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.
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http://dx.doi.org/10.1177/0969141317701166 | DOI Listing |
Am J Hematol
January 2025
Department of Woman's and Child's Health, University of Padova, Padova, Italy.
J Clin Apher
February 2025
Department of Laboratory Medicine and Pathology, Mayo Clinic Arizona, Phoenix, Arizona, USA.
Pediatr Blood Cancer
January 2025
Department of Audiology and Speech Therapy, Universidade Federal de São Paulo, Sao Paulo, Brazil.
Introduction: Sickle cell anemia has a genetic origin characterized by an autosomal recessive inheritance pattern. The nervous system may be subject to vaso-occlusion and, consequently, affect the proper functioning of the central portion of hearing.
Objective: To assess central auditory skills and analyze short- and long-latency auditory evoked potentials in children with sickle cell disease.
Neurol Sci
January 2025
Hematology Unit, Careggi University Hospital, Florence, Italy.
Background: The coexistence of sickle cell anemia and multiple sclerosis in a single patient presents a rare and challenging clinical scenario, possibly favoured by the interplay between chronic inflammatory states and autoimmune processes.
Methos/results: We present the case of a 36-year-old woman with sickle cell anemia who developed progressive neurological symptoms leading to frequent falls and paraparesis; magnetic resonance imaging showed many periventricular, infratentorial, and both cervical and dorsal spinal cord lesions, leading to a diagnosis of multiple sclerosis. After a multidisciplinary approach the patient was successfully started on ofatumumab.
J Nurs Adm
January 2025
Author Affiliations: Nursing Research Consultant (Dr Feetham), Nurse Scientist, and Associate Professor, George Washington University School of Medicine, Washington, DC (Dr Kelly), Nursing Research and Development Programs Manager (Dr Engh), Department Nursing Science, Professional Practice Quality, Director Healthcare Consulting CBRE Washington DC (Dr Frame): Chief Nursing Informatics and Education Officer (Dr King), Nurse Practitioner, Psychiatry Consult Liaison Service (Dr Ojini), Division of Emergency Medicine and Trauma Nursing Director (Dr Schultz), Sickle Cell Disease Lead Translation Research Advanced Practice Nurse and Director of the Sickle Cell Disease Transition Clinic, Associate Professor George Washington University (Dr Barbara Speller-Brown), and Simulation Program Manager (Dr Walsh), Children's National Hospital, Washington, DC; and Assistant Professor (Dr Giordano), Nell Hodgson Woodruff School of Nursing, Emory University, Atlanta, Georgia.
Advancing nursing practice to improve care and system outcomes requires doctoral-prepared nurses to conduct programs of research and translate science to practice. The authors describe a Doctoral support group (DSG) at one hospital designed to support nurses considering and navigating doctoral education while continuing as hospital employees. Strategies from 18 years' experience are provided for others to develop and sustain a DSG as part of an environment to support and retain nurses with doctoral degrees.
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