Igarashi Y, Ohkohchi N, Kikuta A, Suzuki J, Tada K
J Pediatr Gastroenterol Nutr. 1986 Nov-Dec;5(6):899-901. doi: 10.1097/00005176-198611000-00013.
A girl who was clinically suspected of having congenital glucose-galactose malabsorption was investigated for sugar- and amino acid-evoked potential differences in the jejunum in vivo using a jejunal perfusion technique. She was fed an ordinary diet without suffering diarrhea at the time (at age 2) of this study. Her nutritional condition and development were normal. The disaccharidase activities of a biopsy specimen of the jejunal mucosa, which were assayed at 1 year of age when she was not having diarrhea, were normal. Jejunal perfusion of 100-mM solutions of D-glucose or D-maltose evoked no significant change in the electrical potential difference. On the other hand, 100 mM of L-glycine or L-leucine solution evoked a normal increase of the lumen negative potential difference (4.4 mV and 3.6 mV, respectively). Therefore, the patient was diagnosed as having congenital glucose-galactose malabsorption due to a defect of Na-dependent glucose-galactose active transport. These findings indicate that the measurement of sugar- and amino acid-evoked potential differences is a simple and reliable method for the diagnosis of congenital glucose-galactose malabsorption.
一名临床上怀疑患有先天性葡萄糖 - 半乳糖吸收不良的女孩，采用空肠灌注技术，对其体内空肠中糖和氨基酸诱发的电位差异进行了研究。在本研究时（2岁），她食用普通饮食且未出现腹泻。她的营养状况和发育正常。在1岁未腹泻时采集的空肠黏膜活检标本的双糖酶活性正常。用100 mM的D - 葡萄糖或D - 麦芽糖溶液进行空肠灌注时，电位差无显著变化。另一方面，100 mM的L - 甘氨酸或L - 亮氨酸溶液可使肠腔负电位差正常增加（分别为4.4 mV和3.6 mV）。因此，该患者被诊断为因钠依赖性葡萄糖 - 半乳糖主动转运缺陷导致的先天性葡萄糖 - 半乳糖吸收不良。这些发现表明，测量糖和氨基酸诱发的电位差异是诊断先天性葡萄糖 - 半乳糖吸收不良的一种简单可靠的方法。
