Anesthesia and Ullrich Congenital Muscular Dystrophy: Comment

نویسندگان

چکیده

I read with great interest the short report published in Images Anesthesiology section about difficult intubation a 2-yr-old patient Ullrich congenital muscular dystrophy.1 This disease is well known to carry risk of intubation.2–6 The authors nicely described how they used nasopharyngeal airway administer volatile anesthetic and oxygen through one nostril while performing nasotracheal fiberoptic via other. am however surprised that choice agent(s) was not discussed. dystrophy indeed belongs subgroup collagen type 6–related myopathies occurring after mutation COL6A1, COL6A2, or COL6A3 gene. Collagen 6 part large complex anchors basal lamina interstitium muscle cells. myopathy probably caused by membrane fragility an associated mitochondrial dysfunction, which can be decreased cyclosporine A. As close dystrophin-glycoprotein complex, this could at anesthesia-induced rhabdomyolysis presence halogenated agents succinylcholine, as are children Duchenne Becker progressive dystrophy. Very few reports on management patients have been so far all except two5,6 using total intravenous anesthesia.2–4 Carefully titrated anesthesia maintain spontaneous ventilation either dexmedetomidine, propofol, and/or ketamine therefore safe alternative.Dr. Veyckemans financial relationship European Journal Anaesthesiology’s Editorial Board Pediatric Anesthesia’s Board.

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ژورنال

عنوان ژورنال: Anesthesiology

سال: 2021

ISSN: ['0003-3022', '1528-1175']

DOI: https://doi.org/10.1097/aln.0000000000003698