Loading...
Publication
Diagnóstico e terapêutica de complicações agudas da drepanocitose no serviço de urgência
| Name: | Description: | Size: | Format: | |
|---|---|---|---|---|
| 364.14 KB | Adobe PDF |
Authors
Advisor(s)
Abstract(s)
Introdução: A drepanocitose é a doença monogénica mais prevalente no mundo, com
aproximadamente 300 000 novos casos anualmente e afetando cerca de 30 milhões de
indivíduos. Apesar da sua maior prevalência entre populações africanas, representa um
problema de saúde global, reconhecido como prioridade de saúde pública pela
Organização Mundial de Saúde desde 2006. A drepanocitose é caracterizada por
complicações agudas recorrentes, incluindo crises vaso-oclusivas, síndrome torácica
aguda, acidente vascular cerebral, priapismo, sequestro esplénico agudo, infeção aguda
e crise aplásica. Esses eventos contribuem significativamente para a morbidade e
mortalidade da doença, frequentemente exigindo intervenções no serviço de urgência.
Objetivo: Esta revisão de literatura tem como objetivo fornecer uma síntese
abrangente sobre as abordagens diagnósticas e terapêuticas para as complicações
agudas da drepanocitose no serviço de urgência, com foco na melhoria dos desfechos
clínicos.
Métodos: Foi realizada uma pesquisa bibliográfica nas bases de dados PubMed e
Google Scholar, complementada por diretrizes da American Society of Hematology,
American Stroke Association, American Pain Society e American Urological
Association. Os termos de pesquisa incluíram "sickle cell disease" combinados com
complicações específicas. Os critérios de inclusão priorizaram artigos em inglês e
português, sem restrições de data.
Resultados: As complicações agudas da drepanocitose contribuem significativamente
para a morbidade e mortalidade. A crise vaso-oclusiva é a manifestação clínica mais
comum, caracterizada por dor intensa e potencial dano orgânico. A síndrome torácica
aguda, a principal causa de morte em crianças e adultos com a doença, apresenta-se
com sintomas respiratórios e manifestações sistémicas. O acidente vascular cerebral
afeta 25 a 30% dos pacientes, sendo o isquémico mais frequente em crianças e o
hemorrágico mais frequente em adultos. O priapismo, predominantemente isquémico,
está associado a alta morbidade e disfunção erétil. O sequestro esplénico agudo e as
infeções agudas exigem intervenção imediata para prevenir desfechos fatais, enquanto
a crise aplásica, frequentemente associada ao parvovírus B19, resulta em anemia grave
e reticulocitopenia. Conclusão: A drepanocitose constitui um problema de saúde pública global, com
complicações agudas que requerem diagnóstico rápido e tratamento eficaz no serviço
de urgência para reduzir a morbidade e a mortalidade. Apesar dos avanços científicos, a
ausência de marcadores específicos e a falta de diretrizes consensuais dificultam a
padronização das práticas clínicas. Protocolos baseados em evidências robustas e um
acompanhamento multidisciplinar são fundamentais para melhorar os desfechos
clínicos e a qualidade de vida dos doentes.
Introduction: Sickle cell disease is the most prevalent monogenic disorder in the world, with approximately 300 000 new cases annually, affecting around 30 million individuals. Despite its higher prevalence among African populations, it represents a global health concern, recognized as a public health priority by the World Health Organization since 2006. Sickle cell disease is characterized by recurrent acute complications, including vaso-occlusive crises, acute chest syndrome, stroke, priapism, acute splenic sequestration, acute infection, and aplastic crisis. These events contribute significantly to the disease’s morbidity and mortality, often requiring emergency care interventions. Objective: This literature review aims to provide a comprehensive synthesis of diagnostic and therapeutic approaches for acute complications of sickle cell disease in the emergency department, focusing on improving clinical outcomes. Methods: A literature search was conducted in PubMed and Google Scholar databases, complemented by guidelines from the American Society of Hematology, American Stroke Association, American Pain Society, and American Urological Association. Search terms included "sickle cell disease" combined with specific complications. The inclusion criteria prioritized articles in English and Portuguese, without date restrictions. Results: Acute complications of sickle cell disease contribute significantly to morbidity and mortality. Vaso-occlusive crisis is the most common clinical manifestation, characterized by intense pain and potential organic damage. Acute chest syndrome, the main cause of death in children and adults with the disease, presents with respiratory symptoms and systemic manifestations. Stroke affects 25 to 30% of patients, with ischemic stroke being more common in children and hemorrhagic stroke more common in adults. Priapism, predominantly ischemic, is associated with high morbidity and erectile dysfunction. Acute splenic sequestration and acute infections require immediate intervention to prevent fatal outcomes, while aplastic crisis, often associated with parvovirus B19, results in severe anemia and reticulocytopenia. Conclusion: Sickle cell disease is a global public health concern, with acute complications that require rapid diagnosis and effective treatment in the emergency department to reduce morbidity and mortality. Despite scientific advances, the absence of specific markers and the lack of consensual guidelines make it difficult to standardize clinical practices. Protocols based on robust evidence and multidisciplinary monitoring are essential to improve clinical outcomes and patients' quality of life.
Introduction: Sickle cell disease is the most prevalent monogenic disorder in the world, with approximately 300 000 new cases annually, affecting around 30 million individuals. Despite its higher prevalence among African populations, it represents a global health concern, recognized as a public health priority by the World Health Organization since 2006. Sickle cell disease is characterized by recurrent acute complications, including vaso-occlusive crises, acute chest syndrome, stroke, priapism, acute splenic sequestration, acute infection, and aplastic crisis. These events contribute significantly to the disease’s morbidity and mortality, often requiring emergency care interventions. Objective: This literature review aims to provide a comprehensive synthesis of diagnostic and therapeutic approaches for acute complications of sickle cell disease in the emergency department, focusing on improving clinical outcomes. Methods: A literature search was conducted in PubMed and Google Scholar databases, complemented by guidelines from the American Society of Hematology, American Stroke Association, American Pain Society, and American Urological Association. Search terms included "sickle cell disease" combined with specific complications. The inclusion criteria prioritized articles in English and Portuguese, without date restrictions. Results: Acute complications of sickle cell disease contribute significantly to morbidity and mortality. Vaso-occlusive crisis is the most common clinical manifestation, characterized by intense pain and potential organic damage. Acute chest syndrome, the main cause of death in children and adults with the disease, presents with respiratory symptoms and systemic manifestations. Stroke affects 25 to 30% of patients, with ischemic stroke being more common in children and hemorrhagic stroke more common in adults. Priapism, predominantly ischemic, is associated with high morbidity and erectile dysfunction. Acute splenic sequestration and acute infections require immediate intervention to prevent fatal outcomes, while aplastic crisis, often associated with parvovirus B19, results in severe anemia and reticulocytopenia. Conclusion: Sickle cell disease is a global public health concern, with acute complications that require rapid diagnosis and effective treatment in the emergency department to reduce morbidity and mortality. Despite scientific advances, the absence of specific markers and the lack of consensual guidelines make it difficult to standardize clinical practices. Protocols based on robust evidence and multidisciplinary monitoring are essential to improve clinical outcomes and patients' quality of life.
Description
Keywords
Analgesia Anemia Falciforme Complicações Agudas Crise Vaso-Oclusiva Drepanocitose