Some cases of congenital porphyrinuria in cattle: chemical studies upon the living animals and post-mortem material

Abstract

Of several living bovine cases of congenital porphyrinuria, discovered on a farm in Swaziland and all the progeny of a single pure-bred shorthorn bull (see Fourie, 1936), one animal was slaughtered for experimental purposes. This case, a castrated male, 2 years 4 months old, showed definite clinical symptoms of photosensitisation and passed a port wine-red coloured urine, exhibiting porphyrin absorption bands. The bones were found to be coloured a mahogany brown and on transverse section, concentric rings of lighter and deeper pigmentation were seen. The cartilages were normal. Employing, in general, Fischer's methods, the individual organs and tissues were examined for porphyrins and pure crystalline materials (methyl esters) obtained as follows: Urine, Uroporphyrin (275-7°) Coproporphyrin I (233-5°); Faeces, Coproporphyrin I (243-4°) and its Copper complex; Blood plasma, Coproporphyrin I (243-5°); Erythrocytes, Coproporphyrin I (241°); Bones, Uroporphyrin (276-7°) and from a small sample derived from another case Uroporphyrin (273-4°); these esters had copper complexes 311-4° and 310-3° respectively. From the mother liquors of the main crystallisation was isolated an Uroporphyrin with ester M.P. 253-5° but yielding a normal copper salt. From Bone Marrow, Uroporphyrin (278°) and Coproporphyrin I (244-5°); Spleen, Uroporphyrin (278°) ; Liver, Uroporphyrin together with its copper complex (313°); Bile, Coproporphyrin I (237°). In other instances the yields of pure pigment were too small for identification by other than spectroscopic measurements (see chart in text of paper). The significance of these pigments, belonging to the I series of porphyrins, is discussed in relation to normal haemoglobin synthesis and catabolism and the derangements of pigment metabolism occurring in disease and certain states of intoxication such as lead, sulphonal poisoning, etc. A suggestion is made as to the nature of the anomaly in congenital porphyrinuria and a provisional scheme of pigment metabolism mapped out. I wish to thank Mr . G. Roets, B.Sc., for his generous assistance in the laborious task of working up the large quantities of material employed in this investigation and my colleague, Dr. Fourie, for the benefit of many discussions.