Mitochondrial Disease: Difference between revisions

Latest revision as of 16:17, 10 April 2026

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Relevant Article Depot:

See also: Leigh’s Syndrome

A review of anaesthetic outcomes in patients with genetically confirmed mitochondrial disorders

Mitochondrial Disease and Anesthesia

Anesthetic Hypersensitivity in a Case-Controlled Series of Patients With Mitochondrial Disease

Diagnosis and management of mitochondrial disease: a consensus statement from the Mitochondrial Medicine Society

Anesthetic considerations in patients with mitochondrial defects

Hypoxia ameliorates brain hyperoxia and NAD(+) deficiency in a murine model of Leigh syndrome

Hypoxia as a therapy for mitochondrial disease

Open Anesthesia: Anesthetic Considerations in Patients with Mitochondrial Disease

Mitochondrial diseases and anesthesia: a literature review of current opinions

Anesthesia for children with mitochondrial disorders: a national survey and review

Sevoflurane as opposed to propofol anesthesia preserves mitochondrial function and alleviates myocardial ischemia/reperfusion injury

Perioperative management of a child with short-chain acyl CoA dehydrogenase deficiency. Pediatr Anesth 2005; 15: 771-7.

Hsieh VC, Krane EJ, Morgan PG. Mitochondrial disease and anesthesia. JIEMS. 2017;5:1–5

Kishikawa J-I, Inoue Y, Fujikawa M, Nishimura K, Nakanishi A, Tanabe T, et al. General anesthetics cause mitochondrial dysfunction and reduction of intracellular ATP levels. PLoS ONE. 2018;13:e0190213. doi: 10.1371/journal.pone.0190213

Anesthetic Management of a Child With Unspecified Mitochondrial Disease in an Outpatient Dental Setting

Effects of Isoflurane Inhibition of Mitochondrial Complex I on Calcium Removal in Mouse Neuronal Cultures

MT-ND4

ASA Information Page

MT-ND4 gene: mitochondrially encoded NADH dehydrogenase 4

Severe Neurological Complications and Death after General Anesthesia in Venezuelan Children(Forum thread)

Short Communication:Mitochondrial Genetic Susceptibility to Anesthetic Neurotoxicity in Venezuelan Pediatric Patients: A Call for Vigilance and Further Research

Joint Communication from the Society for Pediatric Anesthesia and American Society of Anesthesiologists Regarding Severe Neurological Complications and Death after General Anesthesia in Venezuelan Children

Part of the webinar series Hot Topics in Pediatric Anesthesia, presented by the Pediatric Anesthesia Committee of the Florida Society of Anesthesiologists, Rosalie Tassone, MD, Chair. "Bad Outcomes in Strange Times - Update on the ASA/SPA Communications Regarding Pediatric Patients of Venezuelan Families" presented by Luis Rodriguez, MD (originally presented on November 12, 2025)

Update regarding severe neurological complications and death after general anesthesia in adult and pediatric patients of Venezuelan ancestry (January 27, 2026)

Potential Mitochondrial Pharmacogenetic Susceptibility to Severe Neurologic Events after General Anesthesia: Report from the Chilean Ministry of Health

Hypoxia treatment reverses neurodegenerative disease in a mouse model of Leigh syndrome

Volatile anaesthetic toxicity in the genetic mitochondrial disease Leigh syndrome

Effects of a mitochondrial genetic variant on sevoflurane hypersensitivity

ESAIC News: Joint Statement on Anaesthesia-Related Mitochondrial Pharmacogenetic Risk in Individuals with Venezuelan Maternal Ancestry

KevinMD: How a hidden genetic mutation creates a severe pediatric anesthesia risk

Recomendaciones anestésicas frente a una variante genética infrecuente en población con ascendencia venezolana, con posible asociación a complicaciones neurológicas severas tras anestesia general (Anesthetic recommendations regarding a rare genetic variant in a population with Venezuelan ancestry, with a possible association with severe neurological complications following general anesthesia

Encefalopatía aguda asociada a anestesia general en un paciente adulto portador de la variante m.11232T>C en el gen MT-ND4 (An adult case of acute encephalopathy associated with general anesthesia and the m.11232T>C MT-ND4 variant)

Emerging Reports of Severe Neurological Complications Following General Anesthesia in Patients with Maternal Venezuelan Ancestry

Video for Parents (English)

Video for Parents (Español)

Anesthetic Risk Linked to Venezuelan Maternal Lineage

AIRS Case 2026-03: This Is Why We Have AIRS

Perioperative Practices for Patients of Venezuelan Ancestry

Mitochondrial Genetic Susceptibility to Anesthetics in Patients with Venezuelan Heritage

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 {{Stub Notice}}
+See also: [[Leigh’s Syndrome]]
 [https://pubmed.ncbi.nlm.nih.gov/27885500/ A review of anaesthetic outcomes in patients with genetically confirmed mitochondrial disorders]
@@ Line 5: / Line 8: @@
 [https://med.stanford.edu/content/dam/sm/pedsanesthesia/documents/mitochondrial-disease.pdf Mitochondrial Disease and Anesthesia]
-References:
+[https://pubmed.ncbi.nlm.nih.gov/33591116/  Anesthetic Hypersensitivity in a Case-Controlled Series of Patients With Mitochondrial Disease]
+[https://pubmed.ncbi.nlm.nih.gov/25503498/  Diagnosis and management of mitochondrial disease: a consensus statement from the Mitochondrial Medicine Society]
+[https://onlinelibrary.wiley.com/doi/10.1111/pan.12158 Anesthetic considerations in patients with mitochondrial defects]
+[https://pubmed.ncbi.nlm.nih.gov/33752971/  Hypoxia ameliorates brain hyperoxia and NAD(+) deficiency in a murine model of Leigh syndrome]
+[https://pubmed.ncbi.nlm.nih.gov/26917594/ Hypoxia as a therapy for mitochondrial disease]
+[https://www.openanesthesia.org/vodcasts/anesthetic-considerations-in-patients-with-mitochondrial-disease/ Open Anesthesia: Anesthetic Considerations in Patients with Mitochondrial Disease]
+[https://pubmed.ncbi.nlm.nih.gov/23923677/ Mitochondrial diseases and anesthesia: a literature review of current opinions]
+[https://pubmed.ncbi.nlm.nih.gov/23007905/ Anesthesia for children with mitochondrial disorders: a national survey and review]
+[https://pubmed.ncbi.nlm.nih.gov/32574972/ Sevoflurane as opposed to propofol anesthesia preserves mitochondrial function and alleviates myocardial ischemia/reperfusion injury]
+Perioperative management of a child with short-chain acyl CoA dehydrogenase deficiency.
+Pediatr Anesth 2005; 15: 771-7.
+Hsieh VC, Krane EJ, Morgan PG. Mitochondrial disease and anesthesia. JIEMS. 2017;5:1–5
+Kishikawa J-I, Inoue Y, Fujikawa M, Nishimura K, Nakanishi A, Tanabe T, et al. General anesthetics cause mitochondrial dysfunction and reduction of intracellular ATP levels. PLoS ONE. 2018;13:e0190213. doi: 10.1371/journal.pone.0190213
+[https://pmc.ncbi.nlm.nih.gov/articles/PMC5278534 Anesthetic Management of a Child With Unspecified Mitochondrial Disease in an Outpatient Dental Setting]
+[https://journals.lww.com/anesthesiology/abstract/2026/04000/effects_of_isoflurane_inhibition_of_mitochondrial.23.aspx Effects of Isoflurane Inhibition of Mitochondrial Complex I on Calcium Removal in Mouse Neuronal Cultures]
+<b>MT-ND4</b>
+[https://www.asahq.org/advocating-for-you/genetic-anesthesia-neurologic-mortality-risks ASA Information Page]
+[https://medlineplus.gov/genetics/gene/mt-nd4/ MT-ND4 gene: mitochondrially encoded NADH dehydrogenase 4]
+[https://pedsanesthesia.net/forum/viewtopic.php?t=1352 Severe Neurological Complications and Death after General Anesthesia in Venezuelan Children(Forum thread)]
+[https://www.clinsurgeryjournal.com/index.php/ascr/article/view/ascr-aid1091/pdf Short Communication:Mitochondrial Genetic Susceptibility to Anesthetic Neurotoxicity in Venezuelan Pediatric Patients: A Call for Vigilance and Further Research]
+[https://pedsanesthesia.org/joint-communication-from-the-society-for-pediatric-anesthesia-and-american-society-of-anesthesiologists-regarding-severe-neurological-complications-and-death-after-general-anesthesia-in-venezuelan-chi/ Joint Communication from the Society for Pediatric Anesthesia and American Society of Anesthesiologists Regarding Severe Neurological Complications and Death after General Anesthesia in Venezuelan Children]
+[https://youtu.be/cKuFfUwxSx4?si=v-WqKAO5gQerTKwu Part of the webinar series Hot Topics in Pediatric Anesthesia, presented by the Pediatric Anesthesia Committee of the Florida Society of Anesthesiologists, Rosalie Tassone, MD, Chair. "Bad Outcomes in Strange Times - Update on the ASA/SPA Communications Regarding Pediatric Patients of Venezuelan Families" presented by Luis Rodriguez, MD (originally presented on November 12, 2025)]
+[https://www.asahq.org/advocating-for-you/guidance/asa-spa-neurological Update regarding severe neurological complications and death after general anesthesia in adult and pediatric patients of Venezuelan ancestry (January 27, 2026)]
+[https://journals.lww.com/anesthesiology/fulltext/9900/potential_mitochondrial_pharmacogenetic.904.aspx Potential Mitochondrial Pharmacogenetic Susceptibility to Severe Neurologic Events after General Anesthesia: Report from the Chilean Ministry of Health]
+[https://www.pnas.org/doi/10.1073/pnas.1621511114 Hypoxia treatment reverses neurodegenerative disease in a mouse model of Leigh syndrome]
+[https://pubmed.ncbi.nlm.nih.gov/37770252/ Volatile anaesthetic toxicity in the genetic mitochondrial disease Leigh syndrome]
+[https://journals.lww.com/anesthesiology/abstract/9900/effects_of_a_mitochondrial_genetic_variant_on.912.aspx Effects of a mitochondrial genetic variant on sevoflurane hypersensitivity]
+[https://esaic.org/joint-statement-on-anaesthesia-related-mitochondrial-pharmacogenetic-risk-in-individuals-with-venezuelan-maternal-ancestry/ ESAIC News: Joint Statement on Anaesthesia-Related Mitochondrial Pharmacogenetic Risk in Individuals with Venezuelan Maternal Ancestry]
+[https://kevinmd.com/2026/04/how-a-hidden-genetic-mutation-creates-a-severe-pediatric-anesthesia-risk.html KevinMD: How a hidden genetic mutation creates a severe pediatric anesthesia risk]
+[https://revistachilenadeanestesia.cl/revchilanestv55n2-18/ Recomendaciones anestésicas frente a una variante genética infrecuente en población con ascendencia venezolana, con posible asociación a complicaciones neurológicas severas tras anestesia general (Anesthetic recommendations regarding a rare genetic variant in a population with Venezuelan ancestry, with a possible association with severe neurological complications following general anesthesia]
+[https://www.sciencedirect.com/science/article/abs/pii/S0034935626000708 Encefalopatía aguda asociada a anestesia general en un paciente adulto portador de la variante m.11232T>C en el gen MT-ND4 (An adult case of acute encephalopathy associated with general anesthesia and the m.11232T>C MT-ND4 variant)]
+[https://tools.sodapdf.com/document/6287485d-7db3-42e5-90c2-8f668e846b4a Emerging Reports of Severe Neurological Complications Following General Anesthesia in Patients with Maternal Venezuelan Ancestry]
-.          Hsieh VC, Niezgoda J, Sedensky MM, Hoppel CL, Morgan PG. Anesthetic Hypersensitivity in a Case-Controlled Series of Patients With Mitochondrial Disease. Anesthesia and analgesia. Oct 1 2021;133(4):924-932. doi:10.1213/ane.0000000000005430
+[https://player.vimeo.com/video/1179934920 Video for Parents (English)]
-.          Parikh S, Goldstein A, Koenig MK, et al. Diagnosis and management of mitochondrial disease: a consensus statement from the Mitochondrial Medicine Society. Genet Med. Sep 2015;17(9):689-701. doi:10.1038/gim.2014.177
+[https://player.vimeo.com/video/1179935060 Video for Parents (Español)]
-.          Niezgoda J, Morgan PG. Anesthetic considerations in patients with mitochondrial defects. Paediatric anaesthesia. Sep 2013;23(9):785-93. doi:10.1111/pan.12158
+[https://www.medscape.com/viewarticle/anesthetic-risk-linked-venezuelan-maternal-lineage-2026a10009ni?ecd=a2a&form=fpf Anesthetic Risk Linked to Venezuelan Maternal Lineage]
-.          Ferrari M, Jain IH, Goldberger O, et al. Hypoxia treatment reverses neurodegenerative disease in a mouse model of Leigh syndrome. Proc Natl Acad Sci U S A. May 23 2017;114(21):E4241-e4250. doi:10.1073/pnas.1621511114
+[https://journals.lww.com/monitor/fulltext/2026/03000/airs_case_2026_03__this_is_why_we_have_airs.10.aspx AIRS Case 2026-03: This Is Why We Have AIRS]
-.          Grange RMH, Sharma R, Shah H, et al. Hypoxia ameliorates brain hyperoxia and NAD(+) deficiency in a murine model of Leigh syndrome. Mol Genet Metab. May 2021;133(1):83-93. doi:10.1016/j.ymgme.2021.03.005
+[https://www.apsf.org/article/perioperative-practices-for-patients-of-venezuelan-ancestry/ Perioperative Practices for Patients of Venezuelan Ancestry]
-.          Jain IH, Zazzeron L, Goli R, et al. Hypoxia as a therapy for mitochondrial disease. Science. Apr 1 2016;352(6281):54-61. doi:10.1126/science.aad9642
+[https://sphere.nyssa-pga.org/2026/mitochondrial-genetic-susceptibility-to-anesthetics-in-patients-with-venezuelan-heritage/ Mitochondrial Genetic Susceptibility to Anesthetics in Patients with Venezuelan Heritage]