外分泌性胰腺功能不全（EPI）是胰腺输送特定胰腺消化酶到肠道的最低/阈值水平失败引起的疾病，导致营养物质和大分子营养素的消化不良，从而导致其不同程度的缺乏。EPI经常被低估诊断，因此患者常常得不到适当的治疗。迫切需要增加对该病状的认识和治疗。美国胃肠病学会（AGA）临床实践更新专家回顾的目标是针对EPI的流行病学、评估和管理提供最佳实践建议。该专家回顾由美国胃肠病学会（AGA）研究所临床实践更新委员会（CPUC）和AGA董事会委托和批准，旨在为AGA会员提供有关临床重要性较高主题的及时指导，并经过了胃肠病学的内部同行评审和标准程序的外部同行评审。这些最佳实践建议陈述是从已发表文献的综述和专家意见中得出的。由于未进行系统综述，这些最佳实践建议陈述不具备正式评价证据质量或考虑因素的强度的权威评级。最佳实践建议1：在高风险临床情况下，应怀疑EPI，如慢性胰腺炎、反复发作的急性胰腺炎、胰腺导管腺癌、囊性纤维化以及先前胰腺手术。最佳实践建议2：在中度风险临床情况下，应考虑EPI，如十二指肠疾病，包括乳糜泻和克罗恩病；先前肠道手术；长期存在的糖尿病；以及高分泌状态（例如，佐林格-爱立信综合征）。最佳实践建议3：EPI的临床特点包括伴或不伴腹泻的脂肪粪便、体重减轻、腹胀、过度胀气、脂溶性维生素缺乏和蛋白质-热量营养不良。最佳实践建议4：粪便弹性蛋白酶测试是最合适的初步检测方法，必须在半固态或固体粪便样本上进行。粪便弹性蛋白酶水平<100 μg/g大便表示EPI的很好证据，而100-200 μg/g的水平对EPI的诊断具有不确定性。最佳实践建议5：在进行胰酶替代治疗时，可以进行粪便弹性蛋白酶测试。最佳实践建议6：极少需要粪便脂肪测试，只有在高脂饮食时才需要进行。定量测试通常不适合常规临床使用。最佳实践建议7：对于EPI的诊断，治疗试验的反应不可靠。最佳实践建议8：断层扫描成像方法（计算机断层扫描、磁共振成像和内镜超声）不能识别EPI，但在良性和恶性胰腺疾病的诊断中起重要作用。最佳实践建议9：呼气检测和直接胰腺功能测试具有潜力，但在美国并不常见。最佳实践建议10：一旦确诊为EPI，就需要进行胰酶替代治疗（PERT）。如果不治疗EPI，将导致与脂肪吸收不良和营养不良相关的并发症，对生活质量有负面影响。最佳实践建议11：PERT的制剂全部来自猪源，等效剂量下效果相同。非肠溶包衣制剂需要与H2受体拮抗剂或质子泵抑制剂联合使用。最佳实践建议12：PERT应在进餐期间服用，成人每餐最初治疗剂量至少为40,000 USP单位脂酶，零食时的剂量为原先的一半。随后的剂量可以根据餐量和脂肪含量进行调整。最佳实践建议13：常规补充和监测脂溶性维生素水平是适当的。饮食修改包括低至中等脂肪饮食，频繁的小餐和避免非常低脂肪饮食。最佳实践建议14：判定PERT治疗成功的指标包括减少脂肪粪便和相关的胃肠症状；体重、肌肉质量和肌功能的增长；以及脂溶性维生素水平的改善。最佳实践建议15：应监测EPI，并获取营养状况的基线测量（Body Mass Index，生活质量测量和脂溶性维生素水平）。应进行基线双能量X线吸收测定扫描，每1-2年重测一次。版权 © 2023作者。由Elsevier Inc.出版。保留所有权利。

Exocrine pancreatic insufficiency (EPI) is a disorder caused by the failure of the pancreas to deliver a minimum/threshold level of specific pancreatic digestive enzymes to the intestine, leading to the maldigestion of nutrients and macronutrients, resulting in their variable deficiencies. EPI is frequently underdiagnosed and, as a result, patients are often not treated appropriately. There is an urgent need to increase awareness of and treatment for this condition. The aim of this American Gastroenterological Association (AGA) Clinical Practice Update Expert Review was to provide Best Practice Advice on the epidemiology, evaluation, and management of EPI.This Expert Review was commissioned and approved by the American Gastroenterological Association (AGA) Institute Clinical Practice Updates Committee (CPUC) and the AGA Governing Board to provide timely guidance on a topic of high clinical importance to the AGA membership, and underwent internal peer review by the CPUC and external peer review through standard procedures of Gastroenterology. These Best Practice Advice statements were drawn from a review of the published literature and from expert opinion. Because systematic reviews were not performed, these Best Practice Advice statements do not carry formal ratings regarding the quality of evidence or strength of the presented considerations. Best Practice Advice Statements BEST PRACTICE ADVICE 1: EPI should be suspected in patients with high-risk clinical conditions, such as chronic pancreatitis, relapsing acute pancreatitis, pancreatic ductal adenocarcinoma, cystic fibrosis, and previous pancreatic surgery. BEST PRACTICE ADVICE 2: EPI should be considered in patients with moderate-risk clinical conditions, such as duodenal diseases, including celiac and Crohn's disease; previous intestinal surgery; longstanding diabetes mellitus; and hypersecretory states (eg, Zollinger-Ellison syndrome). BEST PRACTICE ADVICE 3: Clinical features of EPI include steatorrhea with or without diarrhea, weight loss, bloating, excessive flatulence, fat-soluble vitamin deficiencies, and protein-calorie malnutrition. BEST PRACTICE ADVICE 4: Fecal elastase test is the most appropriate initial test and must be performed on a semi-solid or solid stool specimen. A fecal elastase level <100 μg/g of stool provides good evidence of EPI, and levels of 100-200 μg/g are indeterminate for EPI. BEST PRACTICE ADVICE 5: Fecal elastase testing can be performed while on pancreatic enzyme replacement therapy. BEST PRACTICE ADVICE 6: Fecal fat testing is rarely needed and must be performed when on a high-fat diet. Quantitative testing is generally not practical for routine clinical use. BEST PRACTICE ADVICE 7: Response to a therapeutic trial of pancreatic enzymes is unreliable for EPI diagnosis. BEST PRACTICE ADVICE 8: Cross-sectional imaging methods (computed tomography scan, magnetic resonance imaging, and endoscopic ultrasound) cannot identify EPI, although they play an important role in the diagnosis of benign and malignant pancreatic disease. BEST PRACTICE ADVICE 9: Breath tests and direct pancreatic function tests hold promise, but are not widely available in the United States. BEST PRACTICE ADVICE 10: Once EPI is diagnosed, treatment with pancreatic enzyme replacement therapy (PERT) is required. If EPI is left untreated, it will result in complications related to fat malabsorption and malnutrition, having a negative impact on quality of life. BEST PRACTICE ADVICE 11: PERT formulations are all derived from porcine sources and are equally effective at equivalent doses. There is a need for H2 or proton pump inhibitor therapy with non-enteric-coated preparations. BEST PRACTICE ADVICE 12: PERT should be taken during the meal, with the initial treatment of at least 40,000 USP units of lipase during each meal in adults and one-half of that with snacks. The subsequent dosage can be adjusted based on the meal size and fat content. BEST PRACTICE ADVICE 13: Routine supplementation and monitoring of fat-soluble vitamin levels are appropriate. Dietary modifications include a low-moderate fat diet with frequent smaller meals and avoiding very-low-fat diets. BEST PRACTICE ADVICE 14: Measures of successful treatment with PERT include reduction in steatorrhea and associated gastrointestinal symptoms; a gain of weight, muscle mass, and muscle function; and improvement in fat-soluble vitamin levels. BEST PRACTICE ADVICE 15: EPI should be monitored and baseline measurements of nutritional status should be obtained (body mass index, quality-of-life measure, and fat-soluble vitamin levels). A baseline dual-energy x-ray absorptiometry scan should be obtained and repeated every 1-2 years.Copyright © 2023 The Authors. Published by Elsevier Inc. All rights reserved.