Duchenne muscular dystrophy, serum

There is an association between (latent) muscular dystrophy (usually of the Duchenne or Becker type) and the production of rhabdomyolysis by suxamethonium (84,85,89,90). Suxamethonium can cause excessive muscle damage in these patients, as manifested not only by severe myoglobinemia and raised serum creatine kinase activity but also by acute exacerbation of muscle weakness postoperatively (SEDA-11, 121) (7,29,84,91,92). Massive potassium release can result in hyperkalemic cardiac arrest. Such patients may also develop features suggestive of the syndrome of malignant hyperthermia (93,94). Suxamethonium should not be used in patients with Duchenne muscular dystrophy or who have a family history suspect for the condition. 

R13. Roy, 8., and Dubowitz, V., Carrier detection in Duchenne muscular dystrophy. A comparative study of electron microscopy, light microscopy and serum enzymes. 

E. Bertini, and I. Bozzoni (2011). miRNAs as serum biomarkers for Duchenne muscular dystrophy. EMBO Mol Med 3(5) 258-265. 

H. Seol, H. Gordish-Dressman, S. Cirak, L. Bello, K. Nagaraju, T. Partridge, E. P. Hoffman, S. Takeda, J. K. Mah, E. Henricson, and C. McDonald (2014). Discovery of serum protein biomarkers in the mdx mouse model and cross-species comparison to Duchenne muscular dystrophy patients. Hum Mol Genet 23 6458-6469. 

Hu, J., M. Kong, Y. Ye, S. Hong, L. Cheng, and L. Jiang (2014). Serum miR-206 and other muscle-specific microRNAs as non-invasive biomarkers for Duchenne muscular dystrophy. J Neurochem 129(5) 877-883. 

The copious muscle enzyme efflux in Duchenne-type muscular dystrophy, giving gross serum elevations despite the rapid serum clearance, may well deplete some muscle enzymes that so much aldolase still remains may indicate a replacement so rapid that, if applied to the transaminases and to lactic dehydrogenase, the muscle content may be maintained or even increased, since their serum elevations, though considerable, are proportionately much less than that of aldolase. 

Again, continued loss of dystrophic muscle must diminish its total enzyme efflux. In Duchenne-type muscular dystrophy the highest serum enzyme elevations occur in early childhood, diminish as the disease pro-... 

Figures 7, 8, and 9 (pp. 170 and 171) show the eflFects after a week in bed, though free to move about in it (WIO), of 2 days of ordinary ambulation on the serum enzymes of three little boys at difi erent stages of Duchenne-type muscular dystrophy (T8). Such modified rest has little effect, except on SGPT in the earliest case, but the immediate and extreme...

Fig. 4. Relationship between serum aldolase activity and exact age in Duchenne-type muscular dystrophy.

Thus in muscular dystrophy it is apparent that both the mean elevations of the serum enzyme values and the magnitudes of their variations upon physical activity are proportional to the mass of dystrophic muscle remaining and to the severity of the disease in it. Both are thus greater in early than in evident Duchenne-type dystrophy, less in limb-girdle dystrophy, and least in myotonia congenita. Further, though serum creatine kinase has been found to be an exceedingly delicate index of myopathy (A2, S14), for present purposes serum aldolase is suflBciently... 

Al. Aebi, U., Richterich, R., Colombo, J. P., and Rossi, E., Progrossive muscular dystrophy. II. Biochemical identification of the carrier state in the recessive sex-linked juvenile (Duchenne) type by serum creatine-phosphokinase determinations. Enzymol. Biol. Clin. 1, 61 (1961-1962). 

H9. Hughes, B. P., Serum enzyme studies with special reference to the Duchenne type dystrophy. In Research in Muscular Dystrophy pp. 167-179. Pitman, London, 1963. 

Serum CK activity is greatly elevated in all types of muscular dystrophy. In progressive muscular dystrophy (particularly Duchenne sex-linked muscular dystrophy), enzyme activity in serum is highest in infancy and childhood (7 to 10 years of age) and may be increased long before the disease is clinically apparent. Serum CK activity characteristically falls as patients get older and as the mass of functioning muscle diminishes with the progression of the disease. About 50% to 80% of the asymptomatic female carriers of Duchenne dystrophy show threefold to sixfold increases of CK activity, but values may be normal if specimens are obtained after patients have experienced a period of physical inactivity. Quite high values of CK are noted in... 

A3. Askanas, W., Identification of the agent responsible for the abnormal immuno-electrophoretie pattern of serum in Duchenne s progressive muscular dystrophy. Life Sci. 6, 1767-1773 (1966). 

H12. Hudgson, P., Gardner-Medwin, D., Pennington, R. J. T., and Walton, J. N., Studies of the carrier state in the Duchenne type of muscular dystrophy. Part I. Effect of exercise on serum creatine kinase activity. J. Neurol. Neurosurg. Psychiat. 30, 416-419 (1967). 

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