Kato T, Sano M, Mizutani N, Hayakawa C
Department of Pediatrics, Chubu-Rosai Hospital, Nagoya, Japan.
J Inherit Metab Dis. 1987;10(4):391-6. doi: 10.1007/BF01799982.
A 4-year-old boy with hyperargininaemia had an increased urinary excretion of putrescine which was exaggerated with oral ornithine supplementation. It seems unlikely that putrescine was overproduced within the gut, because a loading with a single oral dose of ornithine showed that the intestinal ornithine absorption in the patient was normal. An acceleration of extramitochondrial ornithine metabolism due to impaired mitochondrial ornithine uptake may have caused the hyperexcretion of putrescine.
一名患有高精氨酸血症的4岁男孩尿中腐胺排泄增加,口服鸟氨酸补充剂后这种情况更加明显。腐胺似乎不太可能在肠道内过量产生,因为单次口服鸟氨酸负荷试验表明该患者肠道对鸟氨酸的吸收正常。线粒体鸟氨酸摄取受损导致线粒体外鸟氨酸代谢加速,可能是腐胺排泄过多的原因。