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Related Experiment Videos

Progressive renal insufficiency in methylmalonic acidemia.

K H Molteni1, T D Oberley, J A Wolff

  • 1Department of Pediatrics, University of Wisconsin School of Medicine, Madison 53792.

Pediatric Nephrology (Berlin, Germany)
|May 1, 1991
PubMed
Summary

Methylmalonic acidemia can cause kidney damage even with a low-protein diet. This study suggests lower metabolite levels may be needed to prevent chronic tubulointerstitial nephropathy (CTIN) in patients.

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Area of Science:

  • Biochemistry
  • Nephrology
  • Metabolic Disorders

Background:

  • Methylmalonic acidemia (MMA) is an inborn error of propionate metabolism.
  • Treatment involves a low-protein diet to reduce methylmalonic acid (MMA) levels.
  • Renal dysfunction is a recognized complication, with tubulointerstitial disease noted in prior studies.

Observation:

  • An 18-year-old patient with mut- form methylmalonic acidemia developed renal dysfunction despite dietary management.
  • Renal biopsy confirmed tubulointerstitial injury.
  • The patient lacked risk factors for typical chronic tubulointerstitial nephropathy (CTIN).

Findings:

  • Methylmalonyl-CoA and its precursors (propionyl-CoA, odd-chain fatty acids) are implicated in causing CTIN.
  • This suggests a direct nephrotoxic effect of these metabolites.

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Implications:

  • Preventing renal injury in methylmalonic acidemia may necessitate stricter metabolite control than previously thought.
  • Lowering tissue levels of MMA and its precursors might be crucial for renal health.
  • This highlights the importance of monitoring kidney function in these patients.