THE USE OF HUMAN GLUTATHIONE-S-TRANSFERASE A(1) IN THE DETECTION OF CYSTIC-FIBROSIS LIVER-DISEASE

Objective: To determine the value of serum human glutathione S-transfe rase A(1) (hGST A(1)) in the detection of cystic fibrosis liver diseas e (CFLD). Methods: Sixty-three children (aged 0.5-16 years) with cysti c fibrosis (CF) were screened prospectively for evidence of hepatobili ary abnormalities between February 1993 and February 1995. Comparison was made between clinical examination, abdominal ultrasonic scan, meas urement of conventional liver enzymes (LFTs) and serum hGST A(1) conce ntration in the detection of hepatobiliary abnormalities in children w ith CF, Results: The 5-95% concentration of serum hGST A(1) was 1.7-4. 27 mu g L-1 for the control group. The hGST A(1) levels in the CF pati ents were significantly higher than in the non-CF group. Thirty-eight (60%) children had detectable hepatobiliary abnormalities. Ultrasound scanning detected the highest number of abnormalities (41%), followed by hGST A(1) (30%). The presence of clinical liver disease was found i n 19% of the children. The estimated sensitivities of detecting CFLD b y clinical method, ultrasound scan, serum hGST A(1), and LFTs would be 32%, 68%, 50% and 16%, respectively. Conclusions: Serum hGST A(1) mea surement increases the sensitivity of detecting hepatic abnormalities when included with clinical and ultrasound evaluation although, in som e cases with advanced liver disease, serum hGST A(1) may be normal. Co nventional liver enzyme tests add little information in the detection of CF liver disease.