Comparison of Non-invasive Measurement Methods of Hepatic Fibrosis in Cystic Fibrosis
Condition: Cystic Fibrosis
MeSH terms: Cystic Fibrosis; Fibrosis
Study type: Interventional

Summary: 
      Non-invasive methods of the quantification of fibrosis may help to assess the development of
      fibrosis at a specific moment of the evolution of the disease, in the order to decide the
      initiation of treatment with ursodeoxycholic acid which may slow the progression to hepatic
      cirrhosis:

        -  FibroTest,

        -  The ultrasound impulse elastography, Fibroscan (FS)

        -  Ultrasound Elastography by ShearWave (SWE)

        -  Magnetic resonance elastography (MRE). The purpose of this study is to assess the
           contribution of these methods in the diagnosis of hepatic fibrosis during the evolution
           of the cystic fibrosis.
    

Detailed description: 
      The incidence of hepatobiliary disease in cystic fibrosis in the first 10 years of life is
      2.5 to 1.8 / 100 patient-years, significantly decreasing during the second decade; generally
      30-40% of children develop hepatic disease before 12 years. However, the hepatobiliary damage
      varies in its clinical and histological expression. The typical histological appearance of
      the liver is the focal biliary cirrhosis (or fibrosis) characterized by a heterogeneous
      distribution of portal fibrosis in the liver.

      Complications of cirrhosis are responsible for 2.5% of deaths in patients with cystic
      fibrosis and represent the second cause of death after lung disease if we exclude deaths
      secondary to lung transplantation.

      The diagnosis of hepatic disease at an early stage of development and the assessment of the
      progression of fibrosis are so difficult in the absence of sensitive and specific
      non-invasive diagnostic tests.

      Non-invasive assessment of hepatic fibrosis is an important topic of study in recent years,
      because of the emergence of biological methods and quantitative elastography methods using
      ultrasound or Magnetic Resonance Imagery (MRI) and allowing quantification of the hepatic
      elasticity, which is assumed to be a reflection of fibrosis. The advantages of these methods
      are numerous:

        -  The assessment of wider hepatic parenchyma then hepatic biopsy, allowing overcome the
           sampling bias

        -  Painless and non-invasive methods for the patient

        -  Simple to make

        -  Do not require hospitalization and can be performed as outpatient

        -  Immediately known results

        -  Can be repeated as often as necessary during the patient's follow-up

        -  Lower cost, morbidity and mortality compared to hepatic biopsy. Several non-invasive
           methods of fibrosis diagnosis have been studied in adults primarily for viral diseases
           and helped to limit the indications of hepatic biopsy. However, no method is now
           validated for the early diagnosis of hepatic disease and monitoring of children with
           cystic fibrosis.

      The main objective of the study is to assess the contribution of FibroTest, the ultrasound
      impulse elastography, Fibroscan (FS), the ultrasound elastography by ShearWave (SWE) and
      Magnetic Resonance Elastography (MRE ) in the diagnosis of hepatic fibrosis in cystic
      fibrosis.

      The secondary objectives are following :

        -  To study the correlations between the fibrosis quantitative results obtained through the
           four techniques of measurement and the matches between the results of four techniques
           using the standards available at t0 and after a year (t1).

        -  Study the correlations between clinical, biological and ultrasound endpoints of hepatic
           disease and various non-invasive measurement methods of fibrosis.

        -  Evaluate the area under the ROC curve of the different tests and the diagnostic
           performance tests using analytical methods without gold standard by classifying the
           children into two groups: the presence of advanced fibrosis (stages F2 to F4 ) or not
           (F0 and F1 stages), contracting by a committee according to the results of any biopsy,
           clinical, laboratory and ultrasound endpoints independent on the tests.
    

Intervention name(s): Measure of hepatic elasticity
Intervention type(s): Other
Intervention description(s): Measure of hepatic elasticity by four non-invasive methods:
Fibroscan, Fibrotest, SWE, MRE.
Intervention model: Single Group Assignment
Masking: None (Open Label)
Primary purpose: Screening
Keywords: Cystic fibrosis; hepatic fibrosis; hepatic cirrhosis; non-invasive methods of measurement; hepatic elasticity