The function and morphology of the liver in porphyria cutanea tarda

The aminopyrine breath test, postprandial serum bile acids, and routine liver tests were assessed as indicators of liver dysfunction in 38 patients with porphyria cutanea tarda. In 17 patients needle biopsy specimens of the liver were obtained. Bile acids were increased in almost all the patients studied (97%) but impairment of aminopyrine demethylation was found in only 45%. More than 50% of cases had elevated activities of serum alanine aminopeptidase, leucine aminopeptidase, gammaglutamyl transpeptidase (GGTP), and alanine aminotransferase (ALAT). All liver biopsy specimens showed fluorescence characteristic of porphyrins. Histologic examination of biopsy material revealed cellular lesions in all cases, the most common pathological findings being fatty degeneration of varying degree and iron accumulation. The most frequent electronmicroscopic changes in the liver were fat droplets, granules containing bile material, and siderosomes in the cytoplasm of hepatocytes. However, there was no evident relationship between morphological and functional liver changes.