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Hyperammonaemia in a child with distal renal tubular acidosis

D Seracini1, G M Poggi, I Pela

  • 1Paediatric Nephrology, Department of Paediatrics, University of Florence, via Luca Giordano 13, 50132 Florence, Italy. d.seracini@meyer.it

Pediatric Nephrology (Berlin, Germany)
|September 1, 2005
PubMed

Insights

Distal renal tubular acidosis (RTA) can cause persistent hyperammonaemia, mimicking inborn errors of metabolism. This condition may last days even after correcting metabolic acidosis in infants.

Area of Science:

  • Pediatric Nephrology
  • Metabolic Disorders

Background:

  • Distal renal tubular acidosis (RTA) is a condition affecting kidney function.
  • Hyperammonaemia, elevated ammonia levels in the blood, can occur in infants.

Observation:

  • A 5-month-old infant presented with persistent hyperammonaemia for 12 days despite metabolic acidosis correction.
  • Symptoms included failure to thrive, poor feeding, hypotonia, and vomiting, without identified inborn errors of metabolism.

Findings:

  • Hyperammonaemia in this case was likely due to increased ammonia synthesis and impaired excretion, characteristic of distal RTA.
  • The findings confirm hyperammonaemia can occur in distal RTA, presenting as a metabolic disorder mimic.

Implications:

  • This case highlights the importance of considering distal RTA in infants with unexplained hyperammonaemia.
  • It underscores that hyperammonaemia can persist post-metabolic acidosis correction, requiring continued monitoring.

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