Introduction: Hepatobiliary abnormalities occur commonly in sickle cell anaemia and these have been extensively reported in the adult patients. However, complications have been sparsely reported in children especially in the sub-Saharan African continent. This study aimed to highlight the hepatobiliary complications in this group of children using clinical examination, laboratory testing and abdominal ultrasonography. The challenges in a resource limited country are also highlighted.
Subjects, Materials And Methods: One hundred and thirty- four (134) children aged 1-18years with sickle cell anaemia in steady state were recruited into this crossectional study. Clinical history and physical examination obtained were documented. Relevant basic haematologic and biochemical indices (Full blood count, liver enzymes and viral markers for hepatitis B and C) and abdominal ultrasonographic parameters were documented for all the children. The relationship between the complications and possible risk factors (age, frequency of crisis and blood transfusions) were also documented.
Result: Fifty-three (39.6%) of the children had hepatobiliary abnormalities. Chronic hepatitis B infection was the most prevalent complication (14.9%) seen followed by cholelithiasis (12.7%) and Hepatitis C infection (4.5%). Other complications identified were cholecystitis (3.0%), biliary sludge (1.5%), liver cirrhosis (0.7%). Age was significantly associated with viral hepatitis (p=0.003) and cholelithiasis (p=0.0007) and the conditions were more prevalent in the older age group. The hepatobilary complications were also more prevalent in the males. Frequent blood transfusions was significantly related to viral hepatitis (p=0.03). The use of hydroxyurea was not significantly related to any of the complications.
Conclusion: Hepatobiliary abnormalities are prevalent in children with sickle cell anaemia. Clinical screening and the use of ultrasonography would aid early diagnosis and appropriate therapeutic intervention in this group of children.
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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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