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Hydroxychloroquine and phlebotomy were compared in the treatment of porphyria cutanea tarda (PCT). Thirty patients received hydroxychloroquine (200 mg twice weekly) for 1 year and thirty-one underwent twice-monthly phlebotomies of 400 ml whole blood each, also for 1 year. Clinical signs of disease improved equally in both groups. At the end of the year, urinary porphyrin excretion had significantly improved in twenty-two out of thirty hydroxychloroquine-treated subjects, but in only eight out of the thirty-one patients who received phlebotomy. Liver histology showed significant regression of steatosis and siderosis in both groups compared with the pretrial biopsy, but the activity of liver disease, as judged by the extent of necrosis, inflammation and fibrosis, worsened in twelve hydroxychloroquine and in seven phlebotomy-treated patients. It is concluded that hydroxychloroquine is more effective than phlebotomy in decreasing porphyrin production. However, further work is needed to assess whether long-term hydroxychloroquine treatment favours the progression of the chronic liver disease associated with PCT.

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Epidemiological investigations have revealed that alcoholic cirrhosis is associated with a high frequency of pigment gallstones, but only scanty information is available on the effects of ethanol on biliary secretion of bilirubin. We have injected intravenously 1.0 and 1.5 g/kg body wt of ethanol into six cholecystectomized rabbits and a common bile duct fistula. Experiments were performed ten days after surgery and a stream-splitting apparatus was interposed in the circuit in order to withdraw continuously biliary samples without interruption of enterohepatic bile circulation. Analysis of hourly data showed that both ethanol doses significantly increase the biliary concentration of total bilirubin, without affecting bile flow and lipid composition. Alcohol also promoted the efflux of unconjugated bilirubin into bile. The maximum effect occurred within the first 5 hr following alcohol administration. Thereafter the bile returned to normal. Since excessive concentrations of biliary unconjugated bilirubin favor pigment gallstone development, it can be speculated that alcohol acts as a risk factor for pigment lithiasis by enhancing the biliary levels of this form of pigment.