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Level of liver fibrosis in children diagnosed with cystic fibrosis is possible to be assessed with the use of elastography.

 

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Recently, researchers from the Silesian Medical University in Katowice, Department of Paediatrics, conducted a study with the aim to assess the scale of liver fibrosis with the use of elastography in paediatric patients with diagnosed cystic fibrosis (CF) and compare it with other tests (APRI and Fibrotest).

Cystic fibrosis (CF) is a genetic, multiorgan, and chronic disorder affecting mainly the respiratory and gastrointestinal systems, including the liver and the pancreas. Even though liver abnormalities concern only 5-20% of patients with cystic fibrosis, they shorten the lifespan and deteriorate its quality.

An elastography, also known as liver elastography, is a type of imaging test that checks the liver for fibrosis. Combining the technique with other laboratory tests in clinical practice might allow for the early diagnosis and treatment of liver disease in these patients, the researchers suggested.

The researchers examined 41 patients with diagnosed cystic fibrosis, aged from 2 years to 21 years (the average age of 9.6 years), 24 girls (58.5%) and 17 boys (41.5%), diagnosed and treated in the Department of Paediatrics of the Medical University of Silesia in Katowice. The analysis included clinical picture, laboratory parameters of liver damage, and cholestasis.

Elastography was used to estimate the stage of liver fibrosis (from F0-F4) for each child, with a higher stage reflecting more significant liver stiffness and worse fibrosis. For example, F0 indicated no fibrosis, whereas F4 suggested cirrhosis was present.

Elastography findings were considered normal in 22 children (F0-F1), and abnormal in 19 children, five of whom were advanced (F3-F4). Children with higher elastography stages tended to have a significantly lower body mass index, likely reflecting malnutrition, the researchers noted.

Upon physical examination, signs of CF-associated liver disease (CFLD) were observed in 16 children — seven boys and nine girls. Such signs, including enlarged liver or reduced flow of bile (a digestive fluid released from the liver), were detected with routine lab tests and abdominal ultrasounds.

Of the 16 children diagnosed with CFLD based on their physical exam, 12 had abnormal elastography findings. All five children with advanced elastography abnormalities (F3-4) had a CFLD diagnosis. Seven children who did not show signs of CFLD in other tests were considered to have abnormal elastography results.

The downside of the study is the fact that the groups of patients are not very numerous. Nevertheless, cystic fibrosis is considered a “rare disease” affecting around 1/5000 people in Europe. It would certainly be worth comparing the results of elastography with the assessment of liver fibrosis in the histopathology of the organ. However, liver biopsy is an invasive and not risk-free procedure which only shows topical fibrotic and inflammatory changes, and not the holistic picture.

On the other hand, there are very few studies that assess the suitability of minimally invasive procedures for the detection of early lesions in the liver in paediatric patients with cystic fibrosis. And it is children below the age of 10 that are most frequently diagnosed with CFLD. The researchers are planning on repeating the elastography in this group of patients after 5 and 10 years to assess the progression of fibrosis in paediatric patients.

The study, “Assessment of Liver Fibrosis with the Use of Elastography in Paediatric Patients with Diagnosed Cystic Fibrosis,” was published in Disease Markers.

Reference: Wiecek S., Fabrowicz P., Wos H., Kordys-Darmolinska B., Cebula M., Gruszczynska K., Grzybowska-Chlebowczyk U.2022. “Assessment of Liver Fibrosis with the Use of Elastography in Paediatric Patients with Diagnosed Cystic Fibrosis”, Disease Markers. Vol. 2022: 4798136. https://doi.org/10.1155/2022/4798136
Original article published by Cystic Fibrosis News Today on April, 11, 2022
Photo credit: Freepik

 

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