Protoporphyrin metabolic balance in human protoporphyria.
Short-term maintenance of different stable steady state balances for protoporphyrin production, accumulation in erythrocytes and plasma, and fecal excretion was demonstrated in 5 patients with uncomplicated protoporphyria in whom red blood cell, plasma, and fecal protoporphyrin levels were determined on 5-7 sequential days. The patients were hospitalized in a research unit for dietary control and standardization of specimen collection. Each patient was found to quantitatively partition protoporphyrin among these compartments in a pattern that was reproducible from day to day, and characteristic for that patient. These observations partially substantiate a previously proposed hypothesis that protoporphyrin partitioning patterns may provide a criterion for prospective classification of these patients into groups that may ultimately be shown to have different risk for development of symptomatic liver disease.
D000328:Adult; D004905:Erythrocyte Aging; D004911:Erythrocyte Volume; D004912:Erythrocytes / Q000378:metabolism; D005243:Feces / Q000032:analysis; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D011164:Porphyrias / Q000097:blood / Q000378:metabolism*; D011166:Porphyrins / Q000097:blood*; D011524:Protoporphyrins / Q000097:blood* / Q000378:metabolism; D012871:Skin Diseases / Q000097:blood / Q000378:metabolism*
D011166:Porphyrins; D011524:Protoporphyrins