R. Thompson: intrahepatic cholestasis

Today we are talking to Prof. (and Dr.) Richard Thompson, of London’s King’s College Hospital, who there for twenty-five years has broadened and heightened his leading role in studies of the genetics, physiology, and treatment of forms of intrahepatic cholestasis, disorders that come to clinical attention principally in childhood. Richard and Alex for fifteen years were on the same team at King’s, sometimes shoulder to shoulder, sometimes pushmi-pullyu, but always having fun. Their chat today, after rather a lot of reciprocal congratulation, touches on clinicogenetic correlations in Wilson disease, moving thence to bile salt export pump deficiency – first, with its spectrum of manifestations, as a paradigm of the falsity of Mendelian genetics ; second, with its responses to drugs that inhibit ileal bile acid transport, as a paradigm of how without fixing a programme’s basic code one can apply patches that substantially improve the programme’s utility (that is, how knowledge of clinicogenetic correlations permits patient-tailored choices among surgical and pharmacologic therapies, with earlier and more satisfactory improvements in health). Be warned : You won’t take on board in one hearing what Richard has to share. Make time, then, for a couple of listen-throughs. They will reward you well.