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[Efficacy of various therapeutic measures in metabolic hyperammonemia]
M J Pérez Rodríguez1, J L Vázquez Martínez, M Martínez-Pardo Casanova
1Servicio de Pediatría, Hospital Ramón y Cajal, Madrid.
Insights
Hemodialysis (HD) offers the quickest and most sustained reduction of severe hyperammonemia in children. For infants, combined exchange transfusion (ET) and peritoneal dialysis (DP) is a viable alternative.
Area of Science:
- Pediatric Nephrology
- Metabolic Disorders
- Critical Care Medicine
Background:
- Severe hyperammonemia in children poses a significant threat to neurological outcomes and survival.
- Treatment options for hyperammonemic coma are critical, especially in cases of inborn errors of metabolism and Reye's syndrome.
Observation:
- Six pediatric cases with hyperammonemic coma were treated with exchange transfusion (ET), ET plus peritoneal dialysis (DP), or hemodialysis (HD).
- Initial ammonium levels ranged from 300 to over 2000 µg/dL.
Findings:
- Peritoneal dialysis (DP) achieved the greatest overall reduction in plasma ammonium levels.
- Exchange transfusion (ET) and hemodialysis (HD) provided the quickest reduction in ammonium levels.
- Hemodialysis (HD) showed no significant complications, while one patient experienced hemodynamic deterioration during HD.
Implications:
- Hemodialysis (HD) is recommended as the primary treatment for severe hyperammonemia due to its rapid and sustained ammonium clearance.
- The combination of ET and DP serves as a valuable alternative for neonates and infants, where HD may be less accessible or practical.
Objective:
The aims of this work were to describe the therapeutic options available for severe hyperammonemia in children when ammonium levels are so high that the child's neurologic future or even their life is compromised. In addition, a comparison of the relative efficacy of each therapeutic method is made.
Patients And Methods:
We present six cases, five of which suffered from inborn errors of metabolism and a six which presented with Reye's syndrome, all of whom presented or developed hyperammonemic coma. Their initial ammonium levels fluctuated between 300 and more than 2000 micrograms per deciliter. The treatment was made with exchange transfusion (ET), ET and peritoneal dialysis (DP) together or hemodialysis (HD).
Results:
Peritoneal dialysis was the method that obtained the greatest reduction in plasma ammonium levels. However, the quickest reduction was observed with ET and HD. There were no significant complications with any of the methods except for hemodynamic deterioration in one patient during HD.
Conclusions:
We believe that HD is the treatment of choice for these patients because it is able to obtain a quick and lasting clearance of plasma ammonium. However, this method is not always available and has not been used very often in small babies. In these cases, the combined use of ET and DP can be very useful.
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