We report the long-term clinical results of conservative management of children with unilateral multicystic dysplastic kidneys (MCDK). Between 1989 and 2002, 43 children with MCDK detected by prenatal ultrasonography were prospectively followed. At birth, ultrasonography confirmed the prenatal findings in all cases. Patients underwent a radioisotope scan and micturating cystogram in order to confirm the diagnosis and to exclude other uropathies. Follow-up ultrasound (US) examinations were performed at 6-month intervals during the first 2 years of life and yearly thereafter. The mean follow-up time was 42 months (range 12-156 months). Two children developed hypertension during follow-up. In total 257 US scans were performed. The mean number of US scans per patient was 6 (range 3-10). US scans demonstrated partial involution of the MCDK in 30 (70%) cases and complete involution in 8 (19%). The absolute MCDK length remained almost unchanged in 5 children (11%). The estimated median time of complete involution of the MCDK was 122 months [95% confidence interval (CI)=86-158 months]. A total of 33 (76.7%) contralateral kidneys underwent compensatory hypertrophy, reaching a renal length above the 95th percentile during follow-up. The estimated median time for the occurrence of compensatory hypertrophy was 30 months (95% CI=15-45 months). In conclusion, the natural history of MCDK is usually benign but patients must have long-term follow-up with US scans and blood pressure measurements.

The objective of the work was to prepare an update on imaging methods for bone evaluation during childhood and adolescence. The text was based on original and review articles on imaging methods for clinical evaluation of bone mass in children and adolescents up to 20 years old. They were selected from BIREME and PUBMED by means of the following keywords: bone density; osteoporosis/diagnosis; densitometry; tomography; ultrasonography; magnetic resonance imaging; and radiogrammetry and published in Portuguese or English, in the last 10 years (2006-2016). The article was organized into topics with the description of peculiarities, advantages and disadvantages of each imaging method and their possible clinical applicability. Despite the emergence of new technologies, dual energy X-ray absorptiometry (DXA) remains the gold standard method for low bone mass diagnosis in all age groups. However, interpretation is complex in children and adolescents and demands skilled people. Quantitative computed tomography (QCT) [central QCT, peripheral QCT (pQCT) and high resolution-pQCT (HR-pQCT)] and magnetic resonance imaging (MRI) evaluate real bone density, but are not yet available for routine use. Quantitative bone ultrasound (QUS) shows good perspectives for its use in primary prevention actions. Automated radiogrammetry shows promise as a method able to flag individuals who might benefit from a complete bone assessment, but the clinical value of the measures still needs to be established.

Métodos: A frequência das manifestações clínicas de hipotireoidismo congênito foi analisada em relação aos níveis de hormônio estimulante da tireoide T4L (determinados por quimioluminescência, valores de referência de 0,3 a 5,0 µUI/mL e 0,8 a 1,8 ng/dL, respectivamente) e idades ao diagnóstico e tratamento. Estudou-se 443 crianças, sendo 55,8% do sexo feminino e 95% delas com menos 60 dias de vida. Resultados:Os sinais clínicos mais prevalentes foram: hérnia umbilical (51%), fontanela anterior ampla (50,3%) e fontanela posterior aberta (47,2%). Hipotonia, macroglossia e sucção débil foram os sinais mais associados com a gravidade bioquímica da doença. Em 32,1% das crianças houve atraso na idade óssea ao diagnóstico. As medianas de TSH e T4L séricos foram 120 µUI/mL e 0,62 ng/dL, respectivamente. A mediana da idade de início de tratamento foi de 28 dias. Conclusão:Existem sinais clínicos precoces que sugerem o diagnóstico de hipotireoidismo congênito. Portanto, diante de uma criança com esses sinais, devem ser avaliados TSH e T4L séricos para confirmar ou excluir a doença, independentemente do resultado do teste de triagem. A idade de início de tratamento ainda foi elevada, mas o programa vem adotando estratégias para redução da mesma. Methods: Analysis of factors involved in this profile, including:TSH and FT4 levels (determined by chemiluminescence, with limits of normality set at 0.3-5.0 µUI/mL and 0.8-1.8 ng/dL, respectively), age at diagnosis and age at treatment. The study sample consisted of 443 children, 55.8% were female and 95% were seen before completing 60 days of life. Results:The most prevalent clinical signals were: umbilical hernia (51%), enlarged anterior fontanel (50.3%), and open posterior fontanel (47.2%). Hypotonia, macroglossia and feeding difficulties were the clinical signs most frequently associated with the biochemical severity of the disease. A delay in bone age was present in 32.1% of the children at diagnosis. The median of serum TSH and FT4 was 120 µUI/mL and 0.62 ng/dL, respectively. The median age at start of treatment was 28 days. Conclusion:There are some early clinical signs that suggest a diagnosis of congenital hypothyroidism. Therefore, when presented with a child exhibiting these signs, serum TSH and FT4 should be assayed in order to confirm or rule out the disease, irrespective of the result of screening. Age at start of treatment remains high, but strategies are being implemented to reduce it. J Pediatr (Rio J)

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