Bastani B, Mistry B M, Nahass G T, Joh J, Dundoo G, Solomon H
Division of Nephrology, St. Louis University Health Sciences Center, St. Louis, Mo., USA.
Am J Nephrol. 1999;19(1):64-9. doi: 10.1159/000013428.
We present the case of a young woman with end-stage renal disease secondary to primary hyperoxaluria type 1, who after 3 years and 6 months of maintenance hemodialysis, and despite intensification of the dialytic treatment, developed severe livedo reticularis in her extremities leading to ischemic cutaneous ulcerations, necessitating continuous intravenous infusion of narcotics for pain control. She received a liver transplant after native hepatectomy. However, due to positive crossmatch, she could not receive a kidney from that donor. After transplantation, following serial serum oxalate levels, the hemodialysis regimen was safely reduced from 4 h daily to 3 h three times weekly. Over the course of 6 weeks after liver transplantation, her livedo reticularis resolved, the ischemic ulcers markedly improved, she was weaned off all pain medications, and her erythropoietin-resistant anemia resolved. Our results suggest that in patients with primary hyperoxaluria type 1, who have received a liver transplant and are on maintenance hemodialysis, after serial serum oxalate determinations, some may safely be changed to a thrice-weekly maintenance hemodialysis regimen. Moreover, with this regimen the complications of systemic oxalosis can reverse.
我们报告了一例因1型原发性高草酸尿症继发终末期肾病的年轻女性病例。该患者在维持性血液透析3年零6个月后，尽管加强了透析治疗，但双下肢仍出现严重的网状青斑，并导致缺血性皮肤溃疡，需要持续静脉输注麻醉剂以控制疼痛。她在接受自体肝切除术后接受了肝移植。然而，由于交叉配型阳性，她无法从该供体获得肾脏。移植后，根据系列血清草酸盐水平，血液透析方案从每日4小时安全地减少至每周三次、每次3小时。在肝移植后的6周内，她的网状青斑消退，缺血性溃疡明显改善，所有止痛药物均已停用，并且她的促红细胞生成素抵抗性贫血也得到缓解。我们的结果表明，对于接受肝移植并进行维持性血液透析的1型原发性高草酸尿症患者，在进行系列血清草酸盐测定后，部分患者可安全地改为每周三次的维持性血液透析方案。此外，采用该方案，全身性草酸中毒的并发症可以逆转。
