Fatal Propofol Infusion Syndrome in Association with Ketogenic Diet

Baumeister, F. A. M.; Oberhoffer, Renate; Liebhaber, G.; Kunkel, James M.; Eberhardt, Joshua; Holthausen, Hans; Peters, Jr. John P.

doi:10.1055/s-2004-820992

Cited by 106 publications

(53 citation statements)

References 11 publications

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“…A 10-year-old boy suffered fatal propofol infusion syndrome when administered the ketogenic diet [30]. In this patient, propofol was believed to impair fatty acid oxidation, a necessary step in long chain fatty acid metabolism.…”

Section: Discussionmentioning

confidence: 99%

The Ketogenic Diet for Medically and Surgically Refractory Status Epilepticus in the Neurocritical Care Unit

et al. 2011

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Section: Discussionmentioning

confidence: 99%

The Ketogenic Diet for Medically and Surgically Refractory Status Epilepticus in the Neurocritical Care Unit

et al. 2011

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“…In an intensive care unit, KD should be avoided in children receiving propofol infusion due to the risk of fatal propofol infusion syndrome. 29 Over-the-counter medicines may have carbohydrates and can lead to ketosis reversal and seizure breakthroughs. Adequate counseling of the patient's caregiver is necessary.…”

Section: Special Situationsmentioning

confidence: 99%

The Ketogenic Diet: A Practical Guide for Pediatricians

Luat¹,

Coyle²,

Kamat³

2016

Pediatr Ann

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The ketogenic diet is an effective treatment for drug-resistant epilepsies in children. In addition, it is the first-line treatment for some metabolic disorders, such as glucose transporter 1 deficiency syndrome. This article discusses the proposed mechanisms of a ketogenic diet's antiseizure action, its clinical indications, and its contraindications. The steps involved in ketogenic diet initiation, monitoring, and management of its side effects are also discussed. This review provides general pediatricians with the necessary skills to provide comprehensive care of children using the ketogenic diet and counsel their families and caregivers. [Pediatr Ann. 2016;45(12):e446-e450.]

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“…Excess serum fatty-acid concentrations cause ventricular arrhythmias [31,44]. A case report speculated that PRIS was precipitated by the commencement of a ketogenic diet (high fat, low carbohydrate) as adjuvant therapy for refractory status epilepticus in a 10-year-old boy sedated with propofol [11]. It suggested that the fat load, coupled with propofol-mediated impairment of mitochondrial fatty-acid oxidation, caused the metabolic acidosis.…”

Section: Mechanism Of Prismentioning

confidence: 99%

Propofol infusion syndrome

2007

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SummaryThe clinical features of propofol infusion syndrome (PRIS) are acute refractory bradycardia leading to asystole, in the presence of one or more of the following: metabolic acidosis (base deficit > 10 mmol.l−1), rhabdomyolysis, hyperlipidaemia, and enlarged or fatty liver. There is an association between PRIS and propofol infusions at doses higher than 4 mg.kg−1.h−1 for greater than 48 h duration. Sixty‐one patients with PRIS have been recorded in the literature, with deaths in 20 paediatric and 18 adult patients. Seven of these patients (four paediatric and three adult patients) developed PRIS during anaesthesia. It is proposed that the syndrome may be caused by either a direct mitochondrial respiratory chain inhibition or impaired mitochondrial fatty acid metabolism mediated by propofol. An early sign of cardiac instability associated with the syndrome is the development of right bundle branch block with convex‐curved (‘coved type’) ST elevation in the right praecordial leads (V1 to V3) of the electrocardiogram. Predisposing factors include young age, severe critical illness of central nervous system or respiratory origin, exogenous catecholamine or glucocorticoid administration, inadequate carbohydrate intake and subclinical mitochondrial disease. Treatment options are limited. Haemodialysis or haemoperfusion with cardiorespiratory support has been the most successful treatment.

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Fatal Propofol Infusion Syndrome in Association with Ketogenic Diet

Cited by 106 publications

References 11 publications

The Ketogenic Diet for Medically and Surgically Refractory Status Epilepticus in the Neurocritical Care Unit

The Ketogenic Diet for Medically and Surgically Refractory Status Epilepticus in the Neurocritical Care Unit

The Ketogenic Diet: A Practical Guide for Pediatricians

Propofol infusion syndrome

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