Hereditary pancreatitis: clinical presentation, ERCP findings, and outcome of endoscopic therapy
Hereditary pancreatitis: clinical presentation, ERCP findings, and outcome of endoscopic therapy
复制标题
DOI：
10.1067/mge.2002.125103
复制
发表时间：
2002-07-01
影响因子：
7.7
通讯作者：
Sherman, S
中科院分区：
文献类型：
--
作者：
Choudari, CP;Nicki, NJ;Sherman, S
背景资料：遗传性胰腺炎（HP）是一种常染色体显性遗传疾病，其特征是在儿童期反复发作急性胰腺炎，并经常进展为慢性胰腺炎。关于内镜逆行胰胆管造影（ERCP）在HP患者中的诊断和治疗作用的数据有限。本研究的目的是描述胰腺造影的临床表现和结果，并评估内镜和手术干预的作用，患者HP.Methods：27例连续HP患者被称为ERCP在美国中西部的两个中心。结果：所有患者的胰腺造影均异常。大多数（70%）有慢性胰腺炎的重度（剑桥III级）变化。19例（70%）患者接受了内镜治疗，8例（30%）患者接受了手术干预。内镜治疗后（平均随访32个月），500/6例无症状，38%中度改善，12%症状无改善或短暂改善。手术后（平均随访32个月），38%的患者无症状，25%的患者症状中度改善，12%的患者症状短暂改善。两名患者手术治疗后死于胰腺癌。结论：在短期内，通过内镜手段改善胰腺导管系统的引流可有效缓解疼痛，减少大多数HP患者胰腺炎的发作次数。
Background: Hereditary pancreatitis (HP) is an autosomal dominant disorder characterized by the onset of recurrent attacks of acute pancreatitis in childhood and frequent progression to chronic pancreatitis. There are limited data on the role of diagnostic and therapeutic ERCP in patients with HP. The aims of this study were to describe the clinical presentation and findings on pancreatography and to evaluate the role of endoscopic and surgical intervention in patients with HP.Methods: Twenty-seven consecutive patients with HP were referred for ERCP at two centers in the midwest United States. Medical records and pancreatograms were reviewed.Results: Pancreatography was abnormal in all patients. The majority (70%) had severe (Cambridge Grade III) changes of chronic pancreatitis. Nineteen (70%) patients underwent endoscopic therapy and 8 (30%) surgical intervention. After endoscopic therapy (mean follow-up 32 months), 500/6 were symptom free, 38% had moderate improvement, and 12% had no or transient improvement in symptoms. After surgery (mean follow-up 32 months), 38% were symptom-free, 25% had moderate improvement, and 12% had transient improvement in symptoms. Two patients treated surgically died of pancreatic cancer.Conclusions: In the short-term, improving drainage of the pancreatic ductal system by endoscopic means effectively relieves pain and reduces the number of attacks of pancreatitis in the majority of patients with HP.