Polycystic disease of kidney, liver and pancreas; a possible patholgenesis.

5,6,7,8-tetrahydrocarbzzole-3-acetic acid (AH 2835) given to maternal rats throughout their gestation produces an experimental model of the autosomally inherited human infantile polycystic disease Potter type I in the rat foetuses. The affected animals have cystic lesions in their kidneys, liver and pancreas like those seen in the human. Evidence is presented for the aetiology of the experimental lesion being related to the action of AH 2835 on the specific activity of the ouabain sensitive (Na+ + K+)-ATPase of absorptive epithelia. It is noted that two cystic kidney diseases, the ocngenital nephrotic syndrome and infantile polycystic disease Potter type I are both inherited as autosomal recessive traits and are therefore likely to be caused by enzyme abnormalities, and that the compound AH 2835 can be used to produce experimental models of both of these diseases.