Adult Fanconi syndrom and liver cirrhosis

A female presenting primary adult Fanconi syndrome and liver cirrhosis of obscure aetiology is described. The only other report of this association appeared over four decades ago and concerned a male patient who, however, was subsequently diagnosed as having Wilson's cirrhosis, a well-known cause of acquired Fanconi syndrome. Therefore, the present report is the first account of primary Fanconi syndrome with authentic cryptogenic liver cirrhosis. Since the chronic urinary amino acid leakage is not a pathogenetic mechanism of the cirrhotic process, we believe that this association in fortuitous.