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Parenteral nutrition in propionic and methylmalonic acidemia
S G Kahler1, D S Millington, S D Cederbaum
1Department of Pediatrics, Duke University Medical Center, Durham, North Carolina 27710.
The Journal of Pediatrics
|August 1, 1989
Summary
Long-term parenteral nutrition safely restored health and growth in two patients with propionic acidemia and methylmalonic acidemia. This suggests that suppressing catabolism, not nutrient load, is key to managing these metabolic disorders.
Area of Science:
- Biochemistry
- Metabolic Disorders
- Clinical Nutrition
Background:
- Propionic acidemia and methylmalonic acidemia are branched-chain amino acid metabolism disorders.
- These conditions often present with chronic anorexia and vomiting.
- Parenteral nutrition is typically avoided due to fears of amino acid overload and worsening biochemical derangements.
Observation:
- Two critically ill patients with these disorders received long-term parenteral nutrition.
- Despite initial concerns, health and growth were restored in both patients.
- Minimal production of abnormal metabolites was observed during treatment.
Findings:
- Parenteral nutrition can be safely administered to patients with propionic acidemia and methylmalonic acidemia.
- Clinical and biochemical improvements were significant.
- Toxic metabolite production appears primarily linked to endogenous amino acid turnover, especially during catabolism, rather than administered nutrients.
Implications:
- Suppression of catabolism is a crucial strategy for managing these metabolic disorders.
- Parenteral nutrition, with careful monitoring, is a viable therapeutic option.
- This approach can lead to dramatic clinical and biochemical recovery.