Thompson G N, Chalmers R A, Walter J H, Bresson J L, Lyonnet S L, Reed P J, Saudubray J M, Leonard J V, Halliday D
Nutrition Research Group, Clinical Research Centre, Harrow, UK.
Eur J Pediatr. 1990 Aug;149(11):792-6. doi: 10.1007/BF01957284.
Gut bacteria have been implicated as an important source of propionate in children with inborn errors of propionate metabolism. We have investigated the value of oral metronidazole (10-20 mg/kg per day) in five children with methylmalonic acidaemia (MMA) and four with propionic acidaemia (PA). Urinary excretion of propionate metabolites fell significantly during the treatment in all subjects, the mean decrease being 41% (range 12-76, P less than 0.01), while mean plasma propionate was reduced from 45.0 mumol/l to 25.1 mumol/l (P less than 0.05). Substantial reduction of the gut bacterial population was confirmed by lactulose breath hydrogen tests and by stool culture, and stool propionate concentration was reduced in most subjects. Clinical improvement was noted in three children. These results suggest that long-term antimicrobial therapy may offer significant clinical benefit to children with inborn errors of propionate metabolism.
肠道细菌被认为是丙酸代谢先天性缺陷患儿丙酸的重要来源。我们研究了口服甲硝唑（每天10 - 20毫克/千克）对5名甲基丙二酸血症（MMA）患儿和4名丙酸血症（PA）患儿的疗效。治疗期间，所有受试者尿液中丙酸代谢产物的排泄量均显著下降，平均降幅为41%（范围12 - 76，P < 0.01），而血浆丙酸平均浓度从45.0微摩尔/升降至25.1微摩尔/升（P < 0.05）。乳果糖呼气氢试验和粪便培养证实肠道细菌数量大幅减少，大多数受试者粪便中丙酸浓度降低。3名患儿出现临床改善。这些结果表明，长期抗菌治疗可能对丙酸代谢先天性缺陷患儿具有显著的临床益处。
