Taquicardia paroxística supraventricular en lactantes. A propósito de un caso / No disponible

La taquicardia paroxística supraventricular (TPSV) es la arritmia más frecuente en Pediatríay constituye una urgencia médica. Se presenta en un 0,1-0,4% de la población pediátrica,la mayoría de ocasiones como hallazgo casual o palpitaciones, siendo bien toleraday en ocasiones de resolución espontánea.No obstante, suele requerir ingreso hospitalario preferentemente en una unidad de cuidadosintensivos (UCI), ya que puede desencadenar arritmias malignas, insuficiencia cardiacay miocardiopatía dilatada, sobre todo en lactantes. La evolución a muerte oscila del 1%en pacientes con cardiopatía al 0,25% en los pacientes sin cardiopatía asociada.El tratamiento basado en la realización de maniobras vagales y la administración deadenosina suele ser efectivo aunque recientemente ha venido constatándose la escasa eficaciade las dosis bajas de ATP (50-100 μg/kg), imperando una revisión de los protocolosactuales.En ocasiones, la primera asistencia es prestada en centros de Atención Primaria, inclusocentros rurales con accesibilidad limitada a un centro hospitalario. A nuestro entenderes básico que el médico de Atención Primaria conozca el manejo de esta patología ytambién que Enfermería sea adecuadamente entrenada, sobre todo en la obtención devías venosas periféricas con el fin de prestar una adecuada asistencia al enfermo, más aúnsi no es posible organizar el traslado medicalizado del paciente en un lapso de tiempo razonable.Es nuestra intención llamar la atención sobre dicha patología y con ayuda de un casoclínico centrar los aspectos básicos de su diagnóstico y manejo terapéutico, especialmenteen lactantes pequeños(AU)

Paroxistical supraventricular tachycardia is the most frequent arrhythmia in Pediatrics and itis a medical emergency. It happens in 0.1-0.4% of the pediatric population, mostly as an accidentalfinding or palpitations, it is well tolerated and sometimes with a spontaneous resolution.Nevertheless, it frequently requires admission preferably in an intensive care unit(UCI), since it can be followed by malignant arrhythmias, cardiac insufficiency and dilatedmyocardiopathy, especially in infants. The evolution to death goes from 1% in patientswith cardiopathy to 0,25% in patients without cardiopathy.The treatment based on vagal stimulation manoeuvres and Adenosine (ATP) is usuallysuccessful, although recently has been communicated the low effectiveness of low doses ofATP (50-100 Ìg/kg), making necessary a review of current protocols.Occasionally, the first assistance is given at primary care centres, even at rural medicalcentres. So we think that it is basic that primary care doctors knew about this pathology andalso that nurses must be trained in periferical venous access, especially if the patient cannotbe moved to a reference centre in a brief time.We wish to draw the attention to this condition and with the help of a clinical case toshow the basics of its diagnosis and treatment, especially in infants(AU)

[Pharyngeal-cervical-brachial variant of Guillain-Barré syndrome: a well-defined clinical condition with a heterogeneous immunological profile]. / Variante faringocervicobraquial del síndrome de Guillain-Barré: una entidad clínica bien definida con un perfil inmunológico heterogéneo.

INTRODUCTION: The pharyngeal-cervical-brachial variant of Guillain-Barre syndrome (GBS) is rare. This variant has its own specific clinical aspects but a heterogeneous immunological profile. CASE REPORT: A 38-year-old male who presented progressive symptoms of dysphagia, dysphonia and weakness hindering movement of the upper limbs. Two weeks earlier, the patient had presented acute self-limiting diarrhoea. He displayed predominantly right-side bilateral peripheral facial paresis, and paresis of the 9th and 12th cranial nerves and upper limbs (proximal 0/5, distal 1/5), although strength in the lower limbs was not compromised; sensitivity was preserved and deep tendon reflexes were diminished (0 in the upper limbs and + in the lower extremities). At 24 hours after admission, he suffered severe respiratory distress and had to be moved to the Intensive Care Unit with invasive mechanical ventilation. An electronystagmography/electromyogram study revealed severe demyelinating damage that predominantly involved the brain, but also included a small axonal component. The most striking immunological finding was the presence of positive IgG anti-GQ1b, IgM anti-GMI and IgM anti-asialo GM1 titres. CONCLUSIONS: The pharyngeal-cervical-brachial variant is a clinical condition with its own clinical characteristics and well-established diagnostic criteria that allow it to be distinguished from the other variants of GBS. Our case highlights the wide clinical spectrum of acute inflammatory demyelinating polyradiculoneuropathies and the important degree of heterogeneity that exists as regards the immunological parameters.

Variante faringocervicobraquial del síndrome de Guillain-Barré: una entidad clínica bien definida con un perfil inmunológico heterogéneo / Pharyngel-cervical-brachial variant of guillain-barre syndrome: A well-defined clinical condition with a heterogeneous immunological profile

Introducción. Una variante poco frecuente del síndrome de Guillain-Barré (SGB) es la faringocervicobraquial. Dicha variante posee aspectos clínicos específicos, pero un perfil inmunológico heterogéneo. Caso clínico. Varón de 38 años de edad que presentó cuadro progresivo de disfagia, disfonía y debilidad para movilizar las extremidades superiores. Dos semanas antes, presentó un cuadro diarreico autolimitado. Mostraba paresia facial periférica bilateral de predominio derecho, y paresia de los IX y XII pares craneales y de miembros superiores (proximal 0/5, distal 1/5), sin afectación de la fuerza en miembros inferiores, sensibilidad conservada y reflejos osteotendinosos disminuidos (0 en miembros superiores y + en inferiores). A las 24 horas de ingreso sufrió deterioro respiratorio grave, que requirió traslado a la Unidad de Cuidados Intensivoscon ventilación mecánica invasiva. La electronis-tagmografía/electromiograma mostró un grave daño desmielinizante de predominio cefálico y cierto componente axonal. Inmunológicamente destacó la presencia de títulos positivos de IgG anti-GQ1b, IgM anti-GM1 e IgM anti-asialo GM1. Conclusiones. La variante faringocervicobraquial es una entidad clínica con características clínicas propias y criterios diagnósticos establecidos que permiten distinguirla de las otras variantes del SGB. Nuestro caso pone de relieve el amplio espectro clínico de las polirradiculoneuropatías desmielinizantes inflamatorias agudas y la gran heterogeneidad existente respecto a parámetros inmunológicos

Introduction. The pharyngeal-cervical-brachial variant of Guillain-Barré syndrome (GBS) is rare. This variant has its own specific clinical aspects but a heterogeneous immunological profile. Case report. A 38-year-old male who presented progressive symptoms of dysphagia, dysphonia and weakness hindering movement of the upper limbs. Two weeks earlier, the patient had presented acute self-limiting diarrhoea. He displayed predominantly right-side bilateral peripheral facial paresis, and paresis of the 9th and 12th cranial nerves and upper limbs (proximal 0/5, distal 1/5), although strength in the lower limbs was not compromised; sensitivity was preserved and deep tendon reflexes were diminished (0 in the upper limbs and + in thelower extremities). At 24 hours after admission, he suffered severe respiratory distress and had to be moved to the Intensive Care Unit with invasive mechanical ventilation. An electronystagmography/electromyogram study revealed severe demyelinating damage that predominantly involved the brain, but also included a small axonal component. The most striking immunologicalfinding was the presence of positive IgG anti-GQ1b, IgM anti-GMI and IgM anti-asialo GM1 titres. Conclusions. Thepharyngeal-cervical-brachial variant is a clinical condition with its own clinical characteristics and well-established diagnostic criteria that allow it to be distinguished from the other variants of GBS. Our case highlights the wide clinical spectrum of acute inflammatory demyelinating polyradiculoneuropathies and the important degree of heterogeneity that existsas regards the immunological parameters