Cerebral Cortical Thickness Morphometry and Neurocognitive Correlations in Adolescents With Congenital Hypothyroidism.

CONTEXT: Subtle cognitive impairments have been described in children with congenital hypothyroidism (CH) detected by neonatal screening (NS), even with early and adequate treatment. Patients with CH may present with brain cortical thickness (CT) abnormalities, which may be associated with neurocognitive impairments. OBJECTIVE: This work aimed to evaluate the CT in adolescents with CH detected by the NS Program (Paraná, Brazil), and to correlate possible abnormalities with cognitive level and variables of neurocognitive prognosis. METHODS: A review was conducted of medical records followed by psychometric evaluation of adolescents with CH. Brain magnetic resonance imaging with analysis of 33 brain areas of each hemisphere was performed in 41 patients (29 girls) and in a control group of 20 healthy adolescents. CT values were correlated with Full-scale Intelligence Quotient (FSIQ) scores, age at start of treatment, pretreatment thyroxine levels, and maternal schooling. RESULTS: No significant difference in CT between patients and controls were found. However, there was a trend toward thinning in the right lateral orbitofrontal cortex among patients and in the right postcentral gyrus cortex among controls. CT correlated significantly with FSIQ scores and with age at start of treatment in 1 area, and with hypothyroidism severity in 5 brain areas. Maternal schooling level did not correlate with CT but was significantly correlated with FSIQ. Cognitive level was within average in 44.7% of patients (13.2% had intellectual deficiency). CONCLUSION: There was a trend toward morphometric alterations in the cerebral cortex of adolescents with CH compared with healthy controls. The correlations between CT and variables of neurocognitive prognosis emphasize the influence of hypothyroidism on cortical development. Socioeconomic status exerts a limiting factor on cognitive outcome.

Cognitive outcome of 458 children over 25 years of neonatal screening for congenital hypothyroidism.

OBJECTIVES: To describe the neurocognitive profile of 458 children with congenital hypothyroidism detected by neonatal screening, followed under the same treatment protocol over 25 years. To correlate estimated full-scale IQ (FSIQ) scores with age at the start of treatment, disease severity, and maternal education. METHODS: Observational, analytical, retrospective, and longitudinal cohort study, that evaluated children detected between 1991 and 2014, who underwent at least one psychometric assessment (WPPSI- R and/or WISC-III). Estimated FSIQ scores are described and correlated with prognosis determinants. RESULTS: Median T4 at diagnosis was 2.8 µg/dL (0.0-16.5), the median age at the start of treatment was 18.5 days (3-309). Maternal education (n = 445): 2.7% of illiteracy, 59.8% with basic education. Estimated FSIQ scores were 88.0 (±11.8) in WPPSI-R (age 5.6 ± 0.5 years) and 84.1 (±13.0) in WISC-III (age 9.1 ± 1.4 years). The intellectual deficit was identified in 11.6%. Correlation between age at the start of treatment and estimated FSIQ was found only in the WPPSI-R test (p = 0.02). Initial T4 and maternal education significantly correlated with estimated FSIQ scores in both tests, with the latter being the most important determining factor. CONCLUSIONS: In this large cohort of mainly low socioeconomic status children, most children achieved normal cognitive levels; however, a significant percentage presented with below-average estimated FSIQ scores and intellectual deficits. Maternal education was the main determining factor in cognitive level followed by hypothyroidism severity.

Cognitive outcome of 458 children over 25 years of neonatal screening for congenital hypothyroidism

Abstract Objectives: To describe the neurocognitive profile of 458 children with congenital hypothyroidism detected by neonatal screening, followed under the same treatment protocol over 25 years. To correlate estimated full-scale IQ (FSIQ) scores with age at the start of treatment, disease severity, and maternal education. Methods: Observational, analytical, retrospective, and longitudinal cohort study, that evaluated children detected between 1991 and 2014, who underwent at least one psychometric assessment (WPPSI- R and/or WISC-III). Estimated FSIQ scores are described and correlated with prognosis determinants. Results: Median T4 at diagnosis was 2.8 µg/dL (0.0-16.5), the median age at the start of treatment was 18.5 days (3-309). Maternal education (n = 445): 2.7% of illiteracy, 59.8% with basic education. Estimated FSIQ scores were 88.0 (±11.8) in WPPSI-R (age 5.6 ± 0.5 years) and 84.1 (±13.0) in WISC-III (age 9.1 ± 1.4 years). The intellectual deficit was identified in 11.6%. Correlation between age at the start of treatment and estimated FSIQ was found only in the WPPSI-R test (p = 0.02). Initial T4 and maternal education significantly correlated with estimated FSIQ scores in both tests, with the latter being the most important determining factor. Conclusions: In this large cohort of mainly low socioeconomic status children, most children achieved normal cognitive levels; however, a significant percentage presented with below-average estimated FSIQ scores and intellectual deficits. Maternal education was the main determining factor in cognitive level followed by hypothyroidism severity.

Grupo de apoio para pacientes e familiares do serviço de transplante de medula óssea / Support group for patients and families at the bone marrow transplantation unit

Esse artigo é um relato da experiência resultante das reflexões pertinentes e aprendizagens originárias nos grupos de pacientes adultos e familiares do ambulatório de Transplante de Medula Óssea. OBJETIVO: Constituir um grupo informativo-reflexivo na sala de espera. MÉTODO: Encontros semanais de uma hora sob a coordenação da psicóloga são ofertados para aqueles que demonstram interesse em trocar experiências e dúvidas. O grupo é aberto, tem novos e antigos participantes. RESULTADO: Os participantes apresentaram preocupações, complacência, medos, fé, alegrias, tristezas nas perdas, ameaças reais e imaginárias. A espera de um doador é dolorosa e incerta e relataram dificuldades em relação ao filho adolescente que cresceu, mas requer vigilância constante. CONCLUSÃO: O grupo funciona como espaço continente das vivências propiciando resultados favoráveis em relação ao bem-estar dos participantes. Pensar sobre as ações e reações perante as adversidades da vida contribui para o aproveitamento dos recursos internos e o enfrentamento dos adventos oriundos do binômio doença-saúde(AU)

This article is an account of the experience obtained from gathering the learning and pertinent reflections originated in the groups of adult patients and family members at the Bone Marrow Transplantation Ambulatory Care Unit. OBJECTIVE: To constitute a reflexive-informative group in the waiting room. METHOD: A weekly group of one-hour meetings under the coordination of a psychologist offered to those who demonstrate interest in exchanging experiences and questions. The group is open to new participants. RESULT: The participants presented worries, complacency, fear, faith, and joy, longing, real and imaginary threats. The wait for a donor is painful and uncertain and it were reported difficulties about the adolescent son who has grown up, but requires constant surveillance. CONCLUSION: The group works as a space for exchange of experiences, improving the well-being of patients. Thinking about the actions and reactions facing the adversities of life contributes to the efficient use of internal resources and helps facing the difficulties arising from the illness-health binomial(AU)

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

INTRODUCTION: Amiodarone (AMD) is an antiarrhythmic agent which contains 37% 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 78.2 mU/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) (50 microg/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 (50 microg/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.

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.