Lindley Keith J
Pancreatic and Gastroenterology Services, UCL Institute of Child Health and Great Ormond Street Hospital for Children, 30 Guilford Street, London WC1N 1EH, United Kingdom.
Indian J Pediatr. 2006 Oct;73(10):907-12. doi: 10.1007/BF02859284.
Chronic pancreatitis (CP) is characterised by pancreatic inflammation and fibrosis leading eventually to destruction of pancreatic parenchyma and loss of exocrine and endocrine function. A model of interactions between environmental triggers of pancreatic inflammation and disease susceptibility or modifying genes (including PRSS1, SPINK1 and CFTR) provides a framework within which to understand disease pathogenesis. Early in the disease, when fibrosis is mild and pancreatic damage limited, it is difficult to distinguish CP from recurrent acute pancreatitis (RAP) although it is likely these represent opposite ends of a spectrum of disease with a common aetiology in which CP represents either a later disease stage or disease in individuals predisposed to generate a chronic fibrogenic inflammatory response. Pain is a dominant feature resulting in part from neuroimmune interactions within the pancreas. Diagnosis at an early stage of disease is challenging, though in later stages is dependent upon the demonstration of pancreatic fibrosis and duct ectasia using one or more imaging modalities including transabdominal and endoscopic ultrasound, CT and MRCP or ERCP. Current treatments are largely supportive and reactive. The challenge for pediatricians is to achieve diagnosis at an early stage of the disease and to develop treatments that can alter its natural history.
慢性胰腺炎（CP）的特征是胰腺炎症和纤维化，最终导致胰腺实质破坏以及外分泌和内分泌功能丧失。胰腺炎症的环境触发因素与疾病易感性或修饰基因（包括PRSS1、SPINK1和CFTR）之间的相互作用模型为理解疾病发病机制提供了一个框架。在疾病早期，当纤维化较轻且胰腺损伤有限时，很难将CP与复发性急性胰腺炎（RAP）区分开来，尽管它们可能代表了具有共同病因的疾病谱的两端，其中CP代表疾病的晚期阶段或倾向于产生慢性纤维化炎症反应的个体所患疾病。疼痛是一个主要特征，部分原因是胰腺内的神经免疫相互作用。疾病早期的诊断具有挑战性，而在后期则依赖于使用包括经腹和内镜超声、CT、MRCP或ERCP在内的一种或多种成像方式来证实胰腺纤维化和导管扩张。目前的治疗方法主要是支持性和反应性的。儿科医生面临的挑战是在疾病早期实现诊断，并开发能够改变其自然病程的治疗方法。
