Anesthetic Management of a Pediatric Patient with Leigh Syndrome

Kocamanoğlu, İsmail Serhat; Sarihasan, Esra

doi:10.1016/s0034-7094(13)70219-8

Cited by 2 publications

(2 citation statements)

References 8 publications

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“…13 In a patient with Leigh-syndrome a single application of 20 mg propofol and maintenance dosage of 50-150 µg/kg/min did not cause any side effects (Table 1). 14 A number of other mitochondrial disorder patients were reported who tolerated propofol anesthesia without major side effects (Table 1).…”

Section: Resultsmentioning

confidence: 99%

Propofol Is Mitochondrion-Toxic and May Unmask a Mitochondrial Disorder

Finsterer

Frank

2016

J Child Neurol

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There are indications that preexisting mitochondrial disorders or beta-oxidation defects predispose for propofol infusion syndrome. This review aimed at investigating if propofol infusion syndrome occurs exclusively in patients with mitochondrial disorder and if propofol can unmask a mitochondrial disorder. Propofol infusion syndrome has been reported in genetically confirmed mitochondrial disorder patients. In addition, muscle biopsy of patients with propofol infusion syndrome revealed complex IV or complex II deficiency. In animal studies propofol disrupted the electron flow along the respiratory chain and decreased complex I, complex II, and complex III of the respiratory chain. In addition, propofol disrupted the permeability transition pore and reduced the mitochondrial membrane potential. In conclusion, propofol is mitochondrion-toxic and mitochondrial disorder patients should not receive propofol in high dosages over a prolonged period of time. Short-term application of propofol should be safe even in mitochondrial disorder patients. Not only does propofol infusion syndrome occur in mitochondrial disorder patients, but mitochondrial disorder patients are likely at higher risk to develop propofol infusion syndrome. Patients who develop propofol infusion syndrome should be screened for mitochondrial disorder. Propofol infusion syndrome is preventable if risk factors are thoroughly assessed, and if long-term propofol is avoided in patients at risk for propofol infusion syndrome.

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Section: Resultsmentioning

confidence: 99%

Propofol Is Mitochondrion-Toxic and May Unmask a Mitochondrial Disorder

Finsterer

Frank

2016

J Child Neurol

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“…Spontaneous, gaze-evoked, or pursuit-paretic nystagmus is an infrequent clinical manifestation of a MID and rarely occurs as an isolated phenotypic feature. Together with other CNS or extra-CNS abnormalities, it has been reported most frequently in Leigh syndrome 97 , 98 and more rarely in LHON, 99 MELAS, 100 MDS from DGUOK deficiency, 101 POLG1 -related disorders, 102 or in nsMIDs 103 105 Downbeat nystagmus has been reported in a patient with MELAS syndrome as a result of the tRNA(Leu) mutation m.3271T>C 106 .…”

Section: Cns Manifestations Of Midsmentioning

confidence: 99%

Cerebral Manifestations of Mitochondrial Disorders

Finsterer

Carvalho

2017

Can. J. Neurol. Sci.

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This review aims at summarizing and discussing previous and recent findings concerning the cerebral manifestations of mitochondrial disorders (MIDs). MIDs frequently present as mitochondrial multiorgan disorder syndrome (MIMODS) either already at onset or later in the course. After the muscle, the brain is the organ second most frequently affected in MIMODS. Cerebral manifestations of MIDs are variable and may present with or without a lesion on imaging or functional studies, but there can be imaging/functional lesions without clinical manifestations. The most well-known cerebral manifestations of MIDs include stroke-like episodes, epilepsy, headache, ataxia, movement disorders, hypopituitarism, muscle weakness, psychiatric abnormalities, nystagmus, white and gray matter lesions, atrophy, basal ganglia calcification, and hypometabolism on 2-deoxy-2-[fluorine-18]fluoro-D-glucose positron-emission tomography. For most MIDs, only symptomatic therapy is currently available. Symptomatic treatment should be supplemented by vitamins, cofactors, and antioxidants. In conclusion, cerebral manifestations of MIDs need to be recognized and appropriately managed because they strongly determine the outcome of MID patients.

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Anesthetic Management of a Pediatric Patient with Leigh Syndrome

Cited by 2 publications

References 8 publications

Propofol Is Mitochondrion-Toxic and May Unmask a Mitochondrial Disorder

Propofol Is Mitochondrion-Toxic and May Unmask a Mitochondrial Disorder

Cerebral Manifestations of Mitochondrial Disorders

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