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  • Oral presentation
  • Open Access

Wild-type transthyretin amyloidosis in female patients

  • 1,
  • 1,
  • 1,
  • 2,
  • 3,
  • 4 and
  • 1
Orphanet Journal of Rare Diseases201510 (Suppl 1) :O9

https://doi.org/10.1186/1750-1172-10-S1-O9

  • Published:

Keywords

  • Gender Difference
  • Glomerular Filtration Rate
  • Amyloidosis
  • Clinical Variable
  • Female Gender

Background

Wild-type TTR amyloidosis (wt-ATTR) is a common aging phenomenon in the elderly population. It is claimed to affect exclusively males. Female gender was assumed as a protective factor. Clinical data on wt-ATTR in female gender is lacking. This single center analysis reported on gender differences of clinical variables in wt-ATTR.

Methods

Patient records of 207 consecutive patients with wt-ATTR were analyzed for clinical variables obtained during the initial assessment at Heidelberg Amyloidosis Center, including electrocardiography, echocardiography, and laboratory results. All variables were compared between male and female gender. Finally, predictors of survival (onset of first symptoms to death) were evaluated.

Results

Comparison of clinical findings between males and females affected by wt-ATTR amyloidosis are shown in table 1. Female patients with wt-ATTR did not differ from male patients regarding demographic or clinical parameters except for modified body mass index (1140±184 vs. 1029±154, p<0.05), glomerular filtration rate (66±23 vs. 85±31 ml/min*m2*1.73; p<0.05), NYHA class (2.4±0.7 vs. 2.9±0.3; p<0.01) and PQ interval (211±50 ms vs. 170±26; p<0.01). Interestingly, both groups especially did not differ in age at onset of symptoms, but longer delay between start of symptoms and diagnosis of wt-ATTR in females was observed when compared to male patients with wt-ATTR.

In total, 6 deaths (35%) occurred in females and 45 deaths (24%) in males. No gender differences were observed regarding mean survival (females 54±35 month, males 56±107 months). By multivariate analysis independent predictors of mortality in the whole cohort were use of diuretics (HR 8.657, 95%CI 1.160-64.17; p=0.035) and hs-TnT (HR 1.009, 95%CI 1.004-1.015; p=0.001).

In total, 6 deaths (35%) occurred in females and 45 deaths (24%) in males. No gender differences were observed regarding mean survival (females 54±35 month, males 56±107 months). By multivariate analysis independent predictors of mortality in the whole cohort were use of diuretics (HR 8.657, 95%CI 1.160-64.17; p=0.035) and hs-TnT (HR 1.009, 95%CI 1.004-1.015; p=0.001).

Conclusion

Although wt-ATTR is claimed to be a disease of male gender there is a considerable number of females affected with cardiac manifestation of wt-ATTR. According to this first report on clinical characteristics of a relatively well sized cohort of females no gender-specific differences regarding clinical characteristics and median survival were observed, except for modified body mass index and PQ interval as well as higher glomerular filtration rate and NYHA class in female patients. Moreover, use of diuretics and hs-TnT appeared to be predictors of mortality.

Authors’ Affiliations

(1)
Department of Cardiology, University of Heidelberg, 69120 Heidelberg, Germany
(2)
Department of Hematology, University of Heidelberg, 69120 Heidelberg, Germany
(3)
Institute of Human Genetics, University of Heidelberg, 69120 Heidelberg, Germany
(4)
Institute of Pathology, University of Kiel, 24105 Kiel, Germany

Copyright

© Kristen et al. 2015

This article is published under license to BioMed Central Ltd. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/4.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.

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