Serum Enzyme Activity in Muscular Dystrophy

Thomson, W. H.; Leyburn, Peter; Walton, John N.

doi:10.1136/bmj.2.5208.1276

Cited by 52 publications

(19 citation statements)

References 23 publications

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“…However, the specific reasons for choosing DIES were related to the unusual LDH isozyme distributions found in the serum (16) and muscles (17) of children with DMD and our own findings, that LDH isozyme distributions could be manipulated by several stilbene derivatives including DIES.…”

Section: %B%@%h[ Hrnandhrnmentioning

confidence: 88%

Diethylstilbestrol: Observations on Its Use in Duchenne's Muscular Dystrophy (DMD)

Cohen

Morgan

Schulman

1972

Experimental Biology and Medicine

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Several observations suggest that the course of muscular dystrophy and other myopathies may be sex-influenced. Clinically, muscular dystrophy is less severe in women than in men ( l ) , and the female carrier of the Duchenne gene suffers little muscle impairment though her serum enzymes may be higher than normal ( 2 ) . Experimentally, dystrophic female mice grow better and live longer than do dystrophic male mice ( 3 ) , and steroid-induced myopathy is less severe in the female than the male mouse (4). Finally, the synthetic estrogenic substance, diethylstilbestrol (DIES), appears to be an anabolic agent ( 5 , 6) and has been found in skeletal muscle after administration to dowstic animals (7, 8 ) . These observations and others that will be discussed below suggested that estrogen administration might be useful in DMD. This report summarizes the effects on the serum enzymes and selected muscle parameters in three patients with DMD, treated for 6 months to 3 years with DIES.Methods. The diagnosis of DMD was established in each patient by history, physical and neurological examinations, serum enzyme and lactate dehydrogenase (LDH) isozyme analyses, similar enzyme studies in family members, electromyography, and the histological examination and enzyme assay of several muscle biopsies. T.M., B.C. and D.S. were 13, 11 and 4 years of age, respectively, at the start of their studies, Their muscular disability was quantitated using a 0-10 grade scale (9) (0, normal; 10, totally disabled). T.M.'s disability was grade 6-7; B.C.'s was grade 6-7 ; and D.S.'s was grade 3.Each therapeutic trial consisted of four periods. Period 1 was the control or pretreatment period. Period 2 was the period during which DIES was initially administered. Dur-ing Period 3 DIES was discontinued; and during Period 4 DIES was readministered.The dose of DIES,was 2 to 5 mg/day.During each period, multiple venous blood samples were collected as atraumatically as possible, shortly after arising. Serum was analyzed spectrophotometrically, within 24 hr, for LDH by the method of Cohen and Larson (10) and creatine phosphokinase (CPK) by the method of Rosalki (11). Control serum was analyzed simultaneously, to insure reagent stability. The upper normal limit for LDH is 390 units/ml serum and f o r CPK 50 milli-International Units (mIU) /ml serum.Muscle evaluations were performed at the start of Period 1 and end of each subsequent period by our chief physical therapist. He followed a specific protocol ( 1 2 ) and had no knowledge of the patient's therapy status. These evaluations included manual muscle testing, measurement of limb circumference, joint goniometry, and measurement of the time required to perform certain tasks.Other tests performed at appropriate intervals included complete blood counts; urinalyses; X-ray examination of the chest; radiodensity of the fingers; creatinine clearance and blood urea nitrogen ; electrocardiographic and vectorcardiographic examinations ; electromyography ; serum cholesterol and triglycerides ; glucose tolerance and ...

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Section: %B%@%h[ Hrnandhrnmentioning

confidence: 88%

Diethylstilbestrol: Observations on Its Use in Duchenne's Muscular Dystrophy (DMD)

Cohen

Morgan

Schulman

1972

Experimental Biology and Medicine

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“…A much smaller increase is found in the other forms of muscular dys trophy (limb-girdle type, facioscapulohumeral type, ocular type, oculo-pharingeal and distal types). Even a smaller increase is found in dystrophia myotonica [13,16,24,27,29,36,39]. The very high values found in the pre-clinical and early stages of Duchenne type and to a lesser extent in the Becker type and limb-girdle type are not main-…”

Section: Introductionmentioning

confidence: 86%

Serum and Muscle Creatine Kinase Isoenzymes and Serum Aspartate Aminotransferase Isoenzymes in Progressive Muscular Dystrophy

A¹,

S²,

Lippi³

et al. 1971

Enzyme

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Muscle and serum CPK electrophoretic pattern and serum GOT electrophoretic pattern were studied in Duchenne type, limb-girdle type and distal congenital type muscular dystro- phies. Muscle CPK isoenzyme pattern usually did not show significant changes. In 2 cases a fetal-like pattern was observed. Serum CPK isoenzyme pattern showed in some cases only the MM type. In other cases MB type was found in addition to MM type. Serum electrophoresis zone of GOT displayed in some cases only the cytoplasmic isoenzyme. Other cases showed mitochondrial isoenzyme in addition to the cytoplasmic type. The latter was predominant. GOT mitochondrial isoenzyme and CPK MB isoenzyme were found in serum in the early stages of the disease. These results suggest that a relationship may intercur between the pathological changes in the muscle in early muscular dystrophy and the presence in serum of GOT mitochondrial isoenzyme and CPK MB isoenzyme.

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“…In the absence of significant neurological signs a diagnosis of myopathy in both patients was supported by the abnormally increased serum activities of aldolase (Thomson, Leyburn, and Walton, 1960;Thomson, 1962) and creatine phosphokinase (Schapira and Dreyfus, 1960;Aebi et al, 1962) found in myopathy but not in neuropathy. Both enzymes are particularly abundant in skeletal muscle, and aldolase, but not creatine phosphokinase, in liver and red cells also.…”

Section: Discussionmentioning

confidence: 77%

“…In case 1 the normal electromyogram, the absence of muscular wasting, and a history of full muscular power at rest changing to painful weakness on exercise conformed neither to dystrophy nor to polymyositis. The serum transaminases were scarcely raised compared with their elevations in myopathic wasting (Thomson, 1962); there was no creatinuria, and the E.S.R. was normal.…”

Section: Discussionmentioning

confidence: 83%