CAN THE HISTOLOGIC-CHANGES OF CYSTIC FIBROSIS-ASSOCIATED HEPATOBILIARY DISEASE BE PREDICTED BY CLINICAL-CRITERIA

Background: Correlation between clinical parameters and histology chan ges in cystic fibrosis liver disease has not been documented. The purp ose of this study was to determine the histologic spectrum of cystic f ibrosis liver disease and the degree to which a clinical scoring syste m can identify subjects with significant histologic abnormalities. Met hods: We reviewed the predictive value of physical examination, bioche mical parameters, and a clinical liver score, incorporating physical e xamination and biochemical parameters, in predicting significant abnor malities of liver histology in 43 cystic fibrosis patients who underwe nt hepatic biopsy. Biopsies were scored by two masked pathologists for fibrosis, inflammation, inspissation, fatty infiltration, and congest ion. Results: Significant histologic disease was present in 56% of pat ients despite little biochemical or physical examination evidence of d isease. No single parameter used in the scoring system predicted the t ype or degree of the liver disease. The clinical liver score had a sen sitivity of 85% and a specificity of 82% in predicting significant his tologic changes, yet it was unable to predict the specific lesion. Con clusions: Significant histologic liver disease is common in cystic fib rosis, although the exact nature of the lesion cannot be predicted wit hout liver biopsy. A clinical liver score that was developed for this may be useful in determining which patients require more definitive ev aluation. (C) 1997 Lippincott-Raven Publishers.