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Doença hepática associada à fibrose quística
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A Doença Hepática associada à Fibrose Quística é uma morbilidade crescente nos doentes com Fibrose Quística, manifestando-se ainda na idade pediátrica. É mais prevalente no sexo masculino e está associado às mutações severas (classe I-III) com insuficiência pancreática. O curso é frequentemente assintomático e, portanto, é importante o seguimento destes doentes por uma equipa multidisciplinar, com screening regular através de exame físico, exames laboratoriais e imagiológicos. Dada a pouca sensibilidade e especificidade dos exames na detecção precoce da doença, novos métodos não invasivos têm sido desenvolvidos nas últimas décadas, com resultados promissores nomeadamente no que toca ao Fibroscan. Desse modo poderão ser identificados os doentes que beneficiem de terapêutica dirigida, nomeadamente com UDCA 15-20 mg/kg/dia. Apenas uma pequena parcela de doentes (5-10%) desenvolve cirrose hepática e hipertensão portal.
Liver disease associated with Cystic Fibrosis is an increasing morbidity in patients with this last pathology, manifesting itself from an early age. It is more prevalent among the male gender and frequently associated with severe mutations (class I-III) with pancreatic insufficiency. The course is often asymptomatic therefore enhancing the importance of a close follow up of these patients engaged by a multidisciplinary team, with regular screening through physical examination, laboratory and imaging exams. Given the low sensitivity and specificity of the tests preventing our early detection of the disease, new noninvasive methods have been developed in the past decades, with promising results particularly regarding the Fibroscan. Thus, patients who might benefit from receiving targeted therapy, namely with UDCA 15-20 mg/kg/day, can be promptly identified. Only a small percentage of patients (5-10%) develop cirrhosis and portal hypertension.
Liver disease associated with Cystic Fibrosis is an increasing morbidity in patients with this last pathology, manifesting itself from an early age. It is more prevalent among the male gender and frequently associated with severe mutations (class I-III) with pancreatic insufficiency. The course is often asymptomatic therefore enhancing the importance of a close follow up of these patients engaged by a multidisciplinary team, with regular screening through physical examination, laboratory and imaging exams. Given the low sensitivity and specificity of the tests preventing our early detection of the disease, new noninvasive methods have been developed in the past decades, with promising results particularly regarding the Fibroscan. Thus, patients who might benefit from receiving targeted therapy, namely with UDCA 15-20 mg/kg/day, can be promptly identified. Only a small percentage of patients (5-10%) develop cirrhosis and portal hypertension.
Descrição
Trabalho Final do Curso de Mestrado Integrado em Medicina, Faculdade de Medicina, Universidade de Lisboa, 2017
Palavras-chave
Fibrose quística Doença hepática Cirrose Pediatria