Letter to the Editor
More on noncompaction in Fabry's disease
Mais sobre não compactação na doença de Fabry
Elisabete Martins
, João Paulo Oliveira
Corresponding author
Faculty of Medicine, University of Porto, Portugal
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Squares indicate males, circles indicate females, solid black symbols indicate individuals diagnosed with Fabry disease; symbols with a diagonal line denote deceased individuals. Asterisks indicate a clinical or pedigree-based diagnosis of Fabry disease. Diamonds indicate healthy sibs of different genders, with their number specified by the cardinal inside the symbol. The arrow indicates the patient presented in our paper. Question-marks indicate individuals who have not been clinically or genetically assessed for Fabry disease. The two diagonal vertical lines originating from the same point indicate dizygotic twins. Year of birth is indicated by “b.”; age at death, in full years (“y”), is indicated by “d.”. Patient I-2 was reported to have died from “kidney disease”; patient II-1 was reported to have died from “cerebrovascular disease”, nine years after receiving a kidney transplant; patient II-5 had type 2 diabetes mellitus and died from acute stroke. 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array:2 [ "nombre" => "João Paulo" "apellidos" => "Oliveira" ] ] "afiliaciones" => array:1 [ 0 => array:2 [ "entidad" => "Faculty of Medicine, University of Porto, Portugal" "identificador" => "aff0005" ] ] "correspondencia" => array:1 [ 0 => array:3 [ "identificador" => "cor0005" "etiqueta" => "⁎" "correspondencia" => "Corresponding author." ] ] ] ] "titulosAlternativos" => array:1 [ "pt" => array:1 [ "titulo" => "Mais sobre não compactação na doença de Fabry" ] ] "resumenGrafico" => array:2 [ "original" => 0 "multimedia" => array:7 [ "identificador" => "fig0005" "etiqueta" => "Figure 1" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr1.jpeg" "Alto" => 874 "Ancho" => 2492 "Tamanyo" => 81409 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">Pedigree of the reported Fabry disease patient and of her family. Squares indicate males, circles indicate females, solid black symbols indicate individuals diagnosed with Fabry disease; symbols with a diagonal line denote deceased individuals. Asterisks indicate a clinical or pedigree-based diagnosis of Fabry disease. Diamonds indicate healthy sibs of different genders, with their number specified by the cardinal inside the symbol. The arrow indicates the patient presented in our paper. Question-marks indicate individuals who have not been clinically or genetically assessed for Fabry disease. The two diagonal vertical lines originating from the same point indicate dizygotic twins. Year of birth is indicated by “b.”; age at death, in full years (“y”), is indicated by “d.”. Patient I-2 was reported to have died from “kidney disease”; patient II-1 was reported to have died from “cerebrovascular disease”, nine years after receiving a kidney transplant; patient II-5 had type 2 diabetes mellitus and died from acute stroke. All the affected males presented with the classical phenotype of Fabry disease. Patient II-3, who is followed at another hospital, started hemodialysis at age 35 and received a kidney transplant about two years later. Patients II-11 and III-9 live abroad and are also followed elsewhere. Patients II-2, II-10 and III-2, who are under our clinical care, started enzyme replacement therapy in 2005, 2002 and 2003, respectively. Neither of the two younger sisters of the proband's mother has yet presented any major cardiac or cerebrovascular complications of Fabry disease. Individuals II-4, II-7, II-8, II-9, III-3/4 and III-7/8 have been genetically screened and did not carry the GLA p.R220X mutation.</p>" ] ] ] "textoCompleto" => "<span class="elsevierStyleSections"><p id="par0005" class="elsevierStylePara elsevierViewall">We thank Finsterer et al. for their pertinent comments on our report of the diagnosis of left ventricular noncompaction (LVNC) in a young adult female with Fabry disease,<a class="elsevierStyleCrossRef" href="#bib0095"><span class="elsevierStyleSup">1</span></a> and for the opportunity to describe in more detail her clinical history, which necessarily had to be concise in the original case report format, and to elaborate on the diagnostic value and interpretation of endomyocardial biopsy in females with Fabry disease.</p><p id="par0010" class="elsevierStylePara elsevierViewall">This patient belongs to a large three-generation family with classical Fabry disease (<a class="elsevierStyleCrossRef" href="#fig0005">Figure 1</a>) segregating with a relatively common nonsense mutation in the alpha-galactosidase gene (<span class="elsevierStyleItalic">GLA</span>) that leads to the premature termination of <span class="elsevierStyleItalic">GLA</span> mRNA translation at codon 220 (p.R220X).<a class="elsevierStyleCrossRef" href="#bib0100"><span class="elsevierStyleSup">2</span></a> Because it severely compromises residual alpha-galactosidase enzyme activity, the p.R220X mutation is associated with the classical phenotype of Fabry disease.<a class="elsevierStyleCrossRef" href="#bib0105"><span class="elsevierStyleSup">3</span></a> The <span class="elsevierStyleItalic">GLA</span> gene locus is on the X chromosome, and therefore the inheritance of Fabry disease follows an X-linked pattern with more severe and earlier clinical manifestations in hemizygous males, and more variable and organ-restricted phenotypes in heterozygous females.<a class="elsevierStyleCrossRef" href="#bib0100"><span class="elsevierStyleSup">2</span></a> This is well exemplified by our patient's family, where the affected males in the second generation presented with the classical phenotype of Fabry disease and developed end-stage kidney disease long before the availability of enzyme replacement therapy (ERT), while her affected sisters were asymptomatic or manifested partial clinical phenotypes at later ages.</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><p id="par0015" class="elsevierStylePara elsevierViewall">Both the mother of our patient and one of her younger sisters were diagnosed with Fabry nephropathy on kidney biopsies obtained at ages 45 and 32 years, respectively,<a class="elsevierStyleCrossRef" href="#bib0110"><span class="elsevierStyleSup">4</span></a> but neither of them manifested any other complications of Fabry disease at the baseline evaluation. The <span class="elsevierStyleItalic">GLA</span> mutation p.R220X was originally identified in a 56-year-old Australian woman presenting with hypertrophic cardiomyopathy<a class="elsevierStyleCrossRef" href="#bib0115"><span class="elsevierStyleSup">5</span></a> and typical histopathological findings of Fabry disease were observed in renal and myocardial necropsy specimens from a 71-year-old Japanese woman heterozygous for the same mutation.<a class="elsevierStyleCrossRef" href="#bib0120"><span class="elsevierStyleSup">6</span></a> On the whole, these cases illustrate the clinical variability of Fabry disease in affected females, even when they carry highly pathogenic <span class="elsevierStyleItalic">GLA</span> mutations. As in other X-linked recessive Mendelian disorders, the most striking example of the phenotypic variability of Fabry disease in females is the discordant expression in monozygotic twins,<a class="elsevierStyleCrossRef" href="#bib0125"><span class="elsevierStyleSup">7</span></a> due to highly unbalanced X-chromosome inactivation in opposite directions, with the parental X chromosome carrying the pathogenic <span class="elsevierStyleItalic">GLA</span> mutation preferentially active in the clinically affected twin and the X chromosome inherited from the other parent preferentially active in the asymptomatic twin sister.</p><p id="par0020" class="elsevierStylePara elsevierViewall">However, proper interpretation of the effect of X-chromosome inactivation (lyonization) on the clinical phenotypes seen in females carrying X-linked mutant genes<a class="elsevierStyleCrossRef" href="#bib0130"><span class="elsevierStyleSup">8</span></a> should take into consideration not only the overall ratio of maternal-to-paternal X-chromosome inactivation which occurs at a very early stage in embryonic development, but also the subsequent random cell assortment during organogenesis<a class="elsevierStyleCrossRef" href="#bib0135"><span class="elsevierStyleSup">9</span></a> that may lead to discordant proportions of cells with one or the other functional X chromosome in different tissues. In solid organs, like the heart, the outcome of this process at the cellular level is the mosaic expression of X-linked phenotypes, topographically correlated with X-inactivation patches,<a class="elsevierStyleCrossRef" href="#bib0140"><span class="elsevierStyleSup">10</span></a> i.e., clonally related contiguous groups of cells with the same functionally inactivated parental X chromosome. We are not aware of any published studies on cardiomyocyte X-inactivation patches in the human heart, but in the mouse they can be large.<a class="elsevierStyleCrossRef" href="#bib0145"><span class="elsevierStyleSup">11</span></a></p><p id="par0025" class="elsevierStylePara elsevierViewall">Bearing in mind the above considerations about lyonization, the endomyocardial biopsy unequivocally demonstrated that our patient has Fabry cardiomyopathy, having at least one patch of affected cardiomyocytes in the apical region of the right side of the interventricular septum, from where the biopsy was taken (but a normal biopsy result would not have excluded the diagnosis!); it is quite possible that the sampled patch of affected cardiomyocytes extends to the hypertrabeculated apex of the left ventricle (LV). A likely explanation for the discordant histopathological expression of Fabry disease in cardiomyocytes and endothelial cells is the different progenitor cell populations from which they arise.<a class="elsevierStyleCrossRef" href="#bib0150"><span class="elsevierStyleSup">12</span></a></p><p id="par0030" class="elsevierStylePara elsevierViewall">Regarding the argument raised by Finsterer et al. that our patient may have a second heart disorder, pathogenically unrelated to Fabry disease, the following information is important: (i) all the affected individuals that were alive in this family underwent comprehensive, multidisciplinary assessment at baseline, and have been followed according to expert guidelines for the recognition, evaluation, and surveillance of disease-associated morbidities<a class="elsevierStyleCrossRef" href="#bib0155"><span class="elsevierStyleSup">13</span></a>; (ii) our patient has no clinical manifestations whatsoever of a coexisting neuropathic or myopathic disorder; (iii) her first routine echocardiogram, which had been performed at age 29 years, was unremarkable, but the acoustic window was poor, preventing the exclusion of LV hypertrabeculation at that time; (iv) the patient's mother had no signs of LVNC on the echocardiogram performed at the age of 48 years, for baseline evaluation before initiating ERT, and presented only mild LV hypertrophy at her most recent echocardiographic assessment, at age 56 years; (v) the 72-year-old father of the patient, who is followed at another hospital for severe coronary heart disease, past history of myocardial infarction and LV dysfunction, also has no echocardiographic signs of LVNC or hypertrabeculation. We entirely agree with Finsterer et al. concerning the clinical importance of detailed neuromuscular assessment for the differential diagnosis of cardiomyopathies,<a class="elsevierStyleCrossRef" href="#bib0160"><span class="elsevierStyleSup">14</span></a> including acquired LVNC,<a class="elsevierStyleCrossRef" href="#bib0165"><span class="elsevierStyleSup">15</span></a> and our patient has been evaluated according to such diagnostic concerns.</p><p id="par0035" class="elsevierStylePara elsevierViewall">LVNC is a rare condition that can occur in isolation or coexist with other cardiac and/or systemic anomalies.<a class="elsevierStyleCrossRefs" href="#bib0170"><span class="elsevierStyleSup">16,17</span></a> The majority of cases are congenital and are thought to result from an arrest of the normal compaction process of the myocardium during fetal development. The familial types of LVNC are the most common and follow autosomal-dominant, X-linked, or mitochondrial-inheritance patterns.<a class="elsevierStyleCrossRef" href="#bib0170"><span class="elsevierStyleSup">16</span></a> However, since our patient is a sporadic case of apparently acquired LV hypertrabeculation, the diagnosis of a familial type of LVNC is quite unlikely.</p><p id="par0040" class="elsevierStylePara elsevierViewall">As acknowledged by Finsterer et al., LV hypertrabeculation has been reported in patients with other lysosomal storage disorders (LSDs), including Pompe disease<a class="elsevierStyleCrossRef" href="#bib0160"><span class="elsevierStyleSup">14</span></a> and Danon disease.<a class="elsevierStyleCrossRef" href="#bib0180"><span class="elsevierStyleSup">18</span></a> We suggest that Fabry disease should be added to the list of LSDs that can give rise to acquired LVNC. Prenatal hypertrabeculation and noncompaction of the ventricular wall were observed in a mouse model of severe glycogen storage disease type IV (Andersen disease),<a class="elsevierStyleCrossRef" href="#bib0105"><span class="elsevierStyleSup">3</span></a> in association with increased expression of cell-cycle regulators in cardiomyocytes. Whether similar molecular mechanisms are also operative in LSD-related acquired LVNC is as yet unknown. As recently addressed in a state-of-art paper,<a class="elsevierStyleCrossRef" href="#bib0175"><span class="elsevierStyleSup">17</span></a> further research is needed to clarify whether LVNC is a primary genetic cardiomyopathy or a morphologic trait shared by different cardiomyopathies, as well as to elucidate their molecular pathogenesis.</p><span id="sec0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0005">Conflicts of interest</span><p id="par0045" class="elsevierStylePara elsevierViewall">The authors have no conflicts of interest to declare.</p></span></span>" "textoCompletoSecciones" => array:1 [ "secciones" => array:2 [ 0 => array:2 [ "identificador" => "sec0005" "titulo" => "Conflicts of interest" ] 1 => array:1 [ "titulo" => "References" ] ] ] "pdfFichero" => "main.pdf" "tienePdf" => true "multimedia" => array:1 [ 0 => array:7 [ "identificador" => "fig0005" "etiqueta" => "Figure 1" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr1.jpeg" "Alto" => 874 "Ancho" => 2492 "Tamanyo" => 81409 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">Pedigree of the reported Fabry disease patient and of her family. Squares indicate males, circles indicate females, solid black symbols indicate individuals diagnosed with Fabry disease; symbols with a diagonal line denote deceased individuals. Asterisks indicate a clinical or pedigree-based diagnosis of Fabry disease. Diamonds indicate healthy sibs of different genders, with their number specified by the cardinal inside the symbol. The arrow indicates the patient presented in our paper. Question-marks indicate individuals who have not been clinically or genetically assessed for Fabry disease. The two diagonal vertical lines originating from the same point indicate dizygotic twins. Year of birth is indicated by “b.”; age at death, in full years (“y”), is indicated by “d.”. Patient I-2 was reported to have died from “kidney disease”; patient II-1 was reported to have died from “cerebrovascular disease”, nine years after receiving a kidney transplant; patient II-5 had type 2 diabetes mellitus and died from acute stroke. All the affected males presented with the classical phenotype of Fabry disease. Patient II-3, who is followed at another hospital, started hemodialysis at age 35 and received a kidney transplant about two years later. Patients II-11 and III-9 live abroad and are also followed elsewhere. Patients II-2, II-10 and III-2, who are under our clinical care, started enzyme replacement therapy in 2005, 2002 and 2003, respectively. Neither of the two younger sisters of the proband's mother has yet presented any major cardiac or cerebrovascular complications of Fabry disease. Individuals II-4, II-7, II-8, II-9, III-3/4 and III-7/8 have been genetically screened and did not carry the GLA p.R220X mutation.</p>" ] ] ] "bibliografia" => array:2 [ "titulo" => "References" "seccion" => array:1 [ 0 => array:2 [ "identificador" => "bibs0005" "bibliografiaReferencia" => array:18 [ 0 => array:3 [ "identificador" => "bib0095" "etiqueta" => "1" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Histopathological evidence of Fabry disease in a female patient with left ventricular noncompaction" "autores" => array:1 [ 0 => array:2 [ "etal" => true "autores" => array:3 [ 0 => "E. Martins" 1 => "T. Pinho" 2 => "S. 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| Year/Month | Html | Total | |
|---|---|---|---|
| 2024 November | 7 | 4 | 11 |
| 2024 October | 52 | 29 | 81 |
| 2024 September | 64 | 23 | 87 |
| 2024 August | 42 | 24 | 66 |
| 2024 July | 42 | 31 | 73 |
| 2024 June | 33 | 18 | 51 |
| 2024 May | 42 | 17 | 59 |
| 2024 April | 32 | 19 | 51 |
| 2024 March | 37 | 22 | 59 |
| 2024 February | 31 | 21 | 52 |
| 2024 January | 33 | 29 | 62 |
| 2023 December | 30 | 21 | 51 |
| 2023 November | 37 | 25 | 62 |
| 2023 October | 40 | 19 | 59 |
| 2023 September | 29 | 20 | 49 |
| 2023 August | 27 | 12 | 39 |
| 2023 July | 23 | 5 | 28 |
| 2023 June | 37 | 17 | 54 |
| 2023 May | 27 | 31 | 58 |
| 2023 April | 16 | 3 | 19 |
| 2023 March | 21 | 21 | 42 |
| 2023 February | 16 | 24 | 40 |
| 2023 January | 22 | 12 | 34 |
| 2022 December | 23 | 24 | 47 |
| 2022 November | 38 | 18 | 56 |
| 2022 October | 29 | 22 | 51 |
| 2022 September | 28 | 33 | 61 |
| 2022 August | 28 | 31 | 59 |
| 2022 July | 39 | 27 | 66 |
| 2022 June | 36 | 23 | 59 |
| 2022 May | 33 | 32 | 65 |
| 2022 April | 34 | 26 | 60 |
| 2022 March | 35 | 33 | 68 |
| 2022 February | 36 | 26 | 62 |
| 2022 January | 29 | 11 | 40 |
| 2021 December | 28 | 23 | 51 |
| 2021 November | 36 | 36 | 72 |
| 2021 October | 51 | 28 | 79 |
| 2021 September | 29 | 27 | 56 |
| 2021 August | 32 | 27 | 59 |
| 2021 July | 24 | 24 | 48 |
| 2021 June | 26 | 18 | 44 |
| 2021 May | 37 | 34 | 71 |
| 2021 April | 53 | 23 | 76 |
| 2021 March | 78 | 19 | 97 |
| 2021 February | 56 | 13 | 69 |
| 2021 January | 25 | 13 | 38 |
| 2020 December | 42 | 10 | 52 |
| 2020 November | 34 | 3 | 37 |
| 2020 October | 20 | 9 | 29 |
| 2020 September | 64 | 12 | 76 |
| 2020 August | 26 | 7 | 33 |
| 2020 July | 49 | 4 | 53 |
| 2020 June | 27 | 5 | 32 |
| 2020 May | 47 | 4 | 51 |
| 2020 April | 42 | 9 | 51 |
| 2020 March | 38 | 8 | 46 |
| 2020 February | 107 | 23 | 130 |
| 2020 January | 39 | 6 | 45 |
| 2019 December | 28 | 3 | 31 |
| 2019 November | 49 | 6 | 55 |
| 2019 October | 17 | 3 | 20 |
| 2019 September | 21 | 7 | 28 |
| 2019 August | 42 | 5 | 47 |
| 2019 July | 37 | 13 | 50 |
| 2019 June | 33 | 9 | 42 |
| 2019 May | 46 | 13 | 59 |
| 2019 April | 60 | 13 | 73 |
| 2019 March | 42 | 13 | 55 |
| 2019 February | 31 | 10 | 41 |
| 2019 January | 13 | 9 | 22 |
| 2018 December | 18 | 9 | 27 |
| 2018 November | 38 | 6 | 44 |
| 2018 October | 49 | 12 | 61 |
| 2018 September | 21 | 13 | 34 |
| 2018 August | 14 | 6 | 20 |
| 2018 July | 19 | 2 | 21 |
| 2018 June | 21 | 2 | 23 |
| 2018 May | 31 | 5 | 36 |
| 2018 April | 25 | 7 | 32 |
| 2018 March | 28 | 5 | 33 |
| 2018 February | 26 | 5 | 31 |
| 2018 January | 24 | 2 | 26 |
| 2017 December | 39 | 16 | 55 |
| 2017 November | 29 | 11 | 40 |
| 2017 October | 29 | 17 | 46 |
| 2017 September | 35 | 10 | 45 |
| 2017 August | 41 | 23 | 64 |
| 2017 July | 28 | 19 | 47 |
| 2017 June | 46 | 15 | 61 |
| 2017 May | 42 | 7 | 49 |
| 2017 April | 51 | 2 | 53 |
| 2017 March | 43 | 32 | 75 |
| 2017 February | 31 | 9 | 40 |
| 2017 January | 34 | 4 | 38 |
| 2016 December | 32 | 6 | 38 |
| 2016 November | 23 | 8 | 31 |
| 2016 October | 30 | 3 | 33 |
| 2016 September | 24 | 15 | 39 |
| 2016 August | 11 | 3 | 14 |
| 2016 July | 13 | 4 | 17 |
| 2016 June | 18 | 4 | 22 |
| 2016 May | 17 | 4 | 21 |
| 2016 April | 22 | 1 | 23 |
| 2016 March | 25 | 23 | 48 |
| 2016 February | 25 | 20 | 45 |
| 2016 January | 21 | 20 | 41 |
| 2015 December | 13 | 16 | 29 |
| 2015 November | 16 | 13 | 29 |
| 2015 October | 27 | 21 | 48 |
| 2015 September | 21 | 14 | 35 |
| 2015 August | 23 | 8 | 31 |
| 2015 July | 8 | 4 | 12 |
| 2015 June | 21 | 11 | 32 |
| 2015 May | 25 | 10 | 35 |
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