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.
