Kordac V, Kozáková M, Martásek P
1st Department of Medicine, Charles University School of Medicine, Prague, Czechoslovakia.
Ann Med. 1989 Aug;21(4):273-6. doi: 10.3109/07853898909149205.
In three patients with attacks of acute intermittent porphyria we found markedly impaired functions of the left ventricle accompanied by ECG changes that returned to normal after administration of heme arginate or cytochrome C administration. In nine patients with other types of porphyria, and in one patient with acute intermittent porphyria not suffering an attack, no significant changes of left ventricle function were observed. The changes seen in patients with an acute attack may be ascribed to acute myocardial hypoxia resulting from defective biosynthesis of heme. We assume that the exogenous supply of heme may improve cellular hypoxia.
在3例急性间歇性卟啉病发作患者中,我们发现左心室功能明显受损,并伴有心电图改变,在给予精氨酸血红素或细胞色素C后恢复正常。在9例其他类型卟啉病患者以及1例未发作的急性间歇性卟啉病患者中,未观察到左心室功能有明显变化。急性发作患者出现的这些变化可能归因于血红素生物合成缺陷导致的急性心肌缺氧。我们推测外源性血红素供应可能改善细胞缺氧。