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1型原发性高草酸尿症患者肝肾联合移植后草酸盐骨病的放射学和组织学改善

Radiological and histological improvement of oxalate osteopathy after combined liver-kidney transplantation in primary hyperoxaluria type 1.

作者信息

Toussaint C, De Pauw L, Vienne A, Gevenois P A, Quintin J, Gelin M, Pasteels J L

机构信息

Cliniques Universitaires de Bruxelles, Hôpital Erasme, Belgium.

出版信息

Am J Kidney Dis. 1993 Jan;21(1):54-63. doi: 10.1016/s0272-6386(12)80722-0.

DOI:10.1016/s0272-6386(12)80722-0
PMID:8418628
Abstract

A 15-year-old patient with severe bone disease (with bilateral fractures of hips and shoulders) due to primary hyperoxaluria type 1 (PH1) was treated with combined liver-kidney transplantation after a 4-year hemodialysis period. Normalization of excessive oxalate synthesis brought in by the liver graft combined with the slow excretion of skeletal oxalate stores by the renal graft led to progressive improvement of clinical, radiological, and histological evidence of oxalate osteopathy. This allowed bilateral hip replacement 3 years after transplantation, which led to complete physical rehabilitation of the crippled patient. Combined liver-kidney transplantation constitutes the treatment of choice for end-stage renal failure due to PH1, even in the face of severe oxalate bone disease.

摘要

一名因1型原发性高草酸尿症(PH1)患有严重骨病(双侧髋部和肩部骨折)的15岁患者,在经过4年的血液透析期后接受了肝肾联合移植治疗。肝移植带来的过量草酸盐合成正常化,与肾移植缓慢排出骨骼草酸盐储存相结合,导致草酸盐骨病的临床、放射学和组织学证据逐渐改善。这使得患者在移植后3年能够进行双侧髋关节置换,从而使这名残疾患者完全康复。即使面对严重的草酸盐骨病,肝肾联合移植仍是PH1所致终末期肾衰竭的首选治疗方法。

相似文献

1
Radiological and histological improvement of oxalate osteopathy after combined liver-kidney transplantation in primary hyperoxaluria type 1.1型原发性高草酸尿症患者肝肾联合移植后草酸盐骨病的放射学和组织学改善
Am J Kidney Dis. 1993 Jan;21(1):54-63. doi: 10.1016/s0272-6386(12)80722-0.
2
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引用本文的文献

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Long-term complications of systemic oxalosis in children-a retrospective single-center cohort study.儿童系统性草酸钙沉着症的长期并发症:一项回顾性单中心队列研究。
Pediatr Nephrol. 2021 Oct;36(10):3123-3132. doi: 10.1007/s00467-021-05002-1. Epub 2021 Mar 2.
2
Current approaches to the management of primary hyperoxaluria.原发性高草酸尿症的当前管理方法。
Arch Dis Child. 2000 Jun;82(6):470-3. doi: 10.1136/adc.82.6.470.
3
Initial manifestation of primary hyperoxaluria type I in adults-- recognition, diagnosis, and management.成人原发性I型高草酸尿症的初始表现——识别、诊断与管理
West J Med. 1996 Jan;164(1):42-53.