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Sickle cell disease in Britain.

M Brozović, E Anionwu

    Journal of Clinical Pathology
    |December 1, 1984
    PubMed
    Summary
    This summary is machine-generated.

    Sickle cell disease is increasingly prevalent in urban Britain, particularly London. Early detection in newborns is crucial for preventing infant mortality from infections like pneumococcal disease.

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    Area of Science:

    • Hematology
    • Public Health
    • Pediatrics

    Background:

    • Sickle cell disease (SCD) is a significant health concern in urban UK populations, with London having a substantial patient base.
    • Central Middlesex Hospital manages a considerable number of SCD patients, highlighting the disease's local impact.

    Purpose of the Study:

    • To analyze the clinical features and management of sickle cell disease in a London population.
    • To assess the incidence of sickle cell disease and related traits in newborns through screening programs.
    • To emphasize the need for early detection and management guidelines for SCD in Britain.

    Main Methods:

    • Retrospective analysis of patient admissions and clinical features at Central Middlesex Hospital.
    • Screening of 3165 newborn babies for sickle cell disease and related hemoglobinopathies.

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  • Comparative analysis of clinical presentations between patients in Brent (UK) and Jamaica.

    • Main Results:

    • Vaso-occlusive crisis is the most common reason for hospital admission (80%), followed by acute chest syndrome (12%).
    • Brent patients with sickle cell anemia experienced more frequent painful crises than Jamaican patients; leg ulcers were uncommon.
    • Newborn screening detected five cases of sickle cell anemia and three of sickle cell hemoglobin C disease, with an overall sickle cell trait incidence of 3.2%.

    Conclusions:

    • Sickle cell disease is a growing public health issue in Britain, necessitating robust management strategies.
    • Early identification of newborns with SCD is vital for implementing prophylactic measures against life-threatening infections.
    • Standardized guidelines for managing acute and chronic SCD complications are urgently required in the UK.