Twenty-five patients with overt clinical and biochemical findings of porphyria cutanea tarda took part in a study comparing intensive phlebotomy with slow subcutaneous desferrioxamine treatment. Fifteen male patients (Group A) had intensive venesection therapy. Ten patients (Group B) with associated diseases (minor thalassemia, cardiovascular impairment, pulmonary tuberculosis or severe liver cirrhosis) received 1.5 g of desferrioxamine by slow subcutaneous infusion using an automatic syringe pump 5 days a week. No patient complained of appreciable side effects. Serum iron, ferritin and uroporphyrins were normalized in all subjects by the end of treatment. The mean time necessary for complete recovery was 13.8 months (range 9-19) and 11.2 months (range 6-14) in Groups A and B, respectively. Liver function significantly improved during and after the treatments in both groups. We conclude that recovery from porphyria cutanea tarda can be achieved equally well using phlebotomy or desferrioxamine subcutaneous infusion. Phlebotomy is easily performed and remains the treatment of choice; slow subcutaneous desferrioxamine treatment, although expensive, is recommended when severe associated diseases contra-indicate venesection.

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Based on the assumption that faecal and urinary coproporphyrin excretion is closely dependent on biliary function, coproporphyrin excretion was investigated in severe cholestatic diseases in infants and adults. The following subjects were investigated: biliary atresia (5), neonatal hepatitis (3), normal infants (11), adults with biliary obstruction (5) and normal adults (18). Urinary and faecal coproporphyrin concentrations were determined by solvent partition methods, and the isomers (I and III) were separated by thin-layer chromatography with direct spectrofluorometric scanning. The results showed a significant increase in urinary coproporphyrin in biliary atresia and neonatal hepatitis and in adults with biliary obstruction. All the cholestatic diseases showed the same marked increase in urinary isomer I. In biliary atresia and neonatal hepatitis there was a significant decrease in faecal coproporphyrin and a concomitant increase in isomer III (of bacterial origin) which was related to the extent of the biliary defect. Determination of urinary and faecal coproporphyrin, and particularly of the isomer distribution, may be a sensitive tool for diagnosis.

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The metabolic effects of selected and branched-chain amino acid (BCAA)-enriched parenteral solutions were studied in liver cirrhosis. After 3 days of an oral protein-free diet with balanced amino acid (AA) infusion, 36 cirrhotic patients without encephalopathy were randomly divided into four groups. Groups A and B were infused for 5 days with BCAA (valine, leucine, isoleucine) at doses of 0.5 and 1.0 g/kg/day, respectively, as the only nitrogen source. Group C received 0.8 g/kg of essential and nonessential AA solution with a prevalence of BCAA; the last group (D) continued the basic standard diet, as control. Routine chemistry, urinary nitrogen losses, nitrogen balance, and the whole plasma AA pattern were detected before and after the treatment period. BCAA alone led to an impressive and significant improvement in the basic AA pattern in both the A and B groups. The same results were obtained in group C for plasma AA. In particular, the ratio of BCAA to aromatic amino acids in groups A, B, and C was significantly increased (p less than 0.01, less than 0.02, less than 0.02, respectively). In group D the AA pattern and the BCAA/aromatic amino acid ratio remained unchanged. The negative nitrogen balance of the base state remained unchanged after 0.5 g of BCAA (A); it improved significantly and became positive during and after the infusions of a double dose of BCAA (B), as it did in the case of selective solutions (C), although to a lesser extent; the negative nitrogen balance of the control group showed only a slight improvement.(ABSTRACT TRUNCATED AT 250 WORDS)