The clinical and biochemical features of variegate porphyria: An analysis of 300 cases studied at Groote Schuur Hospital, Cape Town

The main clinical features of 300 cases of VP in respect of their racial and sex incidence and age and mode of presentation, as well as their diagnostic biochemical features have been presented. The abundance of clinical material made it possible to undertake 7 special studies. The 2 most important of these were: (1) the acute attack and its electrolyte disorder, a very high incidence of severe hyponatremia, hypochloremia and azotemia, and a lesser but substantial incidence of alkalosis and hypokalemia. The causes of the electrolyte disorder have been extensively investigated. (2) The structure of the porphyrin peptides and their diagnostic significance have been defined. © 1980.