Disorders of Porphyrin Metabolism (Topics in Hematology)

Each of the four authors of this book has a particular interest in disorders of porphyrin metabolism and special experience in their management. Their individual involvement in the field varies from 12 to 52 years and, combined, represents more than a century of personal experience. Since it has been written by both basic scientists and practicing physicians, the book is intended to be of value to all those involved in porphyrin metab­ olism and the porphyrias. It is hoped that the fascination of porphyrin metabolism and the clinical challenge of the porphyrias experienced by each of the authors will be conveyed to the readers. Michael R. Moore Kenneth E. L. McColl Claude Rimington Abraham Goldberg vii CONTENTS Color Plates ............................................ xvii 1. The History, Classification, and Incidence of the Porphyrias 1 1.1. History ........................................ 1 1.1.1. Early Chemistry . . . . . . . . . . . . . . . . . . . . . . . . . . . 2 1.1.2. Early Descriptions of Porphyria .............. 4 1.1.3. Biochemical Developments .................. 4 1.1.4. Acute Porphyria . . . . . . . . . . . . . . . . . . . . . . . . . . . 7 1.1.5. A Complete Pathway ...................... 8 1.2. Classification of the Porphyrias . . . . . . . . . . . . . . . . . . . . . 9 1.2.1. The Current Classification. . . . . . . . . . . . . . . 12 . . . . Each of the four authors of this book has a particular interest in disorders of porphyrin metabolism and special experience in their management. Their individual involvement in the field varies from 12 to 52 years and, combined, represents more than a century of personal experience. Since it has been written by both basic scientists and practicing physicians, the book is intended to be of value to all those involved in porphyrin metabƯ olism and the porphyrias. It is hoped that the fascination of porphyrin metabolism and the clinical challenge of the porphyrias experienced by each of the authors will be conveyed to the readers. Michael R. Moore Kenneth E.L. McColl Claude Rimington Abraham Goldberg vii CONTENTS Color Plates ... xvii 1. The History, Classification, and Incidence of the Porphyrias 1 1.1. History ... 1 1.1.1. Early Chemistry ... ... ... ... ... 2 1.1.2. Early Descriptions of Porphyria ... 4 1.1.3. Biochemical Developments ... 4 1.1.4. Acute Porphyria ... ... ... ... ... 7 1.1.5. A Complete Pathway ... 8 1.2. Classification of the Porphyrias ... ... ... ... 9 1.2.1. The Current Classification. ... ... . . 12 .. Front Matter....Pages i-xxi The History, Classification, and Incidence of the Porphyrias....Pages 1-20 Porphyrins and Enzymes of the Heme Biosynthetic Pathway....Pages 21-72 The Acute Porphyrias....Pages 73-117 Pathogenesis of the Neuropathy of Acute Porphyria....Pages 119-137 Drugs, Chemicals, and Porphyria....Pages 139-165 The Photochemistry, Photobiology, and Phototherapeutic Use of Porphyrins....Pages 167-177 Cutaneous Hepatic Porphyria....Pages 179-199 Erythropoietic Protoporphyria....Pages 201-211 Congenital Porphyria....Pages 213-226 Porphyrins in Diseases Other than the Porphyrias....Pages 227-255 Porphyria in Animals....Pages 257-277 Perspective....Pages 279-289 References....Pages 291-361 Back Matter....Pages 363-374