Diagnosis and Therapy of Porphyrias and Lead Intoxication: by U. A. Meyer (auth.), Prof. Dr. Manfred Doss (eds.)

By U. A. Meyer (auth.), Prof. Dr. Manfred Doss (eds.)

On the get together of the 450th anniversary of Philipp collage, the porphyrin study workforce of. the dept of medical Biochemistry within the college of drugs prepared a global symposium. This was once held among June 28 and July 1, 1977, less than the joint chairman­ send of Professor Samuel Schwartz and the editor. The organizers and chairmen of the symposium desire to thank the Sozialminister of Hessen, Armin Clauss, the President of the German Society for scientific Chemis­ try out, Professor A. Delbrlick, and the Dean of the college of drugs of Philipp college, Professor F. Neurath, for his or her aid and patronage. we're additionally thankful to Professor A. Treibs of the Institute of natural Chemistry, Technical college of Munich. Professor Treibs, a former scholar, co-worker and now successor to the Nobel laureate Hans Fischer, kindly authorized the honorary presidency of this foreign symposium. the outlet rite used to be inaugurated by means of the chairmen, with welcoming speeches via the President of Philipps collage, Mr. Rudolf Zingel and consumers of the college. The editor defined the ten years of improvement of medical biochemistry at Philipp collage and traced intimately the continuous improvement of its leader examine attempt in porphyria. It was once mentioned that following the 1st overseas symposium at the law of porphyrin and heme biosynthesis in 1973 at Marburg (Doss, M. (ed. ): legislation of Porphyrin and Heme Biosyn­ thesis.

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ALA-S activity was determined according to the method of Sinclair and Granick (6). C. Results Insulin and hydrocortisone were required for cells to spread and adhere to the incubation flask. More cells were retained in the presence of insulin than in its absence. Insulin was required not only for the growth of hepatocytes, but also for the induction of ALA-S and porphyrins by porphyrinogenic chemicals. For example, essentially no porphyrins were induced by allylisopropylacetamide (AlA) in cells grown in the serumfree medium without hormones (Table 1).

In the study of the family (Fig. , also in the feces (Table 8). L .. P. presented an activity of uro-S 15%-20% under the mean of the controls in the overlapping zone. In two grandchildren the Uro-S activity was decreased by 30%-35%, so that one can be sure of an AlP defect. L. 100% higher than the upper normal level), and (d) the clinical manifestation with high excretion of porphyrin precursors and porphyrins in a 67-year-old female patient, although there was no uro-S defect. Together with the results of the family study we at first placed this case among those of AIP.

We have investigated one of the four main lines of descent that, as is shown in Figure 1, extends over ten generations and conforms to the pattern of an autosomal dominantly inherited disorder of high expressivity and penetrance. The designation 'variegate,' which was first applied by Barnes and Dean (1) aptly described the various forms that the disease assumes. They are summarized with their percentage incidence in Table 1. As the title indicates, the first objective of this paper is to provide an account of the biochemical features of VP, but in view of the inordinately high local incidence of alcohol-associated SP and the clinically indistinguishable cutaneous manifestations of these two disorders, it is appropriate to consider the biochemical features of SP pari passu with those of VP.

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