Simple Urine Test Could Improve Cystic Fibrosis Treatment
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An easy-to-administer electrolyte test could offer new insight into response to treatment and disease progression for patients with cystic fibrosis. In a new study, researchers found that urine bicarbonate excretion is a potential marker for the function of the cystic fibrosis transmembrane conductance regulator (CFTR), an ion channel that, when defective, causes mucous obstruction, infection, chronic inflammation, and fibrosis. The prospective, observational study, published October 31 in the Annals of Internal Medicine, investigated the association of urine bicarbonate excretion with disease severity and response to treatment with elexacaftor/tezacaftor/ivacaftor (ETI) in 50 Danish adults with CF (39 of whom completed the 6-month protocol).
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@lata ETI has significantly altered CF care by its ability to restore CFTR function to almost normal. This leads to improvement in lung function and quality of life while reducing pulmonary exacerbations and hospitalization. The study suggests that increased excretion of bicarbonate by the kidneys appears to reflect that improvement, according to the researchers.