Abstract
Summary A 27-year-old Caucasian man had experienced 14 episodes of pruritus, jaundice, bilirubinuria, and weight loss since puberty. Remission occurred spontaneously on each occasion. During a clinical exacerbation, bilirubin, alkaline phosphatase, and serum di- and trihydroxy bile acid concentrations were increased and Tm sulfobromophthalein and hepatic sulfobromophthalein storage were reduced. Examination of a surgical biopsy specimen taken at that time revealed markedly altered bile canaliculi, with distorted and reduced microvilli, almost complete disappearance of nucleoside phosphatase activity, and reduction in the number of acid phosphatase-rich lysosomes. Lipid spheres and glycogen nuclei were abundant and intracanalicular, and intracellular bile pigment was common. During clinical remission, 10 months later, functional, cytochemical, and ultrastructural studies showed a remarkable return to normal patterns. Three exceptions were noted: the presence of small amounts of intracellular and intracanalicular bile pigment, some glycogen-rich nuclei, and the presence of crystalloid nucleoids within the microbodies.
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