Ketogenic parenteral nutrition in 17 pediatric patients with epilepsy

Abstract

SummaryObjectiveKetogenic parenteral nutrition (kPN) is indicated when enteral intake is temporarily limited or impossible, but evidence‐based prescriptions are lacking. Objective was to evaluate the efficacy and safety of kPN in children with epileptic encephalopathies using a new computer‐based algorithm for accurate component calculating.MethodsChildren with epilepsy receiving kPN were included. A computer‐based algorithm was established on the basis of guidelines of the European Society for Paediatric Gastroenterology, Hepatology and Nutrition (ESPGHAN): fat intake not exceeding 4 g/kg/day, age‐adequate supply of protein, electrolytes, vitamins, and trace elements, but reduced carbohydrates. Primary outcome was successfully reaching relevant ketosis, defined as beta‐hydroxybutyrate plasma level of ≥ 2 mmol/L. Efficacy was defined as seizure reduction ≥50% in de novo kPN and maintenance of response in children already on a ketogenic diet (KD). Safety was assessed by adverse effects, laboratory findings, and the appropriateness of nutritional intake.ResultsSeventeen children (median 1.84 years) were studied, of which 76% (13/17) were already on an oral ketogenic diet. Indications for kPN were surgery, status epilepticus, vomiting, food refusal, and introduction of enteral feeding in neonates. The parenteral fat/nonfat ratio was mean 0.9 (±0.3; range 0.6–1.5). Relevant ketosis was reached in 10 children (median 2.9 mmol/L), but not in 7 (median = 1.4 mmol/L). In de novo kPN, significant response was observed in 50% (2/4); in patients previously responding to the KD (77%, 10/13), response was maintained. A significant correlation between the degree of ketosis and seizure reduction (correlation coefficient = 0.691; p = .002) was observed. Only mild and transient adverse events occurred during kPN.Significance KPN with fat intake of 3.5–4.0 g/kg/day was safe and effective. KPN was tailored according to guidelines and individual nutritional needs. In nearly half of the patients, ketosis was lower than during oral KD. Despite this, seizures remained controlled.

Optimal clinical management of children receiving ketogenic parenteral nutrition: a clinical practice guide

“…The use of parenteral nutrition was related to acute illnesses resulting in intestinal ileus, severe vomiting, or severe diarrhea. Few cases reported prolonged use of parenteral ketogenic diet because of conditions in which the use of the enteral route was challenging …”

“…In the literature, studies recommend aiming for 1g protein/kg/d but in daily practice an intake of 0.5g/kg to 0.8g/kg seems to be more realistic . Only the study of Dressler et al . reported a median protein intake of 2g/kg/d with a range of 1.5g/kg to 2g/kg in KD‐PN, owing to very young age (median age of the children 1y 10mo), resulting in a median diet ratio of 0.9:1.…”

“…On the basis of the data from KD‐PN studies and in clinical practice, it is clear that, during KD‐PN, side effects are frequently documented even if parenteral nutrition is only given for a few days.…”

“…Limited data have been published in this area . The White Paper on ketogenic diet includes brief recommendations on KD‐PN monitoring…”

“…Recently updated international guidelines (European Society for Clinical Nutrition and Metabolism/European Society for Paediatric Gastroenterology Hepatology and Nutrition and the American Society for Parenteral and Enteral Nutrition) on parenteral nutrition are not applicable to KDT for the amount of carbohydrates recommended to initiate ketosis. There are currently limited data available on ketogenic diet parenteral nutrition (KD‐PN), with only 35 cases reported in the literature . Only the White Paper from the Charlie Foundation provides brief recommendations for KD‐PN practice…”