Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0026850 (muscular dystrophy)
 5,870 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Non-Fukuyama type congenital muscular dystrophy (n-FCMD), a subtype of progressive muscular dystrophy (PMD), is a very rare autosomal recessive disorder. N-FCMD is characterized by severe and progressive motor weakness and atrophies of proximal muscles during the infant period. A 9-year-old boy with n-FCMD underwent elective surgery for muscle release around the hip joints bilaterally. As many perioperative complications related with volatile anesthetics and muscle relaxants had been reported in the anesthetic management of PMD, these drugs were thought to be contraindicated in patients with n-FCMD. Because n-FCMD seemed to have very similar pathogenesis with PMD, caudal epidural block was chosen, supplemented with the administration of diazepam, pentazocine and nitrous oxide. The operation and anesthesia were conducted uneventfully. No complications occurred postoperatively.
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PMID:[Anesthetic management of a pediatric patient with non-Fukuyama type congenital muscular dystrophy]. 902 88

Among all the drugs used for general anaesthesia, neuromuscular blockers appear to play a prominent role in the incidence of severe adverse reactions. It now seems likely that most serious adverse drug reactions occurring during anaesthesia are immunological in type. The frequency of life-threatening anaphylactic or anaphylactoid reactions occurring during anaesthesia has been estimated to be between 1 in 1000 and 1 in 25,000 anaesthetic procedures, with the neuromuscular blockers being involved in 80% of cases. The mortality from such serious reactions is reported to be in the range of 3.4 to 6%. The highly immunogenic drug, suxamethonium chloride (succinylcholine), was found to be the most hazardous agent. Drug-specific immunoglobulin E antibodies to suxamethonium chloride and other neuromuscular blockers have been demonstrated. This sensitivity to neuromuscular blockers seems to be a long-lasting phenomenon. During anaesthesia, the clinical features of an allergic reaction are often masked. Tachycardia and circulatory collapse may be the only signs of an allergic reaction, and they are easily misdiagnosed. Bronchospasm is reported to be present in about 40% of cases. Successful management of these patients includes stabilisation during the acute reaction and avoidance of future reactions. The latter is based on the identification of the causative drug and potentially cross-reacting compounds. The use of suxamethonium chloride is associated with many other adverse effects, such as fasciculations, myalgia, potassium release, changes in the heart rate, increases in intragastric and intraocular pressures, and malignant hyperthermia. Because of the dangers of hyperkalaemic cardiac arrest after suxamethonium chloride administration in children with unrecognised muscular dystrophy, there have now been moves to limit the use of this drug in children. Although neuromuscular blockers are designed to specifically block nicotinic cholinergic receptors at the neuromuscular junction, many bind to muscarinic cholinergic receptors on ganglia and smooth muscle, and alter parasympathetically mediated heart rate and airway calibre. Most benzylisoquinolinium muscle relaxants can induce histamine release, especially when they are administered rapidly, which can lead to disturbances of cardiovascular function. In addition, nondepolarising neuromuscular blockers have been implicated in causing generalised weakness following their long term administration to patients on an intensive care unit. The problem with these adverse drug reactions is their unpredictable nature. Therefore, prompt recognition with appropriate therapy can help to improve the outcome.
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PMID:Adverse effects of neuromuscular blockers and their antagonists. 951 17

Nineteen patients with Duchenne's muscular dystrophy underwent segmental spinal instrumentation and posterior fusion between 1989 and 1994. The indication for surgery was loss of the ability to walk and development of scoliosis with sitting discomfort. Preoperative assessment included evaluation of pulmonary function. Average age at operation was 12.5 years. Instrumentation and fusion extended from upper thoracic levels to L-5 or the sacrum. A Hartshill rectangle was used in all cases, with banked allograft bone. Severe intraoperative blood loss was avoided by use of hypotensive anaesthesia. Peroperatively, systolic blood pressure was maintained between 75 and 85 mm Hg. Average blood loss was 1,246 ml (range, 400-3,100) or 30% of estimated total blood volume. Average transfusion requirements were 3 units of packed cells. Postoperative analgesia was provided by infusion via an epidural catheter. There were no postoperative wound or chest infections. Three patients required catheterisation for urinary retention. Postoperatively patients were fitted with a Neofract jacket to allow early mobilisation and discharge. Mean postoperative length of stay was 16 days. Posterior spinal fusion by using the Hartshill rectangle provided good correction and fixation. Hypotensive anaesthesia permitted surgery to be performed rapidly in a relatively dry field and avoided the complications of severe intraoperative blood loss and massive transfusion.
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PMID:Spinal instrumentation for Duchenne's muscular dystrophy: experience of hypotensive anaesthesia to minimise blood loss. 959 68

Among all the drugs used for general anesthesia, neuromuscular blockers appear to play a prominent role in the incidence of severe adverse reactions. It now seems likely that most serious adverse drug reactions occurring during anesthesia are immunological in type. The frequency of life-threatening anaphylactic or anaphylactoid reactions occurring during anesthesia has been estimated to be between 1 in 1000 and 1 in 25,000 anesthetic procedures, with the neuromuscular blockers being involved in 80% of cases. The mortality from such serious reactions is reported to be in the range of 3.4 to 6%. The highly immunogenic drug, suxamethonium chloride (succinylcholine), was found to be the most hazardous agent. Drug-specific immunoglobulin E antibodies to suxamethonium chloride and other neuromuscular blockers have been demonstrated. This sensitivity to neuromuscular blockers seems to be a long-lasting phenomenon. During anesthesia, the clinical features of an allergic reaction are often masked. Tachycardia and circulatory collapse may be the only signs of an allergic reaction, and they are easily misdiagnosed. Bronchospasm is reported to be present in about 40% of cases. Successful management of these patients includes stabilisation during the acute reaction and avoidance of future reactions. The latter is based on the identification of the causative drug and potentially cross-reacting compounds. The use of suxamethonium chloride is associated with many other adverse effects, such as fasciculations, myalgia, potassium release, changes in the heart rate, increases in intragastric and intraocular pressures, and malignant hyperthermia. Because of the dangers of hyperkalemic cardiac arrest suxamethonium chloride administration in children with unrecognised muscular dystrophy, there have now been moves to limit the use of this drug in children. Although neuromuscular blockers are designed to specifically block nicotinic cholinergic receptors at the neuromuscular junction, many bind to muscarinic cholinergic receptors on ganglia and smooth muscle, and alter parasympathetically mediated heart rate and airway calibre. Most benzylisoquinolinium muscle relaxants can induce histamine release, especially when they are administered rapidly, which can lead to disturbances of cardiovascular function. In addition, nondepolarising neuromuscular blockers have been implicated in causing generalised weakness following their long term administration to patients on an intensive care unit. The problem with these adverse drug reactions is their upredictable nature. Therefore, prompt recognition with appropriate therapy can help to improve the outcome.
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PMID:Adverse effects of neuromuscular blockers and their antagonists. 978 39

A five year-old boy undergoing elective tonsillectomy sustained cardiac arrest following the administration of a single dose of succinylcholine during induction of anesthesia. With a 10-minute cardiopulmonary resuscitation (CPR) during which intravenous calcium gluconate, epinephrine, and sodium bicarbonate were given and DC counter shock applied, we were successful to restore cardiac activity without neurological sequelae. The cause of cardiac arrest we speculated was hyperkalemia, possibly secondary to succinylcholine-induced rhabdomyolysis. It is suggested that succinylcholine should not be used in patients with known or suspected muscular dystrophy.
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PMID:Succinylcholine-induced cardiac arrest in unsuspected becker muscular dystrophy--a case report. 987 66

A 15-year-old boy with Duchenne's muscular dystrophy experienced prolonged cardiac arrest whilst in the prone position for spinal surgery. He was successfully resuscitated without apparent neurological sequelae by internal cardiac massage via a thoracotomy and external and internal direct current cardioversion. Recommendations are suggested for the pre- and peroperative management of such cases.
Anaesthesia 1999 Apr
PMID:A case of ventricular fibrillation in the prone position during back stabilisation surgery in a boy with Duchenne's muscular dystrophy. 1057 87

Duchenne's muscular dystrophy is a genetic disorder whose features include abnormal responses to muscular relaxants and possible respiratory dysfunction after general anesthesia. The purpose of this report is to describe one management strategy used successfully to anesthetize an obese man with this disorder for atypical mastectomy. The anesthetic management during surgery involves epidural anesthesia using 2% mepivacaine and general anesthesia using the cuffed oropharyngeal airway. Postoperatively 0.25% bupivacaine was infused for epidural analgesia. The patient was observed in the intensive care unit until the first postoperative day and showed an uncomplicated intra- and post-operative course.
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PMID:[Epidural anesthesia and general anesthesia using the cuffed oropharyngeal airway for an obese patient with Duchenne's muscular dystrophy]. 1065 17

A 43-year-old man developed rhabdomyolysis after uvulo- palatopharyngoplasty. After induction with thiopentone and suxamethonium the anesthesia was maintained with halothane. The patient responded to treatment and made an uneventful recovery. In earlier reports of rhabdomyolysis after general anaesthesia with halothane and suxamethonium almost all the patients had malignant hyperthermia (MH) or muscular dystrophy. About 50% of malignant hyperthermia patients carry a mutation in the RYR1 gene. Our patient did not have mutations in the four MH-associated genes tested, but the total amount of different mutations is by now about twenty. Therefore, despite these negative tests rhabdomyolysis may be a sign of subclinical malignant hyperthermia which cannot be ruled out by our investigations. This rare case of rhabdomyolysis in a healthy man suggests careful monitoring of the patient when-ever suxamethonium is used.
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PMID:Suxamethonium-induced rhabdomyolysis in a healthy middle-aged man. 1080 23

Episodes of acute myoglobinuria or cardiac arrest were occasionally complicated in general anesthesia of patients with Duchenne or Becker muscular dystrophy (DMD/BMD). Whether these complications are malignant hyperthermia (MH) or not has several times been discussed. In the present study, we applied the clinical grading scale (CGS) of Larach and modified criteria of caffeine contracture test of the skinned fiber (sIVCT) to solve this problem. When the CGS was applied to reported MH-like episodes of DMD/BMD cases, 9 out of 20 cases were classed as almost certain or very likely MH. According to results of sIVCT in 11 patients with DMD/BMD, 5 patients were judged as MHS (MH-susceptible) and 3 as MHE (MH-equivocal). The diagnostic specificity of present MHS criteria was 100% for the fulminant MH. A possible "false positive" result in European IVCT has been discussed in relation to myopathy such as muscular dystrophy. When we applied our sIVCT to the muscle of mdx mouse, caffeine contracture was rather reduced compared to controls. Present study suggested that a true MH was complicated in some cases of DMD/BMD. In certain stage of muscular degeneration, patients with DMD/BMD become susceptible to MH, probably temporarily, but exact mechanism still awaits clarification.
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PMID:[Malignant hyperthermia of Duchenne muscular dystrophy: application of clinical grading scale and caffeine contracture of skinned muscle fibers]. 1100 22

The paper presents the description of Duchenne progressive muscular dystrophy in an 18-month-old and an 8-year-old boy. The diagnosis was established on the basis of clinical symptoms, such as impaired motor development, hypertrophy of leg muscles, difficulty in walking; elevated serum phosphocreatine kinase activity and pathologic electromyographic recordings. The authors emphasize that the disease is characterized by increased activity of such enzymes as: alanine and aspartate aminotransferases, lactate dehydrogenase and aldolase--observed as early as in the first weeks of life, with normal gammaglutamyltranspeptidase activity suggests progressive muscular dystrophy and makes it possible to establish early diagnosis. Early diagnosis of the disease allows to start rehabilitation earlier, to select an appropriate type of anesthesia in case of surgical treatment and to advise parents within the framework of genetic counseling.
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PMID:Early symptoms of Duchenne muscular dystrophy--description of cases of an 18-month-old and an 8-year-old patient. 1120 76


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