Erdheim-Chester Disease Successfully Treated with Front-Line Single-Agent Dabrafenib.

BACKGROUND Erdheim-Chester disease (ECD) is a clonal disease characterized by histiocytic infiltration of multiple organ systems. As ECD is a rare disorder with variable presentations, its diagnosis and management can present a significant clinical challenge. The diagnosis of ECD requires several clinical, radiological, and histological criteria. Since approximately 75% of ECD patients harbor a mutation in the proto-oncogene BRAF V600E, inhibition of BRAF activation by BRAF inhibitors has significantly improved the management of ECD. Vemurafenib was approved by the U.S. Food and Drug administration for treatment of BRAF-mutated ECD. Another BRAF inhibitor, dabrafenib, has been used in some cases as a single agent and was associated with a lower toxicity profile. CASE REPORT We report the case of a 30-year-old Saudi Arabian woman who initially presented with a history of diffuse abdominal pain and fever. The patient had elevated inflammatory markers, and radiological investigations revealed hypermetabolic regions in the frontoparietal brain lobe, anterior pericardium, kidneys, and the anterior abdominal wall. Histological investigations from the right perinephric soft-tissue mass revealed foamy histiocytes associated with mild chronic inflammation. Furthermore, BRAF V600E was mutated in the biopsy sample, leading to a diagnosis of BRAF-mutated ECD. The patient began single-agent dabrafenib therapy at 75 mg twice daily and experienced an excellent clinical and radiological response with no reported toxicity. CONCLUSIONS Single-agent dabrafenib is effective and well tolerated among ECD patients; therefore, it might be considered as a first-line option for the treatment of BRAF-mutated ECD.

Obstacles faced by healthcare providers during COVID-19 pandemic in Sudan.

The impacts of COVID -19 pandemic have been quite significant on healthcare providers. I was particularly challenging for those in Low and Middle-Income Countries including Sudan . Unfortunately, the pandemic has hit Sudan on extremely difficult time for the country and its people. The country was coming out of long-brutal and devastating dictatorship and transitioning to new democracy with civilian leadership. In addition to the pandemic related issues, trying to rebuild the health system during socioeconomic crisis, healthcare providers  in the country were challenged personally and professionally. These challenges include the stress of working in under-resourced settings with limited access to personal-protection equipment and testing kits raised the fear of contracting the virus and spreading it to their families. The professional, social, and personal life of healthcare providers have been dramatically changed by the ongoing pandemic, however, they are heroically accepting this change in a hope that, this will save the life of many more people. Nevertheless, their fights and sacrifices should at least be rewarded by governments and communities altogether strictly enforce the implementation of other preventive measures including vaccination, face masking, and social distancing and get all protected. We should all understand that, unless we are all protected no one is protected, so all must adapt to the new norm of life and collaborate not only on ending this pandemic but to prevent similar ones in the future.