Copper coproporphyrin excretion in familial coproporphyria.

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