Abstract：
Exocrine pancreatic insufficiency (EPI) is highly prevalent among individuals with cystic fibrosis (CF). Individuals diagnosed with EPI are often labeled as having \"pancreas insufficient cystic fibrosis (PI-CF)\" while those with normal exocrine function are labeled as \"pancreas sufficient CF (PS-CF).\" This diagnosis of EPI relies on clinical and laboratory features and management involves consumption of pancreas enzyme replacement therapy. In this review, we discuss the nuances of diagnosis and management of EPI in CF. We also present emerging evidence on the effects of CFTR modulating agents on the management of EPI, and speculate that these medications may lead to greater heterogeneity in management of EPI in CF moving forward.
摘要：
胰腺外分泌功能不全（EPI）在囊性纤维化（CF）患者中非常普遍。诊断为EPI的人通常被标记为“胰腺囊性纤维化不足（PI-CF）”，而外分泌功能正常的人则被标记为“胰腺足够的CF（PS-CF）”。“EPI的诊断依赖于临床和实验室特征，管理涉及胰腺酶替代疗法的消耗。在这次审查中,我们讨论了CF中EPI的诊断和管理的细微差别。我们还提出了关于CFTR调节剂对EPI管理的影响的新证据，并推测这些药物可能导致CF中EPI管理的更大异质性。
