Biomaterial Database

[Urinary excretion of porphyrins and their precursors. Longterm observations in acute intermittent porphyrias (author's transl)]. 1979

J Brugsch, and H Müller

On the basis of many years of examination and supervision of 26 patients with acute intermittent porphyria three different types of excretion behavior in the urine can be distinguished. 1. After porphyric crises the urinary porphyrin content is completely normalized. 2. Even after the crisis the excretion levels for porphyrins remain very high. 3. The urinary porphyrins return to normal levels but the increases in precursors such as delta-aminolevulinic acid and porphobilinogen still persist. 4. In the diagnosis true acute coproporphyria and increased of excretion of precursors must be differentiated as well as increased coproporphyrin resulting from liver diseases, exogenic, toxic influences and secondary disorders of porphyrin metabolism.

UI	MeSH Term	Description	Entries
D011162	Porphobilinogen
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
D003306	Coproporphyrins	Porphyrins with four methyl and four propionic acid side chains attached to the pyrrole rings. Elevated levels of Coproporphyrin III in the urine and feces are major findings in patients with HEREDITARY COPROPORPHYRIA.
D004967	Estrogens	Compounds that interact with ESTROGEN RECEPTORS in target tissues to bring about the effects similar to those of ESTRADIOL. Estrogens stimulate the female reproductive organs, and the development of secondary female SEX CHARACTERISTICS. Estrogenic chemicals include natural, synthetic, steroidal, or non-steroidal compounds.	Estrogen,Estrogen Effect,Estrogen Effects,Estrogen Receptor Agonists,Estrogenic Agents,Estrogenic Compounds,Estrogenic Effect,Estrogenic Effects,Agents, Estrogenic,Agonists, Estrogen Receptor,Compounds, Estrogenic,Effects, Estrogen,Effects, Estrogenic,Receptor Agonists, Estrogen
D006801	Humans	Members of the species Homo sapiens.	Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000622	Aminolevulinic Acid	A compound produced from succinyl-CoA and GLYCINE as an intermediate in heme synthesis. It is used as a PHOTOCHEMOTHERAPY for actinic KERATOSIS.	5-Amino Levulinic Acid,5-Aminolaevulinate,5-Aminolevulinate,Aminolevulinic Acid Hydrochloride,Delta-Aminolevulinic Acid,Levulan,5 Amino Levulinic Acid,5 Aminolaevulinate,5 Aminolevulinate,Acid Hydrochloride, Aminolevulinic,Acid, 5-Amino Levulinic,Acid, Aminolevulinic,Acid, Delta-Aminolevulinic,Delta Aminolevulinic Acid,Hydrochloride, Aminolevulinic Acid,Levulinic Acid, 5-Amino
D014577	Uroporphyrinogens	Porphyrinogens which are intermediates in heme biosynthesis. They have four acetic acid and four propionic acid side chains attached to the pyrrole rings. Uroporphyrinogen I and III are formed from polypyrryl methane in the presence of uroporphyrinogen III cosynthetase and uroporphyrin I synthetase, respectively. They can yield uroporphyrins by autooxidation or coproporphyrinogens by decarboxylation.	Uroporphyrinogen III
D014578	Uroporphyrins	Porphyrins with four acetic acid and four propionic acid side chains attached to the pyrrole rings.