CFDB - Cystic Fibrosis DataBase

primary studies published, non RCT

Association Between Transient Elastography and Controlled Attenuated Parameter and Liver Ultrasound in Children With Cystic Fibrosis.

Study design (if review, criteria of inclusion for studies)

ELASTIC (Longitudinal Assessment of Transient Elastography in CF) is a nested cohort of 141 patients, ages 7-21, enrolled in the Prediction by US of Risk of Hepatic Cirrhosis in CF (PUSH) Study.

Participants

Children with cystic fibrosis (CF) at risk for development of advanced liver disease. ELASTIC (Longitudinal Assessment of Transient Elastography in CF) is a nested cohort of 141 patients, ages 7-21, enrolled in the Prediction by US of Risk of Hepatic Cirrhosis in CF (PUSH) Study.

Interventions

liver stiffness measurement (LSM)

Outcome measures

The association between LSM with research-grade US patterns (normal [NL], heterogeneous [HTG], homogeneous [HMG], or nodular [NOD]) and conventional hepatic markers. In a subgroup (n = 79), the association between controlled attenuation parameter (CAP) and US pattern was explored.

Main results

Among 133 subjects undergoing VCTE, NOD participants (n = 26) had a significantly higher median (interquartile range) LSM of 9.1 kPa (6.3, 15.8) versus NL (n = 72, 5.1 kPa [4.2, 7.0]; P < 0.0001), HMG (n = 17, 5.9 kPa [5.2, 7.8]; P = 0.0013), and HTG (n = 18, 6.1 kPa [4.7, 7.0]; P = 0.0008) participants. HMG participants (n = 14) had a significantly higher mean CAP (SD) (270.5 dB/m [61.1]) compared with NL (n = 40, 218.8 dB/m [46.5]; P = 0.0027), HTG (n = 10, 218.1 dB/m [60.7]; P = 0.044), and NOD (n = 15, 222.7 dB/m [56.4]; P = 0.041) participants. LSM had a negative correlation with platelet count (r(s) =  -  0.28, P = 0.0071) and positive correlation with aspartate aminotransferase-to-platelet ratio index (r(s) = 0.38, P = 0.0002), Fibrosis-4 index (r(s) = 0.36, P = 0.0007), gamma-glutamyltransferase (GGT; r(s) = 0.35, P = 0.0017), GGT-to-platelet ratio (r(s) = 0.35, P = 0.003), and US spleen size z-score (r(s) = 0.27, P = 0.0073).

Authors' conclusions

VCTE is associated with US patterns and conventional markers in patients with liver disease with CF.

Keywords: Adolescent; Child; Gastrointestinal Diseases; Liver Diseases; non pharmacological intervention - diagn; elastography; diagnostic procedures;