Neonatal screening improves sickle cell disease clinical outcome in Belgium. (June 2018)

• Record Type: Journal Article
• Authors: Lê, Phu-Quoc; Ferster, Alina; Dedeken, Laurence; Vermylen, Christiane; Vanderfaeillie, Anna; Rozen, Laurence; Heijmans, Catherine; Huybrechts, Sophie; Devalck, Christine; Cotton, Frédéric; Ketelslegers, Olivier; Dresse, Marie-Françoise; Fils, Jean-François; Gulbis, Béatrice
• Is Part Of: Journal of medical screening. Volume 25:Number 2(2018)
• Page Start: 57
• Page End: 63
• Publication Date: 2018-06
• Subjects: Sickle cell disease -- neonatal screening programme -- infection -- hospitalization; Medical screening -- Periodicals; 362.177
• DOI: 10.1177/0969141317701166
• Languages: English
• ISSNs: 0969-1413

Abstract

Objectives: To compare the outcomes of sickle cell disease patients diagnosed through neonatal screening with those who were not. Methods: In an observational multicenter study in Belgium, 167 screened and 93 unscreened sickle cell disease patients were analyzed for a total of 1116 and 958 patient-years of follow-up, respectively. Both groups were compared with propensity score analysis, with patients matched on three covariates (gender, genotype, and central Africa origin). Bonferroni correction was applied for all comparisons. Results: Kaplan–Meier estimates of survival without bacteremia were significantly higher in the screened group than the unscreened group (94.47%; [95% CI, 88.64–97.36%] versus 83.78% [95% CI, 72.27–90.42%]), p = 0.032. Non-significant differences between both groups were reported for survival without acute chest syndrome, acute anemia, cerebral complication, severe infection, and vaso-occlusive crisis. Significantly lower hospitalization rate and days per 100 patient-years were observed in the screened compared with the unscreened group (0.27 vs. 0.63 and 1.25 vs. 2.82, p = 0.0006 and <0.0001). Conclusion: These data confirm the benefit of a neonatal screening programme in reducing bacteremia and hospitalization.