Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
. 2023 May 1;15(9):3273-3294.
doi: 10.18632/aging.204681. Epub 2023 May 1.

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 Maeda  1 , Masaya Koshizaka  1 , Mayumi Shoji  1 , Hiyori Kaneko  1 , Hisaya Kato  1 , Yoshiro Maezawa  1 , Junji Kawashima  2 , Kayo Yoshinaga  2 , Mai Ishikawa  3 , Akiko Sekiguchi  3 , Sei-Ichiro Motegi  3 , Hironori Nakagami  4 , Yoshihiko Yamada  5 , Shinji Tsukamoto  6 , Akira Taniguchi  6 , Ken Sugimoto  7 , Yoichi Takami  8 , Yukiko Shoda  9 , Kunihiko Hashimoto  10 , Toru Yoshimura  11 , Asako Kogure  12 , Daisuke Suzuki  12 , Naoki Okubo  13 , Takashi Yoshida  14 , Kazuhisa Watanabe  15 , Masafumi Kuzuya  16 , Minoru Takemoto  17 , Junko Oshima  1   18 , Koutaro Yokote  1
Affiliations

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 Maeda et al. Aging (Albany NY). .

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.

Keywords: Werner syndrome; disease profile; long-term follow-up; malignant neoplasm; renal function.

PubMed Disclaimer

Conflict of interest statement

CONFLICTS OF INTEREST: The authors declare no conflicts of interest related to this study.

Figures

Figure 1
Figure 1
Average renal function in each age group over the entire survey period. The blue bar shows the eGFRcre. The red bar shows the BSA-uncorrected eGFRcre. The green bar shows the eGFRcys.
Figure 2
Figure 2
Percentage of malignant neoplasms onset in each age decile in the patients with Werner syndrome. The blue bar shows the percentage of total malignant neoplasms in each age group. The green bar shows the percentage of epithelial neoplasms in each age group. The red bar shows the percentage of non-epithelial neoplasms in each age group. Patients with thyroid follicular cancer, osteosarcoma, lung cancer/undifferentiated polymorphic sarcoma, and soft tissue sarcoma were excluded because the exact age of onset was unknown.
See this image and copyright information in PMC

Similar articles

See all similar articles

Cited by

References

    1. Matsumoto T, Imamura O, Yamabe Y, Kuromitsu J, Tokutake Y, Shimamoto A, Suzuki N, Satoh M, Kitao S, Ichikawa K, Kataoka H, Sugawara K, Thomas W, et al.. Mutation and haplotype analyses of the Werner's syndrome gene based on its genomic structure: genetic epidemiology in the Japanese population. Hum Genet. 1997; 100:123–30. 10.1007/s004390050477 - DOI - PubMed
    1. Satoh M, Imai M, Sugimoto M, Goto M, Furuichi Y. Prevalence of Werner's syndrome heterozygotes in Japan. Lancet. 1999; 353:1766. 10.1016/S0140-6736(98)05869-3 - DOI - PubMed
    1. Yokote K, Chanprasert S, Lee L, Eirich K, Takemoto M, Watanabe A, Koizumi N, Lessel D, Mori T, Hisama FM, Ladd PD, Angle B, Baris H, et al.. WRN Mutation Update: Mutation Spectrum, Patient Registries, and Translational Prospects. Hum Mutat. 2017; 38:7–15. 10.1002/humu.23128 - DOI - PMC - PubMed
    1. Yamaga M, Takemoto M, Takada-Watanabe A, Koizumi N, Kitamoto T, Sakamoto K, Ishikawa T, Koshizaka M, Maezawa Y, Yokote K. Recent Trends in WRN Gene Mutation Patterns in Individuals with Werner Syndrome. J Am Geriatr Soc. 2017; 65:1853–6. 10.1111/jgs.14906 - DOI - PubMed
    1. Goto M, Matsuura M. Secular trends towards delayed onsets of pathologies and prolonged longevities in Japanese patients with Werner syndrome. Biosci Trends. 2008; 2:81–7. - PubMed

Publication types