ORGANIC ACIDURIA FOLLOWING EXTENSIVE SMALL BOWEL RESECTION
ORGANIC ACIDURIA FOLLOWING EXTENSIVE SMALL BOWEL RESECTION
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大范围小肠切除术后有机酸尿
DOI：
10.1203/00006450-197704000-00463
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发表时间：
1977
影响因子：
3.6
通讯作者：
A. Silverman
中科院分区：
文献类型：
--
作者：
E. McCabe;D. O'brien;S. Goodman;P. Fennessey;B. Miles;A. Silverman
常规筛查发现两名患者在广泛小肠切除术后出现明显的有机酸尿。JK是一只5岁半的雄性动物，由于继发于中肠扭转的坏死，在3日龄时切除了除21 cm外的所有小肠，随后出现复发性酸血症发作。TK是一只5岁雌性动物，在15个月时肠系膜动脉血栓形成后留下15 cm空肠。两人都经历了进行性腹胀、结肠肠梗阻和中枢神经系统抑制的发作。尿乳酸盐（仅JK）、戊二酸盐、丙酸盐、β-羟苯乙酸盐和β-苯乳酸盐升高。JK的白色血细胞戊二酰辅酶A脱氢酶活性正常。JK组血清前列腺素E、F水平明显升高。已报告从粪便中吸收有机酸，这些患者说明小肠切除术后这可能变得定量显著。数据还表明，短肠异常可能不仅仅是由于碳酸氢盐消耗。我们的患者表明，胃肠系统疾病构成了一个以前未被认识的病因，与先天性酶错误相关的谷氨酸尿症。饮食限制、口服碳酸氢盐、导泻和前列腺素合成抑制剂可能在治疗这种疾病中发挥作用。
Routine screening has identified two patients with striking organic aciduria after extensive small bowel resection. JK, a 5 ½ yo male, underwent resection of all but 21 cm of small bowel at 3 days of age because of necrosis secondary to midgut volvulus and subsequently has had recurrent acidemic episodes. TK, a 5 yo female, was left with 15 cm of jejunum following mesenteric artery thrombosis at 15 months. Both have experienced episodes of progressive abdominal distention, colonic ileus, and CNS depression. Urinary lactate (JK only), glutarate, propionate, β-hydroxyphenylacetate and β-phenyllactate were elevated. White blood cell glutaryl-CoA dehydrogenase activity in JK was normal. Serum prostaglandin E and F were significantly elevated in JK. Absorption of organic acids from stool has been reported and these patients illustrate that this may become quantitatively significant following small bowel resection. The data also suggest that the addemia of short bowel may not be solely due to bicarbonate wasting. Our patients demonstrate that gastroenterologic disorders constitute a previously unrecognized etiology to be differentiated from inborn enzymatic errors associated with glutaric aciduria. Dietary restriction, oral bicarbonate, catharsis, and inhibitors of prostaglandin synthesis may play a role in treatment of this disorder.