Biomaterial Database

Responses to nondepolarizing neuromuscular blockers and succinylcholine in von Recklinghausen neurofibromatosis. 1996

M G Richardson, and G K Setty, and S A Rawoof

Department of Anesthesiology, University of Rochester School of Medicine and Dentistry, Strong Memorial Hospital, NY 14642, USA. mrichardson@ccmail.anes.rochester.edu

Patients with type 1 neurofibromatosis (NF-1) have been reported to have prolonged responses to nondepolarizing (ND) neuromuscular blockers (NMBs). Responses to succinylcholine (SCh) have been described as increased, decreased, or normal. The purpose of this study was to assess responses to NMBs in NF-1 patients in order to determine the clinical significance of abnormal responses. We retrospectively identified all NF-1 patients who received anesthetics at Strong Memorial Hospital between January 1, 1984 and December 31, 1994. We then reviewed all anesthetic records to classify responses to NMBs as normal, abnormal, or indeterminate. Records of 114 anesthetics provided to 44 NF-1 patients were reviewed. Nondepolarizing NMBs were used during 73 anesthetic cases in 38 patients. Responses were normal in 69 cases and indeterminate in 4 (3 in patients with normal responses during other anesthetics). SCh was used during 42 anesthetic cases in 23 patients. Responses were normal in all but one case (indeterminate) in a patient who had had other documented normal responses. Standard milligram per kilogram doses of NMBs were used in all cases, and in none was there evidence of abnormal response. The risk of abnormal response to NMBs in individuals with NF-1 appears to be minimal. We recommend no alteration in dosing of either SCh or ND NMBs in patients with NF-1.

UI	MeSH Term	Description	Entries
D009456	Neurofibromatosis 1	An autosomal dominant inherited disorder (with a high frequency of spontaneous mutations) that features developmental changes in the nervous system, muscles, bones, and skin, most notably in tissue derived from the embryonic NEURAL CREST. Multiple hyperpigmented skin lesions and subcutaneous tumors are the hallmark of this disease. Peripheral and central nervous system neoplasms occur frequently, especially OPTIC NERVE GLIOMA and NEUROFIBROSARCOMA. NF1 is caused by mutations which inactivate the NF1 gene (GENES, NEUROFIBROMATOSIS 1) on chromosome 17q. The incidence of learning disabilities is also elevated in this condition. (From Adams et al., Principles of Neurology, 6th ed, pp1014-18) There is overlap of clinical features with NOONAN SYNDROME in a syndrome called neurofibromatosis-Noonan syndrome. Both the PTPN11 and NF1 gene products are involved in the SIGNAL TRANSDUCTION pathway of Ras (RAS PROTEINS).	Peripheral Neurofibromatosis,Recklinghausen Disease of Nerve,von Recklinghausen Disease,Cafe-au-Lait Spots with Pulmonic Stenosis,Molluscum Fibrosum,NF1 (Neurofibromatosis 1),Neurofibromatosis I,Neurofibromatosis Type 1,Neurofibromatosis Type I,Neurofibromatosis, Peripheral Type,Neurofibromatosis, Peripheral, NF 1,Neurofibromatosis, Peripheral, NF1,Neurofibromatosis, Type 1,Neurofibromatosis, Type I,Pulmonic Stenosis with Cafe-au-Lait Spots,Recklinghausen Disease, Nerve,Recklinghausen's Disease of Nerve,Recklinghausens Disease of Nerve,Watson Syndrome,von Recklinghausen's Disease,Cafe au Lait Spots with Pulmonic Stenosis,Neurofibromatoses, Peripheral,Neurofibromatoses, Type I,Neurofibromatosis, Peripheral,Peripheral Neurofibromatoses,Pulmonic Stenosis with Cafe au Lait Spots,Syndrome, Watson,Type 1 Neurofibromatosis,Type 1, Neurofibromatosis,Type I Neurofibromatoses,Type I, Neurofibromatosis,von Recklinghausens Disease
D009467	Neuromuscular Depolarizing Agents	Drugs that interrupt transmission at the skeletal neuromuscular junction by causing sustained depolarization of the motor end plate. These agents are primarily used as adjuvants in surgical anesthesia to cause skeletal muscle relaxation.	Depolarizing Muscle Relaxants,Muscle Relaxants, Depolarizing,Depolarizing Blockers,Agents, Neuromuscular Depolarizing,Blockers, Depolarizing,Depolarizing Agents, Neuromuscular,Relaxants, Depolarizing Muscle
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
D006801	Humans	Members of the species Homo sapiens.	Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000758	Anesthesia	A state characterized by loss of feeling or sensation. This depression of nerve function is usually the result of pharmacologic action and is induced to allow performance of surgery or other painful procedures.
D013390	Succinylcholine	A quaternary skeletal muscle relaxant usually used in the form of its bromide, chloride, or iodide. It is a depolarizing relaxant, acting in about 30 seconds and with a duration of effect averaging three to five minutes. Succinylcholine is used in surgical, anesthetic, and other procedures in which a brief period of muscle relaxation is called for.	Succinyldicholine,Suxamethonium,Anectine,Celocurine,Dicholine Succinate,Ditilin,Listenon,Lysthenon,Myorelaxin,Quelicin,Succicuran,Succinylcholine Chloride,Succinylcholine Dibromide,Succinylcholine Dichloride,Succinylcholine Dichloride, Di-H2O,Succinylcholine Diiodide,Succinylcholine Diperchlorate,Succinylcholine Iodide,Suxamethonium Bromide,Suxamethonium Chloride,Bromide, Suxamethonium,Dibromide, Succinylcholine,Dichloride, Succinylcholine,Diiodide, Succinylcholine,Diperchlorate, Succinylcholine,Succinate, Dicholine,Succinylcholine Dichloride, Di H2O