Probiotics treatment for the prevention of cystic fibrosis-related liver disease

Mr. Dijkstra, 23 years old, is affected by cystic fibrosis (CF). Besides frequent pulmonary exacerbations, Mr. Dijkstra also has exocrine pancreatic insufficiency, frequent abdominal pain and nasal polyps. Mr. Dijkstra is followed at the CF policlinic for adults at the UMCG, where he receives a checkup every 3 months. For the last 6 years, Mr. Dijkstra has had elevated liver enzymes and increased echogenicity on liver echography. He was told that he may have a mild degree of cystic-fibrosis liver disease (CFLD) and he was given ursodeoxycholic acid (UDCA) 20 mg/kg/day. He was told that this treatment could perhaps prevent further progression of his liver disease. However, this year, his yearly liver echography brought bad news: Mr. Dijkstra had developed liver cirrhosis.
Mr. Dijkstra developed progression of CFLD despite treatment with UDCA. Mr. Dijkstra was handled according to the UMCG CF guidelines. These guidelines do not report any other prophylactic or curative treatment for CFLD than UDCA, and further specify that treatment with UDCA is controversial.
Research question
We reviewed the literature concerning the management of CFLD and hypothesized that a possible preventative treatment for CFLD could be probiotics. Our research question is: “Can treatment with probiotics delay the development of CFLD?”



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