Características neuropsicológicas del niño preescolar con hipotiroidismo congénito en la Provincia de Cienfuegos / Neuropsychological characteristic of preschooler children’s with congenital hypothyroidism in Provincia de Cienfuegos

El hipotiroidismo congénito es la causa más frecuente de retraso mental previsible. El diagnóstico clínico en las primeras etapas de la vida es muy impreciso, de ahí que se hayan puesto en marcha programas de diagnóstico y tratamiento precoz cuyo objetivo fundamental es un desarrollo neurocognitivo y su nivel optimización. Método y objetivos: Con el objetivo de evaluar el perfil neurocognitivo en niños hipotiroideos en edad preescolar se realizó un estudio descriptivo prospectivo que incluyó a los pacientes en edades entre 4 a 6 años con seguimiento en consulta de hipotiroidismo congénito del Hospital Pediátrico “Paquito González Cueto” de Cienfuegos. Solo existían 6 niños a los cuales se les aplicó la batería neuropsicológica Luria Inicial, rendimiento intelectual, escala de comportamiento y potenciales evocados. Resultados: Se constató que estos pacientes presentan limitaciones en el sistema óptico- espacial y en el sistema de control inhibitorio. El primero afecta el desarrollo de la orientación espacial del acto motor y la percepción visual con detalles y el otro sistema afecta el control atencional en la edad y conlleva a dificultades para seguir instrucciones verbales. Recomendación: El seguimiento y la vigilancia del neurodesarrollo permiten ir explicando las alteraciones sistemáticas que se presentan en estos niños y hace posible una intervención temprana a favor del posterior proceso de escolarización.

The congenital hipotiroidismo is the most frequent cause of foreseeable mental delay. The clinical diagnosis in the first stages of the life is very vague, for that reason diagnostic programs and precocious treatment have started up whose main target is a neurocognitivo development and its level optimization. Method and objectives: With the aim of evaluating the neurocognitivo profile in hypothyroid children in pre-school age a prospective descriptive study was realised that it included to the patients in ages between 4 to 6 years with pursuit in consultation of congenital hipotiroidismo of the Paediatric Hospital “Paquito González Fortified height” of Cienfuegos. 6 children only existed to who he was applied the neuropsicológica battery Initial Luria, intellectual yield, evoked scale of behavior and potentials to them. Results: It was stated that these patients present/display limitations in the space optical system and in the inhibiting control system. First it affects the development of the spatial orientation of the motor act and the visual perception with details and the other system affects the attention control in the age and entails to difficulties to follow instructions verbal. Recommendation: The pursuit and the monitoring of neurodesarrollo allow to be explaining the systematic alterations that appear in these children and makes an early intervention in favor of the later process possible of schooling.

Hipotireoidismo neonatal transitório causado pelo uso de amiodarona durante a gestação: relato de dois casos e revisão da literatura / Transient neonatal hypothyroidism due to amiodarone administration during pregnancy: two cases report and review of literature

INTRODUÇÃO: A amiodarona (AMD) é uma droga antiarrítmica que contém 37 por cento de iodo. A AMD pode alcançar o feto por via transplacentária e causar hipotireoidismo congênito (HC) ou transitório (HCT). Relatamos dois casos de HCT em virtude de exposição gestacional à AMD, detectados pelo programa de triagem neonatal para HC no Estado do Paraná, Brasil. CASO CLÍNICO 1 (C1): TSH neonatal 78,2 mU/L (normal < 15 mU/L). A AMD foi utilizada durante toda a gestação em virtude de arritmia materna. As dosagens séricas iniciais confirmaram o HC; e na primeira consulta [aos 14 dias de vida (dv)], foi iniciada levotiroxina (L-T4), 50 µg/dia. CASO CLÍNICO 2 (C2): TSH neonatal 134 mU/L. A AMD foi utilizada no último trimestre da gestação em virtude de arritmia materna. As dosagens séricas iniciais confirmaram o HC; aos 13 dv, foi iniciada L-T4 50 µg/dia. ACOMPANHAMENTO: TSH e T4 estavam normais aos 51 dv (C1) e aos 36 dv (C2) sendo então gradativamente reduzida a dose da medicação e suspensa aos 16 meses (C1) e aos dez meses (C2). As pacientes foram acompanhadas até 22 meses (C1) e 16 meses (C2) com testes de função tireoidiana normais. O crescimento e o desenvolvimento neuropsicomotor (DNPM), avaliados pelo teste CAT/CLAMS, eram normais. CONCLUSÃO: As avaliações da função tireoidiana e do DNPM são necessários quando a AMD é utilizada na gestação. O tratamento do HCT deve ser instituído tão logo o diagnóstico seja realizado.

INTRODUCTION: Amiodarone (AMD) is an antiarrhythmic agent which contains 37 percent of iodine. It can reach the fetus by transplacental passage and induce transient congenital hypothyroidism (TCH). We report two cases of TCH caused by gestational exposure to AMD, detected by the Newborn Screening Program for Congenital Hypothyroidism of the State of Paraná - Brazil. CLINICAL CASE 1 (C1): Neonatal TSH value was 78,2mU/L (normal < 15 mU/L). AMD had been given to the mother during pregnancy to treat maternal arrhythmia. The screening results were confirmed by serum thyroid function tests. Levothyroxin (L-T4) (50µg/day) was started on the first visit, on the 14th day of life (dl). CLINICAL CASE 2 (C2): Neonatal TSH value was 134,0 mU/L. AMD had been given to the mother in the third trimester of pregnancy to treat maternal arrhythmia. The screening results were confirmed by serum thyroid function tests: L-T4 (50µg/day) was started on the first visit, with 13 dl. FOLLOW-UP: TSH and T4 normalized on 51 dl (C1) and 36 dl (C2); L-T4 could be diminished gradually and stopped within 16 months (C1) and 10 months (C2). They were followed-up until 22 months (C1) and 16 months (C2) with normal thyroid function tests. Their growth and mental development, evaluated by the Cognitive Adaptive Test/Clinical Linguistic & Auditory Milestone Scale (CAT/CLAMS test), were normal. CONCLUSION: Evaluation of thyroid function and mental development should be performed if AMD is used during pregnancy. Treatment of TCH must be started as soon as the diagnosis is made.