Biomaterial Database

Rocuronium for muscle relaxation in two children with Friedreich's ataxia. 2004

H J Schmitt, and S Wick, and T Münster

Department of Anaesthesiology, Friedrich Alexander University Erlangen-Nuremberg, Krankenhausstrasse 12, D-91054 Erlangen, Germany. hubert.schmitt@kfa.imed.uni-erlangen.de

Friedreich's ataxia is a rare hereditary neurodegenerative disease caused by a defect in the gene that encodes a mitochondrial protein called frataxin. We report the use of rocuronium 0.6 mg kg(-1) in two adolescent girls with Friedreich's ataxia undergoing propofol-sufentanil-oxygen-air anaesthesia for spinal surgery. Neuromuscular transmission was monitored using acceleromyography, and onset and recovery times were recorded. The clinical duration of rocuronium was comparable to that of children without neuromuscular disease (25% recovery T(1)=44 and 24 min for patients 1 and 2 respectively).

UI	MeSH Term	Description	Entries
D007738	Kyphosis	Deformities of the SPINE characterized by an exaggerated convexity of the vertebral column. The forward bending of the thoracic region usually is more than 40 degrees. This deformity sometimes is called round back or hunchback.	Hyperkyphosis,Hyperkyphoses,Kyphoses
D009126	Muscle Relaxation	That phase of a muscle twitch during which a muscle returns to a resting position.	Muscle Relaxations,Relaxation, Muscle,Relaxations, Muscle
D002648	Child	A person 6 to 12 years of age. An individual 2 to 5 years old is CHILD, PRESCHOOL.	Children
D003473	Neuromuscular Nondepolarizing Agents	Drugs that interrupt transmission at the skeletal neuromuscular junction without causing depolarization of the motor end plate. They prevent acetylcholine from triggering muscle contraction and are used as muscle relaxants during electroshock treatments, in convulsive states, and as anesthesia adjuvants.	Curare-Like Agents,Curariform Drugs,Muscle Relaxants, Non-Depolarizing,Neuromuscular Blocking Agents, Competitive,Nondepolarizing Blockers,Agents, Curare-Like,Agents, Neuromuscular Nondepolarizing,Blockers, Nondepolarizing,Curare Like Agents,Drugs, Curariform,Muscle Relaxants, Non Depolarizing,Non-Depolarizing Muscle Relaxants,Nondepolarizing Agents, Neuromuscular
D005260	Female		Females
D005621	Friedreich Ataxia	An autosomal recessive disease, usually of childhood onset, characterized pathologically by degeneration of the spinocerebellar tracts, posterior columns, and to a lesser extent the corticospinal tracts. Clinical manifestations include GAIT ATAXIA, pes cavus, speech impairment, lateral curvature of spine, rhythmic head tremor, kyphoscoliosis, congestive heart failure (secondary to a cardiomyopathy), and lower extremity weakness. Most forms of this condition are associated with a mutation in a gene on chromosome 9, at band q13, which codes for the mitochondrial protein frataxin. (From Adams et al., Principles of Neurology, 6th ed, p1081; N Engl J Med 1996 Oct 17;335(16):1169-75) The severity of Friedreich ataxia associated with expansion of GAA repeats in the first intron of the frataxin gene correlates with the number of trinucleotide repeats. (From Durr et al, N Engl J Med 1996 Oct 17;335(16):1169-75)	Friedreich Disease,Hereditary Spinal Sclerosis,Sclerosis, Hereditary Spinal,Friedreich Familial Ataxia,Friedreich Hereditary Ataxia,Friedreich Hereditary Spinal Ataxia,Friedreich Spinocerebellar Ataxia,Friedreich's Ataxia,Friedreich's Disease,Friedreich's Familial Ataxia,Friedreich's Hereditary Ataxia,Friedreich's Hereditary Spinal Ataxia,Hereditary Spinal Ataxia, Friedreich,Hereditary Spinal Ataxia, Friedreich's,Ataxia, Friedreich,Ataxia, Friedreich Familial,Ataxia, Friedreich Hereditary,Ataxia, Friedreich Spinocerebellar,Ataxia, Friedreich's,Ataxia, Friedreich's Familial,Ataxia, Friedreich's Hereditary,Ataxias, Friedreich,Ataxias, Friedreich's Hereditary,Disease, Friedreich,Disease, Friedreich's,Familial Ataxia, Friedreich,Familial Ataxia, Friedreich's,Friedreich Ataxias,Friedreich's Hereditary Ataxias,Friedreichs Familial Ataxia,Friedreichs Hereditary Ataxia,Hereditary Ataxia, Friedreich,Hereditary Ataxia, Friedreich's,Hereditary Ataxias, Friedreich's,Hereditary Spinal Scleroses,Scleroses, Hereditary Spinal,Spinal Scleroses, Hereditary,Spinal Sclerosis, Hereditary,Spinocerebellar Ataxia, Friedreich
D006801	Humans	Members of the species Homo sapiens.	Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000077123	Rocuronium	An androstanol non-depolarizing neuromuscular blocking agent. It has a mono-quaternary structure and is a weaker nicotinic antagonist than PANCURONIUM.	Androstane-3,17-diol, 2-(4-morpholinyl)-16-(1-(2-propen-1-yl)-1-pyrrolidiniumyl)-, 17-acetate, (2beta,3alpha,5alpha,16beta,17beta)-,1-(17-(Acetoyl)-3-hydroxy-2-(4-morpholinyl)androstan-16-yl)-1-(2-propenyl)pyrrolidinium,Esmeron,Esmerone,ORG 9426,ORG-9426,Pyrrolidinium, 1-((2beta,3alpha,5alpha,16beta,17beta)-17-(acetyloxy)-3-hydroxy-2-(4-morpholinyl)androstan-16-yl)-1-(2-propenyl)-, bromide,Rocuronium Bromide,Zemuron,ORG9426
D000293	Adolescent	A person 13 to 18 years of age.	Adolescence,Youth,Adolescents,Adolescents, Female,Adolescents, Male,Teenagers,Teens,Adolescent, Female,Adolescent, Male,Female Adolescent,Female Adolescents,Male Adolescent,Male Adolescents,Teen,Teenager,Youths
D000732	Androstanols	Androstanes and androstane derivatives which are substituted in any position with one or more hydroxyl groups.	Hydroxyandrostanes

Related Publications

H J Schmitt, and S Wick, and T Münster

Requirements for muscle relaxation in Friedreich's ataxia.

February 1998, Anaesthesia,

H J Schmitt, and S Wick, and T Münster

Friedreich's ataxia with heart disease in children.

February 1961, Pediatrics,

H J Schmitt, and S Wick, and T Münster

Neurophysiological evaluation in children with Friedreich's ataxia.

October 2009, Early human development,

H J Schmitt, and S Wick, and T Münster

Two Cases of Friedreich's Ataxia.

March 1897, Buffalo medical journal,

H J Schmitt, and S Wick, and T Münster

Breast cancer in two sisters with Friedreich's ataxia.

August 2001, European journal of surgical oncology : the journal of the European Society of Surgical Oncology and the British Association of Surgical Oncology,

H J Schmitt, and S Wick, and T Münster

Electroencephalographic findings in two families with Friedreich's ataxia.

January 1962, Confinia neurologica,

H J Schmitt, and S Wick, and T Münster

Nerve conduction studies in children with Friedreich's ataxia and ataxia-telangiectasia.

June 1973, Developmental medicine and child neurology,

H J Schmitt, and S Wick, and T Münster

Near infrared muscle spectroscopy in patients with Friedreich's ataxia.

May 2002, Muscle & nerve,

H J Schmitt, and S Wick, and T Münster

[Unusual molecular changes in two families with Friedreich's ataxia].

September 2003, Neurologia (Barcelona, Spain),