Updates on the role of epigenetics in familial mediterranean fever (FMF).

Familial Mediterranean Fever (FMF) is an autosomal recessive autoinflammatory disease caused by mutations in the MEFV (MEditerranean FeVer) gene that affects people originating from the Mediterranean Sea. The high variability in severity and clinical manifestations observed not only between ethnic groups but also between and within families is mainly related to MEFV allelic heterogeneity and to some modifying genes. In addition to the genetic factors underlying FMF, the environment plays a significant role in the development and manifestation of this disease through various epigenetic mechanisms, including DNA methylation, histone modification, and noncoding RNAs. Indeed, epigenetic events have been identified as an important pathophysiological determinant of FMF and co-factors shaping the clinical picture and outcome of the disease. Therefore, it is essential to better understand the contribution of epigenetic factors to autoinflammatory diseases, namely, FMF, to improve disease prognosis and potentially develop effective targeted therapies. In this review, we highlight the latest updates on the role of epigenetics in FMF.

Hospitalization Events among Children and Adolescents with Sickle Cell Disease in Basra, Iraq.

Objectives. Despite improvements in the management of sickle cell disease (SCD), many patients still experience disease-related complications requiring hospitalizations. The objectives of this study were to identify causes of hospitalization among these patients and factors associated with the length of hospital stay (LOS) and readmission. Methods. Data from 160 patients (<14 years old) with SCD who were admitted to the Basra Maternity and Children's Hospital from the first of January 2012 through July 2012 were analyzed. Results. The main causes of hospitalization were acute painful crises (73.84%), infections (9.28%), acute chest syndrome (8.02%), and acute splenic sequestration crisis (6.32%). The mean LOS was 4.34 ± 2.85 days. The LOS for patients on hydroxyurea (3.41 ± 2.64 days) was shorter than that for patients who were not (4.59 ± 2.86 days), P < 0.05. The readmission rate (23.1%) was significantly higher among patients with frequent hospitalizations in the previous year (OR 9.352, 95% CI 2.011-43.49), asthma symptoms (OR 4.225, 95% CI 1.125-15.862), and opioid use (OR 6.588, 95% CI 1.104-30.336). Patients on hydroxyurea were less likely to be readmitted (OR 0.082, 95% CI 0.10-0.663). Conclusions. There is a relatively high readmission rate among patients with SCD in Basra. The use of hydroxyurea significantly decreases the LOS and readmission rate.