Objective: to describe clinical, laboratory and ultrasonographic abnormalities in the hepatobiliary system of pediatric patients with sickle cell disease in the city of Salvador, Brazil.
Material And Methods: pediatric patients with sickle cell disease were clinically evaluated, their charts were reviewed and findings of supplementary tests were examined to identify hepatobiliary abnormalities.
Results: a total of 134 patients were evaluated, 65 of whom (48.9%) presented hepatomegalia. Elevated transaminases were present in 42.2% and 11.4% presented cholelithiasis. There was a statistically significant association between the presence of hepatomegalia and SS homozygotes and between cholelithiasis and patients over 10 years of age (p = 0.01 and p = 0.00, respectively).
Conclusion: hepatobiliary abnormalities in patients with sickle cell disease were common, particularly in patients with hemoglobin SS and in adolescent patients.
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BMJ Glob Health
January 2025
Sickle Cell Programme, Department of Haematology and Blood Transfusion, Muhimbili University of Health and Allied Sciences, Dar es Salaam, Tanzania.
Despite progress in healthcare services for individuals living with sickle cell disease (SCD) in Africa, substantial gaps remain in advanced treatments for SCD. To help address this burden, Tanzania has established one of the largest single-centre SCD programmes in the world and developed an advanced therapy programme for SCD focused on patient engagement and advocacy, clinical activities involving exchange blood transfusion (ExBT) and haematopoietic stem cell transplant (HSCT), gene therapy (GT) preparedness, and enabling partnerships. This report describes the programme's genesis, structure and progress achieved.
View Article and Find Full Text PDFBlood
January 2025
New York Blood Center, New York, New York, United States.
Babesiosis in sickle cell disease (SCD) is marked by severe anemia but the underlying red blood cell (RBC) rheological parameters remain largely undefined. Here, we describe altered RBC deformability from both primary (host RBC sickle hemoglobin mediated) and secondary changes (Babesia parasite infection mediated) to the RBC membrane using wild type AA, sickle trait AS and sickle SS RBCs. Our ektacytometry (LORRCA) analysis demonstrates that the changes in the host RBC bio-mechanical properties, pre- and post- Babesia infection, reside on a spectrum of severity, with wild type infected AA cells, despite showing a significant reduction of deformability under both shear and osmolarity gradients, exhibiting only a mild phenotype; compared to infected AS RBCs which show median changes in deformability and infected SS RBCs which exhibit the most dramatic impact of infection on cellular rheology, including an increase in Point of Sickling values.
View Article and Find Full Text PDFPak J Med Sci
January 2025
Khalid Khalil Security Forces Hospital Makkah, Makkah, Saudi Arabia.
Objective: To observe the fetomaternal outcome of therapeutic versus prophylactic blood transfusions in patients with sickle cell disease (SCD) during pregnancy.
Method: This single-center retrospective observational study was conducted on consecutive pregnant women with SCD between January 2018 and December 2020. All the pregnant women with SCD were included in this study.
Non-myeloablative hematopoietic cell transplantation (HCT) is a curative option for individuals with sickle cell disease (SCD). Our traditional goal with this approach has been to achieve a state of mixed donor/recipient chimerism. Recently, we reported an increased risk of hematologic malignancies (HMs) in adults with SCD following graft failure or mixed chimerism.
View Article and Find Full Text PDFCureus
December 2024
Internal Medicine, King Fahad Hospital Hofuf, Hofuf, SAU.
Sickle cell anemia (SCA) is one of the known hemoglobinopathies that result in red blood cell (RBC) destruction, among other complications. There are factors that make SCA an environment for autoimmune disease (AID). They include chronic inflammation, immune-mediated processes involved in SCA complications, and susceptibility to infections.
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