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      Incidence of remission and relapse of proteinuria, end-stage kidney disease, mortality, and major outcomes in primary nephrotic syndrome: the Japan Nephrotic Syndrome Cohort Study (JNSCS)

      research-article
      1 , 2 , , 3 , 4 , 5 , 6 , 7 , 8 , 9 , 10 , 11 , 12 , 13 , 14 , 15 , 16 , 17 , 18 , 19 , 20 , 21 , 22 , 23 , 24 , 25 , 26 , 27 , 28 , 29 , 30 , 31 , 32 , 33 , 34 , 35 , 36 , 37 , 38 , 39 , 40 , 41 , 42 , 43 , 44 , 45 , 46 , 47 , 48 , 49 , 50 , 51 , 52 , 53 , 54 , 55 , 4 , 56 , 2
      Clinical and Experimental Nephrology
      Springer Singapore
      Primary nephrotic syndrome, Cohort study, Mortality, End-stage kidney disease, Diabetes, Infection

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          Abstract

          Background

          Despite recent advances in immunosuppressive therapy for patients with primary nephrotic syndrome, its effectiveness and safety have not been fully studied in recent nationwide real-world clinical data in Japan.

          Methods

          A 5-year cohort study, the Japan Nephrotic Syndrome Cohort Study, enrolled 374 patients with primary nephrotic syndrome in 55 hospitals in Japan, including 155, 148, 38, and 33 patients with minimal change disease (MCD), membranous nephropathy (MN), focal segmental glomerulosclerosis (FSGS), and other glomerulonephritides, respectively. The incidence rates of remission and relapse of proteinuria, 50% and 100% increases in serum creatinine, end-stage kidney disease (ESKD), all-cause mortality, and other major adverse outcomes were compared among glomerulonephritides using the Log-rank test. Incidence of hospitalization for infection, the most common cause of mortality, was compared using a multivariable-adjusted Cox proportional hazard model.

          Results

          Immunosuppressive therapy was administered in 339 (90.6%) patients. The cumulative probabilities of complete remission within 3 years of the baseline visit was ≥ 0.75 in patients with MCD, MN, and FSGS (0.95, 0.77, and 0.79, respectively). Diabetes was the most common adverse events associated with immunosuppressive therapy (incidence rate, 71.0 per 1000 person-years). All-cause mortality (15.6 per 1000 person-years), mainly infection-related mortality (47.8%), was more common than ESKD (8.9 per 1000 person-years), especially in patients with MCD and MN. MCD was significantly associated with hospitalization for infection than MN.

          Conclusions

          Patients with MCD and MN had a higher mortality, especially infection-related mortality, than ESKD. Nephrologists should pay more attention to infections in patients with primary nephrotic syndrome.

          Electronic supplementary material

          The online version of this article (10.1007/s10157-020-01864-1) contains supplementary material, which is available to authorized users.

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          Most cited references23

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          Eligibility criteria of randomized controlled trials published in high-impact general medical journals: a systematic sampling review.

          Selective eligibility criteria of randomized controlled trials (RCTs) are vital to trial feasibility and internal validity. However, the exclusion of certain patient populations may lead to impaired generalizability of results. To determine the nature and extent of exclusion criteria among RCTs published in major medical journals and the contribution of exclusion criteria to the representation of certain patient populations. The MEDLINE database was searched for RCTs published between 1994 and 2006 in certain general medical journals with a high impact factor. Of 4827 articles, 283 were selected using a series technique. Trial characteristics and the details regarding exclusions were extracted independently. All exclusion criteria were graded independently and in duplicate as either strongly justified, potentially justified, or poorly justified according to previously developed and pilot-tested guidelines. Common medical conditions formed the basis for exclusion in 81.3% of trials. Patients were excluded due to age in 72.1% of all trials (60.1% in pediatric populations and 38.5% in older adults). Individuals receiving commonly prescribed medications were excluded in 54.1% of trials. Conditions related to female sex were grounds for exclusion in 39.2% of trials. Of all exclusion criteria, only 47.2% were graded as strongly justified in the context of the specific RCT. Exclusion criteria were not reported in 12.0% of trials. Multivariable analyses revealed independent associations between the total number of exclusion criteria and drug intervention trials (risk ratio, 1.35; 95% confidence interval, 1.11-1.65; P = .003) and between the total number of exclusion criteria and multicenter trials (risk ratio, 1.26; 95% confidence interval, 1.06-1.52; P = .009). Industry-sponsored trials were more likely to exclude individuals due to concomitant medication use, medical comorbidities, and age. Drug intervention trials were more likely to exclude individuals due to concomitant medication use, medical comorbidities, female sex, and socioeconomic status. Among such trials, justification for exclusions related to concomitant medication use and comorbidities were more likely to be poorly justified. The RCTs published in major medical journals do not always clearly report exclusion criteria. Women, children, the elderly, and those with common medical conditions are frequently excluded from RCTs. Trials with multiple centers and those involving drug interventions are most likely to have extensive exclusions. Such exclusions may impair the generalizability of RCT results. These findings highlight a need for careful consideration and transparent reporting and justification of exclusion criteria in clinical trials.
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            The incidence of primary glomerulonephritis worldwide: a systematic review of the literature.

            Little is known about the worldwide variation in incidence of primary glomerulonephritis (GN). The objective of this review was to critically appraise studies of incidence published in 1980-2010 so that an overall view of trends of these diseases can be found. This would provide important information for determining changes in rates and understanding variations between countries. All relevant papers found through searches of Medline, Embase and ScienceDirect were critically appraised and an assessment was made of the reliability of the reported incidence data. This review includes 40 studies of incidence of primary GN from Europe, North and South America, Canada, Australasia and the Middle East. Rates for the individual types of disease were found to be in adults, 0.2/100,000/year for membrano-proliferative GN, 0.2/100,000/year for mesangio-proliferative GN, 0.6/100,000/year for minimal change disease, 0.8/100,000/year for focal segmental glomerulosclerosis, 1.2/100,000/year for membranous nephropathy and 2.5/100,000/year for IgA nephropathy. Rates were lower in children at around 0.1/100,000/year with the exception of minimal change disease where incidence was reported to be 2.0/100,000/year in Caucasian children with higher rates in Arabian children (9.2/100,000/year) and Asian children (6.2-15.6/100,000/year). This study found that incidence rates of primary GN vary between 0.2/100,000/year and 2.5/100,000/year. The incidence of IgA nephropathy is at least 2.5/100,000/year in adults; this disease can exist subclinically and is therefore only detected by chance in some patients. In addition, referral policies for diagnostic biopsy vary between countries. This will affect the incidence rates found.
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              Adult minimal-change disease: clinical characteristics, treatment, and outcomes.

              Minimal-change disease (MCD) counts for 10 to 15% of cases of primary nephrotic syndrome in adults. Few series have examined this disease in adults. A retrospective review was performed of 95 adults who had MCD and were seen at a single referral center. Examined were presenting features, response to daily versus alternate-day steroids, response to second-line agents, relapse patterns, complications of the disease and therapy, presence of acute renal failure (ARF), and outcome data. Sixty-five patients received daily and 23 received alternate-day steroids initially. There were no differences in remissions, time to remission, relapse rate, or time to relapse between daily- and alternate-day-treated patients. More than one quarter of patients were steroid resistant. At least one relapse occurred in 73% of patients; 28% were frequently relapsing. A significant proportion of frequently relapsing patients became steroid dependent. Second-line agents were used for steroid dependence, steroid resistance, or frequent relapses. No single agent proved superior. There were more remissions with second-line agents in steroid-dependent patients compared with steroid-resistant patients, and remissions were more likely to be complete in steroid-dependent patients. ARF occurred in 24 patients; they tended to be older and hypertensive with lower serum albumin and more proteinuria than those without ARF. At follow up, patients with an episode of ARF had higher serum creatinine than those without ARF. Four patients progressed to ESRD. These patients were less likely to have responded to steroids and more likely to have FSGS on repeat renal biopsy. In this referral MCD population, response to daily and alternate-day steroids is similar. Second-line agents give greater response in patients who are steroid dependent. ARF occurs in a significant number of adult MCD patients and may leave residual renal dysfunction. Few patients progress to ESRD.
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                Author and article information

                Contributors
                yamamoto@hacc.osaka-u.ac.jp
                Journal
                Clin Exp Nephrol
                Clin. Exp. Nephrol
                Clinical and Experimental Nephrology
                Springer Singapore (Singapore )
                1342-1751
                1437-7799
                7 March 2020
                7 March 2020
                2020
                : 24
                : 6
                : 526-540
                Affiliations
                [1 ]GRID grid.136593.b, ISNI 0000 0004 0373 3971, Health and Counseling Center, , Osaka University, ; 1-17 Machikaneyama-cho, Toyonaka, Osaka 560-0043 Japan
                [2 ]GRID grid.136593.b, ISNI 0000 0004 0373 3971, Department of Nephrology, , Osaka University Graduate School of Medicine, ; 2-2-D11 Yamadaoka, Suita, Osaka 565-0871 Japan
                [3 ]Nakayamadera Imai Clinic, 2-8-18 Nakayamadera, Takarazuka, Hyogo 665-0861 Japan
                [4 ]GRID grid.27476.30, ISNI 0000 0001 0943 978X, Department of Nephrology, , Nagoya University Graduate School of Medicine, ; 65 Tsurumai-cho, Showa-ku, Nagoya, Aichi 466-8550 Japan
                [5 ]GRID grid.411998.c, ISNI 0000 0001 0265 5359, Department of Nephrology, , Kanazawa Medical Univeristy School of Medicine, ; 1-1 Daigaku, Uchinada, Kahoku, Ishikawa 920-0293 Japan
                [6 ]GRID grid.261356.5, ISNI 0000 0001 1302 4472, Department of Nephrology, Rheumatology, Endocrinology and Metabolism, , Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, ; 2-5-1 Shikatacho, Kita-ku, Okayama, Okayama 700-8558 Japan
                [7 ]GRID grid.410818.4, ISNI 0000 0001 0720 6587, Department of Nephrology, , Tokyo Women’s Medical University, ; 8-1 Kawada-cho, Shinjuku-ku, Tokyo, 162-8666 Japan
                [8 ]GRID grid.415392.8, ISNI 0000 0004 0378 7849, Department of Nephrology and Dialysis, Kitano Hospital, , Tazuke Kofukai Medical Research Institute, ; 2-4-20 Ogimachi, Kita-ku, Osaka, Osaka 530-8480 Japan
                [9 ]GRID grid.264706.1, ISNI 0000 0000 9239 9995, Department of Internal Medicine, , Teikyo University School of Medicine, ; 2-11-1 Kaga, Itabashi-ku, Tokyo, 173-8606 Japan
                [10 ]GRID grid.413410.3, Kidney Disease Center, , Japanese Red Cross Nagoya Daini Hospital, ; 2-9 Myokencho, Showa-ku, Nagoya, Aichi 466-8650 Japan
                [11 ]GRID grid.415512.6, ISNI 0000 0004 0618 9318, Department of Nephrology, , JCHO Sendai Hospital, ; 3-16-1 Tsutsumi-machi, Aoba-ku, Sendai, Miyagi 981-8501 Japan
                [12 ]GRID grid.9707.9, ISNI 0000 0001 2308 3329, Department of Nephrology and Laboratory Medicine, , Kanazawa University, ; 13-1 Takara-machi, Kanazawa, Ishikawa 920-8641 Japan
                [13 ]GRID grid.256115.4, ISNI 0000 0004 1761 798X, Department of Nephrology, , Fujita Health University School of Medicine, ; 1-98 Dengakugakubo, Kutsukakecho, Toyoake, Aichi 470-1192 Japan
                [14 ]GRID grid.410814.8, ISNI 0000 0004 0372 782X, First Department of Internal Medicine, , Nara Medical University, ; 840 Shijocho, Kashihara, Nara 634-8522 Japan
                [15 ]GRID grid.417245.1, ISNI 0000 0004 1774 8664, Division of Nephrology, Department of Internal Medicine, , Toyonaka Municipal Hospital, ; 4-14-1 Shibaharacho, Toyonaka, Osaka 560-8565 Japan
                [16 ]GRID grid.177174.3, ISNI 0000 0001 2242 4849, Department of Integrated Therapy for Chronic Kidney Disease, Graduate School of Medical Sciences, , Kyushu University, ; 3-1-1 Maidashi, Higashi-ku, Fukuoka, Fukuoka 812-8582 Japan
                [17 ]GRID grid.411497.e, ISNI 0000 0001 0672 2176, Division of Nephrology and Rheumatology, Department of Internal Medicine, Faculty of Medicine, , Fukuoka University, ; 7-45-1 Nanakuma, Jonan-ku, Fukuoka, Fukuoka 814-0180 Japan
                [18 ]GRID grid.268394.2, ISNI 0000 0001 0674 7277, Department of Cardiology, Pulmonology, and Nephrology, , Yamagata University School of Medicine, ; 2-2 Iida-Nishi, Yamagata-shi, Yamagata, Yamagata 990-9585 Japan
                [19 ]GRID grid.416948.6, ISNI 0000 0004 1764 9308, Department of Kidney Disease and Hypertension, , Osaka General Medical Center, ; 3-1-56 Bandaihigashi, Sumiyoshi-ku, Osaka, Osaka 558-8558 Japan
                [20 ]GRID grid.459633.e, ISNI 0000 0004 1763 1845, Department of Nephrology, , Konan Kosei Hospital, ; 137 Omatsubara, Takayacho, Konan, Aichi 483-8704 Japan
                [21 ]GRID grid.31432.37, ISNI 0000 0001 1092 3077, Division of Nephrology and Kidney Center, , Kobe University Graduate School of Medicine, ; 7-5-1 Kusunokicho, Cuho-ku, Kobe, Hyogo 650-0017 Japan
                [22 ]GRID grid.413779.f, ISNI 0000 0004 0377 5215, Department of Nephrology, , Anjo Kosei Hospital, ; 28 Higashihirokute, Anjocho, Anjo, Aichi 446-8602 Japan
                [23 ]GRID grid.39158.36, ISNI 0000 0001 2173 7691, Division of Rheumatology, Endocrinology and Nephrology, , Hokkaido University Graduate School of Medicine, ; Kita 15, Nishi 7, Kita-ku, Sapporo, Hokkaido 060-8638 Japan
                [24 ]Department of Nephrology, Ichinomiya Municipal Hospital, 2-2-22 Bunkyo, Ichinomiya, Aichi 491-8558 Japan
                [25 ]GRID grid.267335.6, ISNI 0000 0001 1092 3579, Department of Nephrology, Institute of Biomedical Sciences, , Tokushima University Graduate School, ; 3-18-15, Kuramoto-cho, Tokushima, 770-8503 Japan
                [26 ]GRID grid.20515.33, ISNI 0000 0001 2369 4728, Department of Nephrology, Faculty of Medicine, , University of Tsukuba, ; 1-1-1 Tennodai, Tsukuba, Ibaraki 305-8575 Japan
                [27 ]GRID grid.410802.f, ISNI 0000 0001 2216 2631, Department of Nephrology and Hypertension, Saitama Medical Center, , Saitama Medical University, ; 1981 Kamoda, Kawagoe, Saitama 350-850 Japan
                [28 ]GRID grid.505613.4, Internal Medicine 1, , Hamamatsu University School of Medicine, ; 1-20-1 Handayama, Higashi-ku, Hamamatsu, Shizuoka 431-3192 Japan
                [29 ]GRID grid.414932.9, ISNI 0000 0004 0378 818X, Department of Nephrology, , Japanese Red Cross Nagoya Daiichi Hospital, ; 3-35 Michishitacho, Nakamura-ku, Nagoya, Aichi 453-8511 Japan
                [30 ]GRID grid.415067.1, ISNI 0000 0004 1772 4590, Department of Nephrology, , Kasugai Municipal Hospital, ; 1-1-1 Takakicho, Kasugai, Aichi 486-8510 Japan
                [31 ]GRID grid.411873.8, ISNI 0000 0004 0616 1585, Department of Nephrology, , Nagasaki University Hospital, ; 1-7-1 Sakamoto, Nagasaki, Nagasaki 852-8501 Japan
                [32 ]GRID grid.416762.0, ISNI 0000 0004 1772 7492, Department of Diabetology and Nephrology, , Ogaki Municipal Hospital, ; 4-86 Minaminokawacho, Ogaki, Gifu 503-8502 Japan
                [33 ]GRID grid.415804.c, ISNI 0000 0004 1763 9927, Department of Nephrology, , Shizuoka General Hospital, ; 4-27-1 Kitaando, Aoi-ku, Shizuoka, Shizuoak 420-8527 Japan
                [34 ]Department of Nephrology, Chutoen General Medical Center, 1-1 Shobugaike, Kakegawa, Shizuoka 436-8555 Japan
                [35 ]GRID grid.412567.3, Division of Nephrology, , Shimane University Hospital, ; 89-1 Enyacho, Izumo, Shimane 693-8501 Japan
                [36 ]GRID grid.278276.e, ISNI 0000 0001 0659 9825, Department of Endocrinology, Metabolism and Nephrology, Kochi Medical School, , Kochi University, ; Okocho Kohasu, Nankoku, Kochi 783-8505 Japan
                [37 ]GRID grid.470350.5, Kideny Unit, , National Hospital Organization Fukuokahigashi Medical Center, ; 1-1-1 Chidori, Koga, Fukuoka 811-3195 Japan
                [38 ]GRID grid.410849.0, ISNI 0000 0001 0657 3887, Department of Hemovascular Medicine and Artificial Organs, Faculty of Medicine, , University of Miyazaki, ; 5200 Kihara, Kiyotakecho, Miyazaki, Miyazaki 889-1692 Japan
                [39 ]GRID grid.410802.f, ISNI 0000 0001 2216 2631, Department of Nephrology, , Saitama Medical University, ; 38 Morohongo, Moroyama, Iruma, Saitama 350-0495 Japan
                [40 ]GRID grid.261445.0, ISNI 0000 0001 1009 6411, Department of Nephrology, , Osaka City University Graduate School of Medicine, ; 1-4-3 Asahimachi, Abeno-ku, Osaka, 545-8585 Japan
                [41 ]GRID grid.411582.b, ISNI 0000 0001 1017 9540, Department of Nephrology and Hypertension, , Fukushima Medical University School of Medicine, ; 1 Hikariga-oka, Fukushima-City, Fukushima 960-1295 Japan
                [42 ]GRID grid.256642.1, ISNI 0000 0000 9269 4097, Department of Nephrology and Rheumatology, , Gunma University Graduate School of Medicine, ; 3-39-22 Showa-matchi, Maebashi, Gunma 371-8511 Japan
                [43 ]GRID grid.415537.1, ISNI 0000 0004 1772 6537, Department of Nephrology, , Gifu Prefectural Tajimi Hospital, ; 5-161 Maebatacho, Tajimi, Gifu 507-8522 Japan
                [44 ]Department of Nephrology, Kainan Hospital, 396 Minamihonden, Maegasucho, Yatomi, Aichi 498-8502 Japan
                [45 ]GRID grid.417360.7, ISNI 0000 0004 1772 4873, Department of Nephrology, , Yokkaichi Municipal Hospital, ; 2-2-37 Shibata, Yokkaichi, Mie 510-8567 Japan
                [46 ]GRID grid.258331.e, ISNI 0000 0000 8662 309X, Department of Cardiorenal and Cerebrovascular Medicine, , Kagawa University, ; 1750-1 Ikenobe, Miki-cho, Kita-gun, Kagawa 761-0793 Japan
                [47 ]GRID grid.258269.2, ISNI 0000 0004 1762 2738, Division of Nephrology, Department of Internal Medicine, , Juntendo University Faculty of Medicine, ; 2-1-1 Hongo, Bunkyo-ku, Tokyo, 113-8421 Japan
                [48 ]GRID grid.412764.2, ISNI 0000 0004 0372 3116, Division of Nephrology and Hypertension, Department of Internal Medicine, , St. Marianna University School of Medicine, ; 2-16-1 Sugao, Miyamae-ku, Kawasaki, Kanagawa 216-000 Japan
                [49 ]GRID grid.414958.5, ISNI 0000 0004 0569 1891, Division of Internal Medicine, , National Hospital Organization Kanazawa Medical Center, ; 1-1 Shimoishibikimachi, Kanazawa, Ishikawa 920-8650 Japan
                [50 ]Department of Nephrology, Masuko Memorial Hospital, 35-28 Takegashicho, Nakamura-ku, Nagoya, Aichi 453-0016 Japan
                [51 ]GRID grid.410815.9, ISNI 0000 0004 0377 3746, Department of Nephrology, , Chubu Rosai Hospital, ; 1-10-6 Komei, Minato-ku, Nagoya, Aichi 455-8530 Japan
                [52 ]GRID grid.413634.7, ISNI 0000 0004 0604 6712, Department of Nephrology, , Handa City Hospital, ; 2-29 Toyocho, Handa, Aichi 475-8599 Japan
                [53 ]GRID grid.412857.d, ISNI 0000 0004 1763 1087, Department of Nephrology, , Wakayama Medical University, ; 811-1 Kimiidera, Wakayama-City, Wakayama 641-8509 Japan
                [54 ]GRID grid.415086.e, ISNI 0000 0001 1014 2000, Department of Nephrology and Hypertension, , Kawasaki Medical School, ; 577 Matsushima, Kurashiki, Osakayama 701-0192 Japan
                [55 ]GRID grid.69566.3a, ISNI 0000 0001 2248 6943, Department of Nephrology, Endocrinology, and Vascular Medicine, , Tohoku Univeristy Gradaute School of Medicine, ; 1-1 Seiryo-cho, Aoba-ku, Sendai, Miyagi 980-8574 Japan
                [56 ]GRID grid.260975.f, ISNI 0000 0001 0671 5144, Division of Clinical Nephrology and Rheumatology, Kidney Research Center, , Niigata University Graduate School of Medical and Dental Sciences, ; 757 Ichibancho, Asahimachi-dori, Chuo Ward, Niigata, Niigata 951-8510 Japan
                Author information
                http://orcid.org/0000-0003-2610-9824
                Article
                1864
                10.1007/s10157-020-01864-1
                7248042
                32146646
                7273aa6f-c5d7-47fb-b8ca-60870b119057
                © The Author(s) 2020

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                History
                : 24 December 2019
                : 18 February 2020
                Funding
                Funded by: FundRef http://dx.doi.org/10.13039/501100003478, Ministry of Health, Labour and Welfare;
                Categories
                Original Article
                Custom metadata
                © Japanese Society of Nephrology 2020

                Nephrology
                primary nephrotic syndrome,cohort study,mortality,end-stage kidney disease,diabetes,infection

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