Urinary excretion of D-glucaric acid, total glucuronic acid and total porphyrins in porphyria cutanea tarda. 1978

G Budillon, and F Ayala, and M Carrella, and G Mazzacca

The urinary levels of D-glucaric acid, which is an index of hepatic microsome induction, and the excretion of glucuronic acid and porphyrins were measured in nine patients with Porphyria Cutanea Tarda (PCT) and twelve normal controls. The excretion of D-glucaric acid and glucuronic acid were respectively 3.5 and two times higher in PCT patients compared to controls. A statistical correlation could be demonstrated between urinary excretion of total porphyrins with that of glucaric and glucuronic acids. These findings indicate that microsomal function and porphyrin metabolic derangement are strictly related in PCT.

UI MeSH Term Description Entries
D008103 Liver Cirrhosis Liver disease in which the normal microcirculation, the gross vascular anatomy, and the hepatic architecture have been variably destroyed and altered with fibrous septa surrounding regenerated or regenerating parenchymal nodules. Cirrhosis, Liver,Fibrosis, Liver,Hepatic Cirrhosis,Liver Fibrosis,Cirrhosis, Hepatic
D008297 Male Males
D008861 Microsomes Artifactual vesicles formed from the endoplasmic reticulum when cells are disrupted. They are isolated by differential centrifugation and are composed of three structural features: rough vesicles, smooth vesicles, and ribosomes. Numerous enzyme activities are associated with the microsomal fraction. (Glick, Glossary of Biochemistry and Molecular Biology, 1990; from Rieger et al., Glossary of Genetics: Classical and Molecular, 5th ed) Microsome
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
D005234 Fatty Liver Lipid infiltration of the hepatic parenchymal cells resulting in a yellow-colored liver. The abnormal lipid accumulation is usually in the form of TRIGLYCERIDES, either as a single large droplet or multiple small droplets. Fatty liver is caused by an imbalance in the metabolism of FATTY ACIDS. Liver Steatosis,Steatohepatitis,Steatosis of Liver,Visceral Steatosis,Liver Steatoses,Liver, Fatty,Steatohepatitides,Steatoses, Liver,Steatoses, Visceral,Steatosis, Liver,Steatosis, Visceral,Visceral Steatoses
D005937 Glucaric Acid A sugar acid derived from D-glucose in which both the aldehydic carbon atom and the carbon atom bearing the primary hydroxyl group are oxidized to carboxylic acid groups. Glucosaccharic Acid,L-Gularic Acid,Levo-Gularic Acid,Tetrahydroxyadipic Acid,Calcium Glucarate,Calcium Glucarate, Anhydrous,Calcium Saccharate,Calcium Saccharate Anhydrous,Calcium Saccharate Tetrahydrate,Calcium Saccharate, Anhydrous,D-Glucaric Acid,D-Saccharic Acid,Saccharic Acid,Acid, Saccharic,Anhydrous Calcium Glucarate,Anhydrous Calcium Saccharate,D Glucaric Acid,D Saccharic Acid,Glucarate, Anhydrous Calcium,Glucarate, Calcium,L Gularic Acid,Levo Gularic Acid,Saccharate Tetrahydrate, Calcium,Saccharate, Anhydrous Calcium,Saccharate, Calcium,Tetrahydrate, Calcium Saccharate
D005965 Glucuronates Derivatives of GLUCURONIC ACID. Included under this heading are a broad variety of acid forms, salts, esters, and amides that include the 6-carboxy glucose structure. Glucosiduronates,Glucuronic Acids,Acids, Glucuronic
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D013400 Sugar Acids MONOSACCHARIDES and other sugars that contain one or more carboxylic acid moieties. Acids, Sugar

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