The chance of being homozygous for both diseases is, therefore, one in seven million. Alternatively, of every 200 patients with Wilson's disease, one will carry two genes mutated for hereditary haemochromatosis. As the principal site of toxic-metal storage is the liver in both diseases, it is expected that a patient homozygous for both diseases might carry a very poor prognosis.
Further studies are in progress to elucidate the cause of the overload.
A man of 40 years became ill when he was 25 with change of personality, slurred speech, and clumsy hands. This progressed to severe mental illness, aphonia, and tremor. His younger sister was known to have Wilson's disease, which had presented with haemolysis and had been successfully managed with trientine. His first liver biopsy showed established cirrhosis with grade 2 iron overload. He had raised serum iron binding saturation, and normal ferritin.