Multispectral Optoacoustic Tomography in Patients With Cystic Fibrosis
- Conditions
- Cystic FibrosisLiver FibrosisGastrointestinal Transport DisorderDios
- Interventions
- Diagnostic Test: Acoustic Radiation Forced Impulse ImagingDiagnostic Test: Multispectral Optoacoustic Tomography
- Registration Number
- NCT06063785
- Lead Sponsor
- University of Erlangen-Nürnberg Medical School
- Brief Summary
Cystic fibrosis (CF) is the most common hereditary disease in Central Europe. The disease is caused by a mutation in the cystic fibrosis transmembrane conductance regulator gene (CFTR). In the liver, fibrotic remodeling can lead to liver cirrhosis in the long term. Early detection of CF hepatopathy is essential to therapeutically slow down the progression of fibrotic remodeling mechanisms. Newborns suffering from CF have a significantly increased risk for the occurrence of meconium ileus and also with advancing age there are symptoms ranging from chronic constipation to Distal Intestinal Obstruction Syndrome (DIOS), due to a reduction of intestinal motility.
In this study, the degree of liver fibrosis will now be investigated in adult patients with cystic fibrosis using Multispectral Optoacoustic Imaging (MSOT). In addition, gastrointestinal passage will be studied non-invasively to investigate another affection of the gastrointestinal system.
- Detailed Description
Cystic fibrosis (CF) is the most common hereditary disease in Central Europe, with an incidence of approximately 3,300 to 4,800 new cases. The disease follows an autosomal recessive pattern of inheritance, the cause being a mutation in the cystic fibrosis transmembrane conductance regulator gene (CFTR). In the liver, fibrotic remodeling can lead to liver cirrhosis in the long term. Early detection of CF hepatopathy is essential to therapeutically slow down the progression of fibrotic remodeling mechanisms. Over the past decade, measurements of liver stiffness using Acoustic Radiation Force Impulse Imaging (ARFI) have proven to be a valid tool for measuring fibrotic tissue remodeling in CF in adults and children. Furthermore, in the gastrointestinal tract, serious consequences result from the absence of the CFTR channel. Newborns suffering from CF have a significantly increased risk for the occurrence of meconium ileus and also with advancing age there are symptoms ranging from chronic constipation to DIOS (Distal Intestinal Obstruction Syndrome), due to a reduction of intestinal motility.
By means of new imaging methods, such as multispectral optoacoustic tomography, it is possible to examine not only the body's own substances but also substances foreign to the body. With Multispectral Optoacoustic Imaging (MSOT), similar to conventional sonography, a transducer is placed on the skin and instead of sound, energy is applied to the tissue by means of light flashes. This leads to a constant alternation of minimal expansions and contractions (thermoelastic expansion) of individual tissue components or molecules. Previous studies have shown that quantitative determination of hemoglobin can provide information on blood flow and inflammatory activity in the intestines of adult patients with Crohn's disease. Also, fibrotic changes in the liver can probably be detected with this method, similar to that in muscle tissue. Furthermore, we have recently shown that orally ingested Indocyanine green (ICG) can be detected in the small intestine and thus conclusions can be drawn about gastrointestinal passage, without the use of ionizing radiation. In this study, the degree of liver fibrosis will now be investigated in adult patients with cystic fibrosis using MSOT. In addition, gastrointestinal passage will be studied non-invasively to investigate another affection of the gastrointestinal system.
Recruitment & Eligibility
- Status
- RECRUITING
- Sex
- All
- Target Recruitment
- 30
Patient cohort "Cystic Fibrosis without CF-related liver disease":
- Molecular genetic confirmed diagnosis of cystic fibrosis.
- Age over 18 years
- Written informed consent
Patient cohort "Cystic Fibrosis with CF-related liver disease":
-
Molecular genetic confirmed diagnosis of cystic fibrosis
-
Presence of CF-related liver disease based on Colombo criteria:
- Hepato- and/or splenomegaly
- Persistent elevation of transaminases in the serum
- Sonographic evidence of liver involvement
-
Age over 18 years
-
Written informed consent
"Volunteer Subjects":
- Age over 18 years
- Written informed consent
General:
- Pregnancy
- Breastfeeding mothers
- Tattoo in the area of the examination
- Subcutaneous fat tissue over 3 cm
Patient cohort "Cystic fibrosis without CF-related liver disease":
-
Taking systemic glucocorticoids or immunosuppressants as part of a permanent medication regimen.
-
Presence of CF-related liver disease based on Colombo criteria:
- Hepato- and/or splenomegaly.
- Persistent elevation of transaminases in the serum
- Sonographic evidence of liver involvement.
-
Acute exacerbation of infection
Patient cohort "Cystic fibrosis with CF-related liver disease":
- Taking systemic glucocorticoids or immunosuppressants as part of a permanent medication regimen.
- Decompensation of CF-related liver disease
- Acute exacerbation of infection
"volunteer subjects":
- Presence of liver disease
- Use of systemic glucocorticoids or immunosuppressants in the context of permanent medication
Study & Design
- Study Type
- OBSERVATIONAL
- Study Design
- Not specified
- Arm && Interventions
Group Intervention Description CF with liver affection Acoustic Radiation Forced Impulse Imaging CF patients with proven CF hepatopathy CF with liver affection Multispectral Optoacoustic Tomography CF patients with proven CF hepatopathy CF without liver affection Acoustic Radiation Forced Impulse Imaging CF patients without proven CF hepatopathy Healthy volunteers Acoustic Radiation Forced Impulse Imaging Healthy volunteers without liver affection Healthy volunteers Multispectral Optoacoustic Tomography Healthy volunteers without liver affection CF without liver affection Multispectral Optoacoustic Tomography CF patients without proven CF hepatopathy
- Primary Outcome Measures
Name Time Method Quantitative collagen signal (Liver) Day 1 in arbitrary units
Quantitative Indocyanine Green (ICG) signal (Intestinal) 0, 60, 90, 120, 180, 240, 300min post ICG intake Day 1
- Secondary Outcome Measures
Name Time Method Deoxycholic acid (DCA) and respective glycine and taurine conjugates Day 1 µmol/l and /g stool
Cholic acid (CA) and respective glycine and taurine conjugates Day 1 µmol/l and /g stool
Lithocholic acid (LCA) and respective glycine and taurine conjugates Day 1 µmol/l and /g stool
Quantitative oxy/deoxygenated hemoglobin signal (Liver) Day 1 in arbitrary units
Quantitative single wave lengths (Liver) Day 1 in arbitrary units
Optoacoustic spectrum (Intestinal) 0, 60, 90, 120, 180, 240, 300min post ICG intake in arbitrary units, normalized
Optoacoustic spectrum (Liver) Day 1 in arbitrary units, normalized
Quantitative single wave lengths (Intestinal) 0, 60, 90, 120, 180, 240, 300min post ICG intake in arbitrary units
Attenuation coefficient (Liver) Day 1 in dB/cm/MHz
Chenodeoxycholic acid (CDCA) and respective glycine and taurine conjugates Day 1 µmol/l and /g stool
Quantitative oxy/deoxygenated hemoglobin signal (Intestinal) 0, 60, 90, 120, 180, 240, 300min post ICG intake in arbitrary units
Ursodeoxycholic acid (UDCA) and respective glycine and taurine conjugates Day 1 µmol/l and /g stool
Shear wave velocity (Liver) Day 1 in m/s
Trial Locations
- Locations (1)
University Hospital Erlange, Department of Pediatrics
🇩🇪Erlangen, Bavaria, Germany