Cystic Fibrosis Foundation Evidence-based Guidelines for the Management of CRMS/CFSPID

These evidence-based guidelines were created by a multidisciplinary committee to help cystic fibrosis specialists and primary care providers who care for people with CFTR-related metabolic disorder and their families. It should supplement the standard care provided in primary care. 

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These evidence-based guidelines were created by a multidisciplinary committee to help cystic fibrosis specialists and primary care providers who care for people with CFTR-related metabolic disorder (CRMS) and their families. It should supplement the standard care provided in primary care. The guideline will not address how CRMS/cystic fibrosis screen positive, inconclusive diagnosis (CFSPID) is diagnosed, nor criteria for reclassifying people with CRMS as CF since recommendations already exist. It will, however, address genetic testing to better refine the diagnosis. The European CF Society (ECFS) recently published a consensus guidance. These guidelines are evidence-based and are intended to complement the ECFS paper.

Green DM, Lahiri T, Raraigh KS, et al. Cystic Fibrosis Foundation Evidence-based Guidelines for the Management of CRMS/CFSPID. Pediatrics. 2024. Apr 5. 153(5):e2023064657 doi: 10.1542/peds.2023-064657. Online ahead of print.

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