RESUMO
A 68-year-old man diagnosed with Erdheim-Chester disease presented to the emergency department with shortness of breath of one-day duration. Upon presentation, the patient was dyspnoeic and hypoxemic. The initial laboratory workup showed raised inflammation markers, and a chest x-ray showed the presence of bilateral lung infiltrates; therefore, he was managed for community-acquired pneumonia with antimicrobial and other supportive measures. Due to lack of improvement, he had transthoracic echocardiography (ECHO), which showed a large pericardial effusion without tamponade. He was treated with corticosteroids and underwent pericardiocentesis, which resulted in remarkable symptomatic improvement. This case presents a serious manifestation of a rare disease and summarizes treatment options from the literature.
RESUMO
PURPOSE: As treatment options for children with sickle cell anemia (SCA) continue to expand survival, evaluation of factors associated with health-related quality of life (HRQoL) is becoming an important aspect for further improving clinical management. Although the general features of SCA are similar, factors influencing HRQoL within a country may differ from those of other countries, therefore this study aimed to explore factors affecting HRQoL in children with SCA living in the Sultanate of Oman. METHODS: This was a cross-sectional study in which the PedsQL™ Sickle Cell Disease Module was used to evaluate the overall HRQoL in children with SCA. The socio-demographic data, clinical, and treatment outcomes were collected. Univariate and multivariate linear regression analyses were used to identify predictors of HRQoL. RESULTS: A total of 123 children with SCA, aged from 2 to 16 years were enrolled. The mean total HRQoL score was 52 ± 15% (9-94), where Worry II scale recorded the highest score. The multiple regression analysis revealed that the only predictors of total HRQoL score were hemoglobin F (B = 0.64, 95% confidence interval [CI] 0.149-1.118, P = 0.009) and to a lesser degree white blood cell count (B = - 0.99, 95% CI - 1.761 to - 0.198, P = 0.01), independently of other study parameters such as age, gender, spleen status, and hydroxyurea therapy. CONCLUSIONS: Collectively, these findings indicated that hemoglobin F out-weighted white blood cell count in predicting HRQoL in Omani children with SCA. Recognition of these factors could help health professionals to develop effective strategies to improve the overall HRQoL in these young patients.
