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.
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Mccabe, E., O'brien, D., Goodman, S. et al. ORGANIC ACIDURIA FOLLOWING EXTENSIVE SMALL BOWEL RESECTION. Pediatr Res 11, 447 (1977). https://doi.org/10.1203/00006450-197704000-00463