Delay of renal progression in methylmalonic acidemia using angiotensin II inhibition: a case report.

Methylmalonic acidemia (MMA) is a heterogeneous inborn error of propionate metabolism. 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 complication and has been reported previously by others in the small number of renal biopsy specimens from young children. We report the case of a girl with methylmalonic acidemia and chronic renal insufficiency caused by tubulointerstitial nephritis which showed a considerable delay by angiotensin II inhibition.