Diagnosis and Therapy of Porphyrias and Lead Intoxication

1. Front Matter

   Pages I-XXI

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2. Pathobiochemistry of Heme Metabolism

   1. Front Matter

      Pages 1-1

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   2. Regulation of Heme Biosynthesis in Hepatic and Erythroid Cells: A Brief Introduction to the Pathobiochemistry of Human Porphyrias

      • U. A. Meyer

      Pages 3-7

   3. Differential Patterns of Heme and Prophyrin Biosynthesis in Liver and Kidney: Metal Ion Blockade of δ-Aminolevulinate Synthetase Introduction by Porphyrinogenic Agents

      • M. D. Maines, M. J. Tomio, V. Janousek

      Pages 8-14

   4. The Regulatory Function of the Mitochondrial Inner Membrane in the Overall Ferrochelatase Reaction

      • I. Romslo, M. E. Koller

      Pages 15-21

   5. Permissive Effects of Hormones on the Induction of δ-Aminolevulinic Acid Synthase in Cultured Chick-Embryo Liver Cells

      • S. Sassa, A. Kappas

      Pages 22-25

3. Pathogenesis and Differential Diagnosis of Acute Hepatic Porphyrias

   1. Front Matter

      Pages 27-27

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   2. Biochemical and Clinical Transitions Between the Hereditary Hepatic Porphyrias: New Concepts

      • M. Doss, R. v. Tiepermann

      Pages 29-45

   3. A New Approach to Porphyria

      • C. A. Pierach, Z. J. Petryka

      Pages 46-50

   4. The Clinical Chemistry of Variegate Porphyria with Special Reference to the Identification of a New Plasma-Marker Porphyrin

      • L. Eales, R. S. Day, N. R. Pimstone

      Pages 51-65

   5. Expression of the Gene Defects of Acute Intermittent Porphyria (AIP) and Erythropoietic Protoporphyria (EPP) in Mitogen-Stimulated Lymphocytes

      • S. Sassa, G. L. Zalar, A. Kappas

      Pages 66-69

   6. Uroporphyrinogen-Synthase (Uro-S) in Erythrocytes in Acute Intermittent Porphyria (AIP)

      • R. v. Tiepermann, M. Doss

      Pages 70-75

   7. Hereditary Coproporphyria: Demonstration of a Genetic Defect in Coproporphyrinogen Metabolism

      • Y. Nordmann, B. Grandchamp

      Pages 76-81

4. Clinical Course and Therapy of Acute Hepatic Porphyrias

   1. Front Matter

      Pages 83-83

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   2. A Case of Acute Intermittent Porphyria, Relapsing Acute Pancreatitis, and Unconjugated Hyperbilirubinemia (Gilbert’s Syndrome)

      • K. Neuhaus, K. Kobza, K. Gyr, F. Gudat

      Pages 85-86

   3. Acute Intermittent Porphyria; EEG Changes and Their Relationship to the Biochemical Manifestation

      • E. Mühler

      Pages 87-93

   4. Family Studies of Patients with Acute Intermittent Porphyria (AIP): Neurological, Biochemical, and Genetic Investigations

      • K. F. Leonhardt, R. v. Tiepermann, M. Doss

      Pages 94-99

   5. The Effect of Hematin in “Inducible” Hepatic Porphyria

      • C. J. Watson, C. A. Pierach, I. Bossenmaier, R. Cardinal

      Pages 100-102

   6. Therapy of Acute Intermittent Porphyria With Ovulation Inhibitors in Women

      • G. Schley, W. Hengstebeck, K. D. Bock

      Pages 103-104

5. Pathogenesis, Clinical Biochemistry, and Treatment of Chronic Hepatic Porphyrias

   1. Front Matter

      Pages 105-105

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On the occasion of the 450th anniversary of Philipp University, the porphyrin research group of. the Department of Clinical Biochemistry in the Faculty of Medicine organized an international symposium. This was held between June 28 and July 1, 1977, under the joint chairman­ ship of Professor Samuel Schwartz and the editor. The organizers and chairmen of the symposium wish to thank the Sozialminister of Hessen, Armin Clauss, the President of the German Society for Clinical Chemis­ try, Professor A. Delbrlick, and the Dean of the Faculty of Medicine of Philipp University, Professor F. Neurath, for their support and patronage. We are also grateful to Professor A. Treibs of the Institute of Organic Chemistry, Technical University of Munich. Professor Treibs, a former pupil, co-worker and now successor to the Nobel laureate Hans Fischer, kindly accepted the honorary presidency of this international symposium. The opening ceremony was inaugurated by the chairmen, with welcoming speeches by the President of Philipps University, Mr. Rudolf Zingel and patrons of the university. The editor outlined the 10 years of development of clinical biochemistry at Philipp University and traced in detail the continual development of its chief research effort in porphyria. It was pointed out that following the first international symposium on the regulation of porphyrin and heme biosynthesis in 1973 at Marburg (Doss, M. (ed. ): Regulation of Porphyrin and Heme Biosyn­ thesis.

• absorption
• anemia
• biochemistry
• carcinoma
• chemistry
• diagnosis
• diagnostics
• differential diagnosis
• embryo
• metabolism
• pathogenesis
• research
• therapy

• Department of Clinical Biochemistry, Faculty of Medicine, Philipp University of Marburg, Marburg, Federal Republic of Germany

  Manfred Doss

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