Are Serum Type III Procollagen and Prolyl Hydroxylase Useful as Noninvasive Markers of Liver Disease in Patients with Cystic Fibrosis?

Serum markers of hepatic fibrogenesis in cystic fibrosis liver disease

“…Only four underwent liver biopsy and no comparison of serum markers was made between CFLD and CFnoLD. As a group, children with CF had higher serum PH but not PIIIP verses controls [21].…”

“…Previous studies have shown an elevation in collagen type-VI [20], PIIIP, PH [21] and HA [22] in children with CFLD. However, no study has specifically investigated these serum markers in a well-characterised cohort of children with CFLD, compared to age-and sex-matched controls and no correlations have been made with the degree of hepatic fibrosis assessed by liver biopsy.…”

“…Two studies have suggested that human glutathione S transferase A1 (GSTA) was superior to conventional transaminases and comparable to ultrasonography in detecting liver disease, but its utility for detecting hepatic fibrosis was not determined as no biopsies were undertaken [24,25]. Serum PIIIP and PH were studied in 44 children with CF, 26 with suggestion of liver disease by clinical and ultrasound abnormalities [21]. Only four underwent liver biopsy and no comparison of serum markers was made between CFLD and CFnoLD.…”

Hepatobiliary complications of cystic fibrosis

“…Recently, serum markers to detect liver fibrosis have been evaluated in several liver diseases, including CF. Collagen VI [42] and serum prolyl hydroxylase [43] have been shown to be increased in a small number of CF patients with liver disease compared with those without. These serum markers of liver injury, inflammation, and fibrosis require further study in CF patients to determine their usefulness in screening programs.…”

“…Infants with cholestasis may benefit from elemental formulas containing medium-chain triglyceride to promote intestinal absorption of dietary lipid. Fat-soluble vitamin deficiency is common in CF [43], and patients with liver disease may be at even higher risk because of impaired bile acid solubilization. Fat-soluble vitamin levels should be monitored at least annually.…”