Brock W A, Golden J, Kaplan G W
J Urol. 1983 Jul;130(1):157-9. doi: 10.1016/s0022-5347(17)51011-3.
Urinary tract calculi composed primarily of xanthine are rare in adults and children. However, there is risk of xanthine calculi formation in children with hereditary xanthinuria and children on xanthine oxidase inhibitor therapy for hyperuricemia. We describe the clinical presentation and management of 2 children with the Lesch-Nyhan syndrome (a congenital disorder of purine metabolism) and xanthine calculi. Little information has been available to direct the urologic management of such patients. We have based a plan for management upon our clinical experience with these children, as well as upon in vitro dissolution studies of the calculi. We have had some clinical success using an alternating acid/base dissolution therapy developed in the laboratory.
主要由黄嘌呤构成的尿路结石在成人和儿童中都很罕见。然而,患有遗传性黄嘌呤尿症的儿童以及接受黄嘌呤氧化酶抑制剂治疗高尿酸血症的儿童存在形成黄嘌呤结石的风险。我们描述了2例患有莱施-奈恩综合征(一种嘌呤代谢先天性疾病)并伴有黄嘌呤结石的儿童的临床表现及治疗情况。此前几乎没有可用于指导此类患者泌尿外科治疗的信息。我们根据对这些儿童的临床经验以及结石的体外溶解研究制定了治疗方案。我们通过实验室研发的酸碱交替溶解疗法取得了一定的临床成效。