Hipoglycemia secondary to hyperinsulinism in a patient with congenital long QT syndrome

Salamanca Zarzuela, Beatriz; Alcalde Martín, Carlos; Centeno Malfaz, Fernando

doi:10.1016/j.medcle.2019.01.047

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Knowing its presentation and the comorbidities associated with it are essential for proper clinical practice.We present the case of a 3-year-old patient, electrocardiographically diagnosed with LQTS at birth, due to a maternal history of the same syndrome. Maternal genetics had 2 mutations functionally described as benign, related to the Brugada syndrome and arrhythmogenic right ventricular cardiomyopathy, without any classic LQTS mutation. She was diagnosed after resuscitation from sudden death while swimming. She currently has an Automatic Implantable Cardioverter Defibrillator (ICD) implanted. At birth the patient presented a QT interval of 640ms that physiologically decreased over the first days of life to 490ms. This duration has been maintained since then. Treatment with labetalol was started at 9 days of age and continues to date. There were never any episodes of tachycardia or other arrhythmias.At 2 years of age, after 2 days vomiting, she was brought to the paediatric emergency department with an episode of hypoactivity and hyporeactivity, along with pallor and sweating. Associated bradycardia (40bpm), with good peripheral perfusion and age-appropriate blood pressure (116/70mmHg), with no signs of respiratory distress. Upon arrival at the emergency department, support treatment was started, showing a hypoglycaemia level of 30mg/dl. Intravenous glucagon was administered, with progressive general recovery.Eight months later, coinciding with a fever and 2 isolated vomits, she again presented a similar episode, but of less clinical severity than the first. Once the stability of the patient was verified, her hypoglycaemia level was 45mg/dl, which yielded after the bolus administration of 10% glucose serum, with good subsequent clinical progress.In both cases, the patient was hospitalised for observation and serum therapy with glucose input until oral tolerance returned to normal. Glycated haemoglobin A1c (NGSP) and glycated haemoglobin A1 (IFCC) levels were tested, which showed normal results, as well as baseline insulin: 15.8U/ml, in the upper range of normality.LQTS is an arrhythmogenic channelopathy characterised by a severe alteration in the ventricular repolarisation, recognised by a prolonged QT interval on the electrocardiogram. It is also characterised by predisposition to sudden death from malignant ventricular arrhythmias of the “torsade de pointes” type. The channels most affected are those of potassium. Hundreds of mutations have been described which are distributed in 10 syndrome-related genes so far. The genetic study of these patients has shown that although the severe form of the disease is sporadic, there are polymorphisms with regulatory qualities that can exacerbate or silence the severity of a mutation.<a class="elsevierStyleCrossRef" href="#bib0025">1</a>Currently, treatment with beta-blockers is the mainstay of its management especially in children, where ICD implantation is highly restricted due to the physical and developmental characteristics at this age. Beta-blockers have been shown to reduce the cardiac events in LQTS, with different efficacy depending on the type of beta-blocker and the LQT genotype. Nadolol is currently considered as first-line therapy.<a class="elsevierStyleCrossRef" href="#bib0030">2</a>As this group of drugs is the first choice in younger patients, there are cases of LQTS patients who have developed beta-blocker-induced hypoglycaemia with varying clinical severity. As with our patient, all the published cases presented a history of low intake in the previous hours, with the appearance of this phenomenon being more frequent in patients with LQTS2.<a class="elsevierStyleCrossRef" href="#bib0035">3</a>This association has been the subject of studies that have concluded that in addition to having a prolonged QT interval, patients with LQTS with pathogenic genetics in KCNQ1 present a certain degree of hyperinsulinism. This is because the KCNQ1 voltage-gated potassium channel is also involved in insulin secretion.<a class="elsevierStyleCrossRef" href="#bib0040">4</a> Clinically it translates as postprandial hypoglycaemia, severe symptomatic hypoglycaemia, and lower potassium levels. This set of signs, in addition to the acute symptoms, increase the risk of cardiac events.<a class="elsevierStyleCrossRef" href="#bib0040">4</a>The patient we present suffers from a pathogenic mutation in KCNQ1 different from that of her mother. This case shows that the genetic result entails both cardiological as well as metabolic prognostic factors. Given the morbidity associated with severe hypoglycaemia, especially in paediatric patients, it is important to teach the parents to recognise the early symptoms; as well as ensuring the knowledge of health personnel regarding the association of hypoglycaemia with the KCNQ1 mutation in order to identify and treat it early.<a class="elsevierStyleCrossRef" href="#bib0035">3</a>" "pdfFichero" => "main.pdf" "tienePdf" => true "NotaPie" => array:1 [ 0 => array:2 [ "etiqueta" => "☆" "nota" => "Please cite this article as: Salamanca Zarzuela B, Alcalde Martín C, Centeno Malfaz F. Hipoglucemia secundaria a hiperinsulinismo en un paciente con síndrome de QT largo congénito. Med Clin (Barc). 2020;154:415–416." ] ] "bibliografia" => array:2 [ "titulo" => "References" "seccion" => array:1 [ 0 => array:2 [ "identificador" => "bibs0015" "bibliografiaReferencia" => array:4 [ 0 => array:3 [ "identificador" => "bib0025" "etiqueta" => "1" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Clínica y genética en el síndrome de QT largo" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:3 [ 0 => "A. Medeiros-Domingo" 1 => "P. Iturralde-Torres" 2 => "M.J. 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Ackerman" ] ] ] ] ] "host" => array:1 [ 0 => array:1 [ "Revista" => array:5 [ "tituloSerie" => "Hear Rhythm" "fecha" => "2017" "volumen" => "12" "paginaInicial" => "1815" "paginaFinal" => "1819" ] ] ] ] ] ] 3 => array:3 [ "identificador" => "bib0040" "etiqueta" => "4" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "KCNQ1 long QT syndrome patients have hyperinsulinemia and symptomatic hypoglycemia" "autores" => array:1 [ 0 => array:2 [ "etal" => true "autores" => array:6 [ 0 => "S.S. Torekov" 1 => "E. Iepsen" 2 => "M. Christiansen" 3 => "A. Linneberg" 4 => "O. Pedersen" 5 => "J.J. 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Vol. 154. Issue 10.

Pages 415-416 (May 2020)

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