Copper Coproporphyrin Excretion in Familial Coproporphyria

Analysis of stool specimens from a patient with familial coproporphyria by high-performance liquid chromatography revealed that 112 μg/g of dry feces (14% of the total porphyrin present) was Cu coproporphyrin. Examination of stool specimens from other patients with this disease confirmed the presence of significant amtounts of both Cu coproporphyrin and coproporphyrin. Further investigation showed that the Cu coproporphyrin was probably formed by a nonenzymic incorporation of Cu in either the bile or feces.