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    "textoCompleto" => "<span class="elsevierStyleSections"><p id="par0005" class="elsevierStylePara elsevierViewall">Congenital hyperinsulinism &#40;CHI&#41; is the most frequent cause of persistent hypoglycaemia in early childhood&#46; It encompasses a heterogeneous group of genetic disorders whose overall incidence amounts to 1 in 30<span class="elsevierStyleHsp" style=""></span>000 to 50<span class="elsevierStyleHsp" style=""></span>000 live births&#44; although the incidence can be as high as 1 in 2500 births in populations with a high rate of inbreeding&#46;<a class="elsevierStyleCrossRef" href="#bib0035"><span class="elsevierStyleSup">1</span></a></p><p id="par0010" class="elsevierStylePara elsevierViewall">Although neonatal hypoglycaemia is the typical form of CHI&#44; the age at onset and clinical picture are highly variable&#44; and therefore CHI should be included in the differential diagnosis of non-ketotic hypoglycaemia at any age&#46; It should be suspected in cases of persistent non-ketotic hypoglycaemia with abnormally high levels of insulin&#46; The diagnosis of CHI is complicated by the lack of consensus on the biochemical criteria used to define it&#46;<a class="elsevierStyleCrossRefs" href="#bib0040"><span class="elsevierStyleSup">2&#44;3</span></a> Genetic testing identifies the causative mutation in only half of cases&#46; Positron-emission tomography &#40;PET&#41; allows differentiation between focal and diffuse forms of CHI&#46; The management consists of dietary measures and pharmacological treatment&#44; and diazoxide is the first-line drug for treatment of CHI&#46; Adequate glycaemic control is usually achieved in time&#46; There are data suggesting that 50&#37; of patients with CHI have neurologic sequelae secondary to unidentified episodes of hypoglycaemia&#46;<a class="elsevierStyleCrossRefs" href="#bib0045"><span class="elsevierStyleSup">3&#44;4</span></a></p><p id="par0015" class="elsevierStylePara elsevierViewall">We present the cases of 5 patients with CHI diagnosed and treated in our hospital in the past 6 years&#46; <a class="elsevierStyleCrossRefs" href="#tbl0005">Tables 1 and 2</a> summarise the clinical features at diagnosis and during followup&#46; The causative mutation was identified in 3 patients&#46; One patient had a mutation in the <span class="elsevierStyleItalic">KCNJ11</span> gene&#44; which is associated with the most prevalent and severe form of CHI&#44; and another had the second most frequent mutation &#40;in the <span class="elsevierStyleItalic">ABCC8</span> gene&#41;&#46; The third patient had a novel mutation in the <span class="elsevierStyleItalic">GLUD1</span> gene&#44; changes in which are known to cause hyperinsulinism&#47;hyperammonaemia syndrome&#46; All 5 patients required continuous intravenous glucose infusion&#44; whose dose could be tapered off after initiation of treatment with diazoxide&#46; One patient received a diagnosis of hypertrophic cardiomyopathy &#40;HCM&#41;&#46; Four patients underwent an F-18 fluoro-L3&#44;4-dihydroxyphenylalanine PET scan&#44; which revealed focal involvement in only one of them&#46; Two patients had psychomotor retardation&#44; associated with polymalformative syndrome in one&#46; A patient with a phenotype compatible with Beckwith-Wiedemann syndrome showed a favourable response to treatment starting at 6 months of age&#44; and diazoxide was thereon tapered off&#46;</p><elsevierMultimedia ident="tbl0005"></elsevierMultimedia><elsevierMultimedia ident="tbl0010"></elsevierMultimedia><p id="par0020" class="elsevierStylePara elsevierViewall">The incidence of CHI in our community exceeds the one described in the literature&#44; as it amounts to 1 per 2400 live births&#46; Our sample illustrates the clinical variability of CHI&#44; which reflects the heterogeneity of the genetic causes of these disorders&#46; In some mutations there is a clear genotype-phenotype correlation&#46; If the mutation affects potassium channels&#44; hyperinsulinism is severe&#44; has an early onset&#44; and is mostly refractory to pharmacological treatment&#46; In contrast&#44; mutations leading to changes in glutamate dehydrogenase cause late-onset CHI&#44; which is less severe&#44; and hyperammonaemia&#46;<a class="elsevierStyleCrossRef" href="#bib0045"><span class="elsevierStyleSup">3</span></a> The patient corresponding to case 2 had a mutation in this gene&#44; with onset at 14 months and detection of hyperammonaemia&#44; contrary to expectations&#44; detected only on one occasion&#46; He improved on a leucine-free diet&#44; achieving greater stability in blood glucose levels&#46;</p><p id="par0025" class="elsevierStylePara elsevierViewall">Congenital hyperinsulinism is associated with cardiac abnormalities&#44; mainly HCM and ventricular dysfunction&#46; The prevalence of HCM seems increased in the most severe forms of CHI&#44; reaching up to 65&#37; in some case series&#46;<a class="elsevierStyleCrossRef" href="#bib0055"><span class="elsevierStyleSup">5</span></a> Some professionals recommend performance of an electrocardiogram and echocardiographic examination in all children with CHI&#46;<a class="elsevierStyleCrossRef" href="#bib0060"><span class="elsevierStyleSup">6</span></a> We performed electrocardiograms in all our patients&#44; which detected severe HCM with mild ventricular outflow obstruction in only one patient&#44; a problem that resolved gradually without treatment&#46; We believe that detection of ventricular hypertrophy in a newborn with persistent hypoglycaemia is highly suggestive of CHI&#44; and that awareness of this association could facilitate early diagnosis&#46;</p><p id="par0030" class="elsevierStylePara elsevierViewall">Congenital hyperinsulinism may be associated with several syndromes&#44; and Beckwith-Wiedemann syndrome is the most frequent comorbidity out of the overgrowth disorders&#46;<a class="elsevierStyleCrossRef" href="#bib0050"><span class="elsevierStyleSup">4</span></a> In our series&#44; 2 patients had polymalformative syndrome&#44; of who 1 had a phenotype compatible with Beckwith-Wiedemann syndrome&#46;</p><p id="par0035" class="elsevierStylePara elsevierViewall">We detected a single case of focal pancreatic &#946;-cell hypoplasia that could benefit from surgery in the future&#46; Selective partial pancreatectomy in cases with focal hypoplasia would be sufficient for cure of CHI&#44; avoiding the side effects of more aggressive surgical interventions&#46;<a class="elsevierStyleCrossRef" href="#bib0040"><span class="elsevierStyleSup">2</span></a></p><p id="par0040" class="elsevierStylePara elsevierViewall">When it came to neurologic outcomes in our case series&#44; CHI was far from being a benign disease&#46; While most patients achieved adequate blood sugar control&#44; neurodevelopmental impairment was common&#46; The use of continuous glucose monitors could play a significant role in the followup of these patients&#46;</p><p id="par0045" class="elsevierStylePara elsevierViewall">In conclusion&#44; we found considerable variability in the phenotypic expression of CHI&#46; Genetic testing and PET are essential to determine the prognosis and the approach to treatment&#46; The prevention of episodes of hypoglycaemia is essential to improve neurodevelopmental outcomes&#46;</p></span>"
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                  \t\t\t\t</th><th class="td" title="table-head  " align="left" valign="top" scope="col" style="border-bottom: 2px solid black">Symptoms&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</th><th class="td" title="table-head  " align="left" valign="top" scope="col" style="border-bottom: 2px solid black">Glycaemia mg&#47;dL&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</th><th class="td" title="table-head  " align="left" valign="top" scope="col" style="border-bottom: 2px solid black">Insulin &#956;U&#47;mL&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</th><th class="td" title="table-head  " align="left" valign="top" scope="col" style="border-bottom: 2px solid black">Ammonia &#956;mol&#47;L&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</th><th class="td" title="table-head  " align="left" valign="top" scope="col" style="border-bottom: 2px solid black">Glucagon test&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</th><th class="td" title="table-head  " align="left" valign="top" scope="col" style="border-bottom: 2px solid black">Maximum GIR mg&#47;kg&#47;min&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</th></tr></thead><tbody title="tbody"><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Case 1&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">7 days&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">CA&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">33&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">9&#46;69&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">53&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Positive&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">26&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Case 2&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">14 months&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Seizures&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">36&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">27&#46;74&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">95&#46;1&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Positive&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">15&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Case 3&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">4 days&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Seizures&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">40&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">6&#46;86&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">36&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Positive&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">10&#46;7&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Case 4&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">3 months&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Apnoea&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">33&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">2&#46;22&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">50&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Positive&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">15&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Case 5&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">2 days&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">None&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">32&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">18&#46;67&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">43&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Negative&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">19&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr></tbody></table>
                  """
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          "leyenda" => "<p id="spar0020" class="elsevierStyleSimplePara elsevierViewall">BWS&#44; Beckwith-Wiedemann syndrome&#59; ON&#44; optic neuropathy&#59; PET&#44; positron emission tomography&#46;</p>"
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                0 => """
                  <table border="0" frame="\n
                  \t\t\t\t\tvoid\n
                  \t\t\t\t" class=""><thead title="thead"><tr title="table-row"><th class="td" title="table-head  " align="" valign="top" scope="col" style="border-bottom: 2px solid black">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</th><th class="td" title="table-head  " align="left" valign="top" scope="col" style="border-bottom: 2px solid black">Gene mutation&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</th><th class="td" title="table-head  " align="left" valign="top" scope="col" style="border-bottom: 2px solid black">PET&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</th><th class="td" title="table-head  " align="left" valign="top" scope="col" style="border-bottom: 2px solid black">Response to diazoxide&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</th><th class="td" title="table-head  " align="left" valign="top" scope="col" style="border-bottom: 2px solid black">Neurologic manifestations&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</th><th class="td" title="table-head  " align="left" valign="top" scope="col" style="border-bottom: 2px solid black">Other clinical findings&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</th></tr></thead><tbody title="tbody"><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Case 1&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top"><span class="elsevierStyleItalic">KCNJ11</span>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Multifocal pancreas&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Adequate&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Psychomotor retardation<br>Epilepsy&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Bilateral ventricular dysfunction&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Case 2&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top"><span class="elsevierStyleItalic">GLUD1</span>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Diffuse pancreatic involvement&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Partial<br>Adequate after exclusion of leucine from diet&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Asymptomatic&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Normal&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Case 3&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top"><span class="elsevierStyleItalic">ABCC8</span>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Adenoma in head of pancreas&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Adequate&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Asymptomatic&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Hypertrophic cardiomyopathy&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Case 4&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Not identified&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Diffuse involvement of pancreas&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Adequate&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Psychomotor retardation<br>Microcephaly<br>ON&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Polymalformative syndrome&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Case 5&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Results pending&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Not performed yet&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Adequate&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Hypotonia&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Polymalformative syndrome compatible with BWS&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr></tbody></table>
                  """
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      "titulo" => "References"
      "seccion" => array:1 [
        0 => array:2 [
          "identificador" => "bibs0015"
          "bibliografiaReferencia" => array:6 [
            0 => array:3 [
              "identificador" => "bib0035"
              "etiqueta" => "1"
              "referencia" => array:1 [
                0 => array:2 [
                  "contribucion" => array:1 [
                    0 => array:2 [
                      "titulo" => "Hiperinsulinismo cong&#233;nito&#46; Revisi&#243;n de 22 casos"
                      "autores" => array:1 [
                        0 => array:2 [
                          "etal" => false
                          "autores" => array:4 [
                            0 => "J&#46; Guerrero-Fern&#225;ndez"
                            1 => "I&#46; Gonz&#225;lez Casado"
                            2 => "L&#46; Espinosa Colindres"
                            3 => "R&#46; Gracia Bouthelier"
                          ]
                        ]
                      ]
                    ]
                  ]
                  "host" => array:1 [
                    0 => array:1 [
                      "Revista" => array:5 [
                        "tituloSerie" => "An Pediatr &#40;Barc&#41;"
                        "fecha" => "2006"
                        "volumen" => "65"
                        "paginaInicial" => "22"
                        "paginaFinal" => "31"
                      ]
                    ]
                  ]
                ]
              ]
            ]
            1 => array:3 [
              "identificador" => "bib0040"
              "etiqueta" => "2"
              "referencia" => array:1 [
                0 => array:2 [
                  "contribucion" => array:1 [
                    0 => array:2 [
                      "titulo" => "Hiperinsulinismo cong&#233;nito&#58; nuestra casu&#237;stica y revisi&#243;n de la literatura"
                      "autores" => array:1 [
                        0 => array:2 [
                          "etal" => false
                          "autores" => array:4 [
                            0 => "S&#46; Chahin"
                            1 => "G&#46; Edinson Guzm&#225;n G&#243;mez"
                            2 => "I&#46; Dussan"
                            3 => "A&#46; Hoyos-Mart&#237;nez"
                          ]
                        ]
                      ]
                    ]
                  ]
                  "host" => array:1 [
                    0 => array:1 [
                      "Revista" => array:5 [
                        "tituloSerie" => "Rev Esp Endocrinol Pediatr"
                        "fecha" => "2014"
                        "volumen" => "5"
                        "paginaInicial" => "29"
                        "paginaFinal" => "36"
                      ]
                    ]
                  ]
                ]
              ]
            ]
            2 => array:3 [
              "identificador" => "bib0045"
              "etiqueta" => "3"
              "referencia" => array:1 [
                0 => array:2 [
                  "contribucion" => array:1 [
                    0 => array:2 [
                      "titulo" => "Perspectivas actuales en el tratamiento del hiperinsulinismo cong&#233;nito"
                      "autores" => array:1 [
                        0 => array:2 [
                          "etal" => false
                          "autores" => array:4 [
                            0 => "J&#46;R&#46; Fern&#225;ndez Lorenzo"
                            1 => "A&#46; Fern&#225;ndez Marmiesse"
                            2 => "J&#46; Barreiro Conde"
                            3 => "M&#46;L&#46; Couce Pico"
                          ]
                        ]
                      ]
                    ]
                  ]
                  "host" => array:1 [
                    0 => array:1 [
                      "Revista" => array:5 [
                        "tituloSerie" => "Acta Pediatr Esp"
                        "fecha" => "2009"
                        "volumen" => "67"
                        "paginaInicial" => "103"
                        "paginaFinal" => "111"
                      ]
                    ]
                  ]
                ]
              ]
            ]
            3 => array:3 [
              "identificador" => "bib0050"
              "etiqueta" => "4"
              "referencia" => array:1 [
                0 => array:2 [
                  "contribucion" => array:1 [
                    0 => array:2 [
                      "titulo" => "Perspective on the genetics and diagnosis of congenital hyperinsulinism disorders"
                      "autores" => array:1 [
                        0 => array:2 [
                          "etal" => false
                          "autores" => array:1 [
                            0 => "C&#46;A&#46; Stanley"
                          ]
                        ]
                      ]
                    ]
                  ]
                  "host" => array:1 [
                    0 => array:2 [
                      "doi" => "10.1210/jc.2015-3651"
                      "Revista" => array:6 [
                        "tituloSerie" => "J Clin Endocrinol Metab"
                        "fecha" => "2016"
                        "volumen" => "101"
                        "paginaInicial" => "815"
                        "paginaFinal" => "826"
                        "link" => array:1 [
                          0 => array:2 [
                            "url" => "https://www.ncbi.nlm.nih.gov/pubmed/26908106"
                            "web" => "Medline"
                          ]
                        ]
                      ]
                    ]
                  ]
                ]
              ]
            ]
            4 => array:3 [
              "identificador" => "bib0055"
              "etiqueta" => "5"
              "referencia" => array:1 [
                0 => array:2 [
                  "contribucion" => array:1 [
                    0 => array:2 [
                      "titulo" => "The association of cardiac ventricular hypertrophy with congenital hyperinsulinism"
                      "autores" => array:1 [
                        0 => array:2 [
                          "etal" => true
                          "autores" => array:6 [
                            0 => "I&#46; Banerjee"
                            1 => "B&#46; Avatapalle"
                            2 => "A&#46; Petkar"
                            3 => "M&#46; Skae"
                            4 => "R&#46; Padidela"
                            5 => "S&#46; Ehtisham"
                          ]
                        ]
                      ]
                    ]
                  ]
                  "host" => array:1 [
                    0 => array:2 [
                      "doi" => "10.1530/EJE-12-0632"
                      "Revista" => array:6 [
                        "tituloSerie" => "Eur J Endocrinol"
                        "fecha" => "2012"
                        "volumen" => "167"
                        "paginaInicial" => "619"
                        "paginaFinal" => "624"
                        "link" => array:1 [
                          0 => array:2 [
                            "url" => "https://www.ncbi.nlm.nih.gov/pubmed/22894813"
                            "web" => "Medline"
                          ]
                        ]
                      ]
                    ]
                  ]
                ]
              ]
            ]
            5 => array:3 [
              "identificador" => "bib0060"
              "etiqueta" => "6"
              "referencia" => array:1 [
                0 => array:2 [
                  "contribucion" => array:1 [
                    0 => array:2 [
                      "titulo" => "Hypertrophic cardiomyopathy in neonates with congenital hyperinsulinism"
                      "autores" => array:1 [
                        0 => array:2 [
                          "etal" => false
                          "autores" => array:5 [
                            0 => "T&#46; Huang"
                            1 => "A&#46; Kelly"
                            2 => "S&#46;A&#46; Becker"
                            3 => "M&#46;S&#46; Cohen"
                            4 => "C&#46;A&#46; Stanley"
                          ]
                        ]
                      ]
                    ]
                  ]
                  "host" => array:1 [
                    0 => array:1 [
                      "Revista" => array:5 [
                        "tituloSerie" => "Arch Dis Child Fetal Neonatal Ed"
                        "fecha" => "2013"
                        "volumen" => "98"
                        "paginaInicial" => "351"
                        "paginaFinal" => "354"
                      ]
                    ]
                  ]
                ]
              ]
            ]
          ]
        ]
      ]
    ]
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Scientific Letter
Clinical and genetic heterogeneity of congenital hyperinsulinism
Heterogeneidad clínica y genética del hiperinsulinismo congénito
Juncal Reguera Bernardino
Corresponding author
juncalreguera@msn.com

Corresponding author.
, Ignacio Oulego Erroz, Jorge Martínez Sáenz de Jubera, Rocío Quiroga González, Laura Regueras Santos
Servicio de Pediatría, Complejo Asistencial Universitario de León, León, Spain
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    "textoCompleto" => "<span class="elsevierStyleSections"><p id="par0005" class="elsevierStylePara elsevierViewall">Congenital hyperinsulinism &#40;CHI&#41; is the most frequent cause of persistent hypoglycaemia in early childhood&#46; It encompasses a heterogeneous group of genetic disorders whose overall incidence amounts to 1 in 30<span class="elsevierStyleHsp" style=""></span>000 to 50<span class="elsevierStyleHsp" style=""></span>000 live births&#44; although the incidence can be as high as 1 in 2500 births in populations with a high rate of inbreeding&#46;<a class="elsevierStyleCrossRef" href="#bib0035"><span class="elsevierStyleSup">1</span></a></p><p id="par0010" class="elsevierStylePara elsevierViewall">Although neonatal hypoglycaemia is the typical form of CHI&#44; the age at onset and clinical picture are highly variable&#44; and therefore CHI should be included in the differential diagnosis of non-ketotic hypoglycaemia at any age&#46; It should be suspected in cases of persistent non-ketotic hypoglycaemia with abnormally high levels of insulin&#46; The diagnosis of CHI is complicated by the lack of consensus on the biochemical criteria used to define it&#46;<a class="elsevierStyleCrossRefs" href="#bib0040"><span class="elsevierStyleSup">2&#44;3</span></a> Genetic testing identifies the causative mutation in only half of cases&#46; Positron-emission tomography &#40;PET&#41; allows differentiation between focal and diffuse forms of CHI&#46; The management consists of dietary measures and pharmacological treatment&#44; and diazoxide is the first-line drug for treatment of CHI&#46; Adequate glycaemic control is usually achieved in time&#46; There are data suggesting that 50&#37; of patients with CHI have neurologic sequelae secondary to unidentified episodes of hypoglycaemia&#46;<a class="elsevierStyleCrossRefs" href="#bib0045"><span class="elsevierStyleSup">3&#44;4</span></a></p><p id="par0015" class="elsevierStylePara elsevierViewall">We present the cases of 5 patients with CHI diagnosed and treated in our hospital in the past 6 years&#46; <a class="elsevierStyleCrossRefs" href="#tbl0005">Tables 1 and 2</a> summarise the clinical features at diagnosis and during followup&#46; The causative mutation was identified in 3 patients&#46; One patient had a mutation in the <span class="elsevierStyleItalic">KCNJ11</span> gene&#44; which is associated with the most prevalent and severe form of CHI&#44; and another had the second most frequent mutation &#40;in the <span class="elsevierStyleItalic">ABCC8</span> gene&#41;&#46; The third patient had a novel mutation in the <span class="elsevierStyleItalic">GLUD1</span> gene&#44; changes in which are known to cause hyperinsulinism&#47;hyperammonaemia syndrome&#46; All 5 patients required continuous intravenous glucose infusion&#44; whose dose could be tapered off after initiation of treatment with diazoxide&#46; One patient received a diagnosis of hypertrophic cardiomyopathy &#40;HCM&#41;&#46; Four patients underwent an F-18 fluoro-L3&#44;4-dihydroxyphenylalanine PET scan&#44; which revealed focal involvement in only one of them&#46; Two patients had psychomotor retardation&#44; associated with polymalformative syndrome in one&#46; A patient with a phenotype compatible with Beckwith-Wiedemann syndrome showed a favourable response to treatment starting at 6 months of age&#44; and diazoxide was thereon tapered off&#46;</p><elsevierMultimedia ident="tbl0005"></elsevierMultimedia><elsevierMultimedia ident="tbl0010"></elsevierMultimedia><p id="par0020" class="elsevierStylePara elsevierViewall">The incidence of CHI in our community exceeds the one described in the literature&#44; as it amounts to 1 per 2400 live births&#46; Our sample illustrates the clinical variability of CHI&#44; which reflects the heterogeneity of the genetic causes of these disorders&#46; In some mutations there is a clear genotype-phenotype correlation&#46; If the mutation affects potassium channels&#44; hyperinsulinism is severe&#44; has an early onset&#44; and is mostly refractory to pharmacological treatment&#46; In contrast&#44; mutations leading to changes in glutamate dehydrogenase cause late-onset CHI&#44; which is less severe&#44; and hyperammonaemia&#46;<a class="elsevierStyleCrossRef" href="#bib0045"><span class="elsevierStyleSup">3</span></a> The patient corresponding to case 2 had a mutation in this gene&#44; with onset at 14 months and detection of hyperammonaemia&#44; contrary to expectations&#44; detected only on one occasion&#46; He improved on a leucine-free diet&#44; achieving greater stability in blood glucose levels&#46;</p><p id="par0025" class="elsevierStylePara elsevierViewall">Congenital hyperinsulinism is associated with cardiac abnormalities&#44; mainly HCM and ventricular dysfunction&#46; The prevalence of HCM seems increased in the most severe forms of CHI&#44; reaching up to 65&#37; in some case series&#46;<a class="elsevierStyleCrossRef" href="#bib0055"><span class="elsevierStyleSup">5</span></a> Some professionals recommend performance of an electrocardiogram and echocardiographic examination in all children with CHI&#46;<a class="elsevierStyleCrossRef" href="#bib0060"><span class="elsevierStyleSup">6</span></a> We performed electrocardiograms in all our patients&#44; which detected severe HCM with mild ventricular outflow obstruction in only one patient&#44; a problem that resolved gradually without treatment&#46; We believe that detection of ventricular hypertrophy in a newborn with persistent hypoglycaemia is highly suggestive of CHI&#44; and that awareness of this association could facilitate early diagnosis&#46;</p><p id="par0030" class="elsevierStylePara elsevierViewall">Congenital hyperinsulinism may be associated with several syndromes&#44; and Beckwith-Wiedemann syndrome is the most frequent comorbidity out of the overgrowth disorders&#46;<a class="elsevierStyleCrossRef" href="#bib0050"><span class="elsevierStyleSup">4</span></a> In our series&#44; 2 patients had polymalformative syndrome&#44; of who 1 had a phenotype compatible with Beckwith-Wiedemann syndrome&#46;</p><p id="par0035" class="elsevierStylePara elsevierViewall">We detected a single case of focal pancreatic &#946;-cell hypoplasia that could benefit from surgery in the future&#46; Selective partial pancreatectomy in cases with focal hypoplasia would be sufficient for cure of CHI&#44; avoiding the side effects of more aggressive surgical interventions&#46;<a class="elsevierStyleCrossRef" href="#bib0040"><span class="elsevierStyleSup">2</span></a></p><p id="par0040" class="elsevierStylePara elsevierViewall">When it came to neurologic outcomes in our case series&#44; CHI was far from being a benign disease&#46; While most patients achieved adequate blood sugar control&#44; neurodevelopmental impairment was common&#46; The use of continuous glucose monitors could play a significant role in the followup of these patients&#46;</p><p id="par0045" class="elsevierStylePara elsevierViewall">In conclusion&#44; we found considerable variability in the phenotypic expression of CHI&#46; Genetic testing and PET are essential to determine the prognosis and the approach to treatment&#46; The prevention of episodes of hypoglycaemia is essential to improve neurodevelopmental outcomes&#46;</p></span>"
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                  \t\t\t\t</th><th class="td" title="table-head  " align="left" valign="top" scope="col" style="border-bottom: 2px solid black">Age&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</th><th class="td" title="table-head  " align="left" valign="top" scope="col" style="border-bottom: 2px solid black">Symptoms&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</th><th class="td" title="table-head  " align="left" valign="top" scope="col" style="border-bottom: 2px solid black">Glycaemia mg&#47;dL&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</th><th class="td" title="table-head  " align="left" valign="top" scope="col" style="border-bottom: 2px solid black">Insulin &#956;U&#47;mL&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</th><th class="td" title="table-head  " align="left" valign="top" scope="col" style="border-bottom: 2px solid black">Ammonia &#956;mol&#47;L&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</th><th class="td" title="table-head  " align="left" valign="top" scope="col" style="border-bottom: 2px solid black">Glucagon test&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</th><th class="td" title="table-head  " align="left" valign="top" scope="col" style="border-bottom: 2px solid black">Maximum GIR mg&#47;kg&#47;min&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</th></tr></thead><tbody title="tbody"><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Case 1&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">7 days&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">CA&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">33&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">9&#46;69&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">53&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Positive&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">26&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Case 2&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">14 months&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Seizures&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">36&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">27&#46;74&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">95&#46;1&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Positive&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">15&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Case 3&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">4 days&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Seizures&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">40&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">6&#46;86&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">36&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Positive&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">10&#46;7&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Case 4&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">3 months&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Apnoea&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">33&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">2&#46;22&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">50&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Positive&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">15&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Case 5&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">2 days&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">None&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">32&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">18&#46;67&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">43&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Negative&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">19&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr></tbody></table>
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                  <table border="0" frame="\n
                  \t\t\t\t\tvoid\n
                  \t\t\t\t" class=""><thead title="thead"><tr title="table-row"><th class="td" title="table-head  " align="" valign="top" scope="col" style="border-bottom: 2px solid black">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</th><th class="td" title="table-head  " align="left" valign="top" scope="col" style="border-bottom: 2px solid black">Gene mutation&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</th><th class="td" title="table-head  " align="left" valign="top" scope="col" style="border-bottom: 2px solid black">PET&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</th><th class="td" title="table-head  " align="left" valign="top" scope="col" style="border-bottom: 2px solid black">Response to diazoxide&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top"><span class="elsevierStyleItalic">KCNJ11</span>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Multifocal pancreas&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Adequate&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Psychomotor retardation<br>Epilepsy&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Bilateral ventricular dysfunction&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Case 2&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top"><span class="elsevierStyleItalic">GLUD1</span>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Diffuse pancreatic involvement&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Partial<br>Adequate after exclusion of leucine from diet&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Asymptomatic&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Normal&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Case 3&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top"><span class="elsevierStyleItalic">ABCC8</span>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Adenoma in head of pancreas&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Adequate&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Asymptomatic&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Hypertrophic cardiomyopathy&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Case 4&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Not identified&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Diffuse involvement of pancreas&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Adequate&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Psychomotor retardation<br>Microcephaly<br>ON&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Polymalformative syndrome&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Case 5&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Results pending&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Not performed yet&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Adequate&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Hypotonia&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Polymalformative syndrome compatible with BWS&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr></tbody></table>
                  """
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        0 => array:2 [
          "identificador" => "bibs0015"
          "bibliografiaReferencia" => array:6 [
            0 => array:3 [
              "identificador" => "bib0035"
              "etiqueta" => "1"
              "referencia" => array:1 [
                0 => array:2 [
                  "contribucion" => array:1 [
                    0 => array:2 [
                      "titulo" => "Hiperinsulinismo cong&#233;nito&#46; Revisi&#243;n de 22 casos"
                      "autores" => array:1 [
                        0 => array:2 [
                          "etal" => false
                          "autores" => array:4 [
                            0 => "J&#46; Guerrero-Fern&#225;ndez"
                            1 => "I&#46; Gonz&#225;lez Casado"
                            2 => "L&#46; Espinosa Colindres"
                            3 => "R&#46; Gracia Bouthelier"
                          ]
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                    0 => array:1 [
                      "Revista" => array:5 [
                        "tituloSerie" => "An Pediatr &#40;Barc&#41;"
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                        "volumen" => "65"
                        "paginaInicial" => "22"
                        "paginaFinal" => "31"
                      ]
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              "identificador" => "bib0040"
              "etiqueta" => "2"
              "referencia" => array:1 [
                0 => array:2 [
                  "contribucion" => array:1 [
                    0 => array:2 [
                      "titulo" => "Hiperinsulinismo cong&#233;nito&#58; nuestra casu&#237;stica y revisi&#243;n de la literatura"
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                        0 => array:2 [
                          "etal" => false
                          "autores" => array:4 [
                            0 => "S&#46; Chahin"
                            1 => "G&#46; Edinson Guzm&#225;n G&#243;mez"
                            2 => "I&#46; Dussan"
                            3 => "A&#46; Hoyos-Mart&#237;nez"
                          ]
                        ]
                      ]
                    ]
                  ]
                  "host" => array:1 [
                    0 => array:1 [
                      "Revista" => array:5 [
                        "tituloSerie" => "Rev Esp Endocrinol Pediatr"
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                        "volumen" => "5"
                        "paginaInicial" => "29"
                        "paginaFinal" => "36"
                      ]
                    ]
                  ]
                ]
              ]
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              "identificador" => "bib0045"
              "etiqueta" => "3"
              "referencia" => array:1 [
                0 => array:2 [
                  "contribucion" => array:1 [
                    0 => array:2 [
                      "titulo" => "Perspectivas actuales en el tratamiento del hiperinsulinismo cong&#233;nito"
                      "autores" => array:1 [
                        0 => array:2 [
                          "etal" => false
                          "autores" => array:4 [
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                            3 => "M&#46;L&#46; Couce Pico"
                          ]
                        ]
                      ]
                    ]
                  ]
                  "host" => array:1 [
                    0 => array:1 [
                      "Revista" => array:5 [
                        "tituloSerie" => "Acta Pediatr Esp"
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                        "volumen" => "67"
                        "paginaInicial" => "103"
                        "paginaFinal" => "111"
                      ]
                    ]
                  ]
                ]
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                0 => array:2 [
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                      "titulo" => "Perspective on the genetics and diagnosis of congenital hyperinsulinism disorders"
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                          "etal" => false
                          "autores" => array:1 [
                            0 => "C&#46;A&#46; Stanley"
                          ]
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                    ]
                  ]
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                    0 => array:2 [
                      "doi" => "10.1210/jc.2015-3651"
                      "Revista" => array:6 [
                        "tituloSerie" => "J Clin Endocrinol Metab"
                        "fecha" => "2016"
                        "volumen" => "101"
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                      "titulo" => "The association of cardiac ventricular hypertrophy with congenital hyperinsulinism"
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                          "etal" => true
                          "autores" => array:6 [
                            0 => "I&#46; Banerjee"
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                            2 => "A&#46; Petkar"
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                        ]
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                    ]
                  ]
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                      "doi" => "10.1530/EJE-12-0632"
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                        "tituloSerie" => "Eur J Endocrinol"
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                        "volumen" => "167"
                        "paginaInicial" => "619"
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                      "titulo" => "Hypertrophic cardiomyopathy in neonates with congenital hyperinsulinism"
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                          "autores" => array:5 [
                            0 => "T&#46; Huang"
                            1 => "A&#46; Kelly"
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                            3 => "M&#46;S&#46; Cohen"
                            4 => "C&#46;A&#46; Stanley"
                          ]
                        ]
                      ]
                    ]
                  ]
                  "host" => array:1 [
                    0 => array:1 [
                      "Revista" => array:5 [
                        "tituloSerie" => "Arch Dis Child Fetal Neonatal Ed"
                        "fecha" => "2013"
                        "volumen" => "98"
                        "paginaInicial" => "351"
                        "paginaFinal" => "354"
                      ]
                    ]
                  ]
                ]
              ]
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Article information
ISSN: 23412879
Original language: English
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Idiomas
Anales de Pediatría (English Edition)
es en

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