Patients with porphyria cutanea tarda can present with bullous skin lesions, photosensitivity and hypertrichosis and usually respond to venesection therapy. Patients with portacaval shunts will accumulate iron but much less than seen in hemochromatosis and do not usually require iron depletion therapy. Most patients will report an improvement in their energy level and progressive liver disease and heart failure can be prevented by iron depletion even in the cirrhotic patient. It is also of interest that at this major liver transplant centre, with a large collection of hemochromatosis patients, we have yet to transplant a patient with hereditary hemochromatosis. Siblings have a 1 in 4 risk of being affected and can be readily screened for hemochromatosis with a serum ferritin and transferrin saturation.
Children are unlikely to be affected unless the other parent is an occult heterozygote.
They can be screened as teenagers with a serum ferritin and transferrin saturation. Back Although screening with ultrasound and alpha-fetoprotein has led to earlier detection of small hepatocellular carcinomas, the therapeutic options even with an early diagnosis are often not curative. Hepatocellular carcinoma is not an ideal indication for transplantation and is most successful when an unsuspected tumour is discovered by the pathologist after the liver has been transplanted for cirrhosis.10 Although there continues to be enthusiasm for screening, available studies have not shown that this is a cost-effective approach. This is not significantly different than studies in patients with hepatitis B and hepatitis C cirrhosis and is further evidence that it is the cirrhosis rather than the iron which is the major risk factor. Surgical resection is the preferred treatment for the small solitary nodule in a young healthy patient with compensated cirrhosis.