Skeletal involvement in Gaucher disease: extent of bone disease, splenic volume, and quality of life / Envolvimento ósseo na doença de Gaucher: extensão do acometimento ósseo, volume esplênico e qualidade de vida

Abstract Objective: To investigate the correlations among the extent of bone involvement, splenic volume, and quality of life in patients with Gaucher disease. Materials and Methods: This was a descriptive, prospective cross-sectional study of 18 patients with Gaucher disease who underwent 3-T magnetic resonance imaging of both femurs and the lumbar spine. Semiquantitative analyses were performed on the basis of the bone marrow burden (BMB) score. We looked for linear relationships among the variables splenic volume, quality of life score, and BMB score. Results: We identified a linear relationship between the BMB scores and splenic volume. The quality of life score showed no statistically significant relationship with splenic volume or the BMB score. Conclusion: The linear relationship between the BMB score and the splenic volume indicates that the extent of bone disease is greater in individuals with splenomegaly. No correlation was found between the BMB and quality of life scores, illustrating the insidious and silent progression of Gaucher disease.

Resumo Objetivo: Investigar a correlação entre a extensão do envolvimento ósseo, o volume esplênico e a qualidade de vida em pacientes com doença de Gaucher. Materiais e Métodos: Estudo descritivo, prospectivo e transversal de 18 pacientes com doença de Gaucher submetidos a ressonância magnética de 3-T de ambos os fêmures e da coluna lombar. Análise semiquantitativa foi feita utilizando o escore bone marrow burden (BMB). Correlação linear foi estudada para as variáveis volume esplênico, qualidade de vida e escore BMB. Resultados: Uma correlação linear entre os escores BMB e volume esplênico foi demonstrada. Em relação ao índice de qualidade de vida, não foi observada correlação estatisticamente significante nem com o volume esplênico e nem com o escore BMB. Conclusão: Nosso estudo demonstrou uma correlação linear entre o escore BMB e o volume esplênico, correspondendo a maior extensão de doença óssea em indivíduos com maior esplenomegalia. Nenhuma correlação foi encontrada entre o escore BMB e a qualidade de vida, indicando a natureza insidiosa e a progressão silenciosa da doença de Gaucher.

Aspiration pneumonia in children: an iconographic essay / Pneumonia por aspiração na infância: ensaio iconográfico

Abstract In most cases of aspiration pneumonia in children, the disease is specific to this age group. Clinical and radiological correlation is essential for the diagnosis. The present pictorial essay is aimed at showing typical images of the most common etiologies.

Resumo A maioria das pneumonias por aspiração na infância é específica deste grupo etário. Para diagnosticá-las é essencial a correlação clinicorradiológica. O objetivo deste ensaio é mostrar imagens típicas das causas mais frequentes.

Magnetic resonance imaging and BMB score in the evaluation of bone involvement in Gaucher’s disease patients / Ressonância magnética e o escore BMB na avaliação do acometimento ósseo em pacientes com doença de Gaucher

AbstractObjective:To evaluate by magnetic resonance imaging changes in bone marrow of patients undergoing treatment for type I Gaucher’s disease.Materials and Methods:Descriptive, cross-sectional study of Gaucher’s disease patients submitted to 3 T magnetic resonance imaging of femurs and lumbar spine. The images were blindly reviewed and the findings were classified according to the semiquantitative bone marrow burden (BMB) scoring system.Results:All of the seven evaluated patients (three men and four women) presented signs of bone marrow infiltration. Osteonecrosis of the femoral head was found in three patients, Erlenmeyer flask deformity in five, and no patient had vertebral body collapse. The mean BMB score was 11, ranging from 9 to 14.Conclusion:Magnetic resonance imaging is currently the method of choice for assessing bone involvement in Gaucher’s disease in adults due to its high sensitivity to detect both focal and diffuse bone marrow changes, and the BMB score is a simplified method for semiquantitative analysis, without depending on advanced sequences or sophisticated hardware, allowing for the classification of the disease extent and assisting in the treatment monitoring.

ResumoObjetivo:Avaliar, por meio de ressonância magnética, alterações da medular óssea de pacientes em tratamento para doença de Gaucher tipo I.Materiais e Métodos:Estudo descritivo e transversal de pacientes com doença de Gaucher tipo I, com realização de ressonância magnética de 3 T da coluna lombar e fêmures, analisada por meio do escore semiquantitativo denominado bone marrow burden(BMB), sem conhecimento das informações clínicas.Resultados:Dos sete pacientes avaliados (três homens e quatro mulheres), todos apresentaram sinais de infiltração da medula óssea. Osteonecrose da cabeça femoral foi encontrada em três pacientes, deformidade em frasco de Erlenmeyer em cinco e nenhum paciente apresentou colapso de corpo vertebral. A pontuação média do escore BMB total foi 11, variando de 9 a 14.Conclusão:A ressonância magnética é, atualmente, o método de escolha em adultos para avaliar o envolvimento ósseo na doença de Gaucher, em virtude da sua elevada sensibilidade na detecção de alterações tanto focais quanto difusas da medula óssea, sendo o escore BMB um método simplificado de análise semiquantitativa, sem depender de sequências avançadas ou de hardware sofisticado, permitindo classificar a extensão do acometimento da doença e ajudando no monitoramento do tratamento.

Comparison of clinical and laboratory characteristics between children and adults with dengue

Over the past several years, the epidemiological profile of dengue has been changing progressively and is currently characterized by an increase in the number of cases in children under 15 years of age. This study was aimed at comparing the clinical and laboratory features between adults and children with dengue; therefore, we performed a cross-sectional analysis of 5686 individuals with laboratory-confirmed dengue who sought treatment at a healthcare facility in Rio de Janeiro, Brazil from 2010 to 2011. A multivariate analysis indicated that myalgia (OR = 2.58; CI 95% = 2.08-3.18), retro-orbital pain (OR = 1.36; CI 95% = 1.15-1.62), nausea (OR = 1.92; CI 95% = 1.60-2.30), and arthralgia (OR = 3.64; CI 95% = 2.72-4.89) were the most frequent clinical symptoms in adults, whereas vomiting (OR = 0.52; CI 95% = 0.43-0.61) and skin rash (OR = 0.46; CI 95% = 0.25-0.85) were the most prevalent symptoms in children. Adults exhibited a higher hemoconcentration (OR = 3.04; CI 95% = 2.53-3.65), thrombocytopenia (OR = 2.17; CI 95% = 1.80-2.60), increased erythrocyte sedimentation rate (OR = 1.81; CI 95% = 1.53-2.14), and increased ALT (OR = 3.13; CI 95% = 2.44-4.02) than did children. In addition, adults exhibited a higher frequency of the severe forms of the disease (OR = 1.74; CI 95% = 1.12-2.72) and hospitalization (OR = 2.21; CI 95% = 1.59-3.06) relative to children. Based on these results, this study demonstrated significant differences in the clinical and laboratory presentations and disease severity between adults and children affected by dengue.