Update on the diagnosis and management of exocrine pancreatic insufficiency.
Update on the diagnosis and management of exocrine pancreatic insufficiency.
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DOI：
10.12688/f1000research.20779.1
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发表时间：
2019-01-01
期刊：
影响因子：
--
通讯作者：
Forsmark, Chris E
中科院分区：
文献类型：
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作者：
Perbtani, Yaseen;Forsmark, Chris E
胰腺外分泌功能不全（EPI）的特征在于胰腺酶向小肠的递送不足。胰腺外分泌功能不全（EPI）的特征在于胰腺酶向小肠的递送不足，导致吸收不良。EPI的临床表现往往是非特异性的，可导致缺乏及时识别和诊断。这种临床困境的核心是缺乏高度准确或特异性的测试，这导致误诊和次优治疗。识别高风险患者是EPI诊断的关键，这包括胰腺实质疾病患者，如慢性胰腺炎、胰腺恶性肿瘤、囊性纤维化，以及因良性和恶性疾病接受胰腺切除术的患者。较少认识到的是一些额外的条件，也可能有EPI作为一个结果。由于发病率的增加和与这种情况相关的生活质量受损，治疗目标旨在通过口服胰酶替代疗法（PERT）补充外分泌酶缺乏症。PERT的基础是在进餐期间向小肠提供活化的消化酶，主要是导致脂肪和脂溶性维生素的充分吸收。PERT的益处已被证明超出了与EPI相关的体征和症状的改善，包括降低骨病的患病率和改善患有这种疾病的患者亚组的生存结局。然而，尽管在治疗的整体效益，EPI的诊断和管理是次优的。目前的文献表明，处于发展EPI高风险的患者没有进行测试，并且那些被诊断出的患者没有接受足够剂量的治疗。在这篇综述中，我们重点介绍了发生EPI高风险的患者，分析了这种疾病的后果和治疗，回顾了酶替代疗法的原理，并检查了治疗优化的当前证据。
Exocrine pancreatic insufficiency (EPI) is characterized by inadequate pancreatic enzyme delivery to the small intestine Exocrine pancreatic insufficiency (EPI) is characterized by inadequate pancreatic enzyme delivery to the small intestine, resulting in malabsorption. Clinical manifestations of EPI are often nonspecific and can lead to lack of timely recognition and diagnosis. Central to this clinical dilemma is the lack of highly accurate or specific testing which leads to misdiagnosis and suboptimal treatment. Identification of high-risk patients is key in the diagnosis of EPI and this includes patients with pancreatic parenchyma disorders such as chronic pancreatitis, pancreatic malignancy, cystic fibrosis, and those undergoing pancreatic resection for benign and malignant disease. Less recognized are the number of additional conditions which may also have EPI as a consequence. Owing to an increase in morbidity and impaired quality of life associated with this condition, goals of treatment have been aimed at repleting exocrine enzyme deficiency by oral pancreatic enzyme replacement therapy (PERT). The basis of PERT is to provide activated digestive enzymes to the small bowel during the prandial period, mainly, leading to sufficient absorption of fat and fat-soluble vitamins. The benefits of PERT have been shown to go beyond the improvement in signs and symptoms associated with EPI and include decreasing prevalence of osteopathy and improving survival outcomes in subsets of patients with this condition. However, despite the overall benefits in treatment, the diagnosis and management of EPI are suboptimal. Current literature suggests patients at high risk of developing EPI are not tested and those who are diagnosed are not treated with adequate dosages. In this review, we highlight patients who are at high risk for the development of EPI, analyze consequences and treatment of this disorder, review rationale for enzyme replacement therapy, and examine current evidence for treatment optimization.