Thin layer chromatography and counter-current analysis in porphyrias.
The porphyrin metabolism of 100 patients with porphyria and 351 of their relatives has been studied. Thin layer chromatography of methyl esters of the urinary porphyrin was undertaken in sixty-six patients with different types of porphyria, and forty-five relatives, seventeen patients with hepatic cirrhosis, three patients with lead poisoning and twenty normal control subjects. This investigation was also made on the stools of thirty-six patients with porphyria, and then of their relatives. Countercurrent analysis of the bile of nine selected patients with porphyria was also undertaken. The results provide some evidence that symptomatic hepatic porphyria may be familial. Thin layer chromatography was decisive in the characterization of a new type of porphyria described recently by the authors (hepato-erythrocytic porphyria). The counter-current examination of the bile showed the absence of the 'S 411' porphyrin in all the nine cases investigated.
D001646:Bile / Q000032:analysis; D002855:Chromatography, Thin Layer; D003377:Countercurrent Distribution; D005243:Feces / Q000032:analysis; D006801:Humans; D007855:Lead Poisoning / Q000378:metabolism; D008103:Liver Cirrhosis / Q000378:metabolism; D011164:Porphyrias / Q000145:classification / Q000235:genetics / Q000378:metabolism*; D011166:Porphyrins / Q000032:analysis* / Q000652:urine
D011166:Porphyrins