Department of Gastroenterology and Hepatology, Karolinska Institutet, Karolinska University Hospital, Stockholm, Sweden.
Transpl Int. 2010 Jun;23(6):e18-21. doi: 10.1111/j.1432-2277.2009.01035.x. Epub 2009 Dec 16.
We report two patients with acute intermittent porphyria (AIP) who were successfully treated with combined liver and kidney transplantation. Both had a very poor quality of life as a result of years of frequent acute porphyria symptoms, chronic peripheral neuropathy and renal failure requiring dialysis. After transplantation, clinical and biochemical signs of porphyria disappeared. The excretion pattern of porphyrin precursors normalized within the first day and plasma porphyrins returned to normal within a week. These and other recent cases have clarified previous concerns and have helped to formulate the indications for and the timing of transplantation in AIP.
我们报告了两例急性间歇性卟啉症（AIP）患者，他们通过肝肾联合移植成功治疗。由于多年频繁的急性卟啉症症状、慢性周围神经病和需要透析的肾衰竭，两人的生活质量都非常差。移植后，卟啉症的临床和生化迹象消失。卟啉前体的排泄模式在第一天内恢复正常，血浆卟啉在一周内恢复正常。这些和其他最近的病例澄清了以前的担忧，并有助于制定 AIP 移植的适应证和时机。
