From intravenous to enteral ketogenic diet in PICU: A potential treatment strategy for refractory status epilepticus

Chiusolo, Fabrizio; Diamanti, Antonella; Bianchi, Roberto; Fusco, Lucia; Elia, Mirella; Capriati, Teresa; Vigevano, Federico; Picardo, Sergio

doi:10.1016/j.ejpn.2016.08.004

Cited by 35 publications

(53 citation statements)

References 25 publications

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“…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 …”

Section: Methodsmentioning

confidence: 99%

“…In this acute setting, KDT is usually administered by an enteral route (46 of 47 pediatric cases reported by Chiusolo et al.) . However, few patients may require parenteral administration.…”

Section: Recommendationsmentioning

confidence: 99%

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

Section: Recommendationsmentioning

confidence: 99%

“…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…”

mentioning

confidence: 99%

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

Louw

Aldaz

Harvey

et al. 2019

Develop Med Child Neuro

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AimTo give evidence‐based recommendations on the application of ketogenic diet parenteral nutrition (KD‐PN) in emergency situations.MethodAn international group of experts (n=14) researched the literature and distributed a survey among 150 expert centers. International accepted 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) and handbooks for parenteral nutrition were considered general standards of care.ResultsIn the literature, we identified 35 reports of patients treated by KD‐PN. International guidelines and handbooks provided some conflicting information. Twenty‐four expert teams from nine countries responded to the survey, reflecting the limited clinical experience.InterpretationThis paper highlights 23 consensus‐based recommendations for safe and effective KD‐PN (e.g. diet initiation, calculation, application, monitoring, and evaluation) based on the best evidence available and expert opinions.What this paper adds In acute settings, ketogenic diet therapy (KDT) can be administered parenterally.Parenteral administration of KDT should be started only at the intensive care unit.Initiate ketogenic parenteral nutrition stepwise to the highest ratio possible with the lowest level of complications.Evaluate the risk–benefit ratio of parenteral administration continuously.Restart enteral feeding as soon as appropriate.

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Section: Methodsmentioning

confidence: 99%

Section: Recommendationsmentioning

confidence: 99%

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

Section: Recommendationsmentioning

confidence: 99%

mentioning

confidence: 99%

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

Louw

Aldaz

Harvey

et al. 2019

Develop Med Child Neuro

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“…No autoantibodies have been identified. One hypothesis is there is a release of inflammatory molecules following a systemic infection that activates the immune system involved in protecting the blood brain barrier which then gives rise to a neuroinflammatory cascade [4]. This neuroinflammation might lower seizure threshold rather than trigger the seizures.…”

Section: Discussionmentioning

confidence: 99%

DRESS following FIRES: A Clinical Conundrum

Dahl¹,

Knoebel²,

Lloyd³

2018

Clin Pediatr OA

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A previously healthy 2-year old Hmong girl presented to the Emergency Department with first time seizures during a febrile illness. Seizures continued for the next two weeks despite multiple antiepileptic agents. All initial cultures and evaluations were negative, and febrile infection related epilepsy syndrome (FIRES) was diagnosed.Three weeks into her hospital course, the patient developed a morbilliform rash on her thigh that became generalized. Concurrently, she developed fever and tachycardia. Laboratory studies demonstrated eosinophilia, an increase in ALT, AST, and direct/total bilirubin. CMV, EBV, and HHV-6 were negative. Skin biopsy showed lichenoid interface dermatitis. Clinical picture was suggestive of drug reaction with eosinophilia and systemic symptoms (DRESS). Topical and systemic steroids were started. Potential triggers for DRESS were discontinued. The patient deteriorated and cardiorespiratory failure occurred. She required ECMO for eight days. Patient gradually improved over the following weeks despite recurrent seizures and remained in the hospital for 283 days. FIRES is a rare and highly morbid condition in which patients develop status epilepticus in the setting of febrile illness that is often intractable to anticonvulsants and steroids. Typically no causative infection is identified. DRESS, another rare and potentially fatal condition, is a drug hypersensitivity reaction that includes eosinophilia, characteristic skin findings and potential involvement of liver, lungs, kidneys, or other organs. Allopurinol, antiepileptic agents, and antibiotics are the most frequently reported triggers.In this case report, we describe a patient who developed DRESS following FIRES, a sequence not previously described in the literature.

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Ketogenic parenteral nutrition in 17 pediatric patients with epilepsy

et al. 2017

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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.

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From intravenous to enteral ketogenic diet in PICU: A potential treatment strategy for refractory status epilepticus

Cited by 35 publications

References 25 publications

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

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

DRESS following FIRES: A Clinical Conundrum

Ketogenic parenteral nutrition in 17 pediatric patients with epilepsy

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