Arterialization of the venous blood is thought to be indicative of cutaneous shunting, and occurs in patients with sickle cell disease (SCD) during vaso-occlusive crisis (VOC). We performed the present study to quantify the amount of shunting that occurs in sickle cell patients presenting at the Howard University Sickle Cell Center, Washington, D.C., as outpatients and for hospitalizations associated with sickle cell crisis. Peripheral venous blood was drawn anaerobically into heparinized syringes from 9 normal control subjects (NC), 24 outpatients (steady-state group), and 14 inpatients during crisis (VOC group). Spectrophotometric measurements were made for the following species of hemoglobin (Hb): oxy-Hb (O2Hb), reduced Hb (RHb), carboxy-Hb (COHb), and met-Hb (MHb). In addition, fetal hemoglobin (HbF) was measured by high-pressure liquid chromatography (HPLC). The O2Hb saturations of the steady state group were not significantly different than those of the NC group (55 +/- 4% vs. 40 +/- 6%). However, the O2Hb saturations of the VOC group were 73 +/- 3%, and this value was found to be significantly greater than those of both the steady-state and the NC groups (p < 0.05). Reduced hemoglobin saturations were inversely related to the O2Hb values, as expected. Compared to the NC group, the steady-state, and VOC groups had greater dyshemoglobin (COHb and MHb) levels (p < 0.05). These findings suggest that the percentages of venous O2Hb and dyshemoglobins may be increased in sickle cell disease even in the absence of VOC. Therefore, the venous O2Hb saturation may be a useful biochemical marker for the arteriovenous shunting and hemodynamic adaptations associated with sickle cell disease.
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Milbank Q
January 2025
Questrom School of Business, Boston University.
Unlabelled: Policy Points Cell and gene therapies (CGTs) offer treatment for rare and oftentimes deadly disease, but their prices are high, and payers may seek to limit spending. Total annual costs of covering all existing and expected CGTs for the entire US population 2023-2035 to amount to less than $20 per person and concentrate in commercial and state Medicaid plans. Reinsurance fees add to expected costs.
View Article and Find Full Text PDFBMC Health Serv Res
January 2025
Indiana University School of Medicine, 410 W 10th St, Suite 2000A, Indianapolis, IN, 46202, USA.
Background: Individuals with Sickle Cell Disease (SCD) are a minoritized and marginalized community that have disparate health outcomes as a result of systemic racism and disease-related stigma. The purpose of this study was to determine the psychosocial risk factors for families caring for children with SCD at a pediatric SCD center through use of the Psychosocial Assessment Tool (PAT), a validated caregiver-report screener.
Methods: The PAT was administered annually during routine clinical visits and scored by the SCD Social Worker to provide tailored resources to families.
Best Pract Res Clin Rheumatol
January 2025
ICMR-National Institute of Research in Tribal Health, Jabalpur, India; Academy of Scientific and Innovative Research (AcSIR), Ghaziabad, India; Model Rural Health Research Unit, Jheet, Durg, India. Electronic address:
Sickle cell disease (SCD) is a mono-genic disorder causing chronic hemolysis, anemia, and vaso-occlusion, leading to musculoskeletal complications such as osteonecrosis, osteoporosis, and bone fractures affecting 50-70% SCD patients. These complications result from a complex interplay of genetic and physiological factors, including abnormal hemoglobin production, chronic inflammation, and oxidative stress. This review discusses the pathophysiology, pre-clinical symptoms, and clinical manifestations of musculoskeletal complications in SCD, as well as current treatment options, including pharmacological interventions, surgical procedures, and bone marrow transplantation.
View Article and Find Full Text PDFAsian J Transfus Sci
September 2022
Department of Zoology, CCS HAU, Hisar, Haryana, India.
Context: Hemoglobinopathies are the most common heterogeneous group of monogenetic disorder in the world and its prevalence varies with geographical regions. India is developing country and many studies show a significant burden of hemoglobinopathies in India.
Aims: The aim of the present study was to check the prevalence of various hemoglobinopathies in anemic subjects using high-performance liquid chromatography (HPLC) method in Pune region which has multiple ethnic population groups from all parts of India.
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