Objectives: Tanzania has the third highest birth rate of sickle cell anaemia (SCA) in Africa, but few studies describe severity of complications or available treatments, especially in Northwest Tanzania around Lake Victoria where the sickle gene is most prevalent. This is a report of the spectrum of clinical disease and range of interventions available at Bugando Medical Centre (Bugando) in Northwest Tanzania in Africa.
Methods: A cross-sectional study was carried out in Bugando between 1 August 2012 and 30 September 2012. Children (<15 years old) with SCA attending Bugando were sequentially enrolled. A trained research assistant completed a Swahili questionnaire with the parent or guardian of each participant concerning demographic information, clinical features of disease, and treatments received.
Results: Among the 124 participants enrolled, the median age was 6 years (interquartile range [IQR] 4-8.5), and only 13 (10.5%) were < 3 years old. Almost all participants (97.6%) had a prior history of a vaso-occlusive episode, 83 (66.9%) had prior acute chest syndrome, and 21 (16.9%) had prior stroke. In the preceding 12 months, 120 (96.8%) had been hospitalized, and a vaso-occlusive episode was the most common reason for hospitalization (35.5%). Prescriptions for folic acid (92.7%) and malaria prophylaxis (84.7%) were common, but only one had received a pneumococcal vaccine, and none had received hydroxyurea or prophylactic penicillin.
Conclusion: Children with SCA receiving care in Tanzania are diagnosed late, hospitalized frequently, and have severe complications. Opportunities exist to improve care through wider access to screening and diagnosis as well as better coordination of comprehensive care.
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http://dx.doi.org/10.1080/10245332.2015.1101976 | DOI Listing |
Am J Ther
January 2025
Anesthesiology and Perioperative Medicine, David Geffen School of Medicine at UCLA, Los Angeles, CA.
Clinical Features: Sickle cell patients may develop a multitude of antibodies and experience life-threatening events with transfusion such as hyperhemolysis syndrome or delayed hemolytic transfusion reaction. Further transfusion may not be possible in such cases.
Therapeutic Challenge: When conventional blood products are not available for transfusion yet the patient requires additional oxygen-carrying support, artificial oxygen carriers may be required.
Hemoglobin
January 2025
Howard University College of Pharmacy, Washington, DC, USA.
Sickle cell disease (SCD) primarily affects people of African American descent in the United States. Many individuals do not know their sickle cell trait (SCT) status or might not be aware of SCD. The purpose of this study was to assess SCD knowledge, awareness of SCT status, and its impact on relationships.
View Article and Find Full Text PDFPediatr Blood Cancer
January 2025
Division of Pediatric Hematology & Oncology, University Hospitals Rainbow Babies & Children's Hospital, Cleveland, Ohio, USA.
Background: The clinical manifestations of sickle cell disease (SCD) result in significant morbidity and healthcare costs. The effect of weather as a risk factor for the development of vaso-occlusive episodes (VOEs) has been previously studied, although with variable results. The aim of our retrospective, nationwide study was to determine the association between weather patterns and pediatric VOE and acute chest syndrome (ACS).
View Article and Find Full Text PDFCommun Biol
January 2025
Georgia Cancer Center, Augusta University, Augusta, GA, 30912, USA.
The transsulfuration (TSS) pathway is an alternative source of cysteine for glutathione synthesis. Little of the TSS pathway in antioxidant capacity in sickle cell disease (SCD) is known. Here, we evaluate the effects of TSS pathway activation through cystathionine beta-synthase (CBS) to attenuate reactive oxygen species (ROS) and ferroptosis stresses in SCD.
View Article and Find Full Text PDFDrug Test Anal
January 2025
Hemarina SA, Aeropôle Centre, Morlaix, France.
The natural extracellular hemoglobin of the lugworm Arenicola marina (AmHb) has many interesting characteristics: It carries 40 times more oxygen than human hemoglobin; has anti-inflammatory, antibacterial, and antioxidant properties; and is 250 times smaller than a red blood cell. It is nontoxic and nonimmunogenic. It is thus a very promising hemoglobin-based oxygen carrier.
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