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Complicações respiratórias da doença de células falciformes em idade pediátrica
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A doença de células falciformes (DCF) é uma das hemoglobinopatias mais comuns associada a doença grave, condicionando elevada morbilidade, para a qual contribuem significativamente as complicações pulmonares. A presente revisão aborda as principais complicações respiratórias, a sua fisiopatologia, apresentação clínica, diagnóstico e tratamento, bem como as formas de prevenção e rastreio.
A síndrome torácica aguda representa a segunda causa mais comum de internamento destas crianças, e a sua recorrência associa-se a um rápido declínio da função pulmonar, representando um fator de risco major para doença pulmonar crónica. Esta população apresenta maior risco para desenvolver perturbação respiratória obstrutiva do sono, sendo esta co-morbilidade, considerada um preditor de risco de complicações como hipertensão pulmonar e episódios isquémicos do sistema nervoso central. A hipertensão pulmonar, mais frequente no final da adolescência, deve-se à progressiva obliteração da vasculatura pulmonar, sendo normalmente assintomática na idade pediátrica. O diagnóstico diferencial de sibilância pode ser difícil, pois pode ser associada à doença de base, representar uma manifestação de asma ou um quadro de síndrome torácica aguda. As alterações obstrutivas da função pulmonar são as mais frequentes nas crianças com doença de células falciformes. A relativa imunodeficiência para agentes capsulados que a asplenia funcional condiciona torna estes doentes particularmente suscetíveis a pneumonia bacteriana. Em conclusão, o diagnóstico precoce, a terapêutica dirigida, a prevenção de infeção e de complicações respiratórias são fundamentais para reduzir a morbilidade e mortalidade associadas a estas complicações.
Sickle cell disease is one of the most common hemoglobinopathies associated with serious disease in the world. Respiratory complications in these children cause significant morbidity and mortality. The present review addresses the main respiratory complications, their pathophysiology, clinical presentation, diagnosis and treatment, as well as their prevention and screening. Acute chest syndrome represents the second most common cause of hospitalization on those children, and its recurrence is associated with a rapid decline in lung function, representing a major risk factor for sickle cell chronic lung disease. This population has a higher risk of developing obstructive sleep-disordered breathing which can be considered as a predictor for increased risk of serious complications such as pulmonary hypertension and central nervous system ischemia. Pulmonary hypertension is more frequently observed in late adolescence and is caused by the progressive obliteration of pulmonary vasculature, being usually asymptomatic in pediatric age. Differential diagnosis of wheezing is difficult as it may be related with the underlying disease, representing a manifestation of asthma or of acute chest syndrome. Early diagnosis, adequate therapy and preventive measures to infection and lung complications are essential in order to reduce the morbidity and mortality.
Sickle cell disease is one of the most common hemoglobinopathies associated with serious disease in the world. Respiratory complications in these children cause significant morbidity and mortality. The present review addresses the main respiratory complications, their pathophysiology, clinical presentation, diagnosis and treatment, as well as their prevention and screening. Acute chest syndrome represents the second most common cause of hospitalization on those children, and its recurrence is associated with a rapid decline in lung function, representing a major risk factor for sickle cell chronic lung disease. This population has a higher risk of developing obstructive sleep-disordered breathing which can be considered as a predictor for increased risk of serious complications such as pulmonary hypertension and central nervous system ischemia. Pulmonary hypertension is more frequently observed in late adolescence and is caused by the progressive obliteration of pulmonary vasculature, being usually asymptomatic in pediatric age. Differential diagnosis of wheezing is difficult as it may be related with the underlying disease, representing a manifestation of asthma or of acute chest syndrome. Early diagnosis, adequate therapy and preventive measures to infection and lung complications are essential in order to reduce the morbidity and mortality.
Descrição
Trabalho Final do Curso de Mestrado Integrado em Medicina, Faculdade de Medicina, Universidade de Lisboa, 2020
Palavras-chave
Doença de células falciformes Síndrome torácica aguda Perturbação respiratória obstrutiva do sono Hipertensão pulmonar Asma Pediatria