Factors associated with blood pressure variation in sickle cell disease patients: a systematic review and meta-analyses.

OBJECTIVES: Blood pressure (BP) values ≥120/70 mmHg considerably increase the risk of pulmonary hypertension and renal dysfunction in Sickle Cell Disease (CSD) patients and ultimately increased morbidity and mortality. This has led to the development of the term relative systemic hypertension (RSH). RSH was defined as Systolic BP 120-139 mm Hg or diastolic BP 70-89 mm Hg, whereas systemic hypertension is defined as Systolic BP ≥ 140 mm Hg or diastolic BP ≥ 90 mm Hg. Systematic identification of BP variations and risk factors in SCD patients could promote effective management. This review aimed to identify factors associated with BP variation among SCD patients. METHODS: We searched PubMed, Scopus, Web of Science, and Google Scholar up to December 2020 with no geographical or language restrictions. Two reviewers independently screened and summarized data from eligible studies. RESULTS: Advancing age, gender, higher body weight, hemoglobin, eGFR, triglycerides, greater hematocrit, higher blood viscosity, history of blood transfusion, and targeted variants in DRD2 and MIR4301 genes were independently associated with the risk of hypertension in SCD patients. CONCLUSION: Interventions that consider these risk factors may potentially contribute to lower BP pressure in SCD patients and prevent the development of severe complications.

The Sickle Cell Disease Ontology: Enabling Collaborative Research and Co-Designing of New Planetary Health Applications.

Sickle cell disease (SCD) is one of the most common blood disorders impacting planetary health. Over 300,000 newborns are diagnosed with SCD each year globally, with an increasing trend. The sickle cell disease ontology (SCDO) is the most comprehensive multidisciplinary SCD knowledge portal. The SCDO was collaboratively developed by the SCDO working group, which includes experts in SCD and data standards from across the globe. This expert review presents highlights and lessons learned from the fourth SCDO workshop that marked the beginning of applications toward planetary health impact, and with an eye to empower and cultivate multisite SCD collaborative research. The workshop was organized by the Sickle Africa Data Coordinating Center (SADaCC) and attended by 44 participants from 14 countries, with 2 participants connecting remotely. Notably, from the standpoint of democratizing and innovating scientific meeting design, an SCD patient advocate also presented at the workshop, giving a broader real-life perspective on patients' aspirations, needs, and challenges. A major component of the workshop was new approaches to harness SCDO to harmonize data elements used by different studies. This was facilitated by a web-based platform onto which participants uploaded data elements from previous or ongoing SCD-relevant research studies before the workshop, making multisite collaborative research studies based on existing SCD data possible, including multisite cohort, SCD global clinical trials, and SCD community engagement approaches. Trainees presented proposals for systematic literature reviews in key SCD research areas. This expert review emphasizes potential and prospects of SCDO-enabled data standards and harmonization to facilitate large-scale global SCD collaborative initiatives. As the fields of public and global health continue to broaden toward planetary health, the SCDO is well poised to play a prominent role to decipher SCD pathophysiology further, and co-design diagnostics and therapeutics innovation in the field.