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| http://dx.doi.org/10.1016/j.amjmed.2021.12.004 | DOI Listing |
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The modern use of hydroxyurea for children with sickle cell anemia.
Haematologica
January 2025
Division of Hematology, Cincinnati Children's Hospital Medical Center, Cincinnati OH; University of Cincinnati College of Medicine, Cincinnati OH; Global Health Center, Cincinnati Children's Hospital Medical Center, Cincinnati OH.
Over the past 40 years, the introduction and refinement of hydroxyurea therapy has led to remarkable progress for the care of individuals with sickle cell anemia (SCA). From initial small proof-of-principle studies to multi-center Phase 3 controlled clinical trials and then numerous open-label studies, the consistent benefits of once-daily oral hydroxyurea have been demonstrated across the lifespan. Elevated fetal hemoglobin (HbF) serves as the most important treatment response, as HbF delays sickle hemoglobin polymerization and reduces erythrocyte sickling.
View Article and Find Full Text PDFMicrobiome in sickle cell disease: Pathophysiology and therapeutic insights.
Br J Haematol
October 2024
ICMR-CRMCH, National Institute of Immunohaematology, Chandrapur Unit, Chandrapur, Maharashtra, India.
Sickle cell disease (SCD) is a complex genetic blood disorder characterized by abnormal haemoglobin, resulting in sickle-shaped red blood cells. While extensive research has concentrated on the genetic and physiological aspects of SCD, recent studies suggest a potential role of the human microbiome in SCD pathophysiology, adding new dimensions to its understanding. This review synthesizes current knowledge on the microbiome's involvement in SCD, focusing on alterations in the gut microbiome composition and diversity compared to healthy individuals, and their implications for disease pathogenesis.
View Article and Find Full Text PDFDifferential expression of adhesion molecules in sickle cell anemia and gut microbiome effect.
Ann Hematol
February 2024
H&TRC - Health & Technology Research Center, ESTeSL - Escola Superior de Tecnologia da Saúde, Instituto Politécnico de Lisboa, Lisbon, Portugal.
Sickle cell anemia (SCA) causes a long-standing vascular inflammation state, leading to endothelial dysfunction and chronic overexpression of several adhesion molecules, which contributes to acute and constant vaso-occlusive (VOC) episodes. It has been demonstrated that hydroxyurea (HU) can reduce VOC events, organ damage, blood transfusions, and even the adhesion properties to endothelial cells of SCA subjects. Due to VOC episodes, these patients are also more susceptible to recurrent bacterial translocation and dysbiosis.
View Article and Find Full Text PDFDegos disease in a child presenting with acute renal failure.
Pediatr Dermatol
March 2023
Department of Dermatology, KK Women's and Children's Hospital, Singapore, Singapore.
Article Synopsis
- - Degos disease, also known as malignant atrophic papulosis, is a rare, systemic condition that affects blood flow and is not commonly found in young individuals.
- - The key feature of this disease is a specific type of skin lesion: a papule that has a white, sunken center surrounded by a red, spider-web-like area.
- - A case study highlights an adolescent girl who experienced severe kidney failure due to blood clotting issues caused by the disease, along with the distinctive skin lesion observable through a dermatoscope.
Gastrointestinal vaso-occlusive crisis in sickle cell disease.
Proc (Bayl Univ Med Cent)
August 2022
Department of Internal Medicine, University of Connecticut, UConn Health, Farmington, Connecticut.
Although rare, gastrointestinal vaso-occlusive crisis in sickle cell disease results in potentially life-threatening ischemia and death. Here we present a case of a 34-year-old patient with sickle cell disease who developed an acute pain crisis complicated by hypovolemia, hypoxia, and hypotension. The patient was treated with supportive measures.
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