Lebenthal E, Antonowicz I, Shwachman H
Gastroenterology. 1976 Apr;70(4):508-12.
The interrelationship of enterokinase and trypsin activities were investigated in 133 infants and children with a variety of gastrointestinal and pancreatic disorders. Fourteen patients with diarrhea and grade II mucosal injury revealed a significant (P less than 0.01) reduction of enterokinase, trypsin, and disaccharidase activites as compared to 59 children with normal mucosa. Nine patients with cystic fibrosis and pancreatic insufficiency had normal mucosal enterokinase activity and elevated intraluminal enterokinase activity with very low or no trypsin activity. Patients with hypoproteinemia and gastrointestinal protein loss, associated with intestinal lymphangiectasia (4 patients) and intestinal lymphoid nodular hyperplasia (3 patients), had normal or insignificant decrease of enterokinase and trypsin activities. In patients with steatorrhea, a normal sweat test, normal intestinal mucosa, and absent trypsin activity, two entities were defined. One group (3 patients) was diagnosed as Schwachman-Diamond syndrome with pancreatic insufficiency and normal mucosal and intraluminal enterokinase activity. The second group (2 patients) with absent mucosal and intraluminal enterokinase activity and normal lipase and amylase activities was diagnosed as congenital enterokinase deficiency.
对133名患有各种胃肠道和胰腺疾病的婴幼儿和儿童的肠激酶与胰蛋白酶活性的相互关系进行了研究。与59名黏膜正常的儿童相比，14名患有腹泻和II级黏膜损伤的患者的肠激酶、胰蛋白酶和双糖酶活性显著降低（P小于0.01）。9名患有囊性纤维化和胰腺功能不全的患者黏膜肠激酶活性正常，腔内肠激酶活性升高，但胰蛋白酶活性极低或无活性。4名患有肠淋巴管扩张症和3名患有肠道淋巴样结节增生症的低蛋白血症和胃肠道蛋白丢失患者的肠激酶和胰蛋白酶活性正常或仅有轻微降低。在脂肪泻、汗液试验正常、肠黏膜正常但胰蛋白酶活性缺乏的患者中，确定了两种情况。一组（3例患者）被诊断为施瓦赫曼-戴蒙德综合征，伴有胰腺功能不全，黏膜和腔内肠激酶活性正常。第二组（2例患者）黏膜和腔内肠激酶活性缺乏，但脂肪酶和淀粉酶活性正常，被诊断为先天性肠激酶缺乏症。
