Evidence map›Paper›PMID 42435122›Full record

ArticleClinical and experimental nephrology2026

Kidney outcomes of coenzyme Q10 supplementation in patients with genetically confirmed CoQ10 nephropathy in Japan.

China Nagano, Katsumi Ushijima, Yuko Tezuka, Takayuki Okamoto, Yasuhiro Inaba, Akinori Miyazono, Naoko Takeda, Taro Aoki, Akira Mizutani, Hiroki Miyano and 13 more

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Article in Clinical and experimental nephrology, 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.

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1 · What the graph read from it

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The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.

2 · The registry

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3 · Its place in the literature

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0 citing papers in PubMed.

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4 · The record

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5 · Who and what money

Authors and funding

23 authors.

China NaganoDepartment of Pediatrics, Kobe University Graduate School of Medicine, 7-5-1 Kusunoki-Cho, Chuo-Ku, Kobe, Hyogo, 650-0017, Japan. china@med.kobe-u.ac.jp.ORCID http://orcid.org/0000-0003-4946-1345
Katsumi UshijimaDepartment of Pediatrics, Yokkaichi Municipal Hospital, Yokkaichi, Japan.
Yuko TezukaDepartment of Pediatrics, Ehime Prefectural Imabari Hospital, Imabari, Japan.
Takayuki OkamotoDepartment of Pediatrics, Hokkaido University Hospital, Sapporo, Japan.
Yasuhiro InabaDepartment of Pediatrics, Kagoshima University Graduate School of Medical and Dental Sciences, Kagoshima, Japan.
Akinori MiyazonoDepartment of Pediatrics, Kagoshima University Graduate School of Medical and Dental Sciences, Kagoshima, Japan.
Naoko TakedaDepartment of Medicine, Shiga University of Medical Science, Shiga, Japan.
Taro AokiInternal Medicine 1, Hamamatsu University School of Medicine, Hamamatsu, Japan.
Akira MizutaniDepartment of Pediatrics, Dokkyo Medical University Saitama Medical Center, Saitama, Japan.
Hiroki MiyanoDepartment of Pediatrics, Juntendo University Nerima Hospital, Tokyo, Japan.
Masaki YamamotoDepartment of Pediatric Nephrology, Seirei Hamamatsu General Hospital, Hamamatsu, Japan.
Mariko ImaiDepartment of Nephrology, National Hospital Organization Chiba Medical Center Chibahigashi National Hospital, Chiba, Japan.
Takehiko KawaguchiDepartment of Nephrology, National Hospital Organization Chiba Medical Center Chibahigashi National Hospital, Chiba, Japan.
Toshiyuki KomiyaDepartment of Nephrology, Japanese Red Cross Otsu Hospital, Otsu, Shiga, Japan.
Masayuki IshiharaDepartment of Pediatrics, Kochi Medical School, Kochi University, Kochi, Japan.
Yoshiki NagaoDepartment of Pediatrics, Kochi Medical School, Kochi University, Kochi, Japan.
Naoaki MikamiDepartment of Nephrology and Rheumatology, Tokyo Metropolitan Children's Medical Center, Tokyo, Japan.
Yuta InokiDepartment of Pediatrics, Kobe University Graduate School of Medicine, 7-5-1 Kusunoki-Cho, Chuo-Ku, Kobe, Hyogo, 650-0017, Japan.
Nana SakakibaraDepartment of Pediatrics, Kobe University Graduate School of Medicine, 7-5-1 Kusunoki-Cho, Chuo-Ku, Kobe, Hyogo, 650-0017, Japan.
Tomoko HorinouchiDepartment of Pediatrics, Kobe University Graduate School of Medicine, 7-5-1 Kusunoki-Cho, Chuo-Ku, Kobe, Hyogo, 650-0017, Japan.
Tomohiko YamamuraDepartment of Pediatrics, Kobe University Graduate School of Medicine, 7-5-1 Kusunoki-Cho, Chuo-Ku, Kobe, Hyogo, 650-0017, Japan.
Shingo IshimoriDepartment of Pediatrics, Kobe University Graduate School of Medicine, 7-5-1 Kusunoki-Cho, Chuo-Ku, Kobe, Hyogo, 650-0017, Japan.
Kandai NozuDepartment of Pediatrics, Kobe University Graduate School of Medicine, 7-5-1 Kusunoki-Cho, Chuo-Ku, Kobe, Hyogo, 650-0017, Japan.

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

backgroundCoenzyme Q10 (CoQ10) nephropathy is a rare mitochondrial kidney disease caused by defects in CoQ10 biosynthesis and represents a unique form of steroid-resistant nephrotic syndrome with a disease-specific therapy. However, data on treatment outcomes of this disease in Japanese patients remain limited.

methodsWe conducted a retrospective observational study of Japanese patients. Patients with a genetically confirmed diagnosis of CoQ10 nephropathy who received CoQ10 supplementation and had available longitudinal clinical data were included. Changes in the urinary protein-to-creatinine ratio (UPCR) and estimated glomerular filtration rate before and after treatment were evaluated, and adverse events were assessed.

resultsTwelve patients were included in the analysis. The median age at treatment initiation was 9.0 years, and COQ8B was the predominant causative gene (n = 11). One patient harbored a COQ6 variant. CoQ10 supplementation was initiated at a median dose of 10.0 mg/kg/day. The median UPCR decreased from 1.66 g/gCr at baseline to 0.19 g/gCr at 12 months, and 6/7 (86%) patients with available 12-month data achieved a ≥ 50% reduction in proteinuria. Kidney function remained stable, and no patients progressed to end-stage kidney disease during a median follow-up of 28.8 months. Adverse events were mild and did not lead to treatment discontinuation.

conclusionsIn Japanese patients with CoQ10 nephropathy, CoQ10 supplementation was associated with a substantial reduction in proteinuria and stabilization of kidney function. These findings indicate the importance of early genetic diagnosis and prompt initiation of targeted therapy for this treatable hereditary kidney disease.

Indexed as

CoQ10 nephropathyCoQ10 supplementationCOQ6COQ8B

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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the Socratic graph.