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Research
Federica Rossi
Department of Medicine and Surgery, University of Milano-Bicocca, Monza, Italy, Nephrology and Dialysis Unit, ASST-Monza, San Gerardo Hospital, Monza, Italy
Corresponding Author
ORCiD: https://orcid.org/0000-0002-1509-1190
License:
This work is licensed under a CC BY 4.0 License. Read Full License
Background and objectives
Published data on hypertension incidence and management in Anderson-Fabry disease are scanty and it remains to be shown how much an elevated blood pressure contributes to the patient’s organ damage. Therefore, we have assessed blood pressure values and their correlations with clinical findings in a cohort of Fabry patients.
Methods
Between January 2015 and May 2019, all adult Fabry patients (n = 32; n = 24 females, n = 8 males) referred to our institute were enrolled. Data regarding hypertension were obtained by ambulatory blood pressure monitoring, home self-monitoring and repeated office measurements. Organ involvement and hypertension risk factors were also evaluated.
Results
The ambulatory monitoring revealed elevated blood pressure in 18.75% (n = 6) of the Fabry population and 50% of this group was diagnosed with masked hypertension. All these patients were females and they presented a lower glomerular filtration rate and a more advanced cardiac hypertrophy compared with normotensive subjects. They were mostly (66.7%) affected by a progressive form of the disease while the majority of the normotensives (84.6%) were stable. No correlation was found between the category of GLA mutation and the development of hypertension.
Conclusion
Newly detected hypertension can be found in a restricted portion of stable Fabry patients while it becomes more prevalent among clinically progressive cases. The use of the ambulatory blood pressure monitoring is of paramount importance to reveal masked hypertension which can contribute to the progressive worsening of the organ damage. Therefore, a proper diagnosis and therapy of hypertension may improve the outcome of Fabry patients.
Keywords
Anderson-Fabry disease, lysosomal storage disorder, hypertension, masked hypertension, blood pressure monitoring
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This is a preprint. It has not completed peer review.
Research
Federica Rossi
Department of Medicine and Surgery, University of Milano-Bicocca, Monza, Italy, Nephrology and Dialysis Unit, ASST-Monza, San Gerardo Hospital, Monza, Italy
Corresponding Author
ORCiD: https://orcid.org/0000-0002-1509-1190
Badges
Prescreen
This manuscript passed our Prescreen assessment
Complete author information
Appropriate declaration statements
Potential risks to human health
Prescreen
History
CURRENT STATUS: Posted
Version 1
Posted 19 Aug, 2020
No community comments so far
Metrics
Comments: 0
PDF Downloads: ...
HTML Views: ...
Subject Areas
Internal Medicine
License:
This work is licensed under a CC BY 4.0 License. Read Full License
Background and objectives
Published data on hypertension incidence and management in Anderson-Fabry disease are scanty and it remains to be shown how much an elevated blood pressure contributes to the patient’s organ damage. Therefore, we have assessed blood pressure values and their correlations with clinical findings in a cohort of Fabry patients.
Methods
Between January 2015 and May 2019, all adult Fabry patients (n = 32; n = 24 females, n = 8 males) referred to our institute were enrolled. Data regarding hypertension were obtained by ambulatory blood pressure monitoring, home self-monitoring and repeated office measurements. Organ involvement and hypertension risk factors were also evaluated.
Results
The ambulatory monitoring revealed elevated blood pressure in 18.75% (n = 6) of the Fabry population and 50% of this group was diagnosed with masked hypertension. All these patients were females and they presented a lower glomerular filtration rate and a more advanced cardiac hypertrophy compared with normotensive subjects. They were mostly (66.7%) affected by a progressive form of the disease while the majority of the normotensives (84.6%) were stable. No correlation was found between the category of GLA mutation and the development of hypertension.
Conclusion
Newly detected hypertension can be found in a restricted portion of stable Fabry patients while it becomes more prevalent among clinically progressive cases. The use of the ambulatory blood pressure monitoring is of paramount importance to reveal masked hypertension which can contribute to the progressive worsening of the organ damage. Therefore, a proper diagnosis and therapy of hypertension may improve the outcome of Fabry patients.
Figure 1
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