Ros E, García-Pugés A, Reixach M, Cusó E, Rodés J
Gastroenterology. 1984 Jul;87(1):180-7.
Intestinal bile salt deficiency marginally impairs fat absorption in cholestatic patients. The finding of massive steatorrhea in some patients with primary biliary cirrhosis led us to systematically study and compare fat digestion in control subjects (n = 4) and patients with biliary cirrhosis with (n = 11) and without (n = 3) steatorrhea. The jejunum was anatomically and functionally intact in all subjects, as demonstrated by normal gastrointestinal radiology and xylose absorption, respectively. The intestinal contents were recovered during digestion of a fat meal. Lipolysis, pH, and trypsin activity were measured in whole intestinal contents, whereas bile salts, total lipid, and fatty acid were determined in both total and aqueous phases. The results obtained in controls and patients without steatorrhea were similar. Percentage of lipolysis and intraluminal pH were normal in controls and in both patient groups. The intestinal contents of the patients with steatorrhea had a significantly lesser capacity to solubilize both total lipid and fatty acid in relation to abnormally low aqueous bile salt concentrations. No bile salt deconjugation and only minimal bile salt precipitation were found, thus low aqueous bile salts were strictly related to bile secretory failure. Steatorrhea was always present when aqueous bile salt levels were below 3.0 mM. Intestinal trypsin activity was subnormal in patients with steatorrhea; decreased trypsin activity was related (r = 0.82, p less than 0.001) to reduced intestinal bile salt levels. One patient was found to have severe exocrine pancreatic failure. Administration of medium chain triglycerides was uniformly effective in improving nutrition in patients with steatorrhea, but the course of the disease was unaffected. These results indicate that overt pancreatic failure is uncommon in primary biliary cirrhosis, and that fat maldigestion and steatorrhea, regardless of what degree, are due mainly to low intestinal bile salt levels secondary to bile secretory failure. Finally, subnormal pancreatic function in this disease appears to be related to the bile secretory failure, suggesting either that the lack of bile or bile salts in the intestine depresses pancreatic exocrine function or that both biliary and pancreatic secretions decrease in parallel as part of a widespread secretory failure syndrome.
肠道胆汁盐缺乏对胆汁淤积患者的脂肪吸收仅有轻微损害。一些原发性胆汁性肝硬化患者出现大量脂肪泻的情况促使我们系统地研究并比较了对照组（n = 4）以及有（n = 11）和无（n = 3）脂肪泻的胆汁性肝硬化患者的脂肪消化情况。所有受试者的空肠在解剖学和功能上均完好无损，分别通过正常的胃肠放射学检查和木糖吸收试验得以证实。在脂肪餐消化过程中收集肠道内容物。测定全肠道内容物中的脂解作用、pH值和胰蛋白酶活性，而在总相和水相中测定胆汁盐、总脂质和脂肪酸。对照组和无脂肪泻患者的结果相似。对照组以及两组患者的脂解百分比和肠腔内pH值均正常。与异常低的水相胆汁盐浓度相关，脂肪泻患者的肠道内容物溶解总脂质和脂肪酸的能力明显较低。未发现胆汁盐去结合现象，仅发现极少的胆汁盐沉淀，因此低水相胆汁盐与胆汁分泌衰竭密切相关。当水相胆汁盐水平低于3.0 mM时，总是会出现脂肪泻。脂肪泻患者的肠道胰蛋白酶活性低于正常水平；胰蛋白酶活性降低与肠道胆汁盐水平降低相关（r = 0.82，p < 0.001）。发现一名患者存在严重的外分泌性胰腺功能衰竭。给予中链甘油三酯对改善脂肪泻患者的营养状况均有效，但疾病进程未受影响。这些结果表明，明显的胰腺功能衰竭在原发性胆汁性肝硬化中并不常见，并且无论程度如何，脂肪消化不良和脂肪泻主要是由于胆汁分泌衰竭导致肠道胆汁盐水平降低所致。最后，该疾病中胰腺功能低于正常似乎与胆汁分泌衰竭有关，这表明肠道中胆汁或胆汁盐的缺乏会抑制胰腺外分泌功能，或者作为广泛分泌衰竭综合征的一部分，胆汁和胰腺分泌会同时减少。
