In describing the clinical, biochemical, and family findings in five c
hildren with porphyria, we examine initial treatments and, where appro
priate, the effectiveness of long-term therapy. We note that porphyria
diagnosis, particularly in childhood, relies heavily on specialist la
boratory investigations. Because disease expression in some porphyrias
requires exposure to precipitating factors, it may be prevented or de
layed by their avoidance.