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1952年首次描述家族性胰腺炎,至1981年世界文献报告此病已超过40个家庭.本文介绍德国一个第二代患遗传性钙化性胰腺炎的家族,并讨论其典型特征和手术适应症.病例:女性,20岁(Ⅲ/9)1983年因腹痛住院,当即查血清淀粉酶活性上升到966u/L.既往于5岁时发生腹痛,此后14年间无症状,近两年餐后或大量饮酒后发生腹痛伴恶心,2~3天后疼痛可自行缓解.超声证实为钙化性胰腺炎.心肺听诊正常,腹软.实验室检查:胰十二指肠试验T/K系数2%(正常>20%),粪中胰凝乳蛋白酶7u/g(正常30u/g),甲状旁腺激素肽170Pg/ml(正常44~68),腹平片示胰头体部均有明显的
Familial pancreatitis was first described in 1952 and has been reported in more than 40 families by the world literature in 1981. This article describes a family of second-generation hereditary calcific pancreatitis in Germany and discusses its typical features and surgical indications. Case: Female, 20 years old (Ⅲ / 9) In 1983 due to abdominal pain hospital, immediately check the serum amylase activity rose to 966u / L. In the past occurred at the age of 5 abdominal pain, after 14 years asymptomatic, after two years of postprandial or large Abdominal pain with nausea after drinking, 2 to 3 days after the pain can relieve itself. Ultrasound confirmed as calcified pancreatitis. Cardiopulmonary auscultation normal, abdominal soft. Laboratory tests: pancreaticoduodenal test T / K coefficient of 2% (normal> 20%), fecal chymostatin 7u / g (normal 30u / g), parathyroid hormone peptide 170Pg / ml (normal 44-68), abdominal plain film showed significant head and body parts