Rodby R A, Tyszka T S, Williams J W
Department of Medicine, Rush Medical College, Chicago, Illinois.
Am J Med. 1991 Apr;90(4):498-504.
A 23-year-old man with type 1 primary hyperoxaluria, renal failure, and oxalosis developed a severe cardiomyopathy while awaiting combined liver-kidney transplantation. This manifested as radiographic cardiomegaly, a dilated hypokinetic left ventricle with a decreased ejection fraction, ventricular arrhythmias, and cardiac uptake on bone scanning. On liver and kidney transplantation, these abnormalities markedly improved and/or reversed. The cardiac size almost normalized, the left ventricular ejection fraction increased from 20% to 34%, the ventricular arrhythmias resolved, and the cardiac uptake on bone scanning disappeared. This coincided with normalization of oxalate production and excretion. Severe cardiac involvement secondary to oxalosis in patients with primary hyperoxaluria may improve or reverse with combined liver-kidney transplantation.
一名患有1型原发性高草酸尿症、肾衰竭和草酸沉着症的23岁男子在等待肝肾联合移植期间出现了严重的心肌病。表现为影像学上的心脏扩大、左心室扩张运动减弱伴射血分数降低、室性心律失常以及骨扫描时心脏摄取增加。在进行肝肾移植后，这些异常明显改善和/或逆转。心脏大小几乎恢复正常，左心室射血分数从20%增至34%，室性心律失常消失，骨扫描时的心脏摄取也消失了。这与草酸盐生成和排泄的正常化相一致。原发性高草酸尿症患者因草酸沉着症继发的严重心脏受累情况可能会在肝肾联合移植后得到改善或逆转。
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