Jones N L, Hofley P M, Durie P R
Department of Pediatrics, Hospital for Sick Children, Toronto, Ontario, Canada.
J Pediatr. 1994 Sep;125(3):406-8. doi: 10.1016/s0022-3476(05)83286-x.
We compared pancreatic acinar and ductal secretion in two patients with Johanson-Blizzard syndrome, age-matched control subjects, and patients with other primary pancreatic diseases. Patients with Johanson-Blizzard syndrome had preservation of ductular output of fluid and electrolytes, as in patients with Shwachman syndrome but differing from those with cystic fibrosis, who have a primary ductular defect. They also had decreased acinar secretion of trypsin, colipase and total lipase, and low serum immunoreactive trypsinogen levels, consistent with a primary acinar cell defect.
我们比较了两名患有乔汉森-暴雪综合征的患者、年龄匹配的对照受试者以及患有其他原发性胰腺疾病的患者的胰腺腺泡和导管分泌情况。乔汉森-暴雪综合征患者的液体和电解质导管输出功能得以保留，这与施瓦茨曼综合征患者类似，但与囊性纤维化患者不同，后者存在原发性导管缺陷。他们的胰蛋白酶、共脂肪酶和总脂肪酶的腺泡分泌也减少，血清免疫反应性胰蛋白酶原水平较低，这与原发性腺泡细胞缺陷一致。
