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3. Deep learning analysis of clinical course of primary nephrotic syndrome: Japan Nephrotic Syndrome Cohort Study (JNSCS)

Author

Kimura, Tomonori, Yamamoto, Ryohei, Yoshino, Mitsuaki, Sakate, Ryuichi, Imai, Enyu, Maruyama, Shoichi, Yokoyama, Hitoshi, Sugiyama, Hitoshi, Nitta, Kosaku, Tsukamoto, Tatsuo, Uchida, Shunya, Takeda, Asami, Sato, Toshinobu, Wada, Takashi, Hayashi, Hiroki, Akai, Yasuhiro, Fukunaga, Megumu, Tsuruya, Kazuhiko, Masutani, Kosuke, Konta, Tsuneo, Shoji, Tatsuya, Hiramatsu, Takeyuki, Goto, Shunsuke, Tamai, Hirofumi, Nishio, Saori, Nagai, Kojiro, Yamagata, Kunihiro, Yasuda, Hideo, Ichida, Shizunori, Naruse, Tomohiko, Nishino, Tomoya, Sobajima, Hiroshi, Akahori, Toshiyuki, Ito, Takafumi, Terada, Yoshio, Katafuchi, Ritsuko, Fujimoto, Shouichi, Okada, Hirokazu, Mimura, Tetsushi, Suzuki, Satoshi, Saka, Yosuke, Sofue, Tadashi, Kitagawa, Kiyoki, Fujita, Yoshiro, Mizutani, Makoto, Kashihara, Naoki, Sato, Hiroshi, Narita, Ichiei, and Isaka, Yoshitaka

Published
2022
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5. Time to remission of proteinuria and incidence of relapse in patients with steroid-sensitive minimal change disease and focal segmental glomerulosclerosis: the Japan Nephrotic Syndrome Cohort Study

Author

Yamamoto, Ryohei, Imai, Enyu, Maruyama, Shoichi, Yokoyama, Hitoshi, Sugiyama, Hitoshi, Takeda, Asami, Tsukamoto, Tatsuo, Uchida, Shunya, Tsuruya, Kazuhiko, Shoji, Tatsuya, Hayashi, Hiroki, Akai, Yasuhiro, Fukunaga, Megumu, Konta, Tsuneo, Nishio, Saori, Goto, Shunsuke, Tamai, Hirofumi, Nagai, Kojiro, Katafuchi, Ritsuko, Masutani, Kosuke, Wada, Takashi, Nishino, Tomoya, Shirasaki, Arimasa, Sobajima, Hiroshi, Nitta, Kosaku, Yamagata, Kunihiro, Kazama, Junichiro J., Hiromura, Keiju, Yasuda, Hideo, Mizutani, Makoto, Akahori, Toshiyuki, Naruse, Tomohiko, Hiramatsu, Takeyuki, Morozumi, Kunio, Mimura, Tetsushi, Saka, Yosuke, Ishimura, Eiji, Hasegawa, Hajime, Ichikawa, Daisuke, Shigematsu, Takashi, Sato, Hiroshi, Narita, Ichiei, and Isaka, Yoshitaka

Published
2022
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6. Predictors of early remission of proteinuria in adult patients with minimal change disease: a retrospective cohort study

Author

Yamamoto, Ryohei, Imai, Enyu, Maruyama, Shoichi, Yokoyama, Hitoshi, Sugiyama, Hitoshi, Takeda, Asami, Uchida, Shunya, Tsukamoto, Tatsuo, Tsuruya, Kazuhiko, Akai, Yasuhiro, Nitta, Kosaku, Fukunaga, Megumu, Hayashi, Hiroki, Masutani, Kosuke, Wada, Takashi, Konta, Tsuneo, Katafuchi, Ritsuko, Nishio, Saori, Goto, Shunsuke, Tamai, Hirofumi, Shirasaki, Arimasa, Shoji, Tatsuya, Nagai, Kojiro, Nishino, Tomoya, Yamagata, Kunihiro, Kazama, Junichiro J., Hiromura, Keiju, Yasuda, Hideo, Mizutani, Makoto, Naruse, Tomohiko, Hiramatsu, Takeyuki, Morozumi, Kunio, Sobajima, Hiroshi, Saka, Yosuke, Ishimura, Eiji, Ichikawa, Daisuke, Shigematsu, Takashi, Sofue, Tadashi, Fujimoto, Shouichi, Ito, Takafumi, Sato, Hiroshi, Narita, Ichiei, and Isaka, Yoshitaka

Published
2022
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8. Rationale and design of the Japanese Biomarkers in Nephrotic Syndrome (J-MARINE) study.

Author

Kurasawa, Shimon, Kato, Sawako, Ozeki, Takaya, Akiyama, Shin'ichi, Ishimoto, Takuji, Mizuno, Masashi, Tsuboi, Naotake, Kato, Noritoshi, Kosugi, Tomoki, Maruyama, Shoichi, Tamai, Hirofumi, Takeda, Asami, Shinjo, Hibiki, Arata, Hanayo, Naruse, Tomohiko, Watanabe, Tomoharu, Hiromura, Keiju, Fukami, Kei, Nakagawa, Naoki, and Akahori, Toshiyuki

Subjects
NEPHROTIC syndrome, BIOMARKERS, PLASMINOGEN activators, JAPANESE people, FOCAL segmental glomerulosclerosis, GLOMERULAR filtration rate
Abstract

Introduction: Disease subtyping and monitoring are essential for the management of nephrotic syndrome (NS). Although various biomarkers for NS have been reported, their clinical efficacy has not been comprehensively validated in adult Japanese patients. Methods: The Japanese Biomarkers in Nephrotic Syndrome (J-MARINE) study is a nationwide, multicenter, and prospective cohort study in Japan, enrolling adult (≥18 years) patients with minimal change disease (MCD), focal segmental glomerulosclerosis (FSGS), membranous nephropathy (MN), membranoproliferative glomerulonephritis (MPGN), C3 glomerulopathy (C3G), and lupus nephritis (LN). Baseline clinical information and plasma and urine samples will be collected at the time of immunosuppressive therapy initiation or biopsy. Follow-up data and plasma and urine samples will be collected longitudinally based on the designated protocols. Candidate biomarkers will be measured: CD80, cytotoxic T-lymphocyte antigen 4, and soluble urokinase plasminogen activator receptor for MCD and FSGS; anti-phospholipase A2 receptor and thrombospondin type-1 domain-containing protein 7A antibodies for MN; fragment Ba, C3a, factor I, and properdin for MPGN/C3G; and CD11b, CD16b, and CD163 for LN. Outcomes include complete and partial remission, relapse of proteinuria, a 30% reduction in estimated glomerular filtration rate (eGFR), eGFR decline, and initiation of renal replacement therapy. The diagnostic accuracy and predictive ability for clinical outcomes will be assessed for each biomarker. Results: From April 2019 to April 2023, 365 patients were enrolled: 145, 21, 138, 10, and 51 cases of MCD, FSGS, MN, MPGN/C3G, and LN, respectively. Conclusion: This study will provide valuable insights into biomarkers for NS and serve as a biorepository for future studies. [ABSTRACT FROM AUTHOR]

Published
2024
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9. Better remission rates in elderly Japanese patients with primary membranous nephropathy in nationwide real-world practice: The Japan Nephrotic Syndrome Cohort Study (JNSCS)

Author

Yokoyama, Hitoshi, Yamamoto, Ryohei, Imai, Enyu, Maruyama, Shoichi, Sugiyama, Hitoshi, Nitta, Kosaku, Tsukamoto, Tatsuo, Uchida, Shunya, Takeda, Asami, Sato, Toshinobu, Wada, Takashi, Hayashi, Hiroki, Akai, Yasuhiro, Fukunaga, Megumu, Tsuruya, Kazuhiko, Masutani, Kosuke, Konta, Tsuneo, Shoji, Tatsuya, Hiramatsu, Takeyuki, Goto, Shunsuke, Tamai, Hirofumi, Nishio, Saori, Shirasaki, Arimasa, Nagai, Kojiro, Yamagata, Kunihiro, Hasegawa, Hajime, Yasuda, Hidemo, Ichida, Shizunori, Naruse, Tomohiko, Fukami, Kei, Nishino, Tomoya, Sobajima, Hiroshi, Tanaka, Satoshi, Akahori, Toshiyuki, Ito, Takafumi, Terada, Yoshio, Katafuchi, Ritsuko, Fujimoto, Shouichi, Okada, Hirokazu, Ishimura, Eiji, Kazama, Junichiro James, Hiromura, Keiju, Mimura, Tetsushi, Suzuki, Satashi, Saka, Yosuke, Sofue, Tadashi, Suzuki, Yusuke, Shibagaki, Yugo, Kitagawa, Kiyoki, Morozumi, Kunio, Fujita, Yoshiro, Mizutani, Makoto, Shigematsu, Takashi, Furuichi, Kengo, Fujimoto, Keiji, Kashihara, Naoki, Sato, Hiroshi, Matsuo, Seiichi, Narita, Ichiei, and Isaka, Yoshitaka

Published
2020
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10. 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)

Author

Yamamoto, Ryohei, Imai, Enyu, Maruyama, Shoichi, Yokoyama, Hitoshi, Sugiyama, Hitoshi, Nitta, Kosaku, Tsukamoto, Tatsuo, Uchida, Shunya, Takeda, Asami, Sato, Toshinobu, Wada, Takashi, Hayashi, Hiroki, Akai, Yasuhiro, Fukunaga, Megumu, Tsuruya, Kazuhiko, Masutani, Kosuke, Konta, Tsuneo, Shoji, Tatsuya, Hiramatsu, Takeyuki, Goto, Shunsuke, Tamai, Hirofumi, Nishio, Saori, Shirasaki, Arimasa, Nagai, Kojiro, Yamagata, Kunihiro, Hasegawa, Hajime, Yasuda, Hideo, Ichida, Shizunori, Naruse, Tomohiko, Nishino, Tomoya, Sobajima, Hiroshi, Tanaka, Satoshi, Akahori, Toshiyuki, Ito, Takafumi, Terada, Yoshio, Katafuchi, Ritsuko, Fujimoto, Shouichi, Okada, Hirokazu, Ishimura, Eiji, Kazama, Junichiro J., Hiromura, Keiju, Mimura, Tetsushi, Suzuki, Satoshi, Saka, Yosuke, Sofue, Tadashi, Suzuki, Yusuke, Shibagaki, Yugo, Kitagawa, Kiyoki, Morozumi, Kunio, Fujita, Yoshiro, Mizutani, Makoto, Shigematsu, Takashi, Kashihara, Naoki, Sato, Hiroshi, Matsuo, Seiichi, Narita, Ichiei, and Isaka, Yoshitaka

Published
2020
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12. Regional variations in immunosuppressive therapy in patients with primary nephrotic syndrome: the Japan nephrotic syndrome cohort study

Author

Yamamoto, Ryohei, Imai, Enyu, Maruyama, Shoichi, Yokoyama, Hitoshi, Sugiyama, Hitoshi, Nitta, Kosaku, Tsukamoto, Tatsuo, Uchida, Shunya, Takeda, Asami, Sato, Toshinobu, Wada, Takashi, Hayashi, Hiroki, Akai, Yasuhiro, Fukunaga, Megumu, Tsuruya, Kazuhiko, Masutani, Kosuke, Konta, Tsuneo, Shoji, Tatsuya, Hiramatsu, Takeyuki, Goto, Shunsuke, Tamai, Hirofumi, Nishio, Saori, Shirasaki, Arimasa, Nagai, Kojiro, Yamagata, Kunihiro, Hasegawa, Hajime, Yasuda, Hidemo, Ichida, Shizunori, Naruse, Tomohiko, Fukami, Kei, Nishino, Tomoya, Sobajima, Hiroshi, Tanaka, Satoshi, Akahori, Toshiyuki, Ito, Takafumi, Yoshio, Terada, Katafuchi, Ritsuko, Fujimoto, Shouichi, Okada, Hirokazu, Ishimura, Eiji, Kazama, Junichiro James, Hiromura, Keiju, Mimura, Tetsushi, Suzuki, Satashi, Saka, Yosuke, Sofue, Tadashi, Suzuki, Yusuke, Shibagaki, Yugo, Kitagawa, Kiyoki, Morozumi, Kunio, Fujita, Yoshiro, Mizutani, Makoto, Shigematsu, Takashi, Kashihara, Naoki, Sato, Hiroshi, Matsuo, Seiichi, Narita, Ichiei, and Isaka, Yoshitaka

Published
2018
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16. Recent analysis of status and outcomes of peritoneal dialysis in the Tokai area of Japan: the second report of the Tokai peritoneal dialysis registry

Author

Mizuno, Masashi, Ito, Yasuhiko, Suzuki, Yasuhiro, Sakata, Fumiko, Saka, Yosuke, Hiramatsu, Takeyuki, Tamai, Hirofumi, Mizutani, Makoto, Naruse, Tomohiko, Ohashi, Norimi, Kasuga, Hirotake, Shimizu, Hideaki, Kurata, Hisashi, Kurata, Kei, Suzuki, Satoshi, Kido, Satoko, Tsuruta, Yoshikazu, Matsuoka, Teppei, Horie, Masanobu, Maruyama, Shoichi, and Matsuo, Seiichi

Published
2016
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17. Acute Kidney Injury and Remission of Proteinuria in Minimal Change Disease

Author

Yamamoto, Ryohei, primary, Imai, Enyu, additional, Maruyama, Shoichi, additional, Yokoyama, Hitoshi, additional, Sugiyama, Hitoshi, additional, Takeda, Asami, additional, Uchida, Shunya, additional, Tsukamoto, Tatsuo, additional, Tsuruya, Kazuhiko, additional, Akai, Yasuhiro, additional, Nitta, Kosaku, additional, Fukunaga, Megumu, additional, Hayashi, Hiroki, additional, Shoji, Tatsuya, additional, Masutani, Kosuke, additional, Konta, Tsuneo, additional, Katafuchi, Ritsuko, additional, Nishio, Saori, additional, Wada, Takashi, additional, Goto, Shunsuke, additional, Tamai, Hirofumi, additional, Shirasaki, Arimasa, additional, Nagai, Kojiro, additional, Nishino, Tomoya, additional, Yamagata, Kunihiro, additional, Kazama, Junichiro J., additional, Hiromura, Keiju, additional, Yasuda, Hideo, additional, Sofue, Tadashi, additional, Fujimoto, Shouichi, additional, Mizutani, Makoto, additional, Naruse, Tomohiko, additional, Hiramatsu, Takeyuki, additional, Morozumi, Kunio, additional, Sobajima, Hiroshi, additional, Saka, Yosuke, additional, Ishimura, Eiji, additional, Ito, Takafumi, additional, Ichikawa, Daisuke, additional, Shigematsu, Takashi, additional, Sato, Hiroshi, additional, Narita, Ichiei, additional, and Yoshitaka, Isaka, additional

Published
2022
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18. Protocol for a Phase 1, Open-Label, Multiple-Center, Dose-Escalation Study to Evaluate the Safety and Tolerability of ADR-001 in the Treatment of Immunoglobulin A Nephropathy

Author

Tanaka, Akihito, primary, Furuhashi, Kazuhiro, additional, Fujieda, Kumiko, additional, Maeda, Kayaho, additional, Saito, Shoji, additional, Mimura, Tetsushi, additional, Saka, Yosuke, additional, Naruse, Tomohiko, additional, Ishimoto, Takuji, additional, Kosugi, Tomoki, additional, Kinoshita, Fumie, additional, Kuwatsuka, Yachiyo, additional, Shimizu, Shinobu, additional, Nakai, Yasuhiro, additional, and Maruyama, Shoichi, additional

Published
2022
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22. Peritonitis is still an important factor for withdrawal from peritoneal dialysis therapy in the Tokai area of Japan

Author

Mizuno, Masashi, Ito, Yasuhiko, Tanaka, Akio, Suzuki, Yasuhiro, Hiramatsu, Hideki, Watanabe, Midoriko, Tsuruta, Yoshikazu, Matsuoka, Teppei, Ito, Isao, Tamai, Hiroshi, Kasuga, Hirotake, Shimizu, Hideaki, Kurata, Hisashi, Inaguma, Daijo, Hiramatsu, Takeyuki, Horie, Masanobu, Naruse, Tomohiko, Maruyama, Shoichi, Imai, Enyu, Yuzawa, Yukio, and Matsuo, Seiichi

Published
2011
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24. 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)

Author

Yamamoto , Ryohei, Imai , Enyu, Maruyama, Shoichi, Yokoyama, Hitoshi, Sugiyama, Hitoshi, Nitta, Kosaku, Tsukamoto , Tatsuo, Uchida , Shunya, Takeda, Asami, Sato, Toshinobu, Wada, Takashi, Hayashi, Hiroki, Akai, Yasuhiro, Fukunaga, Megumu, Tsuruya, Kazuhiko, Masutani , Kosuke, Konta, Tsuneo, Shoji, Tatsuya, Hiramatsu, Takeyuki, Goto, Shunsuke, Tamai, Hirofumi, Nishio, Saori, Shirasaki , Arimasa, Nagai, Kojiro, Yamagata, Kunihiro, Hasegawa, Hajime, Yasuda, Hideo, Ichida, Shizunori, Naruse, Tomohiko, Nishino, Tomoya, Sobajima, Hiroshi, Tanaka, Satoshi, Akahori, Toshiyuki, ITO, Takafumi, Terada, Yoshio, Katafuchi, Ritsuko, Fujimoto, Shouichi, Okada, Hirokazu, Shimura, Eiji, Kazama, Junichiro J., Hiromura, Keiju, Mimura, Tetsushi, Suzuki, Satoshi, Saka, Yosuke, Sofue, Tadashi, Suzuki , Yusuke, Shibagaki , Yugo, Kitagawa , Kiyoki, Morozumi , Kunio, Fujita , Yoshiro, Mizutani , Makoto, Shigematsu , Takashi, Kashihara , Naoki, Sato , Hiroshi, Matsuo , Seiichi, Narita , Ichiei, Isaka , Yoshitaka, Yamamoto , Ryohei, Imai , Enyu, Maruyama, Shoichi, Yokoyama, Hitoshi, Sugiyama, Hitoshi, Nitta, Kosaku, Tsukamoto , Tatsuo, Uchida , Shunya, Takeda, Asami, Sato, Toshinobu, Wada, Takashi, Hayashi, Hiroki, Akai, Yasuhiro, Fukunaga, Megumu, Tsuruya, Kazuhiko, Masutani , Kosuke, Konta, Tsuneo, Shoji, Tatsuya, Hiramatsu, Takeyuki, Goto, Shunsuke, Tamai, Hirofumi, Nishio, Saori, Shirasaki , Arimasa, Nagai, Kojiro, Yamagata, Kunihiro, Hasegawa, Hajime, Yasuda, Hideo, Ichida, Shizunori, Naruse, Tomohiko, Nishino, Tomoya, Sobajima, Hiroshi, Tanaka, Satoshi, Akahori, Toshiyuki, ITO, Takafumi, Terada, Yoshio, Katafuchi, Ritsuko, Fujimoto, Shouichi, Okada, Hirokazu, Shimura, Eiji, Kazama, Junichiro J., Hiromura, Keiju, Mimura, Tetsushi, Suzuki, Satoshi, Saka, Yosuke, Sofue, Tadashi, Suzuki , Yusuke, Shibagaki , Yugo, Kitagawa , Kiyoki, Morozumi , Kunio, Fujita , Yoshiro, Mizutani , Makoto, Shigematsu , Takashi, Kashihara , Naoki, Sato , Hiroshi, Matsuo , Seiichi, Narita , Ichiei, and Isaka , Yoshitaka

Published
2020

29. Renoprotective effects of topiroxostat for Hyperuricaemic patients with overt diabetic nephropathy study (ETUDE study): A prospective, randomized, multicentre clinical trial

Author

Mizukoshi, Toshihiro, primary, Kato, Sawako, additional, Ando, Masahiko, additional, Sobajima, Hiroshi, additional, Ohashi, Norimi, additional, Naruse, Tomohiko, additional, Saka, Yosuke, additional, Shimizu, Hideaki, additional, Nagata, Takanobu, additional, and Maruyama, Shoichi, additional

Published
2018
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31. Quality of life of elderly patients on peritoneal dialysis versus hemodialysis: a single-center study.

Author

Saka, Yosuke, Naruse, Tomohiko, Tawada, Naoto, Tokomoto, Mitsuhiro, Noda, Yuhei, Taniguchi, Yohei, Nagahara, Yasuko, Yamashita, Ryoko, Karasawa, Munetoshi, Watanabe, Yuzo, and Ito, Yasuhiko

Subjects
*PERITONEAL dialysis, *HEMODIALYSIS, *QUALITY of life, *CHRONIC kidney failure, *OLDER patients
Abstract

Background: The number of elderly dialysis patients in Japan is dramatically increasing. Receiving therapy with better satisfaction through home care is one of the important factors in their daily lives. Thus, the quality of life of elderly patients on hemodialysis (HD) or peritoneal dialysis (PD) was evaluated. Methods: Clinical information of patients aged ≥80 years who started dialysis at our hospital between January 2013 and December 2015 was retrospectively collected. The mortality rate, length of hospitalization, and place of death were identified to evaluate patient quality of life. Results: In total, 56 patients (14 PD and 42 HD) were enrolled. The mean age of study subjects was 85.2 ± 4.0 years. The proportion of PD patients who lived with their family or have professional caregivers who could assist them in their daily life was higher than that of HD patients (100 vs. 76.2%, respectively; p = 0.044). Mortality rate was higher in PD patients than in HD patients ( p = 0.003), but long-term hospitalization of >180 days was observed only in HD patients (PD vs. HD: 0.0 vs. 16.7%; p = 0.102). In patients with Barthel index scores <100, the long-term hospitalization difference was significant (PD vs. HD: 0.0 vs. 30.4%; p = 0.040). Of note, 6 of 7 deceased PD patients and 1 of 10 deceased HD patients died at home ( p = 0.002). Conclusion: PD is a desirable home care therapy for elderly patients, but the burden on caregivers should be considered. [ABSTRACT FROM AUTHOR]

Published
2017
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38. Safety and Tolerability of ADR-001 Therapy for Immunoglobulin A Nephropathy.

Author

Tanaka A, Furuhashi K, Fujieda K, Horinouchi A, Maeda K, Saito S, Mimura T, Saka Y, Naruse T, Ishimoto T, Kato N, Kosugi T, Kinoshita F, Kuwatsuka Y, Nakai Y, and Maruyama S

Abstract

Background: Immunoglobulin A nephropathy often requires kidney replacement therapy because of its refractoriness and because corticosteroids pose infection risks. However, mesenchymal stem cells offer clinical benefits because of their regenerative and immunomodulatory properties. This prospective clinical trial assessed the safety and tolerability of adipose-derived mesenchymal stem cell therapy and evaluated its therapeutic efficacy., Methods: This phase 1 study included adult patients with refractory immunoglobulin A nephropathy that was difficult to treat with traditional therapies. Adipose-derived mesenchymal stem cell therapy comprising one intravenous dose of 1 × 108 cells was administered to three to six patients in cohort 1. The same intravenous dose was administered twice with a 2-week interval to six patients in cohort 2. Heparin was administered simultaneously. This study continued for 52 weeks, and the primary endpoints were safety and tolerability during the 6-week period after treatment administration. Secondary endpoints included adverse events and efficacy measures such as clinical remission, partial remission, urine protein remission, hematuria remission, time to remission, changes in the urine protein and hematuria levels, and changes in the estimated glomerular filtration rate., Results: The three patients in cohort 1 and six patients in cohort 2 who received adipose-derived mesenchymal stem cell therapy achieved the primary endpoints. No severe adverse clinical events were observed. Therefore, the safety and tolerability of adipose-derived mesenchymal stem cells were confirmed. Improvements such as significantly decreased kidney damage markers and urinary protein levels were observed immediately after adipose-derived mesenchymal stem cell administration., Conclusions: The safety and tolerability of adipose-derived mesenchymal stem cells are acceptable for patients with immunoglobulin A nephropathy., Trial Registration: This trial was registered with the Japan Registry of Clinical Trials (jRCT2043200002; registration date: April 14, 2020) and ClinicalTrials.gov (NCT04342325; registration date: April 13, 2020)., (Copyright © 2024 The Author(s). Published by Wolters Kluwer Health, Inc. on behalf of the American Society of Nephrology.)

Published
2024
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39. Successful rituximab treatment of TAFRO syndrome with pathological findings of glomerular endothelial damage.

Author

Noda Y, Saka Y, Kato A, Mimura T, and Naruse T

Abstract

Thrombocytopenia, anasarca, fever, renal insufficiency, and organomegaly constitute TAFRO syndrome, a variant of Castleman disease. We describe a patient with TAFRO syndrome who underwent renal biopsy. A 79-year-old woman was referred to us with fever and leg edema. She also had thrombocytopenia, pleural effusion, ascites, and acute kidney injury, and was admitted to our hospital. Her response to initial therapy with corticosteroid and cyclosporine was poor. Therefore, she received 4 doses of rituximab per week, which resulted in clinical improvement, including recovery of thrombocytopenia. A kidney biopsy thereafter showed diffuse, global glomerular endothelial injury indicating thrombotic microangiopathy (TMA). These findings suggested that TMA is associated with the thrombocytopenia and renal insufficiency of TAFRO syndrome.

Published
2018
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40. [Case of thrombotic thrombocytopenic purpura with a positive Coomb test].

Author

Urahama Y, Saka Y, Yaomura T, Naruse T, Watanabe Y, Yuzawa Y, and Matsuo S

Subjects
Acidosis, Lactic, Anemia, Hemolytic, Autoimmune, Fatal Outcome, Female, Humans, Middle Aged, Plasma Exchange, Prednisolone administration & dosage, Purpura, Thrombotic Thrombocytopenic complications, Purpura, Thrombotic Thrombocytopenic therapy, Respiratory Distress Syndrome complications, Shock, von Willebrand Factor, Coombs Test, Purpura, Thrombotic Thrombocytopenic diagnosis
Abstract

We report a case of thrombotic thrombocytopenic purpura (TTP) with a positive Coombs' test. A 59-year-old female was admitted to our hospital in February, 1997 with symptoms of heart failure. Ultrasound cardiography showed moderate pericardiac effusion and she was diagnosed as having pericarditis. After admission she had anorexia and her urine volume was reduced. Laboratory tests showed anemia and thrombocytopenia. Her Coombs' test result was positive. Her renal function gradually worsened and her conscious level was reduced. We diagnosed her as TTP and judged that she needed hemodialysis. We performed plasma exchange and started steroid therapy. The renal biopsy was compatible with TTP. After treatment, her level of consciousness improved, but her renal function did not improve. On the 51st hospital day she fell into acute respiratory distress syndrome (ARDS) and entered ICU. We considered ARDS caused by infection and continued treatment, but she died of shock and lactate acidosis. Activity of von Willebrand factor-cleaving protease in our case was 15% before the first PE, and 25 % just before death. A case of TTP without collagen disease usually shows a negative Coombs' test result. We think that this was a rare case in which autoimmune hemolytic anemia was supervened with TTP.

Published
2005

41. [Diet therapy for diabetic patients on dialysis].

Author

Watanabe Y, Saka Y, and Naruse T

Subjects
Dietary Fats administration & dosage, Dietary Proteins administration & dosage, Electrolytes administration & dosage, Energy Intake, Humans, Water administration & dosage, Diabetic Nephropathies diet therapy, Dialysis
Published
2004

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