Clinical disorders of porphyrin metabolism: Part 2
digital file Black & White Sound 1972 52:47
Summary: The second part of a talk by Professor Abe Goldberg from the University of Glasgow. A summary accompanying the cassette says: Haems and porphyrins are present in every living cell of the body and disorders of their metabolism can take place in a number of clinical states. Acute intermittent porphyria is the most important of the porphyria disorders, due to a defect of an hepatic enzyme and presenting with neuro-psychiatric manifestations, hypertension and severe abdominal pain. The disease can be provoked by drugs such as barbiturates, and an endogenous abnormality of 17-oxosteroid metabolism has been identified in a majority of cases. The cutaneous porphyrias present with skin photosensitivity, due to a defect in the liver, sometimes associated with alcoholism or hepatic cirrhosis and sometimes with excessive porphyrin production in erythrocyte precursors of the bone marrow. Abnormalities of porphyrin metabolism also occur in lead poisoning, a number of anaemias, including the secondary anaemias of infectin and neoplasm, and the sideroachrestic anaemias.
Title number: 18381
LSA ID: LSA/21538
Description: Segment 1 Goldberg introduces the second lecture. He talks about porphyria and describes how it can be passed on genetically. Time start: 00:00:00:00 Time end: 00:05:39:00 Length: 00:05:39:00 Segment 2 We see a photograph of the hands and face of a female with porphyria. He reads from a postcard and a journal of a woman suffering from porphyria. Time start: 00:05:39:00 Time end: 00:11:25:17 Length: 00:05:46:17 Segment 3 Goldberg shows a slide of a teenager with porphyria after eating bread contaminated with hexachlorobenzene. He discusses hexachlorobenzene. He talks about the inherited aspects of porphyria and shows a family tree consisting of people suffering from the condition. Time start: 00:11:25:17 Time end: 00:17:15:00 Length: 00:05:49:06 Segment 4 We are shown a family tree of hereditary coproporphyria and slides of journal pages which feature a photograph of a man with erythropoietic porphyria. Time start: 00:17:15:00 Time end: 00:22:45:14 Length: 00:05:30:14 Segment 6 We see how lines in the gum can be indicative of porphyria. Some of the symptoms of the disease can be the same as in acute lead poisoning and Goldberg compares the two. Time start: 00:29:16:19 Time end: 00:31:23:23 Length: 00:02:07:04. Segment 7 Goldberg discusses the problem of lead in water. He refers to studies of this in groups of households in Glasgow. Time start: 00:31:23:23 Time end: 00:36:13:00 Length: 00:04:49:05. Segment 8 We hear about the results of a test in which lead was fed to rats, then lead levels in their organs measured. Goldberg shows a chart which compares levels of protoporphyrin in healthy patients compared to those with iron deficiency anaemia, then after the anaemic patients have been treated with ferrous sulphate. Time start: 00:36:13:00 Time end: 00:42:09:00 Length: 00:05:56:00. Segment 9 Goldberg shows a photomicrograph of the bone marrow of a patient with sideroachrestic anaemia. Then slides showing erythrocyte protoporphyrin levels in various disorders. Time start: 00:42:09:00 Time end: 00:48:10:00 Length: 00:06:01:00. Segment 10 Goldberg concludes the lecture by returning to a photograph of a woman with porphyria, now lying in a hospital bed. She eventually died and he suggests that this was preventable with further research. Time start: 00:48:10:00 Time end: 00:52:47:05 Length: 00:04:37:05
Credits: Presented by Professor Abe Goldberg, University of Glasgow. Produced by Peter Bowen.
Further information: This video is one of more than 120 titles, originally broadcast on Channel 7 of the ILEA closed-circuit television network, given to Wellcome Trust from the University of London Audio-Visual Centre shortly after it closed in the late 1980s. Although some of these programmes might now seem rather out-dated, they probably represent the largest and most diversified body of medical video produced in any British university at this time, and give a comprehensive and fascinating view of the state of medical and surgical research and practice in the 1970s and 1980s, thus constituting a contemporary medical-historical archive of great interest. The lectures mostly take place in a small and intimate studio setting and are often face-to-face. The lecturers use a wide variety of resources to illustrate their points, including film clips, slides, graphs, animated diagrams, charts and tables as well as 3-dimensional models and display boards with movable pieces. Some of the lecturers are telegenic while some are clearly less comfortable about being recorded all are experts in their field and show great enthusiasm to share both the latest research and the historical context of their specialist areas.
Keywords: Heme; Porphyrins; Porphyrias; Hexaclorophine
Locations: United Kingdom; England; London; University of London