Table 2.

Summary of 23 recommendations for application of KD‐PN

Ketogenic parenteral nutrition should only be started in an intensive care unit

1. In general, indications and contraindications (e.g. fatty acid oxidation deficiencies) for KD‐PN and enteral KDT are similar

2. Start parenteral administration of KDT exclusively in case nothing by mouth >48h is expected

3. Check for contraindications in case of a newly starting KDT

4. Careful evaluation of the medical and nutritional status: infants born preterma and malnourished children are at high risk of complications and therefore KD‐PN should be omitted

5. Change all medications to non‐carbohydrate forms if possible (pill, tablet, pharmacy compounded). Consider using toothpaste with the lowest glycerol and carbohydrate content. The carbohydrate and alcohol content of the intravenous product should be calculated into the final KD‐PN diet ratio

6. Additional fasting can be performed at KDT initiation for a maximum of 24h to induce ketosis for first‐time KDT initiation. Note: hyperketosis and/or hypoglycemia might be accelerated by caloric restriction

7. Obtain baseline nutritional assessment: use ideal weight/height and height/age

8. Fluid: determine the net fluid volume available for parenteral nutrition. Notice the medical status, body weight, excretion, blood electrolytes, acid base status, hematocrit, specific gravity of urine, and urine electrolytes

9. Calories: start with 50% of goal and build this up within a maximum of 1wk when lipids are increased. Aim for resting energy expenditure or 70–80% of calculated energy requirements. Intake of 50% of calculated energy goal might be accepted for a limited period (3–4d). If available, measure resting energy expenditure and respiratory quotient. Notice the risk of hyperalimentation

10. Carbohydrates: the use of glucose/dextrose might be avoided for 3–4d at KD‐PN initiation to benefit ketosis, when this is clinically appropriate as a certain amount of carbohydrates will be delivered by glycerol metabolization from intravenous lipid emulsions. Administer sodium chloride 0.45% or other fluids required on the basis of blood electrolytes. Subsequently, on the basis of ketone and glucose levels and laboratory checks, use the lowest dextrose percentage available or start dextrose 5% solution delivering the amount of carbohydrates of the original enteral ketogenic diet as the maximum

11. Protein: aim for 100% requirements as the goal, with 1.5g/kg/d as a minimum requirement. To maximize ketosis, 0.5–0.8g/kg/d temporary restriction is accepted. During critical illness, additional protein may be required to promote positive nitrogen balance

12. Lipids: start with 50% of goal lipids or 1–2g/kg/d. Increase lipids every 1–2d (on the basis of triglyceride level) up to a maximum of 4g/kg/d. Run lipids continuously. Notice possible allergy or hypersensitivity of the patient to fish, egg, soybean, or peanut protein

13. Diet ratio: start with 1:1 ratio and increase every 1–2d to the highest ratio possible. Aim for a diet ratio within the range 2.0:1–2.9:1 (including glycerol from lipid emulsion) within 3–4d. Aim for the highest diet ratio possible with the lowest level of complications. Consider carnitine supplementation (50mg/kg to a maximum of 1000mg/d) to benefit ketosis

14. Vitamin/mineral supplementations should be age‐appropriate, and consider the weight of the patient

15. Delayed treatment (after 7d) should be avoided for children already on ketogenic diet (to maintain ketosis) or when KDT is required urgently (to reach ketosis) for epilepsy control (RSE or FIRES)

16. Appropriate level of ketosis might not always be achieved; when seizure burden improves this might be accepted

17. Use ASPEN and ESPGHAN/ESPEN guidelines to prevent general complications of parenteral nutrition

18. Side effects of parenteral nutrition during KDT that are most frequently observed are elevated lipids (up to <400mg/dL per 10mmol/L), insufficient ketosis (<1.5mmol/L), hypoglycemia (<2.5mmol/L per 45mg/dL). To a smaller degree: hyperketosis (>6.5mmol/L), hyperbilirubin (>40μmol/L), altered liver function tests, and pancreatic enzymes

19. A tailored nutritional regimen must be carefully chosen and closely monitored

20. KD‐PN initiation during propofol anesthesia is not recommended and should be undertaken only with caution

21. Close monitoring should be completed at baseline, maintained during follow‐up, and tailored to the patient's medical and nutritional status

22. Evaluation will be based on the maintenance of seizure control provided by the initial enteral prescription of the ketogenic diet; in the case of RSE or FIRES, the efficacy should be evaluated both clinically and electroencephalographically

23. Transition to an enteral feed has to be tailored to the medical situation and nutritional status. Start trophic feeds as soon as possible and appropriate

^aInfants born preterm: <36wks postmenstrual age. KD‐PN, ketogenic parenteral nutrition; KDT, ketogenic diet therapy; RSE, refractory status epilepticus; FIRES, febrile infection related epilepsy syndrome; ASPEN, American Society for Parenteral and Enteral Nutrition; ESPGHAN, European Society for Paediatric Gastroenterology Hepatology and Nutrition; ESPEN, European Society for Clinical Nutrition and Metabolism.