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.

MANAGEMENT OF ENDOCRINE DISEASE: Growth and growth hormone therapy in short children born preterm.

Approximately 15 million babies are born preterm across the world every year, with less than 37 completed weeks of gestation. Survival rates increased during the last decades with the improvement of neonatal care. With premature birth, babies are deprived of the intense intrauterine growth phase, and postnatal growth failure might occur. Some children born prematurely will remain short at later ages and adult life. The risk of short stature increases if the child is also born small for gestational age. In this review, the effects of being born preterm on childhood growth and adult height and the hormonal abnormalities possibly associated with growth restriction are discussed, followed by a review of current information on growth hormone treatment for those who remain with short stature during infancy and childhood.

Endovascular Therapeutic Occlusion of the Posterior Cerebral Artery: An Option for Ruptured Giant Aneurysm in a Child.

The incidence of intracranial aneurysms in the pediatric population is low, and surgical clipping remains a good long-term treatment option. However, posterior circulation aneurysms are even more complex to manage in children than in adults. We report a case of a giant aneurysm of the posterior cerebral artery in a 10-year-old boy presenting with subarachnoid hemorrhage. Endovascular treatment with platinum coils was performed with total occlusion of the aneurysm and the affected arterial segment without complications. The patient achieved good recovery, and a late control angiogram confirmed exclusion of the aneurysm. Occurrence of special features of cerebral aneurysm in children, in comparison to adults, is also described. Parent artery sacrifice is an effective therapeutic option, but long-term follow-up is necessary to avoid recurrence and rebleeding.

Crescimento de crianças nascidas prematuras / Growth of preterm-born children

Crianças nascidas prematuras podem passar por um período de restrição do crescimento logo após o nascimento. A normalização do crescimento tem início nos primeiros meses de vida, podendo ocorrer de forma lenta e progressiva. Muitas vezes essas crianças mantêm-se mais baixas e com menor peso durante toda a infância quando comparadas àquelas nascidas a termo. Em alguns casos, a recuperação completa só ocorre na adolescência. Entretanto, algumas crianças não conseguem recuperar totalmente o ganho de peso e altura, e adultos nascidos prematuros apresentam maior risco de baixa estatura. O comprometimento do crescimento é mais significativo naquelas nascidas prematuras e pequenas para a idade gestacional. Fatores como estatura-alvo, peso ao nascimento, idade gestacional, intercorrências neonatais e escolaridade materna interferem no potencial de crescimento. Especial atenção deve ser dada aos nascidos prematuros durante todo o período de crescimento.

Children born prematurely might experience a period of growth restriction just after birth. Catch-up growth begins during the first months of life and can be slow and progressive. These children may remain shorter and thinner throughout infancy and childhood compared to children born at term. In some cases, complete catch-up growth occurs only during adolescence. However, some children do not completely recover growth, and adults born prematurely are at increased risk of short stature. Impaired growth is more frequent in those born preterm and small for gestational age. Factors such as target height, birth weight, gestational age, neonatal morbidities and maternal education interfere in growth potential. Special attention should be given to children born preterm during the whole growth period.

[Growth of preterm-born children]. / Crescimento de crianças nascidas prematuras.

Children born prematurely might experience a period of growth restriction just after birth. Catch-up growth begins during the first months of life and can be slow and progressive. These children may remain shorter and thinner throughout infancy and childhood compared to children born at term. In some cases, complete catch-up growth occurs only during adolescence. However, some children do not completely recover growth, and adults born prematurely are at increased risk of short stature. Impaired growth is more frequent in those born preterm and small for gestational age. Factors such as target height, birth weight, gestational age, neonatal morbidities and maternal education interfere in growth potential. Special attention should be given to children born preterm during the whole growth period.

Prevalência de obesidade em crianças e adolescentes com diabetes melito tipo 1 / Prevalence of obesity in children and adolescents with type 1 diabetes mellitus

OBJETIVO: Conhecer a prevalência de obesidade e sua distribuição por sexo e idade em pacientes com diabetes melito tipo 1 (DM1), em acompanhamento em Serviço de Endocrinologia Pediátrica. MÉTODOS: Estudo transversal descritivo de pacientes em acompanhamento no Serviço de Endocrinologia Pediátrica do Hospital de Base de São José do Rio Preto, avaliando as seguintes variáveis: sexo, idade, dose e número de aplicações diárias de insulina, hemoglobina glicada (HbA1c), peso, estatura e índice de massa corpórea (IMC). A definição de sobrepeso e obesidade baseou-se nas curvas de IMC para a idade do Centers for Disease Control and Prevention (CDC, 2000). RESULTADOS: Foram incluídos 77 portadores de DM1 (44 meninas e 33 meninos) com idade entre três e 18 anos (média: 13,4). A determinação de HbA1c variou de 4,1 a 10,6 por cento (média: 7,7 por cento) e o IMC, de 15,1 a 28,6 kg/m² (média: 23,2). Conforme critérios do CDC, 8 por cento dos pacientes (três meninas e três meninos) apresentavam obesidade e 8 por cento (duas meninas e quatro meninos), sobrepeso. CONCLUSÕES: Nesta casuística de pacientes diabéticos, a prevalência de sobrepeso e obesidade não se mostrou diferente de populações não diabéticas.

OBJECTIVE: To describe the prevalence of obesity according to gender and age in patients with type 1 diabetes mellitus of a pediatric endocrinology follow-up clinic. METHODS: This cross-sectional descriptive study enrolled patients of the follow-up clinic of the Pediatric Endocrinology Unit of Hospital de Base of São José do Rio Preto, São Paulo, Brazil. The following data were collected: gender, age, insulin dosage, glycated hemoglobin (HbA1c), weight, height and body mass index (BMI). The cutoff criteria for overweight and obesity were based on BMI-for-age growth charts of the Centers for Disease Control and Prevention (CDC, 2000). RESULTS: 77 patients with type 1 diabetes mellitus were included (44 females and 33 males), with three to 18 years old (mean: 13.4). The value of HbA1c varied from 4.1 to 10.6 percent (mean: 7.7 percent) and the BMI ranged from 15.1 to 28.6 kg/m² (average: 23.2). Based on BMI charts, 8 percent (three girls and three boys) were obese and 8 percent (two girls and four boys) had overweight. CONCLUSION: Compared to the prevalence of obesity and overweight in non-diabetic pediatric population, the presence of these nutritional disturbances in the studied children was not significantly different.