An international survey to inform priorities for new guidelines on von Willebrand disease.

INTRODUCTION: von Willebrand disease (VWD) is an inherited bleeding disorder caused by a quantitative or qualitative dysfunction of von Willebrand factor. Clinicians, patients and other stakeholders have many questions about the diagnosis and management of the disease. AIM: To identify topics of highest importance to stakeholders that could be addressed by guidelines to be developed by the American Society of Hematology (ASH), the International Society on Thrombosis and Haemostasis (ISTH), the National Hemophilia Foundation (NHF) and the World Federation of Hemophilia (WFH). METHODS: A survey to determine and prioritize topics to be addressed in the collaborative development of guidelines for VWD was distributed to international stakeholders including patients, caregivers and healthcare providers (HCPs). Representatives of the four organizations coordinated the distribution strategy. The survey focused on both diagnosis and management of VWD, soliciting 7-point Likert-scale responses and open-ended comments, in English, French and Spanish. We conducted descriptive analysis with comparison of results by stakeholder type, gender and countries' income classification for the rating questions and qualitative conventional content data analysis for the open-ended responses. RESULTS: A total of 601 participants responded to the survey (49% patients/caregivers and 51% healthcare providers). The highest priority topics identified were diagnostic criteria/classification, bleeding assessment tools and treatment options for women and surgical patients. In contrast, screening for anaemia and differentiating plasma-derived therapy versus recombinant therapies received lower ratings. CONCLUSION: This survey highlighted areas of importance to a diverse representation of stakeholders in the diagnosis and management of VWD, providing a framework for future guideline development and implementation.

Risk factors and prevention strategies for diabetic ketoacidosis in people with established type 1 diabetes.

Diabetic ketoacidosis (DKA) is a serious acute complication of type 1 diabetes, which is receiving more attention given the increased DKA risk associated with SGLT inhibitors. Sociodemographic and modifiable risk factors were identified with strong evidence for an increased risk of DKA, including socioeconomic disadvantage, adolescent age (13-25 years), female sex, high HbA1c, previous DKA, and psychiatric comorbidities (eg, eating disorders and depression). Possible prevention strategies, which include the identification of people at risk based on non-modifiable sociodemographic risk factors, are proposed. As a second risk mitigation strategy, structured diabetes self-management education that addresses modifiable risk factors can be used. Evidence has found that structured education leads to reduced DKA rates. Knowledge of these risk factors and potent risk mitigation strategies are important to identify subgroups of people with an elevated DKA risk. This knowledge should also be used when adjunct therapy options with an increased DKA risk are considered. Prevention of DKA in people with type 1 diabetes is an important clinical task, which should also be addressed when SGLT inhibitors are part of therapy.

Hepatitis B care pathway in Jordan: current situation, gaps and recommended actions.

OBJECTIVES: Hepatitis B virus (HBV) infection remains a worldwide public health problem. The last major review of the HBV care pathway in the Middle East was published in 2011. This article aims to assess the current situation of the HBV care pathway in Jordan, identify gaps and barriers and recommend initiatives that could be taken to improve disease management across all stages of the care pathway. METHODS: A Delphi process was used between February and October 2017, including a literature review (PubMed and Google Scholar) for published articles on HBV in Jordan, followed by individual interviews with various healthcare professionals and group discussions with leading Jordanian hepatologists and gastroenterologists to provide context to the results of the literature review. RESULTS AND DISCUSSION: The national prevalence of HBV is estimated to be around 2.4% and has declined from 9.9% in the pre-vaccination era. There is limited data on the burden of HBV-related hepatocellular carcinoma in the country. Awareness of various aspects of the disease is considered low among the general population and medium among primary care providers. There are several mandated national screening structures, however, established protocols for those that test positive and subsequent linkage-to-care are not optimal. In conclusion, significant improvements have been made in the past 30 years in Jordan in terms of decline in HBV prevalence. However, efforts must be made by all key stakeholders to optimise screening and linkage-to-care of patients and reduce both morbidity and mortality in the country.