Maynard J, Benson P
Department of Surgery, Al Qassimi Hospital, Sharjah, United Arab Emirates.
J Urol. 1988 Feb;139(2):338-9. doi: 10.1016/s0022-5347(17)42404-9.
We describe a 3-year-old Pakistani girl who presented with recurrent urinary infections. She had a nonfunctioning hydronephrotic right kidney and hypertension. At operation a calculus was impacted in the right ureter with dilatation of the pelviocaliceal system. Nephrectomy was performed. Histology revealed end stage pyelonephritis. The calculus consisted of pure xanthine. Further investigations demonstrated low serum uric acid and absent urinary uric acid with increased excretion of xanthine. Eight months after nephrectomy blood pressure had decreased to normal. Her 5-year-old sister, who has beta-thalassemia, also has a low serum uric acid concentration and xanthinuria. The treatment of choice is to increase fluid intake so that the urine xanthine concentration remains below the level at which xanthine crystallizes. This may require adjustment of the urine pH.
我们描述了一名3岁的巴基斯坦女孩，她反复出现泌尿系统感染。她有一个无功能的肾积水右肾和高血压。手术时，一枚结石嵌顿在右输尿管，肾盂肾盏系统扩张。进行了肾切除术。组织学检查显示为终末期肾盂肾炎。结石由纯黄嘌呤组成。进一步检查发现血清尿酸水平低，尿中无尿酸，黄嘌呤排泄增加。肾切除术后8个月，血压已降至正常。她患有β地中海贫血的5岁妹妹血清尿酸浓度也低，且有黄嘌呤尿症。治疗的首选方法是增加液体摄入量，使尿中黄嘌呤浓度保持在低于黄嘌呤结晶的水平。这可能需要调整尿液pH值。
