Igarashi T, Hayakawa H, Shiraga H, Kawato H, Yan K, Kawaguchi H, Yamanaka T, Tsuchida S, Akagi K
Department of Pediatrics, University of Tokyo, Japan.
Nephron. 1995;69(3):242-7. doi: 10.1159/000188464.
Idiopathic low-molecular-weight (LMW) proteinuria is a newly described renal disease in Japan and Italy. We report on 7 patients who manifested bilateral or unilateral nephrocalcinosis, as demonstrated by abdominal computed tomography scans. Renal histology revealed calcinosis of renal tubules in 2 patients. Computed tomography is a reliable method for the detection of nephrocalcinosis in this disorder. Hypercalciuria was also seen in 6 patients. A calcium-loading test performed in 2 patients suggested that hypercalciuria was of renal origin. Although the true pathogenesis is still not known, hypercalciuria and nephrocalcinosis appear to be a common complication in patients with idiopathic LMW proteinuria. These complications and clinical features suggest that idiopathic LMW proteinuria in Japan is likely to be identical to Dent's disease in the United Kingdom.
特发性低分子量（LMW）蛋白尿是日本和意大利新发现的一种肾脏疾病。我们报告了7例患者，腹部计算机断层扫描显示其存在双侧或单侧肾钙质沉着症。肾脏组织学检查发现2例患者肾小管有钙质沉着。计算机断层扫描是检测该疾病中肾钙质沉着症的可靠方法。6例患者还出现了高钙尿症。对2例患者进行的钙负荷试验表明，高钙尿症源于肾脏。尽管真正的发病机制尚不清楚，但高钙尿症和肾钙质沉着症似乎是特发性LMW蛋白尿患者的常见并发症。这些并发症和临床特征表明，日本的特发性LMW蛋白尿可能与英国的丹特病相同。
