ABSTRACT
A study was conducted to assess and compare the knowledge of blood transfusion practices among medical students and residents in Lebanese and Saudi medical institutions. The online survey consisted of 26 questions: 4 about personal data and experience with transfusion and 22 about knowledge on transfusion practices in the areas of blood donation and donor selection, production and storage of blood components, selection of appropriate blood components, administration of blood components, transfusion reactions, and complications. One hundred and twenty-six students from Saudi Arabia, 84 students from Lebanon, 31 residents from Saudi Arabia, and 23 residents from Lebanon participated in the survey. There were no significant differences between students' and residents' levels of knowledge. Similarly, there was no difference between the students' level of knowledge in the two countries. The correct responses (48% and 46%, for students and residents, respectively) were below the acceptable limit of 60% for both groups. This reflects the need for more vigorous and well-structured education and training for both students and residents.
ABSTRACT
Diamond-Blackfan anaemia (DBA), first described over 80 years ago, is a congenital disorder of erythropoiesis with a predilection for birth defects and cancer. Despite scientific advances, this chronic, debilitating, and life-limiting disorder continues to cause a substantial physical, psychological, and financial toll on patients and their families. The highly complex medical needs of affected patients require specialised expertise and multidisciplinary care. However, gaps remain in effectively bridging scientific discoveries to clinical practice and disseminating the latest knowledge and best practices to providers. Following the publication of the first international consensus in 2008, advances in our understanding of the genetics, natural history, and clinical management of DBA have strongly supported the need for new consensus recommendations. In 2014 in Freiburg, Germany, a panel of 53 experts including clinicians, diagnosticians, and researchers from 27 countries convened. With support from patient advocates, the panel met repeatedly over subsequent years, engaging in ongoing discussions. These meetings led to the development of new consensus recommendations in 2024, replacing the previous guidelines. To account for the diverse phenotypes including presentation without anaemia, the panel agreed to adopt the term DBA syndrome. We propose new simplified diagnostic criteria, describe the genetics of DBA syndrome and its phenocopies, and introduce major changes in therapeutic standards. These changes include lowering the prednisone maintenance dose to maximum 0·3 mg/kg per day, raising the pre-transfusion haemoglobin to 9-10 g/dL independent of age, recommending early aggressive chelation, broadening indications for haematopoietic stem-cell transplantation, and recommending systematic clinical surveillance including early colorectal cancer screening. In summary, the current practice guidelines standardise the diagnostics, treatment, and long-term surveillance of patients with DBA syndrome of all ages worldwide.