Related Experiment Videos
A patient with propionic acidemia managed with continuous insulin infusion and total parenteral nutrition
A Kalloghlian1, H Gleispach, P T Ozand
1Department of Pediatrics, King Faisal Specialist Hospital and Research Centre, Riyadh, Saudi Arabia.
Insights
Total parenteral nutrition and continuous insulin infusion successfully managed propionic acidemic coma in an infant. This treatment reduced organic acid excretion but did not prevent thrombocytopenia or resolve hyperlactic acidemia.
Area of Science:
- Biochemistry
- Pediatric Medicine
- Metabolic Disorders
Background:
- Propionic acidemia is a rare inherited metabolic disorder leading to toxic accumulation of organic acids.
- Infantile propionic acidemic coma presents a critical medical challenge requiring immediate and effective management strategies.
Observation:
- A neonate diagnosed with propionic acidemic coma was treated with total parenteral nutrition (TPN) and continuous insulin infusion.
- The treatment regimen led to a significant reduction in urinary 3-hydroxypropionic acid excretion within 16 days.
Findings:
- The infant's 3-hydroxypropionic acid levels decreased from admission values to 1.5% within 16 days.
- While TPN was tolerated, persistent hyperlactic acidemia was noted.
- Thrombocytopenia occurred as a prolonged complication, and metabolic derangements recurred only during sepsis.
Implications:
- This case highlights the efficacy of TPN and insulin infusion in managing propionic acidemic coma.
- The findings underscore the importance of monitoring for complications like thrombocytopenia and hyperlactic acidemia.
- Successful management in this infant suggests a potential therapeutic approach for similar metabolic emergencies in neonates.
Abstract:
An infant newly diagnosed with propionic acidemic coma was managed successfully with total parenteral nutrition (TPN) and continuous infusion of insulin. The urinary excretion of 3-hydroxypropionic acid was reduced to 3% of the admission value in 4 days, gradually decreasing to 1.5% in 16 days. The treatment did not prevent a prolonged episode of thrombocytopenia. The infant tolerated TPN well, except for continued hyper-lactic acidemia (2 to 4 times normal). Metabolic acidosis and mild hyperammonemia recurred only when the patient had sepsis secondary to Candida albicans and Staphylococcus aureus infection.