Protoporphyrin metabolic balance in human protoporphyria. 1985

M B Poh-Fitzpatrick

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.

UI MeSH Term Description Entries
D008297 Male Males
D008875 Middle Aged An adult aged 45 - 64 years. Middle Age
D011164 Porphyrias A diverse group of metabolic diseases characterized by errors in the biosynthetic pathway of HEME in the LIVER, the BONE MARROW, or both. They are classified by the deficiency of specific enzymes, the tissue site of enzyme defect, or the clinical features that include neurological (acute) or cutaneous (skin lesions). Porphyrias can be hereditary or acquired as a result of toxicity to the hepatic or erythropoietic marrow tissues. Porphyria,Porphyrin Disorder,Disorder, Porphyrin,Disorders, Porphyrin,Porphyrin Disorders
D011166 Porphyrins A group of compounds containing the porphin structure, four pyrrole rings connected by methine bridges in a cyclic configuration to which a variety of side chains are attached. The nature of the side chain is indicated by a prefix, as uroporphyrin, hematoporphyrin, etc. The porphyrins, in combination with iron, form the heme component in biologically significant compounds such as hemoglobin and myoglobin. Porphyrin
D011524 Protoporphyrins Porphyrins with four methyl, two vinyl, and two propionic acid side chains attached to the pyrrole rings. Protoporphyrin IX occurs in hemoglobin, myoglobin, and most of the cytochromes.
D004905 Erythrocyte Aging The senescence of RED BLOOD CELLS. Lacking the organelles that make protein synthesis possible, the mature erythrocyte is incapable of self-repair, reproduction, and carrying out certain functions performed by other cells. This limits the average life span of an erythrocyte to 120 days. Erythrocyte Survival,Aging, Erythrocyte,Survival, Erythrocyte
D004911 Erythrocyte Volume Volume of circulating ERYTHROCYTES . It is usually measured by RADIOISOTOPE DILUTION TECHNIQUE. Red Cell Mass,Erythrocyte Volumes,Mass, Red Cell,Masses, Red Cell,Red Cell Masses,Volume, Erythrocyte,Volumes, Erythrocyte
D004912 Erythrocytes Red blood cells. Mature erythrocytes are non-nucleated, biconcave disks containing HEMOGLOBIN whose function is to transport OXYGEN. Blood Cells, Red,Blood Corpuscles, Red,Red Blood Cells,Red Blood Corpuscles,Blood Cell, Red,Blood Corpuscle, Red,Erythrocyte,Red Blood Cell,Red Blood Corpuscle
D005243 Feces Excrement from the INTESTINES, containing unabsorbed solids, waste products, secretions, and BACTERIA of the DIGESTIVE SYSTEM.
D005260 Female Females

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