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Neonatal screening improves sickle cell disease clinical outcome in belgium show less

par 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 Françoise;Gulbis, Béatrice
Référence Journal of medical screening, 25, 2, page (57-63)
Publication Publié, 2018

Article révisé par les pairs

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Résumé :

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, p¼0.0006 and <0.0001). Conclusion: These data confirm the benefit of a neonatal screening programme in reducing bacteremia and hospitalization.