Aging
Navigate
Research Paper|Volume 15, Issue 9|pp 3273—3294

Renal dysfunction, malignant neoplasms, atherosclerotic cardiovascular diseases, and sarcopenia as key outcomes observed in a three-year follow-up study using the Werner Syndrome Registry

Yukari Maeda1, Masaya Koshizaka1, Mayumi Shoji1, Hiyori Kaneko1, Hisaya Kato1, Yoshiro Maezawa1, Junji Kawashima2, Kayo Yoshinaga2, Mai Ishikawa3, Akiko Sekiguchi3, Sei-Ichiro Motegi3, Hironori Nakagami4, Yoshihiko Yamada5, Shinji Tsukamoto6, Akira Taniguchi6, Ken Sugimoto7, Yoichi Takami8, Yukiko Shoda9, Kunihiko Hashimoto10, Toru Yoshimura11, Asako Kogure12, Daisuke Suzuki12, Naoki Okubo13, Takashi Yoshida14, Kazuhisa Watanabe15, Masafumi Kuzuya16, Minoru Takemoto17, Junko Oshima1,18, Koutaro Yokote1
  • 1Department of Endocrinology, Hematology, and Gerontology, Chiba University Graduate School of Medicine, Chiba, Japan
  • 2Department of Metabolic Medicine, Faculty of Life Sciences, Kumamoto University, Kumamoto, Japan
  • 3Department of Dermatology, Gunma University Graduate School of Medicine, Maebashi, Japan
  • 4Department of Health Development and Medicine, Osaka University Graduate School of Medicine, Osaka, Japan
  • 5Department of Medicine, Division of Diabetes, Metabolism and Endocrinology, Atami Hospital, International University of Health and Welfare, Atami, Japan
  • 6Department of Orthopaedic Surgery, Nara Medical University, Nara, Japan
  • 7General Geriatric Medicine, Kawasaki Medical School, Okayama, Japan
  • 8Department of Geriatric and General Medicine, Osaka University Graduate School of Medicine, Osaka, Japan
  • 9Department of Dermatology, Sumitomo Hospital, Osaka, Japan
  • 10Department of Endocrinology and Metabolic Medicine, Nippon Life Hospital, Osaka, Japan
  • 11Diabetes and Endocrinology, Saga-Ken Medical Centre Koseikan, Saga, Japan
  • 12Department of Dermatology, Showa General Hospital, Tokyo, Japan
  • 13Department of Orthopaedics, Graduate School of Medical Science, Kyoto Prefectural University of Medicine, Kyoto, Japan
  • 14Department of Orthopaedic Surgery, North Medical Center, Kyoto Prefectural University of Medicine, Kyoto, Japan
  • 15Department of Community Healthcare and Geriatrics, Nagoya University Graduate School of Medicine, Nagoya, Japan
  • 16Geriatric Medicine, Meitetsu Hospital, Nagoya, Japan
  • 17Department of Medicine, Division of Diabetes, Metabolism and Endocrinology, International University of Health and Welfare, Narita, Japan
  • 18Department of Laboratory Medicine and Pathology, University of Washington, Seattle, WA 98195, USA
Received: December 1, 2022Accepted: April 15, 2023Published: May 1, 2023

Copyright: © 2023 Maeda et al. This is an open access article distributed under the terms of the Creative Commons Attribution License (CC BY 3.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.

Abstract

Werner syndrome is an adult-onset progeria syndrome that results in various complications. This study aimed to clarify the profile and secular variation of the disease. Fifty-one patients were enrolled and registered in the Werner Syndrome Registry. Their data were collected annually following registration. A cross-sectional analysis at registration and a longitudinal analysis between the baseline and each subsequent year was performed. Pearson's chi-squared and Wilcoxon signed-rank tests were used. Malignant neoplasms were observed from the fifth decade of life (mean onset: 49.7 years) and were observed in approximately 30% of patients during the 3-year survey period. Regarding renal function, the mean estimated glomerular filtration rate calculated from serum creatinine (eGFRcre) and eGFRcys, which were calculated from cystatin C in the first year, were 98.3 and 83.2 mL/min/1.73 m2, respectively, and differed depending on the index used. In longitudinal analysis, the average eGFRcre for the first and fourth years was 74.8 and 63.4 mL/min/1.73 m2, showing a rapid decline. Secular changes in Werner syndrome in multiple patients were identified. The prevalence of malignant neoplasms is high, and renal function may decline rapidly. It is, therefore, necessary to carry out active and detailed examinations and pay attention to the type and dose of the drugs used.