Description: Segment 1 Dr Gilliland introduces Dr Gompertz and the topic of the organic acidaemias. Dr Gompertz explains what the organic acidaemias are - a second generation of inborn errors of amino acid metabolism. He also discusses the first generation of inborn errors of amino acid metabolism and the diseases associated with them. Charts show the degradation pathways of the three branched-chain amino acids leucine, isoleucine and valine and Gompertz talks about the metabolic reactions of these pathways and the organic acidaemias associated with them. Time start: 00:00:00:00 Time end: 00:05:02:00 Length: 00:05:02:00 Segment 2 Gompertz continues to talk about the leucine degradation pathway and episodes of acute acidosis associated with it. He describes various patient case studies who have presented with second generation inborn errors of amino acid metabolism. He uses charts to describe the three small molecular weight intermediates that finish the degradation pathways and the function of enzymes in the pathways. Time start: 00:05:02:00 Time end: 00:10:20:00 Length: 00:05:18:00 Segment 3 The inborn error of propionic acidaemia is discussed. Gompertz mentions a case study of two babies who had untreatable metabolic acidoses and died within two weeks. He then turns to discussing how the organic acidaemias present in patients, describing symptoms and types of disease. He shows photographs of two children who both developed diseases and talks about their cases. Time start: 00:10:20:00 Time end: 00:15:34:22 Length: 00:05:14:22 Segment 4 Gompertz summarises the three possible presentations of organic acidaemias and moves on to the treatment of these conditions. A chart shows the effects of treatment by peritoneal dialysis on a newborn. He also talks about treatment with massive doses of vitamin B12. Charts help explain how this treatment works. Time start: 00:15:34:22 Time end: 00:19:31:15 Length: 00:03:56:18 Segment 6 Next, Gompertz talks about the inheritance of these conditions, saying that many are assumed to be autosomal recessive and that there is a high degree of consanguinity in these families. A family tree of a family with propionic acidaemia is seen. The family tree has two first cousin marriages. Gompertz also talks about the prognosis for children affected by these conditions and how the conditions affect their mental capabilities. He finishes the lecture by summarising the points made within it. Time start: 00:24:25:00 Time end: 00:29:26:24 Length: 00:05:01:24