In a 52-year-old female who was jaundiced since early childhood, marked conjugated hyperbilirubinaemia and a delay of bromsulfophthalein and indocyanine green plasma disappearance rates were observed. Liver histology, plasma bile acids and plasma clearance of 14-C-cholic acid were normal. There was a pronounced impairment of the relative storage capacity of bromsulfophthalein (S = 2,4 mg/mg%) and a moderate decrease of BSP-transport maximum.
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