Abstracts

Rationale: The ketogenic diet is a well-established treatment for refractory epilepsy, but when enteral feeding is contraindicated, few data show whether a ketogenic total parenteral nutrition (TPN) is efficacious and safe. In 2 previous case reports, use of a highly ketogenic TPN (ratio 3:1 to 4:1) was associated with electrolyte disturbances and sepsis; it is unclear whether these complications were due to the TPN therapy or due to preexisting malnutrition. We report on the successful maintenance of ketosis using a lower ratio ketogenic TPN in a 34-month-old girl with seizure disorder of unknown etiology who required prolonged bowel rest. Methods: The girl initiated the ketogenic diet at 20 months of age, and within 1 month, seizure frequency decreased from daily seizures with intermittent episodes of status epilepticus to 2 - 3 seizures per month using a 4:1 ketogenic ratio. In addition, she continued to require 4 antiepileptic drugs. She presented at 34 months of age with megacolon, pneumoperitoneum, and rectal bleeding requiring bowel rest, intubation and TPN. Given the severity of her epilepsy and efficacious response to ketosis, a ketogenic TPN was prescribed. TPN provided a mean daily energy of 40 kcal/kg/day (82% of estimated energy needs) from a 0.5% dextrose solution, with 2 g/kg/day protein, and a 20% lipid emulsion providing 2.5 - 4.0 g/kg/day of fat. During the 12 days of TPN therapy, ketogenic ratio ranged from 1:1 to 1.65:1. Results: Ketosis was maintained as measured by plasma beta-hydroxybutyrate of 2.93 mmol/L on the 11th day of TPN. At a 1.65:1 ratio and 4.0 g/kg of fat on day 3 of TPN, patient s triglyceride level rose to 836 mg/dL. Due to the hypertriglyceridemia, the TPN was stopped for 24 hours, then restarted and increased slowly over 3 days to provide a final ratio of 1.3:1 and 3.0 g/kg/day of fat for the remaining period of bowel rest. Seizures requiring lorazepam and phenobarbital bolus occurred on days 8 and 9 of TPN. The patient s weight after the course of TPN was unchanged from her baseline weight. Conclusions: When enteral feeding is contraindicated, a lower-ratio ketogenic TPN can be used to maintain ketosis and provide nutrition support.