[Chronic nephritis--porphyrin biosynthesis and nephrogenic anemia]. / Chronische Nephritis--Biosynthese von Porphyrinen und nephrogene Anämie.

Disturbances in the biosynthesis of porphyrins in erythrocytes were investigated in 50 patients with chronic nephritis after incubation with delta-aminolevulinic acid. A medium to strong correlation was found among the parameters of porphyrin metabolism and those of haemoglobin synthesis and the functional state of the kidneys. This gives rise to the assumption that the disturbances in porphyrin biosynthesis are to be classified among the pathogenetic mechanisms of anaemia in the course of chronic nephritis.

[Uroporphyrinogen-I-synthetase in erythrocytes in acute intermittent porphyria]. / Uroporphyrinogen-I-Synthetase in den Erythrozyten bei akuter intermittierender Porphyrie.

The primary genetic defect in acute intermittent porphyria is a decreased uroporphyrinogen I-synthetase [EC.4.3.1.8] activity. As a beginning of a genealogical study of the known families with members suffering from this disease in the People's Republic of Bulgaria, the red cell uroporphyrinogen I-synthetase was determined in 3 families by the method of Mandel et al [8]. Except for the three propositi, an enzyme deficiency was established in 3 latent carriers of the pathological gene, two of whom had normal values of the urinary epsilon-aminolevulinic acid and porphobilinogen. The determination of red cell uroporphyrinogen I-synthetase proved to be a valuable parameter for revealing the latent AIP.