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Purpose: Hip deformity is frequent after childhood osteonecrosis in patients with sickle cell disease (SCD). When they are adults, they present a challenge as candidates for total hip arthroplasty (THA) because of abnormal bone development, their relative youth, and also because of their disease. Performing subtrochanteric osteotomy associated with THA is technically challenging, and healing of osteotomies has never been reported in this population with frequent osteonecrotic bone, whether using cemented or uncemented arthroplasties.
View Article and Find Full Text PDFSickle cell disease in India: the journey and hope for the future.
Hematology Am Soc Hematol Educ Program
December 2024
Department of Paediatrics, Government Medical College, Nagpur, Maharashtra, India.
India, the most populous nation in the world, also has a high frequency of the sickle hemoglobin (HbS) allele globally. The Arab Indian HbS haplotype in India is characterized by a relatively high percentage of fetal Hb, with widely varying frequencies of α-thalassemia. Hence, sickle cell disease (SCD) in India was perceived to be mild.
View Article and Find Full Text PDFScreening for pulmonary hypertension in pregnant women with sickle cell disease in sub-Saharan Africa.
AJOG Glob Rep
November 2024
Department of Family Medicine, Indiana University School of Medicine, Indianapolis, IN (Okuyemi).
Background: Sickle cell disease (SCD) has evolved from a condition predominantly fatal in childhood to a chronic illness impacting many adults, including women of reproductive age. For females with SCD, pregnancy represents one of the greatest health threats, exacerbating existing health challenges and introducing new risks. Despite advancements in healthcare, routine screening for existing complications like pulmonary hypertension (PH) remains inconsistent, particularly in low- and middle-income countries (LMICs), where the prevalence of SCD is highest.
View Article and Find Full Text PDFLate diagnosis of sickle cell disease in adults still a challenge in developing countries: a case report.
J Med Case Rep
November 2024
Department of Internal Medicine, Faculty of Clinical Medicine and Dentistry, Kampala International University, Kampala, Uganda.
Article Synopsis
- Sickle cell disease is a genetic disorder that significantly affects health, with a high prevalence in Sub-Saharan Africa; Uganda sees about 20,000 affected children born each year, most of whom face severe health challenges.
- A case study of a 22-year-old Ugandan male highlights the importance of recognizing sickle cell disease symptoms in adults, as he experienced multiple joint pain and anemia before diagnosis.
- The report emphasizes the need for comprehensive screening for sickle cell disease across all age groups to improve outcomes, particularly in regions where the disease is common.
Trends in Sickle Cell Disease Mortality: 1979-2020.
Pediatrics
December 2024
Department of Pediatrics, Warren Alpert Medical School of Brown University, Providence, Rhode Island.
Background And Objectives: Although sickle cell disease (SCD)-related childhood mortality in the United States significantly improved in the 1990s, unclear is the trend in SCD-related mortality more recently given the continued disparities faced by this minoritized population. In this analysis, we aimed to (1) compare the overall and age-specific mortality rates from 1999 to 2009 vs 2010 to 2020 with a particular focus on the age of transition and (2) determine the most common causes of death for the US SCD population for 2010 to 2020.
Methods: We analyzed publicly available data from the Centers for Disease Control and Prevention WONDER database, a compilation of national-level mortality statistics from 1979 to 2020 derived from death certificates compiled by the National Center for Health Statistics.
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