Pallet Nicolas, Karras Alexandre, Thervet Eric, Gouya Laurent, Karim Zoubida, Puy Hervé
INSERM U1147, Centre Universitaire des Saints Pères, Paris, France.
Université Paris Descartes, Paris, France.
Clin Kidney J. 2018 Apr;11(2):191-197. doi: 10.1093/ckj/sfx146. Epub 2018 Jan 10.
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.
就每日合成血红素的量而言，肾脏仅次于骨髓和肝脏，位列第三。血红素被整合到对肾脏生理功能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功能，并与肾脏疾病密切相关。急性间歇性卟啉症会导致肾损伤，而与终末期肾病相关的医学情况，如卟啉蓄积、铁过载和丙型肝炎，会参与抑制尿卟啉原脱羧酶，并使个体易患迟发性皮肤卟啉症。即使其中一些相互作用早已为人所知，但随着透析治疗新策略的出现以及对卟啉相关肾脏疾病病理生理机制的更好理解，与这些相互关系相关的临床情况也随着时间的推移发生了显著变化。医生应该了解这些相互作用。本综述的目的是总结卟啉症背景下肾脏生理与病理之间的复杂相互作用，并强调它们常常被低估的重要性。
