acid-phosphatase and Porphyrias

Topics: Abortion, Induced; Acid Phosphatase; Adrenal Hyperplasia, Congenital; Amnion; Amniotic Fluid; Carbohydrate Metabolism, Inborn Errors; Congenital Abnormalities; Counseling; Culture Techniques; Cystic Fibrosis; Economics, Medical; Ethics, Medical; Female; Fetal Diseases; Fibroblasts; Genetic Diseases, Inborn; Glycogen Storage Disease; Heterozygote; Homozygote; Humans; Lysosomes; Mannose; Marfan Syndrome; Methods; Mucopolysaccharidoses; Muscular Dystrophies; Porphyrias; Pregnancy; Punctures; Xeroderma Pigmentosum

Topics: Acid Phosphatase; Blood Group Antigens; Chromosome Mapping; Female; Genetic Linkage; Genetic Markers; Humans; Hydroxymethylbilane Synthase; Lod Score; Male; Orosomucoid; Pedigree; Pepsinogens; Porphyrias

In the present study the participation of lysosomal enzymes in the primary photosensitizing process in the skin of porphyric patients were investigated. Cutaneous photosensitivity reactions were produced in patients with porphyria cutanea tarda by irradiation with near UV light and also to a lesser degree with visible light. The reactions occurred almost exclusively in patients with urinary uroporphyrin excretion exceeding 900 nmol/24 h and preceeded any noticeable increase of lysosomal enzyme activity by several hours. These results indicate that lysosomal enzymes are not primarily involved in the photosensitizing reaction in porphyric patients but that they participate in the cutaneous inflammatory process in porphyric skin at a later stage.

Topics: Acid Phosphatase; Adult; Aged; Coproporphyrins; Erythema; Female; Glycoside Hydrolases; Humans; Hydrolases; Lysosomes; Male; Middle Aged; Photosensitivity Disorders; Porphyrias; Skin; Skin Diseases; Ultraviolet Rays; Uroporphyrins

Topics: Acid Phosphatase; Acute Disease; Adult; Alkaline Phosphatase; Autopsy; Electron Transport Complex IV; Female; Glucosephosphate Dehydrogenase; Glycerolphosphate Dehydrogenase; Histocytochemistry; Humans; Hydroxybutyrate Dehydrogenase; L-Lactate Dehydrogenase; Liver; Liver Diseases; NAD; Peroxidases; Porphyrias; Succinate Dehydrogenase

Topics: Acid Phosphatase; Animals; Hexachlorocyclohexane; Humans; Lysosomes; Photosensitivity Disorders; Porphyrias; Porphyrins; Radiation Effects; Self Medication; Thiocarbamates

Topics: Acid Phosphatase; Amides; Animals; Bile; Endoplasmic Reticulum; Female; Glycogen; Golgi Apparatus; Histocytochemistry; Levulinic Acids; Lipid Metabolism; Liver; Lysosomes; Microscopy, Electron; Mitochondria, Liver; Nucleosides; Porphyrias; Rats