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    "textoCompleto" => "<span class="elsevierStyleSections"><p id="par0005" class="elsevierStylePara elsevierViewall">In this issue of the <span class="elsevierStyleItalic">Journal</span>&#44; Brito et al&#46; present a very interesting work<a class="elsevierStyleCrossRef" href="#bib0035"><span class="elsevierStyleSup">1</span></a> based on data extracted from the Portuguese Registry of Hypertrophic Cardiomyopathy&#46;<a class="elsevierStyleCrossRef" href="#bib0040"><span class="elsevierStyleSup">2</span></a></p><p id="par0010" class="elsevierStylePara elsevierViewall">Focusing on a non-mandatory question from this registry&#44; concerning the exclusion of Anderson-Fabry disease in the differential diagnosis&#44; the authors concluded that this entity is seldom studied in the workup of patients with unexplained left ventricular hypertrophy&#46; According to their data&#44; Fabry disease was recorded as excluded in only 27&#37; of the patients included in the registry&#46; Alpha-galactosidase A &#40;&#945;-Gal A&#41; activity was assessed in 18&#37; and <span class="elsevierStyleItalic">GLA</span> gene testing was only performed in 23&#37; of cases&#46; Among patients with potential red flags for Fabry disease &#40;including concentric left ventricular hypertrophy&#44; short or prolonged PR interval&#44; intraventricular conduction disturbances or bradyarrhythmias requiring pacemaker implantation&#41;&#44; fewer than half &#40;47&#37;&#41; underwent specific tests &#40;<span class="elsevierStyleItalic">GLA</span> gene testing and&#47;or &#945;-Gal A activity&#41;&#46;</p><p id="par0015" class="elsevierStylePara elsevierViewall">These results are even more striking if we note that only cardiology departments were included in the registry&#44; which is totally voluntary&#44; and that inclusion was not sequential&#46; Bearing this in mind&#44; there is a high probability of bias toward the inclusion of better-studied patients and a higher participation of centers with better overall performance in cardiomyopathies&#46; This means that in real-world practice the rates of Fabry disease exclusion in these patients are probably even lower than reported here&#46;</p><p id="par0020" class="elsevierStylePara elsevierViewall">Portugal has some of the largest series of Fabry disease patients in Europe&#44; and these numbers are mainly due to the systematic diagnostic workup performed in reference centers for lysosomal storage diseases&#46;<a class="elsevierStyleCrossRefs" href="#bib0045"><span class="elsevierStyleSup">3&#8211;6</span></a> Although some geographic distribution patterns are clearly discernible&#44; as can be expected from an X-linked genetic disease&#44; it is essential to maintain general awareness of this disease&#44; for which specific therapies are available that can modify prognosis&#46; Since cardiac involvement is frequent and is sometimes the primary or sole manifestation of Fabry disease&#44; all cardiologists should be constantly on the alert for the possibility of Fabry disease in the study of unexplained left ventricular hypertrophy&#46; Early diagnosis and&#44; if appropriate&#44; initiation of enzyme replacement therapy can change the course of this disease and may improve both symptoms and prognosis&#46; Furthermore&#44; identification of an index case can help identify relatives affected by disease&#44; who may also benefit from diagnostic workup&#44; structured follow-up and early initiation of therapy&#44; if indicated&#46;</p><p id="par0025" class="elsevierStylePara elsevierViewall">In an era of advanced imaging and readily available genetic testing&#44; efforts should be made to ensure that red flags for Fabry disease in patients with left ventricular hypertrophy are identified and to encourage the use of dedicated tools for its exclusion&#46; This paper has a clear message for all cardiologists&#58; Fabry disease is something that must always be borne in mind in the study of patients with unexplained left ventricular hypertrophy&#46;</p><span id="sec0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0005">Conflicts of interest</span><p id="par0030" class="elsevierStylePara elsevierViewall">The author has no conflicts of interest to declare&#46;</p></span></span>"
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Editorial comment
Fabry disease: Something cardiologists must always bear in mind
Doença de Fabry – um lembrete obrigatório para os cardiologistas!
Nuno Bettencourt
Unidade de Investigação Cardiovascular, Faculdade de Medicina da Universidade do Porto, Porto, Portugal
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    "textoCompleto" => "<span class="elsevierStyleSections"><p id="par0005" class="elsevierStylePara elsevierViewall">In this issue of the <span class="elsevierStyleItalic">Journal</span>&#44; Brito et al&#46; present a very interesting work<a class="elsevierStyleCrossRef" href="#bib0035"><span class="elsevierStyleSup">1</span></a> based on data extracted from the Portuguese Registry of Hypertrophic Cardiomyopathy&#46;<a class="elsevierStyleCrossRef" href="#bib0040"><span class="elsevierStyleSup">2</span></a></p><p id="par0010" class="elsevierStylePara elsevierViewall">Focusing on a non-mandatory question from this registry&#44; concerning the exclusion of Anderson-Fabry disease in the differential diagnosis&#44; the authors concluded that this entity is seldom studied in the workup of patients with unexplained left ventricular hypertrophy&#46; According to their data&#44; Fabry disease was recorded as excluded in only 27&#37; of the patients included in the registry&#46; Alpha-galactosidase A &#40;&#945;-Gal A&#41; activity was assessed in 18&#37; and <span class="elsevierStyleItalic">GLA</span> gene testing was only performed in 23&#37; of cases&#46; Among patients with potential red flags for Fabry disease &#40;including concentric left ventricular hypertrophy&#44; short or prolonged PR interval&#44; intraventricular conduction disturbances or bradyarrhythmias requiring pacemaker implantation&#41;&#44; fewer than half &#40;47&#37;&#41; underwent specific tests &#40;<span class="elsevierStyleItalic">GLA</span> gene testing and&#47;or &#945;-Gal A activity&#41;&#46;</p><p id="par0015" class="elsevierStylePara elsevierViewall">These results are even more striking if we note that only cardiology departments were included in the registry&#44; which is totally voluntary&#44; and that inclusion was not sequential&#46; Bearing this in mind&#44; there is a high probability of bias toward the inclusion of better-studied patients and a higher participation of centers with better overall performance in cardiomyopathies&#46; This means that in real-world practice the rates of Fabry disease exclusion in these patients are probably even lower than reported here&#46;</p><p id="par0020" class="elsevierStylePara elsevierViewall">Portugal has some of the largest series of Fabry disease patients in Europe&#44; and these numbers are mainly due to the systematic diagnostic workup performed in reference centers for lysosomal storage diseases&#46;<a class="elsevierStyleCrossRefs" href="#bib0045"><span class="elsevierStyleSup">3&#8211;6</span></a> Although some geographic distribution patterns are clearly discernible&#44; as can be expected from an X-linked genetic disease&#44; it is essential to maintain general awareness of this disease&#44; for which specific therapies are available that can modify prognosis&#46; Since cardiac involvement is frequent and is sometimes the primary or sole manifestation of Fabry disease&#44; all cardiologists should be constantly on the alert for the possibility of Fabry disease in the study of unexplained left ventricular hypertrophy&#46; Early diagnosis and&#44; if appropriate&#44; initiation of enzyme replacement therapy can change the course of this disease and may improve both symptoms and prognosis&#46; Furthermore&#44; identification of an index case can help identify relatives affected by disease&#44; who may also benefit from diagnostic workup&#44; structured follow-up and early initiation of therapy&#44; if indicated&#46;</p><p id="par0025" class="elsevierStylePara elsevierViewall">In an era of advanced imaging and readily available genetic testing&#44; efforts should be made to ensure that red flags for Fabry disease in patients with left ventricular hypertrophy are identified and to encourage the use of dedicated tools for its exclusion&#46; This paper has a clear message for all cardiologists&#58; Fabry disease is something that must always be borne in mind in the study of patients with unexplained left ventricular hypertrophy&#46;</p><span id="sec0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0005">Conflicts of interest</span><p id="par0030" class="elsevierStylePara elsevierViewall">The author has no conflicts of interest to declare&#46;</p></span></span>"
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Article information
ISSN: 21742049
Original language: English
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2020 April 25 4 29
2020 March 35 9 44
2020 February 28 11 39
2020 January 29 4 33
2019 December 21 3 24
2019 November 25 12 37
2019 October 23 9 32
2019 September 14 11 25
2019 August 26 3 29
2019 July 31 6 37
2019 June 22 15 37
2019 May 38 14 52
2019 April 20 16 36
2019 March 18 10 28
2019 February 24 7 31
2019 January 18 5 23
2018 December 18 11 29
2018 November 34 11 45
2018 October 33 12 45
2018 September 25 18 43
2018 August 27 21 48
2018 July 54 17 71
2018 June 23 12 35
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Revista Portuguesa de Cardiologia (English edition)
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