Propofol-related infusion syndrome heralding a mitochondrial disease: Case report

Parness, Jerome; Savard, Martin; Turgeon, Alexis F.

doi:10.1212/01.wnl.0000443933.18269.20

Cited by 5 publications

(3 citation statements)

References 5 publications

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“…We have already discussed strategies for limiting propofol infusions in patients with traumatic head injury. Considering the real possibility of a mitochondrial aetiology and reports in patients with inborn errors of mitochondrial function, 54 it would seem prudent to avoid propofol infusions in patients with suspected mitochondrial impairment. The position with the use of vasopressors 52 and steroids 4 in conjunction with propofol infusions and their role in the pathophysiology of propofol infusion syndrome is less clear.…”

Section: Strategies To Reduce the Risk Of Propofol Infusion Syndromementioning

confidence: 99%

Propofol infusion syndrome: a structured literature review and analysis of published case reports

Hemphill

McMenamin

Bellamy

et al. 2019

British Journal of Anaesthesia

213

182

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Propofol infusion syndrome is a rare, potentially fatal condition first described in children in the 1990s and later reported in adults. We provide a narrative review of what is currently known about propofol infusion syndrome, including a structured analysis of all published case reports; child and adult cases were analysed separately as propofol is no longer used for long-term sedation in children. The review contains an update on current knowledge of the pathophysiology of this condition along with recommendations for its diagnosis, prevention, and management. We reviewed 108 publications documenting 168 cases of propofol infusion syndrome. We evaluated clinical features and analysed factors influencing mortality in child and adult cases using separate multivariate analysis models. We used separate multiple linear regression models to analyse relationships between cumulative dose of propofol and the number of features seen and organ systems involved. Lipidaemia, fever, and hepatomegaly occurred more frequently in children than in adults, whilst rhabdomyolysis and hyperkalaemia were more frequent in adults. Mortality from propofol infusion syndrome is independently associated with fever and hepatomegaly in children, and electrocardiogram changes, hypotension, hyperkalaemia, traumatic brain injury, and a mean propofol infusion rate >5 mg kg À1 h À1 in adults. The cumulative dose of propofol was associated with an increased number of clinical features and the number of organ systems involved in adult cases only. Clinicians should consider propofol infusion syndrome in cases of unexplained metabolic acidosis, ECG changes, and rhabdomyolysis. We recommend early consideration of continuous haemofiltration in the management of propofol infusion syndrome.

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Section: Strategies To Reduce the Risk Of Propofol Infusion Syndromementioning

confidence: 99%

Propofol infusion syndrome: a structured literature review and analysis of published case reports

Hemphill

McMenamin

Bellamy

et al. 2019

British Journal of Anaesthesia

213

182

View full text Add to dashboard Cite

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“…From the first reported cases, a wide variety of risk factors for developing PRIS have been identified, including cumulative propofol dose [12, 13], infusion duration [13], sepsis [14], steroids [15], vasopressors [16], fasting [15], critical illness [10], rich fat and low carbohydrate diet [14], inborn errors in mitochondrial fatty-acid oxidation [17], and cranioencephalic trauma [12]. The main risk factors consistently associated with PRIS are accumulated dose and duration of propofol infusion [11, 13].…”

Section: Discussionmentioning

confidence: 99%

Propofol Infusion Syndrome in the Postoperative Period of a Kidney Transplant

Dehesa-López

Irizar-Santana

Granado³

et al. 2019

Case Reports in Nephrology

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Sedation during medical procedures poses a risk to any patient, and the use of specific anesthetic agents should be carefully considered to avoid adverse outcomes. We report on a patient with propofol infusion syndrome diagnosed during the post-operative period of a renal transplant. A 58-year-old female on chronic hemodialysis due to end stage kidney disease secondary to microscopic polyangiitis underwent kidney transplant from a deceased donor. Anesthetic induction was performed with fentanyl, propofol, and cisatracurium, and maintained with continuous propofol infusion. In the recovery room, the patient developed somnolence, tachypnea, and thoracoabdominal dissociation secondary to residual neuromuscular block. An arterial-blood gas test indicated acidemia, high pCO2, low HCO3, and mildly increased serum lactate. The patient remained hemodynamically stable, on volume-controlled ventilation, with sedation by continuous propofol infusion. Blood gas tests revealed persistent acidemia without tissue hypoperfusion. Doppler ultrasound of the renal graft reported adequate blood flow and serum triglycerides were elevated. A diagnosis of propofol infusion syndrome was made, and infusion ceased. A decrease in serum lactate levels was observed, with normalization 4 h later. This case highlights the importance of considering adverse effects of anesthetic agents as the cause of post-operative complications when prolonged sedation is required.

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“…Other potential risk factors for the development of PRIS are critical illness (sepsis, head trauma, status epilepticus, etc. ), use of vasopressors and glucocorticosteroids, carbohydrate depletion (liver disease, starvation, or malnutrition), carnitine deficiency, and subclinical mitochondrial disease [ 28 , 57 – 59 ]. It is not clear whether these factors represent only a marker of a severe illness or if they play a direct role in the development of PRIS.…”

Section: Risk Factors For the Development Of Propofol Infusion Synmentioning

confidence: 99%

Propofol Infusion Syndrome in Adults: A Clinical Update

Mirrakhimov

Voore

Halytskyy

et al. 2015

Critical Care Research and Practice

114

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Propofol infusion syndrome is a rare but extremely dangerous complication of propofol administration. Certain risk factors for the development of propofol infusion syndrome are described, such as appropriate propofol doses and durations of administration, carbohydrate depletion, severe illness, and concomitant administration of catecholamines and glucocorticosteroids. The pathophysiology of this condition includes impairment of mitochondrial beta-oxidation of fatty acids, disruption of the electron transport chain, and blockage of beta-adrenoreceptors and cardiac calcium channels. The disease commonly presents as an otherwise unexplained high anion gap metabolic acidosis, rhabdomyolysis, hyperkalemia, acute kidney injury, elevated liver enzymes, and cardiac dysfunction. Management of overt propofol infusion syndrome requires immediate discontinuation of propofol infusion and supportive management, including hemodialysis, hemodynamic support, and extracorporeal membrane oxygenation in refractory cases. However, we must emphasize that given the high mortality of propofol infusion syndrome, the best management is prevention. Clinicians should consider alternative sedative regimes to prolonged propofol infusions and remain within recommended maximal dose limits.

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Propofol-related infusion syndrome heralding a mitochondrial disease: Case report

Cited by 5 publications

References 5 publications

Propofol infusion syndrome: a structured literature review and analysis of published case reports

Propofol infusion syndrome: a structured literature review and analysis of published case reports

Propofol Infusion Syndrome in the Postoperative Period of a Kidney Transplant

Propofol Infusion Syndrome in Adults: A Clinical Update

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