MicroRNA expression patterns in HbE/ß-thalassemia patients: The passwords to unlock fetal hemoglobin expression in ß-hemoglobinopathies.

Hemoglobin E (HbE)/ß-thalassemia is a form of ß-hemoglobinopathy that is well-known for its clinical heterogeneity. Individuals suffering from this condition are often found to exhibit increased fetal hemoglobin (HbF) levels - a factor that may contribute to their reduced blood transfusion requirements. This study hypothesized that the high HbF levels in HbE/ß-thalassemia individuals may be guided by microRNAs and explored their involvement in the disease pathophysiology. The miRNA expression profile of hematopoietic progenitor cells in HbE/ß-thalassemia patients was investigated and compared with that of healthy controls. Using miRNA PCR array experiments, eight miRNAs (hsa-miR-146a-5p, hsa-miR-146b-5p, hsa-miR-148b-3p, hsa-miR-155-5p, hsa-miR-192-5p, hsa-miR-335-5p, hsa-miR-7-5p, hsa-miR-98-5p) were identified to be significantly up-regulated whereas four miRNAs (hsa-let-7a-5p, hsa-miR-320a, hsa-let-7b-5p, hsa-miR-92a-3p) were significantly down-regulated. Target analysis found them to be associated with several biological processes and molecular functions including MAPK and HIF-1 signaling pathways - the pathways known to be associated with HbF upregulation. Results of dysregulated miRNAs further indicated that miR-17/92 cluster might be of critical importance in HbF regulation. The findings of our study thus identify key miRNAs that can be extrinsically manipulated to elevate HbF levels in ß-hemoglobinopathies.

Mediastinal Pseudocyst and Cardiac Tamponade Due to Massive Pericardial Effusion in Pediatric Chronic Calcific Pancreatitis.

A 7-year-old male patient with a history of recurrent abdominal pain over 1 year presented with cardiac tamponade due to massive pericardial effusion, which was percutaneously drained. Contrast-enhanced computed tomography revealed a large posterior mediastinal cyst and calcified, heterogeneous pancreatic parenchyma. Elevated amylase and lipase levels of the cyst fluid confirmed the diagnosis of pancreatic pseudocyst, which was treated with an octreotide infusion and Roux-en-Y cystojejunostomy. The child was discharged on pancreatic enzyme supplement and was asymptomatic on follow-up.