{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,6,12]],"date-time":"2025-06-12T07:53:14Z","timestamp":1749714794072,"version":"3.37.3"},"reference-count":40,"publisher":"Springer Science and Business Media LLC","issue":"1","license":[{"start":{"date-parts":[[2021,6,12]],"date-time":"2021-06-12T00:00:00Z","timestamp":1623456000000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"},{"start":{"date-parts":[[2021,6,12]],"date-time":"2021-06-12T00:00:00Z","timestamp":1623456000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"}],"funder":[{"DOI":"10.13039\/501100003593","name":"Conselho Nacional de Desenvolvimento Cient\u00edfico e Tecnol\u00f3gico","doi-asserted-by":"publisher","award":["306723\/2019-0"],"award-info":[{"award-number":["306723\/2019-0"]}],"id":[{"id":"10.13039\/501100003593","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100002322","name":"Coordena\u00e7\u00e3o de Aperfei\u00e7oamento de Pessoal de N\u00edvel Superior","doi-asserted-by":"publisher","award":["001","001"],"award-info":[{"award-number":["001","001"]}],"id":[{"id":"10.13039\/501100002322","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["Pediatr Rheumatol"],"published-print":{"date-parts":[[2021,12]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec>\n                <jats:title>Background<\/jats:title>\n                <jats:p>Fabry disease (FD) is a rare, X-linked, multisystemic lysosomal storage disorder (LSD) that results from a deficiency in the hydrolase alpha-galactosidase A (\u237a-GalA). During childhood, classic FD symptomatology is rare. The majority of children may show non-specific symptoms, including in the musculoskeletal system. The prevalence of FD among juvenile idiopathic arthritis (JIA) patients is unknown.<\/jats:p>\n              <\/jats:sec><jats:sec>\n                <jats:title>Objective<\/jats:title>\n                <jats:p>This study aimed to identify the frequency of FD in a JIA cohort, characterizing early clinical symptoms, enzyme titers, and <jats:italic>GLA<\/jats:italic> genotyping.<\/jats:p>\n              <\/jats:sec><jats:sec>\n                <jats:title>Methods<\/jats:title>\n                <jats:p>Children with JIA followed in a tertiary Children Hospital cohort were selected. Clinical, laboratory and familiar information were recorded. Molecular genetic testing to detect <jats:italic>GLA<\/jats:italic> gene mutations was performed in girls and enzymatic analysis in boys.<\/jats:p>\n              <\/jats:sec><jats:sec>\n                <jats:title>Results<\/jats:title>\n                <jats:p>In 89 patients (56.2% female, age at disease onset: 8.93\u2009\u00b1\u20094.35\u2009years), one male (1.12%) patient presented pathogenic mutation in <jats:italic>GLA<\/jats:italic> gene, <jats:italic>c.1244\u2009T\u2009&gt;\u2009C p.L415P<\/jats:italic>, one female patient had a variant of uncertain significance <jats:italic>c.38C\u2009&gt;\u2009T (p.Ala13Val).<\/jats:italic> Three additional (3.4%) patients had the enzymatic activity of alpha-galactosidase slightly decreased. We observed the presence of intronic variants in 44.44% of patients in our cohort: <jats:italic>c.1000-22C\u2009&gt;\u2009T<\/jats:italic>; <jats:italic>c.370-81_-77del<\/jats:italic>; <jats:italic>c.640-16A\u2009&gt;\u2009G<\/jats:italic>; <jats:italic>c.10C\u2009&gt;\u2009T<\/jats:italic>; <jats:italic>c.548-125C\u2009&gt;\u2009G<\/jats:italic> and <jats:italic>c.-12G\u2009&gt;\u2009A.<\/jats:italic> These variants and their combination were associated with clinical symptoms in our cohort.<\/jats:p>\n              <\/jats:sec><jats:sec>\n                <jats:title>Conclusions<\/jats:title>\n                <jats:p>The incidence of FD in our cohort was 1.12%. Intronic variants were associated with symptoms previously described in the literature. Screening for FD in JIA may be a reasonable strategy for those with an atypical pattern of pain.<\/jats:p>\n              <\/jats:sec>","DOI":"10.1186\/s12969-021-00563-9","type":"journal-article","created":{"date-parts":[[2021,6,12]],"date-time":"2021-06-12T20:02:32Z","timestamp":1623528152000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":8,"title":["Frequency of Fabry disease in a juvenile idiopathic arthritis cohort"],"prefix":"10.1186","volume":"19","author":[{"given":"Luciana","family":"Paim-Marques","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Amanda Virginia","family":"Cavalcante","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Islane","family":"Ver\u00e7osa","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Paula","family":"Carneiro","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Marcia","family":"Souto-Maior","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Erlane","family":"Marques","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5075-4474","authenticated-orcid":false,"given":"Simone","family":"Appenzeller","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"297","published-online":{"date-parts":[[2021,6,12]]},"reference":[{"key":"563_CR1","doi-asserted-by":"publisher","unstructured":"Biegstraaten M, Arngr\u00edmsson R, Barbey F, Boks L, Cecchi F, Deegan PB, et al. 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All patients (and legal representatives if children under 18\u2009years) included signed the informed consent and assent form.","order":2,"name":"Ethics","group":{"name":"EthicsHeading","label":"Ethics approval and consent to participate"}},{"value":"Not applicable.","order":3,"name":"Ethics","group":{"name":"EthicsHeading","label":"Consent for publication"}},{"value":"All authors declare that they have no conflict of interest related to the study.","order":4,"name":"Ethics","group":{"name":"EthicsHeading","label":"Competing interests"}}],"article-number":"91"}}