Abstract:We would like to thank Mr. W. D. Park, consultant surgeon, and Mr. Bernard Fairburn, consultant neurosurgeon, for permission to publish details of cases under their care. We are indebted to Mr. Sheppard for the photographs, and to the neurological department of the London Hospital for their assistance in the diagnosis of Barnes's myopathy.
“…Some reports comment upon muscle hypertrophy in susceptible subjects (Steers et al, 1970;King et al, 1972;Harriman et al, 1973). The pathological changes observed in muscle biopsies are variable and non-specific (Harriman et al, 1973;Isaacs et al, 1973;Ellis et al, 1975).…”
“…Some reports comment upon muscle hypertrophy in susceptible subjects (Steers et al, 1970;King et al, 1972;Harriman et al, 1973). The pathological changes observed in muscle biopsies are variable and non-specific (Harriman et al, 1973;Isaacs et al, 1973;Ellis et al, 1975).…”
“…TM In addition, there are statistical 1~176176 and genetic analyses. 44,46,61,81,1~176 There are a number of descriptions of malignant h v]3er-thermia in pigs ~~ in which the manifestations are similar to those in man. Several biochemical and microscopic 12~ studies exploring the cause of porcine malignant hyperthermia have been published, but their results and conclusions are not uniform.…”
Section: Introductionmentioning
confidence: 99%
“…10,17,26,74,81,99,101,108,109,141,143,145,146,150,158,187,188,189 This is not to say that the defects must be confined to skeletal muscle, nor does this necessarily mean that a defect in muscle is the primary lesion. 99,14~…”
“…In addition to their confirmation of the value of CPK in carrier detection, they found clinical evidence of myopathy. Steers et al (1970) found evidence of a myopathy in their susceptible family similar to that described by Barnes (1932). King, Denborough, and Zapf (1972) have suggested that susceptible individuals may show one of a number of specific myopathies consisting of a group with a dominantly inherited myopathy, a group who have myotonia congenita, and a third group with physical abnormalities and a progressive congenital myopathy.…”
SUMMARY The history, clinical presentation, and management of malignant hyperpyrexia are presented. The aetiology seems to be associated with some inherited abnormality which affects the movement and binding of calcium ions in the sarcoplasmic reticulum, sarcoplasm, and mitochondria. Whether this is a primary muscular defect or secondary to some trophic neural influence is yet to be established. The subjects carrying the abnormal trait show evidence of a myopathy which is subclinical in most instances and revealed only by estimation of serum CPK or biopsy. In some families where the myopathy is clinically obvious there may be, in addition, a variety of musculoskeletal abnormalities. A plea is made for routine monitoring of temperature during anaesthesia and for procainamide or procaine to be readily available in all operating theatres. A history of anaesthetic deaths in a family calls for special care, and, if the serum CPK is elevated, suxamethonium and halothane are to be avoided. Families with orthopaedic and muscular abnormalities are at increased risk and should have estimation of serum CPK before surgery. As a bonus of this study it is suggested that serum CPK estimations be used to screen pigs for selective breeding and so eliminate the disease, which causes soft exudative pork.
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