Transient elastography in patients with cystic fibrosis

Area of Science:

• Hepatology
• Pediatric Gastroenterology
• Medical Imaging

Background:

• Cystic fibrosis (CF) frequently involves the liver, potentially leading to CF-associated liver disease (CFLD) in about 25% of patients.
• Liver biopsy, the gold standard for diagnosis, is invasive and prone to sampling errors.
• Ultrasound (US) is limited, as a normal scan does not rule out significant liver fibrosis.

Purpose of the Study:

• To prospectively compare the efficacy of transient elastography (TE) and transabdominal US in evaluating liver conditions in pediatric and adult CF patients.
• To establish TE as a reliable, non-invasive tool for assessing liver stiffness in CF patients.

Main Methods:

• 134 CF patients underwent TE immediately following routine liver US.
• Liver US appearance was graded (1-5); TE measurements (kPa) were taken ten times per patient, with the median value used.
• Control groups included 31 children without CF.

Main Results:

• No age-related correlation with liver stiffness was found in CF patients or controls.
• Lower elasticity values (US score <3) were observed in controls, pancreatic-sufficient CF patients, and pancreatic-insufficient CF patients compared to those with US score >=3 (all PI).
• Median liver stiffness was significantly higher in male CF patients (4.7 kPa) than in females (3.9 kPa).

Conclusions:

• Transient elastography (TE) presents a promising, non-invasive alternative to liver biopsy for assessing and monitoring liver disease in CF patients.
• TE's user-friendliness and reproducibility make it an attractive tool, especially given the limitations of US and the risks associated with biopsy.