The Enigma of Familial Polycystic Kidneys

Abstract

Familial polycystic kidney disease of the adult type, recognized for several centuries, has attracted the attention of clinicians, pathologists and nephrologists since Virchow first suggested in 1856¹ that the deposition of salts or casts caused obstruction of the renal tubules that was followed by cyst formation. Seventy years later Cairns² clearly established that the disorder was inherited. Subsequently, data were accumulated indicating that the gene responsible is an autosomal dominant that has virtually 100 per cent penetrance if the individual bearing it lives to the age of 80. Rarely manifest before the age of 20, the disease is most often . . .