Singh S, Tai C, Ganz G, Yeung C K, Magil A, Rosenberg F, Applegarth D, Levin A
Division of Pathology, St Paul's Hospital and Vancouver General Hospital, Canada.
Am J Kidney Dis. 1999 Apr;33(4):e5. doi: 10.1016/s0272-6386(99)70246-5.
We present a case of a 54-year-old woman with rapidly progressive renal failure of uncertain origin, who developed pleuropericarditis and livedo reticularis 6 weeks after initiation of hemodialysis (HD). The presentation with acute renal failure, the development of serositis, and the dramatic clinical response to empiric steroid therapy initially suggested the diagnosis of a systemic inflammatory disorder or vasculitis. Renal biopsy, performed 3 days after presentation, suggested crystal deposition disease, and subsequent investigations, using both dialysate oxalate concentrations and liver biopsy, led to the diagnosis of primary hyperoxaluria (PH). We discuss this atypical adult presentation of PH and propose a role for the use of steroids in the management of the acute inflammatory symptoms of oxalosis. We also briefly discuss the current medical management of patients with PH, including transplantation.
我们报告一例54岁女性患者，患有病因不明的快速进展性肾衰竭，在开始血液透析（HD）6周后出现了胸膜心包炎和网状青斑。急性肾衰竭的表现、浆膜炎的发生以及对经验性类固醇治疗的显著临床反应最初提示诊断为全身性炎症性疾病或血管炎。就诊3天后进行的肾活检提示晶体沉积病，随后通过透析液草酸盐浓度和肝活检进行的进一步检查确诊为原发性高草酸尿症（PH）。我们讨论了PH这种非典型的成人表现，并提出类固醇在草酸中毒急性炎症症状管理中的作用。我们还简要讨论了PH患者目前的药物治疗，包括移植。
