The earliest symptom of glomerular injury in patients with sickle cell disease (SCD) is microalbuminuria. The effect of hydroxyurea (HU) on urine albumin-to-creatinine ratio (ACR) is unclear and should be determined, because increasing numbers of patients with SCD take this drug to improve red blood cell function. In this cohort study of 58 SS-homozygous adults with SCD who initiated HU therapy, we evaluated ACR changes and relationships of these changes with demographic, clinical, and biologic parameters at HU initiation (baseline) and 6 months later (follow-up). Between baseline and follow-up, ACR declined significantly for the entire population (3.0-1.7 mg/mmol; P<0.01), but this was primarily driven by the ACR reduction in the microalbuminuria subgroup (8.1-2.3 mg/mmol; P=0.03; n=23). According to bivariate analyses on 39 patients who did not receive a blood transfusion during the study period, the baseline to follow-up ACR decline was strongly associated with decreases in levels of hemolysis markers, percentage of dense red blood cells, and systolic BP. Bivariate analysis also revealed a close association between the ACR decrease and high baseline levels of hemolysis markers and percentage of dense red blood cells. These results show that urine ACR decreased significantly after 6 months of HU and confirm a close relationship between ACR and hemolysis evolution in patients with SCD.
Download full-text PDF |
Source |
---|---|
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4884099 | PMC |
http://dx.doi.org/10.1681/ASN.2014111126 | DOI Listing |
Pediatr Blood Cancer
January 2025
Blood and Marrow Transplant/Cellular Therapy Program, Division of Hematology/Oncology, The Hospital for Sick Children, Toronto, Ontario, Canada.
With advances in conditioning strategies and graft-versus-host disease (GvHD) prevention, hematopoietic stem cell transplantation (HSCT) is a safe, curative treatment option for pediatric patients with sickle cell disease (SCD). However, donor options have been limited in non-myeloablative matched sibling donor (MSD) setting by excluding recipients with major ABO blood group incompatible donors due to concern of the risk of significant complications such as pure red cell aplasia (PRCA). We present three cases of successful HSCT with major ABO incompatibility with their donors, and discuss strategies to safely expand the donor pool to include these donors.
View Article and Find Full Text PDFInt J Nurs Stud
December 2024
Service de Médecine Interne, Centre National de Référence des Syndromes Drépanocytaires Majeurs de l'Adulte, AP-HP, Hôpital Européen Georges Pompidou, Assistance Publique-Hôpitaux de Paris, 20 rue Leblanc, F-75908 Paris cedex 15, France; Service d'aval des urgences, hopital Henri-Mondor Assistance Publique-Hôpitaux de Paris, 1 rue Gustave Eiffeil, 94000 Créteil, France; Université Paris Cité, Paris, France. Electronic address:
Background: Intravenous (IV) access is often required for the treatment of vaso-occlusive crises in patients with sickle cell disease, but can be particularly challenging due to recurrent venous damage. The AccuVein® device, uses near-infrared light technology to visualise veins for easier venepuncture.
Methods: A randomised, controlled trial of the efficacy of the AccuVeinAV400® device in the replacement of peripheral venous lines during a vaso-occlusive crisis was conducted at two centres in France.
Rheumatoid arthritis (RA) is rarely reported among patients with sickle cell disease (SCD). RA treatment in these patients is believed to be more challenging due to fear of increasing the risk of infection and complications of SCD. We are reporting 7 patients with concurrent SCD and RA.
View Article and Find Full Text PDFExpert Rev Respir Med
January 2025
Department of Surgery, Dentistry, Pediatrics and Gynaecology, Pediatric Division, University of Verona, Verona, Italy.
Introduction: Sickle cell disease (SCD) is an inherited hemoglobinopathy characterized by the production of sickle hemoglobin, leading to red blood cells sickling and hemolysis in hypoxic conditions. The resulting acute and chronic endothelial inflammation leads to chronic organ damage. Respiratory manifestations in SCD usually start from childhood and represent the leading causes of morbidity and mortality.
View Article and Find Full Text PDFRev Med Interne
January 2025
Service de médecine interne, centre national de référence des syndromes drépanocytaires majeurs de l'adulte, hôpital européen Georges-Pompidou, AP-HP, 20, rue Leblanc, 75015 Paris, France; Inserm U1163, laboratoire « Mécanismes cellulaires et moléculaires des désordres hématologiques et implications thérapeutiques », institut Imagine, université Paris-Cité, 75015 Paris, France; Laboratoire d'Excellence GR-Ex, 75015 Paris, France; Faculté de santé, université Paris-Cité, 75006 Paris, France. Electronic address:
Introduction: Extramedullary hematopoiesis (EMH) is very rarely described during sickle cell disease (SCD). Our aim was to describe six cases of EMH occurring in adult SCD patients and to conduct a literature review.
Methods: Retrospective, descriptive, and monocentric study, identifying all cases of EMH recorded in our cohort of adult SCD patients, up to April 2024.
Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!