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首页> 外文期刊>Clinical kidney journal. >Porphyria and kidney diseases
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Porphyria and kidney diseases

机译：卟啉病和肾脏疾病

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The kidneys, after the bone marrow and liver, are third in terms of the amounts of haem synthesized daily. Haem is incorporated into haemoproteins that are critical to renal physiology. In turn, disturbances in haem metabolism interfere with renal physiology and are tightly interrelated with kidney diseases. Acute intermittent porphyria causes kidney injury, whereas medical situations associated with end-stage renal disease, such as porphyrin accumulation, iron overload and hepatitis C, participate in the inhibition of uroporphyrinogen decarboxylase and predispose the individual to porphyria cutanea tarda. Even if some of these interactions have been known for a long time, the clinical situations associated with these interrelations have strikingly evolved over time with the advent of new therapeutic strategies for dialysis therapy and a better understanding of the pathophysiological mechanisms of porphyria-associated kidney disease. Physicians should be aware of these interactions. The aim of this review is to summarize the complex interactions between kidney physiology and pathology in the settings of porphyria and to emphasize their often-underestimated importance.
机译：在每日合成血红素的数量方面，仅次于骨髓和肝脏的肾脏排名第三。血红素被掺入对肾生理至关重要的血红蛋白中。反过来，血红素代谢紊乱会干扰肾脏生理，并与肾脏疾病紧密相关。急性间歇性卟啉症会导致肾脏损伤，而与终末期肾脏疾病相关的医学情况（如卟啉积聚，铁超负荷和丙型肝炎）会参与抑制尿卟啉原原脱羧酶，并使个体易患皮肤卟啉症。即使其中一些相互作用早已为人所知，但随着时间的推移，随着透析治疗新治疗策略的出现以及对卟啉症相关性肾脏疾病的病理生理机制的更深入了解，与这些相关性相关的临床情况已发生了惊人的变化。 。医师应意识到这些相互作用。这篇综述的目的是总结在卟啉症环境中肾脏生理学与病理学之间复杂的相互作用，并强调它们经常被低估的重要性。

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