急性间歇性卟啉病与糖代谢

Acute intermittent porphyria and glucose metabolism

急性间歇性卟啉病(AIP)临床表现复杂多样,可累及全身多个系统。部分卟啉病患者可伴发有糖尿病,故卟啉病一直被列为特殊类型糖尿病之中。AIP可能通过血红素缺乏、过氧化物酶体增殖物激活受体-γ共激活物-1α(PGC-1α)的表达增强、炎性作用、抵抗素的升高、线粒体损伤等多方面机制影响血糖水平。糖代谢也可对卟啉代谢产生影响。禁食可导致AIP的急性发作,而2型糖尿病的发病和高糖饮食对卟啉病有一定的"保护作用"。葡萄糖在急性肝卟啉病发作时的有益作用可能得益于胰岛素水平的升高。机体是一个有机的整体,卟啉代谢与糖代谢之间存在着联系,但仍需要进一步关注和明确。

Acute intermittent porphyria(AIP)has complex and diverse clinical manifestations,of ten involves multiple systems.Some patients with porphyria may be accompanied by diabetes,so porphyria has always been listed as a special type of diabetes.AIP may affect the level of blood glucose through heme deficiency,enhanced expression of peroxisome proliferator-activated receptorγcoactivator-1α(PGC-1α),inflammation,increased resistin,mitochondrial dysfunction and other mechanisms.Glucose metabolism also affects porphyrin metabolism.Fasting can lead to acute attacks of AIP;the onset of type 2 diabetes and a high carbohydrate diet have a"protective effect"on porphyria.The beneficial effect of glucose on AIP may be due to increased insulin levels.The body is an organic whole,and there is a association between porphyrin metabolism and glucose metabolism,but it still needs further attention and clarification.