Acute Intermittent Porphyria : a thesis and 68 follow-up publications 1964-2016

Lennart Wetterberg, M.D., Ph.D., was in 1973 elected both as a member of the Nobel Prize Assembly and as a Professor in Psychiatry at the Karolinska Institute, Stockholm, Sweden. He is now an Emeritus Professor. The book presents more than 50 years of research in porphyria. It starts with Wetterberg´s thesis “A Neuropsychiatric and Genetical Investigation of ACUTE INTERMITTENT PORPHYRIA”. The major hit was the link between AIP and mental illness. A 50-year follow-up of the 40 index cases gave eight gene variations. In 1964 a woman 26 y with an AIP crisis after oral contraceptives and in 1966 a man with an AIP attack after lead poisoning were reported as well as a young lady who died after anaesthesia. The mortality of the porphyrias in Sweden 1890-1956 is graphically compiled. Wetterberg reported the first broad list of unsafe drugs in AIP in 1975 and advised that AIP patients bring a card telling illness and unsafe drugs. The talk at the inauguration of the Swedish Porphyria Association Nov. 26, 1976, 40 years ago is added. His studies about glucose treatment in AIP, the Harderian gland as a model for hormonal regulation, and a kryptopyrrole-like compound in AIP patients of potential clinical importance, melatonin in AIP, and increased hepatocellular carcinoma in AIP are also reviewed. Cytochrome P-450 metabolism in the brain is a new field for research in AIP. Titles of Swedish academic theses about the porphyrias, web addresses and an author index of some four hundred AIP researchers are included. The book concludes with an update until 2016 regarding acute porphyria in other countries like Argentina, Finland, France, South Africa, Spain and Taiwan.