The management of organic acidaemias: The role of transplantation
The management of organic acidaemias: The role of transplantation
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DOI：
10.1023/a:1010395724012
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发表时间：
2001-04-01
影响因子：
4.2
通讯作者：
McKiernan, PJ
中科院分区：
文献类型：
--
作者：
Leonard, JV;Walter, JH;McKiernan, PJ
该研讨会讨论了丙酸血症（PA）和甲基丙二酸血症（MMA）的治疗问题，特别是与器官移植相比，传统治疗的结果。虽然通过药物治疗，早发性疾病的死亡率似乎有所改善，但长期结果仍然很差。然而，肝和肝/肾移植与高围手术期死亡率和长期并发症相关。它只能提供部分治疗。移植的适应症仍不清楚。建议建立PA和MMA患者登记册。
This workshop addressed the issue of treatment in propionic acid-aemia (PA) and methylmalonic acidaemia (MMA) and in particular the outcome of conventional management compared with organ transplantation. Although it appears that with medical treatment the mortality for early-onset disease has improved, long-term outcome remains poor. However, liver and liver/kidney transplantation is associated with a high perioperative mortality and long-term complications. It offers only a partial cure. The indications for transplantation remain unclear. It is recommended that a register of PA and MMA patients should be established.