Korean J Nephrol.  2003 Nov;22(6):749-752.

A Case of Chronic Tubulointerstitial Nephritis in Methylmalonic Acidemia

Affiliations
  • 1Department of Pediatrics, College of Medicine, Chungbuk National University, Cheongju, Korea. tsha@med.chungbuk.ac.kr

Abstract

Methylmalonic acidemia (MMA) is a heterogeneous inborn error of propionate metabolism and its management frequently includes a low-protein diet to minimize precursors of methylmalonic acid and reduce its concentration in body tissues. In the long- term follow-up, renal dysfunction in these patients has been increasingly recognized. Tubulointerstitial nephritis is the most frequent renal complications and has been reported in the small number of renal biopsy specimens from young children previously by others. We report a case of a 18-year-old girl with MMA and renal dysfunction in whom renal biopsy demonstrated chronic tubulointerstitial nephritis.

Keyword

Methylmalonic acidemia; Tubulointerstitial nephritis; Renal biopsy

MeSH Terms

Adolescent
Biopsy
Child
Diet, Protein-Restricted
Diethylpropion
Female
Follow-Up Studies
Humans
Metabolism
Methylmalonic Acid
Nephritis, Interstitial*
Diethylpropion
Methylmalonic Acid
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