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Query: UMLS:C0026850 (muscular dystrophy)
 5,870 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Under ether anesthesia electrocardiograms were derived from Syrian hamsters (strain BIO 8262) suffering from cardiomyopathy and muscular dystrophy. In addition, ventricular weights and body weight were determined. Young hamsters -- not yet showing morphological signs of the cardiomyopathy with the exception of possible left ventricular hypertrophy -- demonstrated only a longer ventricular activation time than normal hamsters. With the onset of cardiac necrotization left axis deviation in frontal plane projection and right bundle branch blocks are developing in the cardiomyopathic hamsters followed by first degree atrioventricular conduction defects. During the late stage of the cardiomyopathy left bundle branch blocks are additionally arising, while left ventricular hypertrophy is disappearing. Since no overt heart failure is occurring in this strain of cardiomyopathic hamsters, gradual development of high degree conduction defects is assumed to terminate their lives. The electrocardiographic pattern of the hamster cardiomyopathy fits partly into that of human primary as well as secondary cardiomyopathy. Nevertheless, it seems to form an entity of its own, as arrhythmias, higher degree atrioventricular conduction disturbances, typical signs of ventricular or septal hypertrophy, abnormal P and Q waves, ST segment and T wave changes are lacking.
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PMID:Electrocardiographic changes in cardiomyopathic Syrian hamsters (strain BIO 8262). 14 76

A total of 600 handicapped patients had dental rehabilitation under general anesthesia during an eight-year period. Handicaps included mental retardation, cerebral palsy, Down syndrome, seizure disorders, autism, cystic fibrosis, osteogenesis imperfecta, and muscular dystrophy. No significant complications developed in the majority of patients. This is attributed to thorough preoperative evaluation, appropriate anesthetic management, and vigilant postoperative observation.
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PMID:Complications related to the administration of general anesthesia in 600 developmentally disabled dental patients. 15 47

Our purpose was to determine whether an apparently healthy patient who died under general anaesthesia had malignant hyperpyrexia by examining her relatives and to suggest protective measures for the relatives of the deceased patient against this complication during future general anaesthetics. The family members of the deceased patient were examined systematically to determine whether or not they were prone to develop malignant hyperpyrexia. Raised serum CPK and aldolase levels, EMG changes, histopathological examination of the striated muscle, diminished muscle power during an ergometric test, and subjective symptoms revealed that other members of her family had muscular dystrophy. Our results support the theory that during general anaesthesia patients with muscular dystrophy are prone to develop malignant hyperpyrexia. Although muscular dystrophy is uncommon in Finland, affected persons should be catalogued, and preventive measures against malignant hyperpyrexia taken if they ever have to have a general anaesthetic.
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PMID:Malignant hyperpyrexia. A study of an affected family. 113 33

The case is reported of a four-year-old boy with progressive muscular dystrophy who underwent anal fistulectomy under neuroleptanaesthesia with Thalamonal. Recovery was uneventful. Anaesthetic problems associated with the disease include sudden death due to myocardial involvement and high postoperative mortality due to pulmonary infection. Mental retardation and ECG abnormalities may be present and acute postoperative gastric dilatation has been reported. Narcotics or minor tranquilizers and hyoscine are recommended for premedication. Light anaesthesia should be maintained throughout surgery and minimal doses of non-depolarizing muscle relaxants may be used if necessary. Suxamethonium chloride should be avoided because muscle damage and cardiac arrest might occur.
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PMID:General anaesthesia for a patient with progressive muscular dystrophy. 126 19

A 9 year old male previously diagnosed as progressive muscular dystrophy whose serum CPK5430IU.l-1 was very high received general anesthesia. Before anesthesia, dantrolene sodium 2 mg.kg-1 was given. Anesthesia was induced with thiamylal 100 mg and vecuronium bromide 3 mg. Anesthesia was maintained with sevoflurane (0.5%) in nitrous oxide (66%) and oxygen (33%). The course of anesthesia was uneventful. The operative time was 80 minutes. At the end of the operation, the patient recovered smoothly from anesthesia. A 46 year old female with dystrophia myotonia also received general anesthesia. The patient was diagnosed as having this disease 26 years previously. Preoperatively, the patient was suspected to have cardiac damage. Anesthesia was induced with thiamylal 100 mg, fentanyl 100 micrograms, midazolam 5 mg and vecuronium bromide 4 mg, and maintained with sevoflurane (1.0%) in nitrous oxide (66%) and oxygen (33%). Anesthesia was uneventful, but at the end of the operation, the patient could not breath fully by herself. She was placed on a ventilator and observed carefully. The endotracheal tube was removed 150 minutes after the induction of anesthesia. In these two cases, sevoflurane and vecuronium bromide were used safely.
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PMID:[General anesthesia with sevoflurane and vecuronium for patients with dystrophia myotonica and progressive muscular dystrophy]. 168

A 6-yr-old boy who presented with brown urine due to myoglobinuria and who was otherwise virtually asymptomatic was diagnosed as having Becker muscular dystrophy on the basis of a greatly elevated creatine kinase, muscle biopsy, dystrophin analysis, and a deletion of exons 3-7 in the dystrophin gene. Fifteen months later, during a general anaesthetic for dental treatment, he had a cardiac arrest associated with acute rhabdomyolysis, hyperkalaemia and hypocalcaemia. He died 4 days later. This case is reported to highlight this rare but potentially fatal complication of anaesthesia in muscular dystrophy, and to discuss possible ways of preventing such a catastrophe.
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PMID:Fatal rhabdomyolysis complicating general anaesthesia in a child with Becker muscular dystrophy. 182 95

Emery-Dreifuss syndrome is a rare form of muscular dystrophy associated with cardiac complications that lead to sudden death. The disorder and its potential anaesthetic implications in the management of a patient who presented for orthopaedic surgery is described.
Anaesthesia 1991 Jan
PMID:Emery-Dreifuss muscular dystrophy. 199 51

An 8-year-old boy known to have Duchenne's muscular dystrophy suffered a cardiac arrest 10 minutes after he regained consciousness after isoflurane anaesthesia for an orchidopexy procedure. Resuscitation was successful 2 hours after the start of external cardiac compression and after correction of hyperkalaemia and the administration of dantrolene. He later developed myoglobinuria elevated creatine kinase and a metabolic and respiratory acidosis. He demonstrated a delayed increase in rectal temperature.
Anaesthesia 1990 Jan
PMID:Cardiac arrest after isoflurane anaesthesia in a patient with Duchenne's muscular dystrophy. 231 33

Genetic cardiac dysrhythmias, although rare in pediatrics, may be often the first manifestation of the main disease or may determine the vital prognosis in some of these diseases (e. g. Friedreich's ataxia and Duchenne's progressive muscular dystrophy). This is more so, as at present, technical possibilities of implanting pacemakers, at ever smaller ages, are ever so great. The first part of the paper deals with these aspects, reviews, and analyses a large variety of genotypic diseases (metabolism genetic diseases, cardio-skeletal syndromes, neuromuscular diseases, cardio-auditory syndromes, etc.) and also a series of tachydysrhythmias or isolated familial conduction disturbances (blocks). Within each entity, the authors describe the main elements allowing the diagnosis, with special references on the rhythm and cardiac conduction disturbances that may appear, and with the necessary therapeutical considerations. The second part of the material presented is devoted to the cardiac dysrhythmias following the surgical intervention on heart, frequently used at present in the congenital heart diseases, at ever smaller ages due to the special progress in anesthesia and intensive care techniques. Correlation of various types of surgeries with the anatomical lesions of the excitation-conduction system they can involve, makes easier the understanding of the nature and appearance risks of some postsurgical rhythm and cardiac conduction disturbances. The distinction is made between early postsurgical dysrhythmias--that are often episodic--and tardy dysrhythmias that may relapse or become chronic, thus raising difficult treatment problems and sometimes require a secondary pacemaker implantation, given the major handicap they represent for the child.
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PMID:[Genetic cardiac dysrhythmias and the sequelae of heart surgery in children]. 251 77

The genetic etiology, pathophysiology, natural progression, prognosis, and complications of Landouzy-Dejerine facio-scapulo-humeral benign muscular dystrophy are presented, as well as the risks of anesthesia and surgery. Patients with relatively benign myopathies may be candidates for reconstructive orthognathic surgery to improve oral function, facial esthetics, social interchange, and general quality of life. The surgery can be a gratifying experience for the patient, the family, and the surgeon, but the procedure, the anesthesia, and the postoperative care must be carefully planned to minimize the genuine risks.
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PMID:Correction of facial-skeletal deformities in two patients with facio-scapulo-humeral muscular dystrophy. 252 95


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