Rabassa A A, Schwartz M R, Ertan A
Department of Internal Medicine, Baylor College of Medicine, Houston, Texas, USA.
Dig Dis Sci. 1995 Sep;40(9):1997-2001. doi: 10.1007/BF02208669.
Alpha 1-antitrypsin deficiency is a genetic disorder commonly associated with pulmonary and hepatic injury. Low serum levels of this glycoprotein result in an imbalance between circulating protease and protease inhibitors, which is thought to play a role in the development of emphysema. In recent studies, a protease-to-protease inhibitor imbalance in patients with alpha 1-antitrypsin deficiency was thought to be a mechanism contributing to the development of chronic pancreatitis. The heterozygous phenotype and low levels of this glycoprotein have been reported to occur more frequently in patients with chronic pancreatitis than in healthy controls. We report a patient with Pi-SS phenotype alpha 1-antitrypsin deficiency and chronic pancreatitis complicated by recurrent pancreatic pseudocysts and chronic abdominal pain. Our case supports the association between chronic pancreatitis and alpha 1-antitrypsin deficiency. Furthermore, this case provides support for the use of pancreatic stent drainage in the management of intractable abdominal pain in patients with chronic pancreatitis and a dominant stricture.
α1-抗胰蛋白酶缺乏症是一种常与肺和肝损伤相关的遗传性疾病。这种糖蛋白的血清水平较低会导致循环中的蛋白酶和蛋白酶抑制剂之间失衡，这被认为在肺气肿的发展中起作用。在最近的研究中，α1-抗胰蛋白酶缺乏症患者中蛋白酶与蛋白酶抑制剂的失衡被认为是导致慢性胰腺炎发展的一种机制。据报道，这种杂合子表型和该糖蛋白的低水平在慢性胰腺炎患者中比在健康对照者中更频繁出现。我们报告了一名患有Pi-SS表型α1-抗胰蛋白酶缺乏症且并发复发性胰腺假性囊肿和慢性腹痛的慢性胰腺炎患者。我们的病例支持慢性胰腺炎与α1-抗胰蛋白酶缺乏症之间的关联。此外，该病例为在慢性胰腺炎伴主要狭窄的患者中使用胰管支架引流治疗顽固性腹痛提供了支持。
