Oral Coenzyme Q10 supplementation leads to better preservation of kidney function in steroid-resistant nephrotic syndrome due to primary Coenzyme Q10 deficiency

辅酶Q10 肾病综合征 医学 蛋白尿 内科学 肾脏疾病 胃肠病学 肾功能 内分泌学
作者
Stefania Drovandi,Beata S. Lipska‐Ziętkiewicz,Fatih Özaltın,Francesco Emma,Bora Gülhan,Olivia Boyer,Agnes Trautmann,Hong Xu,Qian Shen,Jia Rao,Korbinian M. Riedhammer,Uwe Heemann,Julia Hoefele,Sarah L. Stenton,А.Н. Цыгин,Kar Hui Ng,Svitlana Fomina,Elisa Benetti,Manon Aurelle,Larisa Prikhodina
出处
期刊:Kidney International [Elsevier BV]
卷期号:102 (3): 604-612 被引量:39
标识
DOI:10.1016/j.kint.2022.04.029
摘要

Primary Coenzyme Q10 (CoQ10) deficiency is an ultra-rare disorder caused by defects in genes involved in CoQ10 biosynthesis leading to multidrug-resistant nephrotic syndrome as the hallmark kidney manifestation. Promising early results have been reported anecdotally with oral CoQ10 supplementation. However, the long-term efficacy and optimal prescription remain to be established. In a global effort, we collected and analyzed information from 116 patients who received CoQ10 supplements for primary CoQ10 deficiency due to biallelic pathogenic variants in either the COQ2, COQ6 or COQ8B genes. Median duration of follow up on treatment was two years. The effect of treatment on proteinuria was assessed, and kidney survival was analyzed in 41 patients younger than 18 years with chronic kidney disease stage 1-4 at the start of treatment compared with that of an untreated cohort matched by genotype, age, kidney function, and proteinuria. CoQ10 supplementation was associated with a substantial and significant sustained reduction of proteinuria by 88% at 12 months. Complete remission of proteinuria was more frequently observed in COQ6 disease. CoQ10 supplementation led to significantly better preservation of kidney function (5-year kidney failure-free survival 62% vs. 19%) with an improvement in general condition and neurological manifestations. Side effects of treatment were uncommon and mild. Thus, our findings indicate that all patients diagnosed with primary CoQ10 deficiency should receive early and life-long CoQ10 supplementation to decelerate the progression of kidney disease and prevent further damage to other organs.
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