Management of Glanzmann's Thrombasthenia – Guidelines based on an expert panel consensus from gulf cooperation council countries

Glanzmann thrombasthenia is a rare disorder, due to quantitative and/or qualitative abnormalities of the platelet integrin αIIbβ3 and/or αIIbβ3. Although it is considered a rare disorder with a global incidence of 1/1,000,000 population, the case is different at the Gulf Cooperation Council countries, where prevalence rate as high as 1/40,000 in Madinah, Saudi Arabia. This makes it necessary to develop patient's management guidelines. Due to limited resources in the literature, experts' consensus was important to develop such guidelines. Experts panel elected to use recombinant activated factor VII (rFVIIa) as the first line of treatment of acute bleeds and reserve platelets transfusion for nonresponding patients or severe bleeds, rFVIIa at high dose (270 μg/kg body weight) may tried upfront. rFVIIa may be tried as prophylactic treatment in patients with frequents bleeds. Experts panel elected to allow girls with Glanzmann thrombasthenia to have menstruation and to adapt a special protocol for this purpose (Madinah protocol). Pregnancy should be managed carefully, where normal delivery encouraged under coverage of rFVIIa. Risk of bleeding should be expected in neonates. Minor surgeries and dental workup can be managed under coverage of rFVIIa. Glanzmann thrombasthenia patients with severe disease should be encouraged for hematopoietic stem cell transplant

Knowledge and response of the community to premarital screening program (Sickle Cell Anemia\Thalassemia); AlMadinah, Saudi Arabia

BACKGROUND: Premarital screening (PMS) is a mandatory laboratory examination before marriage to identify genetic blood and some infectious diseases. Sickle cell anemia (SCA) and thalassemia are genetic disorders caused by errors in the hemoglobin genes and are prevalent in Saudi Arabia OBJECTIVE: This study assessed the knowledge and response of the Al-Madinah community regarding PMS program. METHODOLOGY: This cross-sectional study assessed the knowledge and attitude of Al-Madinah community regarding hereditary blood disorders. Thirty-seven interviewers conducted direct and electronic interviews from October 2016 to January 2017. RESULTS: The total number of respondents was 2554, among the participants, 61% noted that PMS can diagnose SCA, 5.3% noted that PMS cannot diagnose SCA, and 33.4% answered “I don't know.” Regarding thalassemia, 50.2% answered that PMS can diagnose thalassemia, 7.4% answered “no,” and 42% answered “I don't know.” Furthermore, 76.4% of the participants answered that PMS can prevent SCA, whereas 71% of the participants answered that PMS can prevent thalassemia. Approximately 88.6% of the participants agreed to undergo elective PMS, 78.2% of them will not marry someone who has SCA or thalassemia, and 79.5% of them will not marry someone with a genetic trait if they themselves have a trait. In terms of raising the community's awareness on various hereditary blood disorders, 95.9%, 93.9%, and 92.5% agreed on the importance of media, medical education in school, and seminars, respectively. CONCLUSION: As half of the population were unaware about the screened diseases, it is necessary to raise the community's awareness on the importance of PMS