Dundee Philip, Bouchier-Hayes David, Iles Linda, Costello Anthony
Department of Urology, Royal Melbourne Hospital, Parkville, Melbourne, Australia.
Int Urol Nephrol. 2005;37(3):453-6. doi: 10.1007/s11255-004-0020-4.
Erdheim-Chester Disease (ECD) is a non-inherited multifocal lipid storing histiocytosis. It is a rare disease characterised by lipid-laden monocyte infiltration of long bones causing cortical sclerosis and characteristic X-ray appearances. It also involves extraskeletal tissue in up to 50% of cases including retroperitoneal and renal infiltration. We report a patient with long standing ECD with widespread extraskeletal involvement, including significant renal infiltration, presenting with left hydronephrosis secondery obstruction from a proximal ureteric calculas.
厄尔德海姆-切斯特病（ECD）是一种非遗传性多灶性脂质贮积性组织细胞增多症。它是一种罕见疾病，其特征是长骨中充满脂质的单核细胞浸润，导致皮质硬化和特征性X线表现。在高达50%的病例中，它还累及骨骼外组织，包括腹膜后和肾脏浸润。我们报告了一名患有长期ECD且骨骼外广泛受累的患者，包括显著的肾脏浸润，该患者因近端输尿管结石导致左肾积水继发梗阻。
