Tinti Francesca, Umbro Ilaria, D'Alessandro Mariadomenica, Lai Silvia, Merli Manuela, Noce Annalisa, Di Daniele Nicola, Mazzaferro Sandro, Mitterhofer Anna Paola
Nephrology Unit, Department of Translational and Precision Medicine, Sapienza University of Rome, 00185 Rome, Italy.
Clinical Pathology Unit, Department of General Surgery "P.Stefanini", Sapienza University of Rome, 00161 Rome, Italy.
Life (Basel). 2021 Nov 6;11(11):1200. doi: 10.3390/life11111200.
Cholemic nephropathy (CN) is a recognized cause of acute kidney injury (AKI) in patients with severe hyperbilirubinemia (sHyb) and jaundice. Pathophysiological mechanisms of CN are not completely understood, but it seems caused both by direct toxicity of cholephiles and bile casts formation in nephrons enhanced by prolonged exposure to sHyb, particularly in the presence of promoting factors, as highlighted by a literature reviewed and by personal experience. The aim of our update is to retrace CN in its pathophysiology, risk factors, diagnosis and treatment, underlining the role of sHyb, promoting factors, and CN-AKI diagnostic criteria in the different clinical settings associated with this often-concealed disease. Our purpose is to focus on clinical manifestation of CN, exploring the possible transition to CKD. Cholemic nephropathy is an overlooked clinical entity that enters differential diagnosis with other causes of AKI. Early diagnosis and treatment are essential because renal injury could be fully reversible as rapidly as bilirubin levels are reduced. In conclusion, our proposal is to introduce an alert for considering CN in diagnostic and prognostic scores that include bilirubin and/or creatinine with acute renal involvement, with the aim of early diagnosis and treatment of sHyb to reduce the burden on renal outcome.
胆血症肾病（CN）是严重高胆红素血症（sHyb）和黄疸患者急性肾损伤（AKI）的一个公认病因。CN的病理生理机制尚未完全明确，但似乎是由胆亲和物的直接毒性以及因长期暴露于sHyb（尤其是在存在促发因素的情况下）导致肾单位内胆汁管型形成共同引起的，正如一篇综述文献及个人经验所强调的那样。我们此次更新的目的是追溯CN在病理生理学、危险因素、诊断及治疗方面的情况，强调sHyb、促发因素以及CN-AKI诊断标准在与这种常被隐匿的疾病相关的不同临床环境中的作用。我们的目的是聚焦于CN的临床表现，探讨其向慢性肾脏病（CKD）转变的可能性。胆血症肾病是一个被忽视的临床实体，在与其他AKI病因进行鉴别诊断时需要考虑。早期诊断和治疗至关重要，因为随着胆红素水平迅速降低，肾损伤可能完全可逆。总之，我们的建议是在包括胆红素和/或肌酐以及急性肾受累情况的诊断和预后评分中加入对CN的警示，以期早期诊断和治疗sHyb，减轻对肾脏转归的负担。
