Clinical disorders of porphyrin metabolism: Part 1
digital file Black & White Sound 1972 40:31
Summary: This is the first 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: 18388
Description: Segment 1 Gilliland introduces Goldberg. Goldbert shows a picture of a female patient lying in a hospital bed and tells her case history. Time start: 00:00:00:00 Time end: 00:06:11:15 Length: 00:06:11:15 Segment 2 Goldberg talks in detail about the chemical properties of porphobilinogen and haem. He then refers to a slide detailing the different presentations of porphyria. Time start: 00:06:11:15 Time end: 00:09:46:00 Length: 00:03:35:00 Segment 3 Goldberg shows a bar chart detailing the different presenting symptoms of porphyria. He then shows a slide which reveals the course of porphyria and a diagram detailing the clinical manifestations of the disease. Time start: 00:09:46:00 Time end: 00:15:36:00 Length: 00:05:50:00 Segment 4 We see a picture of a patient with paresis of the hands. Goldberg refers to slides of photomicrographs of specimens from patients with porphyria. Time start: 00:15:36:00 Time end: 00:20:03:00 Length: 00:04:27:00 Segment 6 Goldberg talks about the gender and age factors that make porphyria more likely to occur. He shows graphs and charts which measure these factors. Time start: 00:25:16:00 Time end: 00:29:29:00 Length: 00:04:13:00. Segment 7 We see a photograph of a mother, baby and child. The mother had had porphyria but has survived and leads a good life.He then discusses four out of his thirty patients in a twenty year follow upstudy, who died. Time start: 00:29:29:00 Time end: 00:34:32:14 Length: 00:05:03:14. Segment 8 We see a slide of an intubated male patient who has had an acute attack of porphyria and is lying in a hospital bed, intubated. Goldberg describes the typical treatment for acute porphyria. Time start: 00:34:32:14 Time end: 00:40:31:14 Length: 00:05:59:14
Credits: Presented by Professor Abe Goldberg, University of Glasgow. Introduced by Ian Gilliland. 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
Locations: United Kingdom; England; London; University of London