Werner Syndrome Follow-up

  • Author: Camila K Janniger, MD; Chief Editor: Dirk M Elston, MD   more...
 
Updated: May 27, 2011
 

Complications

  • Complications of Werner syndrome (WS) are related to the concomitant diseases and symptoms described above (see Physical). Pregnancy complicated by the presence of Werner syndrome has been reported.[10]
  • The variant genotype of WRN Leu1074Phe has been associated with a 1.36-fold significantly increased risk of breast cancer in Chinese women, particularly pronounced in those carrying Phe/Phe genotype and having an earlier age at menarche, to produce a 3.58-fold increased risk of breast cancer.[11]
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Prognosis

  • The prognosis is unfavorable.
  • Death usually occurs when patients are aged 30-50 years.
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Contributor Information and Disclosures
Author

Camila K Janniger, MD  Clinical Professor of Dermatology, Clinical Associate Professor of Pediatrics, Chief of Pediatric Dermatology, University of Medicine and Dentistry of New Jersey-New Jersey Medical School

Camila K Janniger, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

Coauthor(s)

Anna Wozniacka, MD, PhD  Adjunct Lecturer, Department of Dermatology, Medical University of Lodz, Poland

Anna Wozniacka, MD, PhD is a member of the following medical societies: European Academy of Dermatology and Venereology

Disclosure: Nothing to disclose.

Specialty Editor Board

Franklin Flowers, MD  Chief, Division of Dermatology, Professor, Department of Medicine and Otolaryngology, Affiliate Associate Professor of Pediatrics and Pathology, University of Florida College of Medicine

Franklin Flowers, MD, is a member of the following medical societies: American College of Mohs Micrographic Surgery and Cutaneous Oncology

Disclosure: Nothing to disclose.

Michael J Wells, MD  Associate Professor, Department of Dermatology, Texas Tech University Health Sciences Center, Paul L Foster School of Medicine

Michael J Wells, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, and Texas Medical Association

Disclosure: Nothing to disclose.

Robert A Schwartz, MD, MPH  Professor and Head, Dermatology, Professor of Pathology, Pediatrics, Medicine, and Preventive Medicine and Community Health, University of Medicine and Dentistry of New Jersey-New Jersey Medical School

Robert A Schwartz, MD, MPH is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American College of Physicians, and Sigma Xi

Disclosure: Nothing to disclose.

Glen H Crawford, MD  Assistant Clinical Professor, Department of Dermatology, University of Pennsylvania School of Medicine; Chief, Division of Dermatology, The Pennsylvania Hospital

Glen H Crawford, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, Phi Beta Kappa, and Society of USAF Flight Surgeons

Disclosure: Nothing to disclose.

Chief Editor

Dirk M Elston, MD  Director, Department of Dermatology, Geisinger Medical Center

Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

References

  1. Nehlin JO, Skovgaard GL, Bohr VA. The Werner syndrome. A model for the study of human aging. Ann N Y Acad Sci. Jun 2000;908:167-79. [Medline].

  2. Davis T, Wyllie FS, Rokicki MJ, Bagley MC, Kipling D. The role of cellular senescence in Werner syndrome: toward therapeutic intervention in human premature aging. Ann N Y Acad Sci. Apr 2007;1100:455-69. [Medline].

  3. Zhao N, Hao F, Qu T, Zuo YG, Wang BX. A novel mutation of the WRN gene in a Chinese patient with Werner syndrome. Clin Exp Dermatol. May 2008;33(3):278-81. [Medline].

  4. Barrios Sanjuanelo A, Munoz Otero C. [Atypical Werner syndrome: Atypical progeroid syndrome: A case report.]. An Pediatr (Barc). May 7 2010;[Medline].

  5. Friedrich K, Lee L, Leistritz DF, et al. WRN mutations in Werner syndrome patients: genomic rearrangements, unusual intronic mutations and ethnic-specific alterations. Hum Genet. May 5 2010;[Medline].

  6. Pirzio LM, Pichierri P, Bignami M, Franchitto A. Werner syndrome helicase activity is essential in maintaining fragile site stability. J Cell Biol. Jan 28 2008;180(2):305-14. [Medline].

  7. Kyng KJ, Bohr VA. Gene expression and DNA repair in progeroid syndromes and human aging. Ageing Res Rev. Nov 2005;4(4):579-602. [Medline].

  8. Doh YJ, Kim HK, Jung ED, Choi SH, Kim JG, Kim BW, et al. Novel LMNA gene mutation in a patient with Atypical Werner's Syndrome. Korean J Intern Med. Mar 2009;24(1):68-72. [Medline].

  9. Massip L, Garand C, Paquet ER, et al. Vitamin C restores healthy aging in a mouse model for Werner syndrome. FASEB J. Jan 2010;24(1):158-72. [Medline].

  10. Solek-Pastuszka J, Zagrodnik-Ulan E, Plonka T, et al. Pregnancy complicated by Werner syndrome. Acta Obstet Gynecol Scand. Feb 2011;90(2):201-2. [Medline].

  11. Wang Z, Xu Y, Tang J, Ma H, Qin J, Lu C, et al. A polymorphism in Werner syndrome gene is associated with breast cancer susceptibility in Chinese women. Breast Cancer Res Treat. Feb 10 2009;[Medline].

  12. Ariyoshi K, Suzuki K, Goto M, Watanabe M, Kodama S. Increased Chromosome Instability and Accumulation of DNA Double-strand Breaks in Werner Syndrome Cells. J Radiat Res (Tokyo). May 2007;48(3):219-31. [Medline].

  13. Barak Y, Sirota P, Kimhi R, Slor H. Werner's syndrome (adult progeria): an affected mother and son presenting with resistant psychosis. Compr Psychiatry. Nov-Dec 2001;42(6):508-10. [Medline].

  14. Bohr VA, Souza Pinto N, Nyaga SG, Dianov G, Kraemer K, Seidman MM, et al. DNA repair and mutagenesis in Werner syndrome. Environ Mol Mutagen. 2001;38(2-3):227-34. [Medline].

  15. Brosh RM Jr, Bohr VA. Roles of the Werner syndrome protein in pathways required for maintenance of genome stability. Exp Gerontol. Apr 2002;37(4):491-506. [Medline].

  16. Fossel M. Human aging and progeria. J Pediatr Endocrinol Metab. 2000;13 Suppl 6:1477-81. [Medline].

  17. Harrigan JA, Wilson DM, Prasad R, Opresko PL, Beck G, May A, et al. The Werner syndrome protein operates in base excision repair and cooperates with DNA polymerase beta. Nucleic Acids Res. Jan 30 2006;34(2):745-54. [Medline].

  18. Hrabko RP, Milgrom H, Schwartz RA. Werner's syndrome with associated malignant neoplasms. Arch Dermatol. Feb 1982;118(2):106-8. [Medline].

  19. Ishikawa Y, Miller RW, Machinami R, Sugano H, Goto M. Atypical osteosarcomas in Werner Syndrome (adult progeria). Jpn J Cancer Res. Dec 2000;91(12):1345-9. [Medline].

  20. Machwe A, Xiao L, Orren DK. Length-dependent degradation of single-stranded 3' ends by the Werner syndrome protein (WRN): implications for spatial orientation and coordinated 3' to 5' movement of its ATPase/helicase and exonuclease domains. BMC Mol Biol. 2006;7:6. [Medline].

  21. Navarro CL, Cau P, Levy N. Molecular bases of progeroid syndromes. Hum Mol Genet. Oct 15 2006;15 Spec No 2:R151-61. [Medline].

  22. Opresko PL, Laine JP, Brosh RM Jr, Seidman MM, Bohr VA. Coordinate action of the helicase and 3' to 5' exonuclease of Werner syndrome protein. J Biol Chem. Nov 30 2001;276(48):44677-87. [Medline].

  23. Oshima J. The Werner syndrome protein: an update. Bioessays. Oct 2000;22(10):894-901. [Medline].

  24. Shibuya H, Kato A, Kai N, Fujiwara S, Goto M. A case of Werner syndrome with three primary lesions of malignant melanoma. J Dermatol. Sep 2005;32(9):737-44. [Medline].

 
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