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rheumatology&#44; hematology and internal medicine departments&#44; with an initial diagnosis of systemic onset Still&#39;s disease&#44; which was treated with NSAID and paracetamol&#46;</p><p id="par0025" class="elsevierStylePara elsevierViewall">His laboratory tests showed persistent leukocytosis and elevation of acute phase reactants&#44; C-reactive protein and erythrocyte sedimentation rate&#46; However&#44; the levels of ferritin&#44; rheumatoid factor&#44; antinuclear antibodies &#40;ANA&#41;&#44; the complement system and immunoglobulins were always negative or within the normal range&#46;</p><p id="par0030" class="elsevierStylePara elsevierViewall">When the patient was 23 years old&#44; the presence of proteinuria in the nephrotic range was detected&#44; with no changes in the sediment&#46; Given the suspicion of proteinuria in relation to the administration of NSAID or his underlying inflammatory process&#44; we requested a renal biopsy&#44; which revealed secondary amyloidosis &#40;<a class="elsevierStyleCrossRef" href="#fig0005">Fig&#46; 1</a>&#41;&#46; At that time&#44; with the above description of the clinical findings and analytical results&#44; and after the development of renal amyloidosis&#44; we decided to reconsider the diagnosis&#46; We thought of the possibility that it could be a hereditary systemic autoinflammatory disease rather than Still&#39;s disease&#44; and we requested genetic studies in that respect&#46; As the result was positive for mutation of the <span class="elsevierStyleItalic">NLRP3</span> gene&#44; the diagnosis was Muckle-Wells syndrome&#46;</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><p id="par0035" class="elsevierStylePara elsevierViewall">Treatment was begun with subcutaneous anakinra at a dose of 100<span class="elsevierStyleHsp" style=""></span>mg daily&#44;<a class="elsevierStyleCrossRef" href="#bib0050"><span class="elsevierStyleSup">4</span></a> but that failed to achieve adequate control of proteinuria&#46; Given the poor response and following the finding of elevated IL-6 levels&#44; anakinra was discontinued and treatment was initiated with tocilizumab &#40;an IL-6 inhibitor&#41;&#46; Over the past 6 years&#44; the patient has been asymptomatic&#44; with good control of the inflammation and normal renal function without proteinuria&#46;</p></span><span id="sec0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0035">Discussion</span><p id="par0040" class="elsevierStylePara elsevierViewall">Since infancy&#44; our patient had had recurrent episodes of fever and arthralgia&#44; in addition to episodes of abdominal pain and skin rash&#46; Rheumatoid factor and ANA were negative&#44; all of which is compatible both with Still&#39;s disease<a class="elsevierStyleCrossRef" href="#bib0055"><span class="elsevierStyleSup">5</span></a> and cryopyrinopathies&#46; The coexistence of hepatosplenomegaly&#44; according to the criteria of Yamaguchi&#44;<a class="elsevierStyleCrossRef" href="#bib0060"><span class="elsevierStyleSup">6</span></a> indicated the diagnosis of Still&#39;s disease&#46; It was not until the development of renal amyloidosis&#44; 20 years later&#44; that the diagnosis was reconsidered&#46; The genetic study confirmed the diagnosis of Muckle-Wells syndrome&#46;</p></span><span id="sec0020" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0040">Conclusion</span><p id="par0045" class="elsevierStylePara elsevierViewall">Given the rarity of these syndromes in routine clinical practice&#44; it is necessary to stress the importance of being aware of their different and sometimes incomplete forms of presentation and maintain a high index of suspicion&#46; This will help in the design of an adequate differential diagnosis&#44; in deciding the genetic tests that confirm the definitive diagnosis and in implementing a suitable biological therapy&#46; This will help to curb&#44; or at least slow&#44; the advance of the disease and with it the development of complications &#8211; renal amyloidosis &#8211; that can effect the prognosis&#44; as occurred in this case&#46; Nevertheless&#44; the IL-6 inhibitor&#44; tocilizumab&#44; proved effective in achieving the total remission of nephrotic syndrome&#44; changing the prognosis of this disease&#46;</p></span><span id="sec0025" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0045">Ethical Disclosures</span><span id="sec0030" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0050">Protection of human and animal subjects</span><p id="par0050" class="elsevierStylePara elsevierViewall">The authors declare that no experiments were performed on humans or animals for this study&#46;</p></span><span id="sec0035" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0055">Confidentiality of data</span><p id="par0055" class="elsevierStylePara elsevierViewall">The authors declare that they have followed the protocols of their work center on the publication of patient data&#46;</p></span><span id="sec0040" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0060">Right to privacy and informed consent</span><p id="par0060" class="elsevierStylePara elsevierViewall">The authors declare that no patient data appear in this article&#46;</p></span></span><span id="sec0045" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0065">Conflicts of Interest</span><p id="par0065" class="elsevierStylePara elsevierViewall">The authors declare they have no conflicts of interest&#46;</p></span></span>"
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            2 => "Cryopyrin-associated periodic syndromes"
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        "resumen" => "<span id="abst0005" class="elsevierStyleSection elsevierViewall"><p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">Muckle-Wells syndrome is a systemic autoinflammatory disease included in the group of hereditary periodic febrile syndromes&#46;</p><p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">We report the case of a patient with this rare disease to call the attention to the singularity of this condition&#44; its low incidence&#44; its atypical presentation and the subsequent delay in the diagnosis&#44; which is reached when late and devastating consequences have taken place&#46;</p><p id="spar0015" class="elsevierStyleSimplePara elsevierViewall">In this case&#44; the first-line therapy&#44; anti-interleukin 1 &#40;IL-1&#41;&#44; failed to control the disease&#46; Nevertheless&#44; the IL-6 inhibitor&#44; tocilizumab&#44; proved effective&#44; achieving the total remission of nephrotic syndrome associated with AA secondary amyloidosis&#44; changing the bleak prognosis of this disease&#46;</p></span>"
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        "resumen" => "<span id="abst0010" class="elsevierStyleSection elsevierViewall"><p id="spar0020" class="elsevierStyleSimplePara elsevierViewall">El s&#237;ndrome de Muckle-Wells es una enfermedad autoinflamatoria sist&#233;mica incluida dentro del grupo de fiebres peri&#243;dicas hereditarias&#46;</p><p id="spar0025" class="elsevierStyleSimplePara elsevierViewall">Presentamos a un paciente con esta enfermedad para llamar la atenci&#243;n sobre la singularidad de esta entidad&#44; su baja frecuencia y presentaci&#243;n at&#237;pica&#44; que conllevan generalmente demora en el diagn&#243;stico&#44; cuando ya hay consecuencias tard&#237;as y muchas veces devastadoras&#46;</p><p id="spar0030" class="elsevierStyleSimplePara elsevierViewall">En este caso&#44; la terapia de primera l&#237;nea antiinterleucina 1 &#40;IL-1&#41; no consigui&#243; frenar la enfermedad&#44; consiguiendo&#44; sin embargo&#44; su control el inhibidor de IL-6 tocilizumab&#44; mostr&#225;ndose eficaz en la remisi&#243;n total del s&#237;ndrome nefr&#243;tico asociado a amiloidosis secundaria AA&#44; cambiando su oscuro pron&#243;stico&#46;</p></span>"
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        "nota" => "<p class="elsevierStyleNotepara" id="npar0005">Please cite this article as&#58; Sol&#237;s Marqu&#237;nez MN&#44; Garc&#237;a Fern&#225;ndez E&#44; Mor&#237;s de la Tassa J&#46; Fiebres peri&#243;dicas&#58; de la enfermedad de Still al s&#237;ndrome de Muckle-Wells&#46; Reumatol Clin&#46; 2019&#59;15&#58;e39&#8211;e40&#46;</p>"
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Case Report
Periodic Fever: From Still's Disease to Muckle-Wells Syndrome
Fiebres periódicas: de la enfermedad de Still al síndrome de Muckle-Wells
Marta Nataya Solís Marquíneza,
Corresponding author
natayasolis@gmail.com

Corresponding author.
, Edilia García Fernándezb, Joaquín Morís de la Tassaa,c
a Servicio de Medicina Interna, Hospital Universitario de Cabueñes, Gijón, Asturias, Spain
b Servicio de Reumatología, Hospital Universitario de Cabueñes, Gijón, Asturias, Spain
c Departamento de Medicina, Facultad de Medicina y Ciencias de la Salud, Universidad de Oviedo, Oviedo, Asturias, Spain
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cutaneous&#44; articular &#40;CINCA&#41; syndrome and neonatal onset multisystem inflammatory disease &#40;NOMID&#41;&#46;</p><p id="par0010" class="elsevierStylePara elsevierViewall">There are no clearly defined diagnostic criteria but&#44; according to the medical literature&#44; its diagnosis is based on 3 findings&#46; In the first place&#44; the clinical onset begins with episodes characterized by fever&#44; rash and&#47;or urticaria and arthralgia and&#47;or arthritis associated with abdominal pain and previous conjunctivitis and&#47;or uveitis&#46; These episodes are self-limiting and recurrent&#44; and have a duration of 2&#8211;5 days&#46; Subsequently&#44; the patient may be found to have progressive sensorineural deafness and&#47;or secondary &#40;AA&#41; amyloidosis &#40;fundamentally with renal involvement&#41;&#59; the first develops in approximately 60&#37; of the patients&#44; whereas the second occurs in around 25&#37;&#46;<a class="elsevierStyleCrossRef" href="#bib0040"><span class="elsevierStyleSup">2</span></a></p><p id="par0015" class="elsevierStylePara elsevierViewall">Treatment is initially based on nonsteroidal anti-inflammatory drugs &#40;NSAID&#41;&#44; colchicine and corticosteroids&#44; in addition to anti-IL-1 therapies<a class="elsevierStyleCrossRef" href="#bib0045"><span class="elsevierStyleSup">3</span></a> &#40;mainly anakinra&#44; rilonacept and canakinumab&#41;&#46;<a class="elsevierStyleCrossRef" href="#bib0050"><span class="elsevierStyleSup">4</span></a></p></span><span id="sec0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0030">Case Report</span><p id="par0020" class="elsevierStylePara elsevierViewall">The patient was a 25-year-old man who had had recurrent episodes of prolonged fever since he was 2 years old&#44; occurring 1 to 2 times a year&#46; The presence of hepatosplenomegaly had been detected in a pediatric examination&#46; He also had joint and muscle pain in the absence of inflammatory signs&#59; he had a few episodes of abdominal pain and evanescent rash on 2 occasions&#46; There was no family history of recurrent fevers or connective tissue diseases&#46; He was evaluated in the pediatrics&#44; rheumatology&#44; hematology and internal medicine departments&#44; with an initial diagnosis of systemic onset Still&#39;s disease&#44; which was treated with NSAID and paracetamol&#46;</p><p id="par0025" class="elsevierStylePara elsevierViewall">His laboratory tests showed persistent leukocytosis and elevation of acute phase reactants&#44; C-reactive protein and erythrocyte sedimentation rate&#46; However&#44; the levels of ferritin&#44; rheumatoid factor&#44; antinuclear antibodies &#40;ANA&#41;&#44; the complement system and immunoglobulins were always negative or within the normal range&#46;</p><p id="par0030" class="elsevierStylePara elsevierViewall">When the patient was 23 years old&#44; the presence of proteinuria in the nephrotic range was detected&#44; with no changes in the sediment&#46; Given the suspicion of proteinuria in relation to the administration of NSAID or his underlying inflammatory process&#44; we requested a renal biopsy&#44; which revealed secondary amyloidosis &#40;<a class="elsevierStyleCrossRef" href="#fig0005">Fig&#46; 1</a>&#41;&#46; At that time&#44; with the above description of the clinical findings and analytical results&#44; and after the development of renal amyloidosis&#44; we decided to reconsider the diagnosis&#46; We thought of the possibility that it could be a hereditary systemic autoinflammatory disease rather than Still&#39;s disease&#44; and we requested genetic studies in that respect&#46; As the result was positive for mutation of the <span class="elsevierStyleItalic">NLRP3</span> gene&#44; the diagnosis was Muckle-Wells syndrome&#46;</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><p id="par0035" class="elsevierStylePara elsevierViewall">Treatment was begun with subcutaneous anakinra at a dose of 100<span class="elsevierStyleHsp" style=""></span>mg daily&#44;<a class="elsevierStyleCrossRef" href="#bib0050"><span class="elsevierStyleSup">4</span></a> but that failed to achieve adequate control of proteinuria&#46; Given the poor response and following the finding of elevated IL-6 levels&#44; anakinra was discontinued and treatment was initiated with tocilizumab &#40;an IL-6 inhibitor&#41;&#46; Over the past 6 years&#44; the patient has been asymptomatic&#44; with good control of the inflammation and normal renal function without proteinuria&#46;</p></span><span id="sec0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0035">Discussion</span><p id="par0040" class="elsevierStylePara elsevierViewall">Since infancy&#44; our patient had had recurrent episodes of fever and arthralgia&#44; in addition to episodes of abdominal pain and skin rash&#46; Rheumatoid factor and ANA were negative&#44; all of which is compatible both with Still&#39;s disease<a class="elsevierStyleCrossRef" href="#bib0055"><span class="elsevierStyleSup">5</span></a> and cryopyrinopathies&#46; The coexistence of hepatosplenomegaly&#44; according to the criteria of Yamaguchi&#44;<a class="elsevierStyleCrossRef" href="#bib0060"><span class="elsevierStyleSup">6</span></a> indicated the diagnosis of Still&#39;s disease&#46; It was not until the development of renal amyloidosis&#44; 20 years later&#44; that the diagnosis was reconsidered&#46; The genetic study confirmed the diagnosis of Muckle-Wells syndrome&#46;</p></span><span id="sec0020" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0040">Conclusion</span><p id="par0045" class="elsevierStylePara elsevierViewall">Given the rarity of these syndromes in routine clinical practice&#44; it is necessary to stress the importance of being aware of their different and sometimes incomplete forms of presentation and maintain a high index of suspicion&#46; This will help in the design of an adequate differential diagnosis&#44; in deciding the genetic tests that confirm the definitive diagnosis and in implementing a suitable biological therapy&#46; This will help to curb&#44; or at least slow&#44; the advance of the disease and with it the development of complications &#8211; renal amyloidosis &#8211; that can effect the prognosis&#44; as occurred in this case&#46; Nevertheless&#44; the IL-6 inhibitor&#44; tocilizumab&#44; proved effective in achieving the total remission of nephrotic syndrome&#44; changing the prognosis of this disease&#46;</p></span><span id="sec0025" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0045">Ethical Disclosures</span><span id="sec0030" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0050">Protection of human and animal subjects</span><p id="par0050" class="elsevierStylePara elsevierViewall">The authors declare that no experiments were performed on humans or animals for this study&#46;</p></span><span id="sec0035" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0055">Confidentiality of data</span><p id="par0055" class="elsevierStylePara elsevierViewall">The authors declare that they have followed the protocols of their work center on the publication of patient data&#46;</p></span><span id="sec0040" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0060">Right to privacy and informed consent</span><p id="par0060" class="elsevierStylePara elsevierViewall">The authors declare that no patient data appear in this article&#46;</p></span></span><span id="sec0045" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0065">Conflicts of Interest</span><p id="par0065" class="elsevierStylePara elsevierViewall">The authors declare they have no conflicts of interest&#46;</p></span></span>"
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          "titulo" => "Discussion"
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          "titulo" => "Conclusion"
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          "titulo" => "Ethical Disclosures"
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            0 => array:2 [
              "identificador" => "sec0030"
              "titulo" => "Protection of human and animal subjects"
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              "titulo" => "Confidentiality of data"
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              "titulo" => "Right to privacy and informed consent"
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        ]
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          "titulo" => "Conflicts of Interest"
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          "titulo" => "References"
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          "palabras" => array:6 [
            0 => "Periodic fever"
            1 => "Muckle-Wells syndrome"
            2 => "Cryopyrin-associated periodic syndromes"
            3 => "Interleukin 1 receptor antagonist protein"
            4 => "Interleukin 6 inhibitor"
            5 => "Still&#39;s disease"
          ]
        ]
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          "palabras" => array:6 [
            0 => "Fiebre peri&#243;dica"
            1 => "S&#237;ndrome de Muckle-Wells"
            2 => "S&#237;ndromes peri&#243;dicos asociados a criopirinas"
            3 => "Antagonista del receptor interleucina 1"
            4 => "Inhibidor de interleucina 6"
            5 => "Enfermedad de Still"
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        "titulo" => "Abstract"
        "resumen" => "<span id="abst0005" class="elsevierStyleSection elsevierViewall"><p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">Muckle-Wells syndrome is a systemic autoinflammatory disease included in the group of hereditary periodic febrile syndromes&#46;</p><p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">We report the case of a patient with this rare disease to call the attention to the singularity of this condition&#44; its low incidence&#44; its atypical presentation and the subsequent delay in the diagnosis&#44; which is reached when late and devastating consequences have taken place&#46;</p><p id="spar0015" class="elsevierStyleSimplePara elsevierViewall">In this case&#44; the first-line therapy&#44; anti-interleukin 1 &#40;IL-1&#41;&#44; failed to control the disease&#46; Nevertheless&#44; the IL-6 inhibitor&#44; tocilizumab&#44; proved effective&#44; achieving the total remission of nephrotic syndrome associated with AA secondary amyloidosis&#44; changing the bleak prognosis of this disease&#46;</p></span>"
      ]
      "es" => array:2 [
        "titulo" => "Resumen"
        "resumen" => "<span id="abst0010" class="elsevierStyleSection elsevierViewall"><p id="spar0020" class="elsevierStyleSimplePara elsevierViewall">El s&#237;ndrome de Muckle-Wells es una enfermedad autoinflamatoria sist&#233;mica incluida dentro del grupo de fiebres peri&#243;dicas hereditarias&#46;</p><p id="spar0025" class="elsevierStyleSimplePara elsevierViewall">Presentamos a un paciente con esta enfermedad para llamar la atenci&#243;n sobre la singularidad de esta entidad&#44; su baja frecuencia y presentaci&#243;n at&#237;pica&#44; que conllevan generalmente demora en el diagn&#243;stico&#44; cuando ya hay consecuencias tard&#237;as y muchas veces devastadoras&#46;</p><p id="spar0030" class="elsevierStyleSimplePara elsevierViewall">En este caso&#44; la terapia de primera l&#237;nea antiinterleucina 1 &#40;IL-1&#41; no consigui&#243; frenar la enfermedad&#44; consiguiendo&#44; sin embargo&#44; su control el inhibidor de IL-6 tocilizumab&#44; mostr&#225;ndose eficaz en la remisi&#243;n total del s&#237;ndrome nefr&#243;tico asociado a amiloidosis secundaria AA&#44; cambiando su oscuro pron&#243;stico&#46;</p></span>"
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    "NotaPie" => array:1 [
      0 => array:2 [
        "etiqueta" => "&#9734;"
        "nota" => "<p class="elsevierStyleNotepara" id="npar0005">Please cite this article as&#58; Sol&#237;s Marqu&#237;nez MN&#44; Garc&#237;a Fern&#225;ndez E&#44; Mor&#237;s de la Tassa J&#46; Fiebres peri&#243;dicas&#58; de la enfermedad de Still al s&#237;ndrome de Muckle-Wells&#46; Reumatol Clin&#46; 2019&#59;15&#58;e39&#8211;e40&#46;</p>"
      ]
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          "en" => "<p id="spar0035" class="elsevierStyleSimplePara elsevierViewall">Image of the glomerulus of the patient stained with Congo red &#40;40&#215;&#41;&#44; showing positivity for amyloid deposited in the mesangium &#40;arrow&#41;&#46;</p>"
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    ]
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                      "titulo" => "Enfermedades autoinflamatorias sist&#233;micas hereditarias"
                      "autores" => array:1 [
                        0 => array:2 [
                          "etal" => false
                          "autores" => array:1 [
                            0 => "J&#46;I&#46; Ar&#243;stegui"
                          ]
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                    ]
                  ]
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                    0 => array:2 [
                      "doi" => "10.1016/j.reuma.2010.01.010"
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                        "tituloSerie" => "Reumatol Clin"
                        "fecha" => "2011"
                        "volumen" => "7"
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                      "titulo" => "S&#237;ndrome de Muckle-Wells y s&#237;ndrome autoinflamatorio familiar inducido por fr&#237;o"
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                      "titulo" => "Muckle-Wells treatment with anakinra"
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                        0 => array:2 [
                          "etal" => false
                          "autores" => array:4 [
                            0 => "J&#46; Eungdamrong"
                            1 => "K&#46;P&#46; Boyd"
                            2 => "S&#46;A&#46; Meehan"
                            3 => "J&#46;A&#46; Latkowski"
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Article information
ISSN: 21735743
Original language: English
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