Tubulointerstitial nephritis associated with acute intermittent porphyria. 2001

M L Onozato, and A Tojo, and A Kamijo, and S Taniguchi, and K Kimura, and A Goto, and T Fujita
Department of Internal Medicine, University of Tokyo, Japan.

Progressive renal impairment associated with acute intermittent porphyria is not well recognized and the mechanism of renal damage remains unclear. We report a case of a 51-year-old female with acute intermittent porphyria and long-term follow-up who developed proteinuria and renal insufficiency. Her biopsy showed marked tubulointerstitial damage with mitochondrial abnormalities. Urinary excretion of lipid peroxidation was increased compared to healthy controls. The porphyrin precursors may increase lipid peroxidation products and damage mitochondria leading to tubulointerstitial nephritis.

UI MeSH Term Description Entries
D008854 Microscopy, Electron Microscopy using an electron beam, instead of light, to visualize the sample, thereby allowing much greater magnification. The interactions of ELECTRONS with specimens are used to provide information about the fine structure of that specimen. In TRANSMISSION ELECTRON MICROSCOPY the reactions of the electrons that are transmitted through the specimen are imaged. In SCANNING ELECTRON MICROSCOPY an electron beam falls at a non-normal angle on the specimen and the image is derived from the reactions occurring above the plane of the specimen. Electron Microscopy
D009395 Nephritis, Interstitial Inflammation of the interstitial tissue of the kidney. This term is generally used for primary inflammation of KIDNEY TUBULES and/or surrounding interstitium. For primary inflammation of glomerular interstitium, see GLOMERULONEPHRITIS. Infiltration of the inflammatory cells into the interstitial compartment results in EDEMA, increased spaces between the tubules, and tubular renal dysfunction. Interstitial Nephritis,Nephritis, Tubulointerstitial,Tubulointerstitial Nephritis,Interstitial Nephritides,Nephritides, Interstitial,Nephritides, Tubulointerstitial,Tubulointerstitial Nephritides
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
D011507 Proteinuria The presence of proteins in the urine, an indicator of KIDNEY DISEASES. Proteinurias
D005260 Female Females
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000208 Acute Disease Disease having a short and relatively severe course. Acute Diseases,Disease, Acute,Diseases, Acute
D000328 Adult A person having attained full growth or maturity. Adults are of 19 through 44 years of age. For a person between 19 and 24 years of age, YOUNG ADULT is available. Adults
D015227 Lipid Peroxidation Peroxidase catalyzed oxidation of lipids using hydrogen peroxide as an electron acceptor. Lipid Peroxidations,Peroxidation, Lipid,Peroxidations, Lipid

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