Liver Fibrosis in Sickle Cell Disease
Condition: Sickle Cell Disease
MeSH terms: Liver Cirrhosis; Anemia, Sickle Cell; Fibrosis
Study type: Observational

Summary: 
      Patients with sickle cell disease many have a number of systemic complications, including
      liver problems. Some of these liver problems lead to liver fibrosis/cirrhosis, secondary to
      chronic blood transfusions. The purpose of this study is to investigate FibroScan readings in
      patients with sickle cell disease and iron overload secondary to blood transfusions, and to
      correlate the FibroScan results with Ferriscan. A comparison with the results of FibroScan to
      patients with Sickle cell without known liver disease, who have never been on chronic
      transfusions and with normal liver function profiles will also be made.The primary hypothesis
      is that the results of FibroScan will correlate with the results of Ferriscan and liver
      biopsy.
    

Detailed description: 
      Liver biopsy is the gold standard to examine the liver for iron deposits and histology.
      However, liver biopsy is invasive and involves a risk of bleeding and pain. Biopsy may also
      miss significant pathology if the small biopsy specimen is taken from an uninvolved part of
      the liver. Non-invasive techniques such as MRI are now used to evaluate the liver iron
      content. MRI can visualize the whole liver and measure liver iron content. MRI, however, will
      not detect liver scarring.

      Liver transient elastography (FibroScan) is a non-invasive ultrasound tool for assessing
      liver fibrosis or scarring by measuring liver stiffness (LSM). Compared with liver biopsy,
      FibroScan provides immediate results and is a painless, short (3 mins), simple procedure to
      perform. In some studies FibroScan reports have correlated well with liver biopsy results of
      fibrosis and cirrhosis, and with Ferriscan, ferritin and liver function tests.

      This purpose of this study is to investigate the role of FibroScan in individuals with sickle
      cell anemia and iron overload or who have a diagnosis of liver disease, and to compare
      FibroScan readings with magnetic resonance imaging.

      We will also compare the results of the Fibroscan with liver biopsy.

      This study will also compare the results of FibroScan to patients with Sickle cell without
      any known liver disease, who have never been on chronic transfusions (defined as no more than
      4 transfusions in lifetime) and who have normal liver function profiles.
    

Intervention name(s): Liver transient elastography (Fibroscan); Ferriscan; Liver biopsy in iron overloaded subjects.
Intervention type(s): Other; Other; Procedure
Intervention description(s): Fibroscan will be performed as a measure of liver stiffness. The study will be obtained free of charge in this study.; Ferriscan will be done to assess the quantity of liver iron in iron overloaded subjects.; Liver biopsy will be done and the results will be compared to Fibroscan results.
Other intervention name(s): FibroScan
Intervention MeSH terms: Liver Extracts
Observational model: Case-Control
Time perspective: Cross-Sectional
Sampling method: Non-Probability Sample
Study population: 
        30 subjects with sickle cell disease and iron overload. 30 control subjects without history
        of chronic transfusions (less than 4 transfusions in lifetime) and without evidence of
        liver pathology.
      
Keywords: sickle cell disease; hemochromatosis; liver fibrosis; liver cirrhosis; FibroScan; Ferriscan; liver iron