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Porphyrin synthesis and metabolism in iron deficiency anaemia. I. In-vivo-studies

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Summary

The results of studies done to ascertain whether the rate of protoporphyrin synthesis from δ-aminolaevulic acid is decreased in iron deficiency anaemia and whether iron is essential for the synthesis of the intermediate compounds in the porphyrin metabolism are reported. By measuring the urinary δ-amino-laevulic acid, porphobilinogen, coproporphyrin and uroporphyrin excretion and the faecal coproporphyrin and protoporphyrin excretion a decrease in the rate of porphyrin synthesis was demonstrated in cases of idiopathic hypochromic anaemia. The relative increase in the urinary porphobilinogen excretion points to a partial block of porphyrin synthesis from porphobilinogen. Iron treatment normalises the porphyrin metabolism.

Zusammenfassung

Die Ergebnisse von Untersuchungen zur Feststellung, ob die Höhe der Protoporphyrinsynthese aus δ-Aminolävulinsäure bei Eisenmangelanämie geringer ist und ob Eisen für die Synthese von Zwischenprodukten im Porphyrinstoffwechsel wesentlich ist, werden berichtet. Durch das Messen von δ-Aminolävulinsäure im Harn, Porphobilinogen-, Koproporphyrin- und Uroporphyrinausscheidung im Harn und die fäkale Ausscheidung von Koproporphyrin und Protoporphyrin wurde ein Absinken der Höhe der Porphyrinsynthese bei Fällen von idiopathischer hypochromer Anämie nachgewiesen. Der relative Anstieg in der Porphobilinogenausscheidung im Harn deutet auf eine Teilblockierung der Porphyrinsynthese von Porphobilinogen. Eisenbehandlung normalisiert den Porphyrinstoffwechsel.

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Prato, V., Mazza, U., Massaro, A.L. et al. Porphyrin synthesis and metabolism in iron deficiency anaemia. I. In-vivo-studies. Blut 16, 333–341 (1968). https://doi.org/10.1007/BF01632077

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