BackgroundMaternal and Perinatal Death Surveillance and Review (MPDSR) can reduce mortality but its implementation is often suboptimal, especially in low- and middle-income countries (LMICs)... Show moreBackgroundMaternal and Perinatal Death Surveillance and Review (MPDSR) can reduce mortality but its implementation is often suboptimal, especially in low- and middle-income countries (LMICs).ObjectivesTo understand the determinants of behaviors influencing implementation of MPDSR in LMICs (through a systematic review of qualitative studies), in order to plan an intervention to improve its implementation.Search StrategyTerms for maternal or perinatal death reviews and qualitative studies.Selection CriteriaQualitative studies regarding implementation of MPDSR in LMICs.Data Collection and AnalysisWe coded the included studies using the Theoretical Domains Framework and COM-B model of behavior change (Capability, Opportunity, Motivation). We developed guiding principles for interventions to improve implementation of MPDSR.Main ResultsFifty-nine studies met our inclusion criteria. Capabilities required to conduct MPDSR (knowledge and technical/leadership skills) increase cumulatively from community to health facility and leadership levels. Physical and social opportunities depend on adequate data, human and financial resources, and a blame-free environment. All stakeholders were motivated to avoid negative consequences (blame, litigation, disciplinary action).ConclusionsImplementation of MPDSR could be improved by (1) introducing structural changes to reduce negative consequences, (2) strengthening data collection tools and information systems, (3) mobilizing adequate resources, and (4) building capabilities of all stakeholders.Implementation of MPDSR could be improved by introducing changes to reduce negative consequences, strengthening data collection tools, mobilizing resources, and building capabilities of all stakeholders. Show less
Purpose Deficiency of adenosine deaminase 2 (DADA2) is an inherited inborn error of immunity, characterized by autoinflammation (recurrent fever), vasculopathy (livedo racemosa, polyarteritis... Show morePurpose Deficiency of adenosine deaminase 2 (DADA2) is an inherited inborn error of immunity, characterized by autoinflammation (recurrent fever), vasculopathy (livedo racemosa, polyarteritis nodosa, lacunar ischemic strokes, and intracranial hemorrhages), immunodeficiency, lymphoproliferation, immune cytopenias, and bone marrow failure (BMF). Tumor necrosis factor (TNF-alpha) blockade is the treatment of choice for the vasculopathy, but often fails to reverse refractory cytopenia. We aimed to study the outcome of hematopoietic cell transplantation (HCT) in patients with DADA2. Methods We conducted a retrospective study on the outcome of HCT in patients with DADA2. The primary outcome was overall survival (OS). Results Thirty DADA2 patients from 12 countries received a total of 38 HCTs. The indications for HCT were BMF, immune cytopenia, malignancy, or immunodeficiency. Median age at HCT was 9 years (range: 2-28 years). The conditioning regimens for the final transplants were myeloablative (n = 20), reduced intensity (n = 8), or non-myeloablative (n = 2). Donors were HLA-matched related (n = 4), HLA-matched unrelated (n = 16), HLA-haploidentical (n = 2), or HLA-mismatched unrelated (n = 8). After a median follow-up of 2 years (range: 0.5-16 years), 2-year OS was 97%, and 2-year GvHD-free relapse-free survival was 73%. The hematological and immunological phenotypes resolved, and there were no new vascular events. Plasma ADA2 enzyme activity normalized in 16/17 patients tested. Six patients required more than one HCT. Conclusion HCT was an effective treatment for DADA2, successfully reversing the refractory cytopenia, as well as the vasculopathy and immunodeficiency. Clinical Implications HCT is a definitive cure for DADA2 with > 95% survival. Show less
