso teve uma recuperação satisfatória. A HIP possui clínica bem definida, porém seu manejo é altamente complexo, principalmente nos pacientes com outras comorbidades. O diagnóstico rápido, bem como o tratamento adequado são cruciais na recuperação dos pacientes, diminuindo assim os índices de mortalidade de pessoas acometidas por ela. Faculdade de Medicina de Campos (FMC)2017-12-15info:eu-repo/semantics/articleinfo:eu-repo/semantics/publishedVersionapplication/pdfhttps://www.fmc.br/ojs/index.php/RCFMC/article/view/10110.29184/1980-7813.rcfmc.101.vol.12.n3.2017Scientific Journal of the Medical School of Campos; Vol. 12 No. 3 (2017)Revista Científica da Faculdade de Medicina de Campos; v. 12 n. 3 (2017)1980-7813reponame:Revista Científica da Faculdade de Medicina de Camposinstname:Faculdade de Medicina de Campos (FMC)instacron:FMCporhttps://www.fmc.br/ojs/index.php/RCFMC/article/view/101/159Copyright (c) 2017 Revista Científica da Faculdade de Medicina de Camposinfo:eu-repo/semantics/openAccessBernardo, Thiago TavaresFadlallah, Abner Davidde Carvalho, Gabriela MedeirosBorges, Camila de FariaFadlallah, Jussara David2021-01-06T19:42:12Zoai:ojs.www.fmc.br:article/101Revistahttps://www.fmc.br/ojs/index.php/RCFMC/PRIhttps://www.fmc.br/ojs/index.php/RCFMC/oai||revista@fmc.br1980-78131980-7813opendoar:2021-01-06T19:42:12Revista Científica da Faculdade de Medicina de Campos - Faculdade de Medicina de Campos (FMC)false |
dc.title.none.fl_str_mv |
Intraparenchymal cerebral hemorrhage in patient with Christmas disease Hemorragia cerebral intraparenquimatosa em paciente com doença de Christmas |
title |
Intraparenchymal cerebral hemorrhage in patient with Christmas disease |
spellingShingle |
Intraparenchymal cerebral hemorrhage in patient with Christmas disease Bernardo, Thiago Tavares Acidente Vascular Cerebral Hematologia Hemofilia B Transtornos Hemorrágicos Medicina Clínica Clinical Medicine Hematology Hemophilia B Hemorrhagic Disorders Stroke |
title_short |
Intraparenchymal cerebral hemorrhage in patient with Christmas disease |
title_full |
Intraparenchymal cerebral hemorrhage in patient with Christmas disease |
title_fullStr |
Intraparenchymal cerebral hemorrhage in patient with Christmas disease |
title_full_unstemmed |
Intraparenchymal cerebral hemorrhage in patient with Christmas disease |
title_sort |
Intraparenchymal cerebral hemorrhage in patient with Christmas disease |
author |
Bernardo, Thiago Tavares |
author_facet |
Bernardo, Thiago Tavares Fadlallah, Abner David de Carvalho, Gabriela Medeiros Borges, Camila de Faria Fadlallah, Jussara David |
author_role |
author |
author2 |
Fadlallah, Abner David de Carvalho, Gabriela Medeiros Borges, Camila de Faria Fadlallah, Jussara David |
author2_role |
author author author author |
dc.contributor.author.fl_str_mv |
Bernardo, Thiago Tavares Fadlallah, Abner David de Carvalho, Gabriela Medeiros Borges, Camila de Faria Fadlallah, Jussara David |
dc.subject.por.fl_str_mv |
Acidente Vascular Cerebral Hematologia Hemofilia B Transtornos Hemorrágicos Medicina Clínica Clinical Medicine Hematology Hemophilia B Hemorrhagic Disorders Stroke |
topic |
Acidente Vascular Cerebral Hematologia Hemofilia B Transtornos Hemorrágicos Medicina Clínica Clinical Medicine Hematology Hemophilia B Hemorrhagic Disorders Stroke |
description |
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. |
publishDate |
2017 |
dc.date.none.fl_str_mv |
2017-12-15 |
dc.type.driver.fl_str_mv |
info:eu-repo/semantics/article info:eu-repo/semantics/publishedVersion |
format |
article |
status_str |
publishedVersion |
dc.identifier.uri.fl_str_mv |
https://www.fmc.br/ojs/index.php/RCFMC/article/view/101 10.29184/1980-7813.rcfmc.101.vol.12.n3.2017 |
url |
https://www.fmc.br/ojs/index.php/RCFMC/article/view/101 |
identifier_str_mv |
10.29184/1980-7813.rcfmc.101.vol.12.n3.2017 |
dc.language.iso.fl_str_mv |
por |
language |
por |
dc.relation.none.fl_str_mv |
https://www.fmc.br/ojs/index.php/RCFMC/article/view/101/159 |
dc.rights.driver.fl_str_mv |
Copyright (c) 2017 Revista Científica da Faculdade de Medicina de Campos info:eu-repo/semantics/openAccess |
rights_invalid_str_mv |
Copyright (c) 2017 Revista Científica da Faculdade de Medicina de Campos |
eu_rights_str_mv |
openAccess |
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application/pdf |
dc.publisher.none.fl_str_mv |
Faculdade de Medicina de Campos (FMC) |
publisher.none.fl_str_mv |
Faculdade de Medicina de Campos (FMC) |
dc.source.none.fl_str_mv |
Scientific Journal of the Medical School of Campos; Vol. 12 No. 3 (2017) Revista Científica da Faculdade de Medicina de Campos; v. 12 n. 3 (2017) 1980-7813 reponame:Revista Científica da Faculdade de Medicina de Campos instname:Faculdade de Medicina de Campos (FMC) instacron:FMC |
instname_str |
Faculdade de Medicina de Campos (FMC) |
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FMC |
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FMC |
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Revista Científica da Faculdade de Medicina de Campos |
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Revista Científica da Faculdade de Medicina de Campos |
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Revista Científica da Faculdade de Medicina de Campos - Faculdade de Medicina de Campos (FMC) |
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