A carregar...
Publicação
Diagnóstico e intervenção da insuficiência pancreática exócrina em idade pediátrica
| Nome: | Descrição: | Tamanho: | Formato: | |
|---|---|---|---|---|
| 2 MB | Adobe PDF |
Resumo(s)
A Insuficiência Pancreática Exócrina (IPE) é caracterizada por uma diminuição da secreção de enzimas pancreáticas, condicionando malabsorção de micro e macronutrientes. Manifesta-se como malnutrição e sintomatologia gastro-intestinal inespecífica, nomeadamente diarreia/esteatorreia, distensão abdominal e flatulência, associando-se em idade pediátrica, a má progressão estaturo-ponderal. A etiologia da IPE poderá ser primária (pancreática) ou secundária (extra-pancreática). De entre as causas mais frequentes destacam-se a Pancreatite Crónica Primária, a Fibrose Quística e a Síndrome de Schwachman-Diamond. A IPE Transitória é uma entidade raramente descrita, sem etiologia esclarecida, mas que deverá igualmente fazer parte do diagnóstico diferencial de IPE, após exclusão das causas conhecidas. O diagnóstico da IPE inclui a avaliação direta e/ou indireta da função pancreática, sendo o método mais generalizado na prática clínica o doseamento de elastase fecal. A intervenção baseia-se na terapêutica de substituição enzimática, tendo como objetivo a normalização do estado nutricional e o alívio sintomático. O presente trabalho constitui uma revisão narrativa relativa ao diagnóstico e terapêutica da IPE em geral, salientando a importância do seu reconhecimento e diagnóstico diferencial no contexto de esteatorreia e/ou má progressão ponderal em idade pediátrica. Com base na análise de três algoritmos atuais, é feita uma proposta de síntese que inclui recomendações sobre o diagnóstico, terapêutica e seguimento destes doentes.
Exocrine Pancreatic Insufficiency (EPI) is characterized by a decrease in the secretion of pancreatic enzymes, resulting in malabsorption of micro and macronutrients. Its manifestations include malnutrition and unspecific gastro-intestinal symptoms, namely diarrhea/steatorrhea, bloating and flatulence and, in pediatric age, it associates to poor height-weight progression. The etiology of EPI can be primary (pancreatic) or secondary (extra-pancreatic). Among the most frequent causes are Primary Chronic Pancreatitis, Cystic Fibrosis and Schwachman- Diamond Syndrome. Transitory EPI without a clarified etiology is a rarely described entity, but it should also be part of the differential diagnosis of EPI after exclusion of known causes. The diagnosis of EPI includes the direct and/or indirect assessment of pancreatic function, with the most used method in clinical practice being the measurement of fecal elastase. The approach to these patients is based on enzyme replacement therapy and the main goal is to normalize the nutritional status and relieve symptoms. The present work comprises a narrative review regarding the diagnosis and treatment of EPI in general, emphasizing the importance of its recognition and differential diagnosis in the context of steatorrhea and/or poor weight progression in pediatric age. Based on the analysis of three recent algorithms, a summary that includes recommendations on the diagnosis, therapy and follow-up of these patients is presented.
Exocrine Pancreatic Insufficiency (EPI) is characterized by a decrease in the secretion of pancreatic enzymes, resulting in malabsorption of micro and macronutrients. Its manifestations include malnutrition and unspecific gastro-intestinal symptoms, namely diarrhea/steatorrhea, bloating and flatulence and, in pediatric age, it associates to poor height-weight progression. The etiology of EPI can be primary (pancreatic) or secondary (extra-pancreatic). Among the most frequent causes are Primary Chronic Pancreatitis, Cystic Fibrosis and Schwachman- Diamond Syndrome. Transitory EPI without a clarified etiology is a rarely described entity, but it should also be part of the differential diagnosis of EPI after exclusion of known causes. The diagnosis of EPI includes the direct and/or indirect assessment of pancreatic function, with the most used method in clinical practice being the measurement of fecal elastase. The approach to these patients is based on enzyme replacement therapy and the main goal is to normalize the nutritional status and relieve symptoms. The present work comprises a narrative review regarding the diagnosis and treatment of EPI in general, emphasizing the importance of its recognition and differential diagnosis in the context of steatorrhea and/or poor weight progression in pediatric age. Based on the analysis of three recent algorithms, a summary that includes recommendations on the diagnosis, therapy and follow-up of these patients is presented.
Descrição
Trabalho Final do Curso de Mestrado Integrado em Medicina, Faculdade de Medicina, Universidade de Lisboa, 2022
Palavras-chave
Insuficiência pancreática exócrina Terapêutica de substituição enzimática Esteatorreia Elastase fecal Má progressão estaturo-ponderal Pediatria