Progress in medical genetics. Volume 5 / edited by Arthur G. Steinberg and Alexander G. Bearn.

Date:
1967
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Credit: Progress in medical genetics. Volume 5 / edited by Arthur G. Steinberg and Alexander G. Bearn. Source: Wellcome Collection.

    110/168 page 98
    98 WALDENSTROM AND HAEGER-ARONSEN cause stimulation of the corresponding synthetase. At present it seems as if induction of ALA synthetase is the most Hkely explanation for porphyria acuta intermittens. Watson (1966) has recently discussed the hypothesis that a similar ALA synthetase induction could also explain erythropoietic porphyria, if this enzyme increase happens in the normo¬ blasts in the latter disease and in liver cells in the former. The reader who is interested in these problems should consult recent reviews by Rimington (1966) and by Watson (1966). While all these theories are very stimulating, it must be agreed that we still know too little about the basic mechanisms. The fact that spontaneous recovery is so common in porphyria acuta intermittens makes one believe that a correct picture of the biochemical mechanisms could lead to rapid restoration of health by correction of the basic biochemical disturbance. References Amoroso, E. C., R. M. Loosmore, C. Rimington, and B. E. Tooth. 1957. Congenital porphyria in bovines: first living case in Britain. Nature 180: 230. Barker, L. F., and W. L. Estes. 1912. Family hematoporphyrinuria and its association with chronic gastroduodenal dilatation, peculiar fits and acute polyneuritis. J.A.M.A. 59: 718. Barnes, H. D. 1958. Porphyria in South Africa: The faecal excretion of porphyrin. S. Afr. Med. J. 32: 680. —, and N. Whittaker. 1965. Hereditary coproporphyria with acute intermittent manifestations. Brit. Med. J. 2: 1102. Berger, H., and A. Goldberg. 1955. Hereditary coproporphyria. Brit. Med. ]. 2: 85. Berman, J., and A. Braun. 1962. Incidence of hepatoma in porphyria cutanea tarda. Rev. Czechoslovak. Med. 8: 290. Borst, M., and H. Königsdörffer Jr. 1929. Untersuchungen über Porphyrie mit besonaercr Berücksichtigung der Porphyria congenita. Leipzig: Verlag von S. Hirzel. Chaudhuri, A., J. N. Chaudhuri, and C. C. Chaudhuri. 1958. Congenital porphyria in siblings. Indian J. Pediat. 25: 157. Cowger, M. L., and R. F. Labbe. 1965. Contraindications of biological-oxidation inhibitors in the treatment of porphyria. Lancet i : 88. Curnow, D. H., E. H. Morgan, and G. A. Sarfaty. 1959. Acute intermittent porphyria. A family study. Aust. Ann. Med. 8: 267. Dean, G. 1963. The Porphyrias. A Story of Inheritance and Environment. London; Pitman Med. Pubi. Co. —. 1963. The prevalence of the porphyrias. S. Afr. J. Lab. Clin. Med. 9:145. —, and H. D. Barnes. 1958. Porphyria. A South African screening experiment. Brit. Med. J. i: 298. —, and —. 1959. Porphyria in Sweden and South Africa. S. Afr. Med. J. 33: 246. Derrien, E. 1926. Sur la biologie des porphyrines naturelles. Bull. Soc. Chim. Biol. 8: 218. Dobriner, K. 1936. Simultaneous excretion of coproporphyrin I and III in a case of chronic porphyria. Proc. Soc. Exp. Biol. Med. 35: 175.
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