Durie P R, Largman C, Brodrick J W, Johnson J H, Gaskin K J, Forstner G G, Geokas M C
Pediatr Res. 1981 Oct;15(10):1351-5. doi: 10.1203/00006450-198110000-00010.
Plasma immunoreactive cationic trypsin(ogen) levels were determined in 32 control subjects and 43 patients with varying degrees of pancreatic insufficiency including 35 with cystic fibrosis (CF) and eight with Shwachman's syndrome. In six CF infants less than 2 years of age, plasma trypsin(ogen) levels were significantly elevated (97.3 +/- 62.2 ng/ml) above the normal range for nine controls (7.0 +/- 5.9 ng/ml; P less than 0.025). Four of these infants had steatorrhea, three of whom had undetectable duodenal trypsin activity after stimulation with secretin-cholecystokinin. In two CF infants, molecular size fractionation by gel filtration of plasma followed by radioimmunoassay of the column fractions demonstrated that trypsinogen was the only immunoreactive species in the circulation. In contrast, in older CF patients with steatorrhea (mean age, 15.3 +/- 4.6 years), plasma cationic trypsin(ogen) levels were undetectable or low (1.1 +/- 1.7 ng/ml). This finding clearly distinguished them from older CF patients without steatorrhea (mean age, 14.3 +/- 3.9 years) in whom cationic trypsin(ogen) levels were significantly higher (23.3 +/- 17.6 ng/ml; P less than 0.01). The mean trypsin(ogen) concentration in the older CF patients without steatorrhea did not differ from the mean value for 23 normal subjects of similar age. Plasma cationic trypsin(ogen) levels in two Schwachman's patients with steatorrhea (0.19 and 0.86 ng/ml) were significantly lower than the values found in six Shwachman's patients without steatorrhea (5.9 +/- 2.3 ng/ml; P less than 0.025). Furthermore, in nine older CF patients and eight Schwachman's patients, circulating trypsin(ogen) levels were highly correlated with duodenal trypsin output after secretin-cholecystokinin stimulation (r = 0.946, P less than 0.01; r = 0.899, P less than 0.01, respectively). These results suggest that in CF infants high levels of circulating trypsin(ogen) persist even in those with Shwachman's syndrome, however, circulating trypsin(ogen) accurately reflects residual pancreatic function.
在32名对照受试者和43例不同程度胰腺功能不全的患者中测定了血浆免疫反应性阳离子胰蛋白酶（原）水平，其中包括35例囊性纤维化（CF）患者和8例施瓦茨曼综合征患者。在6名年龄小于2岁的CF婴儿中，血浆胰蛋白酶（原）水平显著升高（97.3±62.2 ng/ml），高于9名对照者的正常范围（7.0±5.9 ng/ml；P<0.025）。这些婴儿中有4例患有脂肪泻，其中3例在促胰液素-缩胆囊素刺激后十二指肠胰蛋白酶活性检测不到。在2例CF婴儿中，通过血浆凝胶过滤进行分子大小分级，然后对柱分级进行放射免疫测定，结果表明胰蛋白酶原是循环中唯一的免疫反应性物质。相比之下，在患有脂肪泻的老年CF患者（平均年龄15.3±4.6岁）中，血浆阳离子胰蛋白酶（原）水平检测不到或很低（1.1±1.7 ng/ml）。这一发现将他们与没有脂肪泻的老年CF患者（平均年龄14.3±3.9岁）明显区分开来，后者的阳离子胰蛋白酶（原）水平显著更高（23.3±17.6 ng/ml；P<0.01）。没有脂肪泻的老年CF患者的平均胰蛋白酶（原）浓度与23名年龄相仿的正常受试者的平均值没有差异。2例患有脂肪泻的施瓦茨曼患者的血浆阳离子胰蛋白酶（原）水平（0.19和0.86 ng/ml）显著低于6例没有脂肪泻的施瓦茨曼患者的值（5.9±2.3 ng/ml；P<0.025）。此外，在9名老年CF患者和8名施瓦茨曼患者中，循环胰蛋白酶（原）水平与促胰液素-缩胆囊素刺激后的十二指肠胰蛋白酶分泌量高度相关（r分别为0.946，P<0.01；r为0.899，P<0.01）。这些结果表明，在CF婴儿中，即使患有施瓦茨曼综合征，循环中胰蛋白酶（原）水平仍会持续升高，然而，循环胰蛋白酶（原）能准确反映胰腺残余功能。
