Abstract
A 41-year-old woman was admitted to our hospital because of abdominal pain, limb weakness, and red-wine-colored urine. Laboratory data showed renal insufficiency and hyperchloremic metabolic acidosis due to type 4 renal tubular acidosis. Clinical manifestations and investigation of porphyrins in her urine, erythrocytes, and feces established the diagnosis of hereditary coproporphyria (HCP). Renal biopsy revealed chronic tubulointerstitial nephritis. She was treated with corticosteroid, but her renal function showed no improvement. Although rare, renal involvement has been described in several types of porphyrias other than HCP. Previously reported cases of porphyrias with renal histology share the findings of chronic tubulointerstitial nephritis. To our knowledge, this is the first report of a patient with HCP presenting with renal involvement.
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