Serum Muscle Enzyme Levels Research Articles

Effects of ubiquinone (coenzyme Q10) on myopathy in statin users

Statins are associated with muscle complaints, including myositis. The mechanism through which statin use causes muscle toxicity is unknown. One of the theories is that statin therapy reduces coenzyme Q10 levels in muscle mitochondria, which leads to muscle injury and myopathy. The aim of the present article is to review published data on the association between coenzyme Q10 and statin-associated myopathy. Studies have consistently shown that statins reduce coenzyme Q10 levels in serum and that supplementation of coenzyme Q10 increases these levels. However, the effect of statin therapy on coenzyme Q10 levels in muscle has been conflicting. Recently, two pilot studies on coenzyme Q10 supplementation in statin-induced myopathy and one study on the effect of coenzyme Q10 supplementation on serum muscle enzyme levels were published. These three studies were the first randomized trials with coenzyme Q10 supplementation in hypercholesterolemic patients treated with statins. The results of these trials have been contradictory; whereas one seems to support supplementation with coenzyme Q10, the other two do not. This review summarizes the current evidence on coenzyme Q10 supplementation in statin-induced myopathy. We conclude that the present evidence does not support coenzyme Q10 supplementation in statin-induced myopathy.

Histopathological evidence of small-vessel vasculitis within the skin and lungs associated with interstitial pneumonia in an adult patient with dermatomyositis

Dermatomyositis (DM) often has a poor prognosis, due to complications associated with malignancy or interstitial pneumonia (IP). It is uncommon to find histopathological small-vessel vasculitis within cutaneous lesions and pulmonary capillaritis in patients with DM. A 64-year-old woman was diagnosed with DM based on the presence of quadriparesis, typical heliotropic rash, Gottron's papules, increased serum levels of muscle enzymes and typical muscle biopsy findings. She also had associated IP, which subsequently developed into a rapidly progressive condition. We found a high titre of Krebs von den Lunge (KL)-6 in her serum. Measurement of serum KL-6 level is widely accepted as a diagnostic test to monitor the activity of interstitial lung diseases. Histopathological examinations of the cutaneous and lung-associated features showed small-vessel vasculitis. We suggest that investigation of KL-6 levels in patients with DM and associated conditions should be carried out to determine if raised KL-6 levels are useful at predicting outcome or severity of various features.

Renal manifestations of Dent disease and Lowe syndrome

To date, two responsible genes for the development of Dent disease have been identified: CLCN5 and OCRL1. In this study, genotype-phenotype correlations were studied in patients with Dent disease and those with Lowe syndrome. Among the 12 boys with a phenotype typical of Dent disease, nine had a mutation in CLCN5 (Dent disease 1), two had a mutation in OCRL1 (Dent disease 2), and one had no mutations in either gene. All seven boys with a clinical diagnosis of Lowe syndrome had a mutation in OCRL1. Patients with Lowe syndrome showed more frequent hypophosphatemia/rickets and more prominent tubular proteinuria than patients with Dent disease 1, and patients with Dent disease 2 had higher degree of tubular proteinuria and hypercalciuria than patients with Dent disease 1. Additionally, one patient with Dent disease 2 showed a mild degree of developmental delay, elevated serum muscle enzyme levels, and cryptorchidism. In this study, the genetic heterogeneity in Dent disease and the phenotypic heterogeneity in Lowe syndrome were confirmed. In patients with Dent disease, the presence of the above-mentioned extrarenal manifestations indicates that it is more likely that the patient is affected by Dent disease 2 than by Dent disease 1.

Inflammatory muscle disease: Clinical presentation and assessment of patients

Muscle weakness and muscle fatigue are the most common manifestations in patients with idiopathic inflammatory myopathies (ie, myositis), but other organs are frequently involved such as skin, lungs, joints, and the heart. These could occur before, simultaneously with, or after the onset of muscle symptoms. One tool to structure outcome measure is the International Classification of Functioning, Disability, and Health. Measures of "disability" can be divided into impairment and activity limitation/participation restriction. Most of the available outcome measures for myositis could be classified as measures of impairment, such as muscle strength, serum levels of muscle enzymes, and pulmonary function tests. Measures of activity limitation/participation restriction are also important, such as the myositis activities profile, the visual analog scale to assess impact on general well-being, and the generic short form-36 to capture impact on health-related quality of life.

Intermediate Syndrome Following Acute Organophosphate Poisoning: Correlation with Initial Serum Levels of Muscle Enzymes*

Following acute organophosphate poisoning, intermediate syndrome essentially relates to the impairment of neuromuscular transmission due to prolonged inhibition of acetylcholinesterase activity at the muscle end-plate. However, the role of muscle injury in the development of intermedicate syndrome is not clear. The aim of this study was to determine whether the initial serum levels of the muscle enzymes can predict the subsequent development of intermediate syndrome. We reviewed the files of 47 adult patients with organophosphate poisoning between April 2003 and February 2006. The muscle enzymes were obtained within first 24 hr from each patient. Among the patients, 17% (n = 8) had severe poisoning and 83% (n = 39) mild poisoning. In 10 patients (21%), intermediate syndrome was observed. Of the patients with severe poisoning, 63% (n = 5) developed subsequent intermediate syndrome; among those with mild poisoning, 13% (n = 5) developed intermediate syndrome. There was no significant difference in initial serum levels of creatine kinase and aspartate aminotransferase between the patients with severe poisoning and mild poisoning, and there was no difference in initial serum levels of creatine kinase and aspartate aminotransferase between the patients with and without intermediate syndrome. The serum levels of the muscle enzymes measured within the first 24 hr may not predict the subsequent development of intermediate syndrome.

Effect of Manual Lymph Drainage on the Course of Serum Levels of Muscle Enzymes After Treadmill Exercise

Improving muscular recovery after exercise is an important topic in sports medicine. The aim of the present study was to evaluate the effect of manual lymph drainage on the course of serum levels of muscle enzymes after an extended treadmill exercise. Fourteen recreational athletes (seven women, seven men) were included in the study. The participants underwent a graded exercise test on a treadmill ergometer to determine the individual anaerobic threshold (IAT). Seven days after the graded exercise test, all subjects performed 30 mins of treadmill exercise at an intensity equivalent to IAT. The subjects were randomized into two groups of seven persons. One group was treated with manual lymph drainage (ML), whereas the control group (CG) received no treatment after the endurance exercise at IAT level. After an increase immediately after exercise, a fast decrease in lactate dehydrogenase (LDH) and in aspartate aminotransferase (AST) concentration was observed, with significantly lower values for LDH after 48 hrs in the subjects having received lymph drainage treatment. The course of creatine kinase (CK) levels was comparable, but did not reach significance. Manual lymph drainage after treadmill exercise was associated with a faster decrease in serum levels of muscle enzymes. This may indicate improved regenerative processes related to structural damage of muscle cell integrity.

Differential diagnosis of idiopathic inflammatory myopathies

Symmetric proximal muscle weakness has many potential etiologies. An onset over weeks to months and elevated serum levels of muscle enzymes point to the diagnosis of an idiopathic inflammatory myopathy, including dermatomyositis, polymyositis, and inclusion body myositis. However, there is a broad differential diagnosis, including certain muscular dystrophies, metabolic myopathies, drug- or toxin-induced myotoxicity, neuropathies, and infectious myositides. The differentiation is critical for defining appropriate treatment. In addition, an alternative diagnosis may explain the lack of response to immunosuppressive treatment for some patients with polymyositis. Careful clinical evaluation and choice of available diagnostic tests are required to establish the correct diagnosis.

Rhabdomyolysis after Bariatric Surgery

Rhabdomyolysis is a potential threat after bariatric surgey. The severity ranges from asymptomatic elevations of serum muscle enzyme levels to life-threatening cases associated with muscle necrosis, compartment syndrome, acute renal failure and cardiac arrest. We studied 98 consecutive obese patients who underwent primary uncomplicated bariatric surgery during a 1-year period. A database was created for all patients (sex, age, BMI, duration of the operation); serum creatinine phosphokinase (CPK) was systematically measured before surgery and on the first and second postoperative day. The study sample consisted of 35 males (35.7%) and 63 females (64.3%) with preoperative CPK level 156.6 +/- 41.1 U/L (40 to 220), 24 hours postoperatively 1,075.2 +/- 596.5 U/L, (85 to 2,790 U/L) and 48 hours postoperatively 967.3 +/- 545.3 U/L (79 to 2,630). There was no difference in mean BMI (P=0.1) and mean duration of operation (P=0.5) between males and females. However, a statistically significant difference in mean elevation of CPK between males and females (P=0.003) was found. The variables sex, age, weight and duration of surgery were analyzed by multivariate logistic regression, but did not show a statistically significant difference. Rhabdomyolysis is a potentially fatal complication of surgical procedures in obese patients, and can be minimized with simple measures such as additional padding, aggressive hydration and urine alkalinization. Diagnosis requires a high level of physician awareness.

Unusually high frequency of autoantibodies to PL‐7 associated with milder muscle disease in Japanese patients with polymyositis/dermatomyositis

Autoantibodies to aminoacyl transfer RNA synthetases, such as histidyl (Jo-1), threonyl (PL-7), alanyl (PL-12), glycyl (EJ), and isoleucyl (OJ), are closely associated with a subset of patients with polymyositis/dermatomyositis (PM/DM) complicated by interstitial lung disease (ILD). Anti-Jo-1 is by far the most common, found in 15-25% of patients with PM/DM, whereas the other types are found in only approximately 3% of these patients. In this study, the clinical associations of these autoantibodies in Japanese patients with PM/DM were investigated. The diagnoses of PM/DM and amyopathic DM (ADM) were based on the Bohan and Peter criteria and Sontheimer's definition, respectively. Sera from 36 Japanese patients with PM/DM (13 with PM, 20 with DM, 3 with ADM) were screened by immunoprecipitation and by enzyme-linked immunosorbent assay (for Jo-1). Clinical and laboratory data were collected. The frequencies of autoantibodies to Jo-1 (22%) and to EJ, OJ, and PL-12 (3-6%) were similar to those found in previous studies, including studies of Japanese subjects. However, anti-PL-7 was found in 17% of patients, in contrast to a frequency of 1-4% in previous studies (P < 0.02-0.0002). The 6 anti-PL-7-positive patients were not related, and no skewing in year or month of disease development, place of residence or work, or occupation was found. All patients had ILD, consistent with the clinical features of antisynthetase-positive patients. The patients with anti-PL-7 had lower serum muscle enzyme levels and milder muscle weakness (P < 0.05) compared with anti-Jo-1-positive patients. Anti-PL-7 was found at an unusually high frequency in this group of Japanese patients with myositis. Although anti-PL-7, similar to anti-Jo-1, is associated with PM/DM with ILD, muscle involvement in the patients with anti-PL-7 appeared to be milder than that in the anti-Jo-1 subset.

Chronic Steroid and Immunosuppressant Therapy in Children

1. Abraham Gedalia, MD* 2. Avinash K. Shetty, MD† 1. *Professor of Pediatrics and Head, Division of Pediatric Rheumatology, Louisiana State University Health Sciences Center and Children’s Hospital, New Orleans, La 2. †Assistant Professor of Pediatrics, Division of Infectious Diseases, Wake Forest University School of Medicine, Winston-Salem, NC After completing this article, readers should be able to: 1. Describe the mechanism of action, list indications for therapy, and discuss the adverse effects of corticosteroid therapy in children. 2. Delineate the mechanism of action and adverse effects of other immunosuppressive agents frequently used in children. 3. Discuss the immunization recommendations for children receiving immunosuppressive therapy. Corticosteroids are the most potent, naturally occurring anti-inflammatory agents known. Despite more than 50 years of controversy regarding their clinical use, risks, and benefits, corticosteroids still play a significant role in the management of a wide variety of diseases. They have reduced the morbidity and mortality significantly in children afflicted with leukemia, asthma, rheumatic diseases, and other inflammatory disorders. In this article, we review the mechanism of action, clinical indications, and adverse effects of corticosteroids and other commonly used immunosuppressive agents in children. We also review immunization recommendations for children receiving immunosuppressive therapy. Corticosteroids have a diverse role in the human body at the physiologic level. The actions of glucocorticoids include: 1) negative feedback modulation of corticotropin-releasing factor and adrenocorticotropic hormone; 2) regulation of blood glucose and liver glycogen levels; 3) maintenance of water-electrolyte homeostasis; 4) influence on the metabolism of protein, fat, and purine; 5) maintenance of normal cardiovascular, central nervous system (CNS), and renal functions; 6) influence on circadian rhythmicity; 7) maintenance of bone and muscle work capacity; and 8) protection of the body against moderate stress. Glucocorticoids also have pharmacologic properties that are used in the treatment of rheumatic and other inflammatory diseases. Cellular receptors for cortisol are ubiquitous in cell cytoplasm, reflecting the critical role of the hormone in maintaining cell homeostasis. The type 1 glucocorticoid receptor, known as the mineralocorticoid receptor, has high affinity for aldosterone. The type 2 glucocorticoid receptor, known as the glucocorticoid receptor, exhibits strong affinity for dexamethasone. Although …

Coexistência de hipotiroidismo e polimiosite

Pacientes com hipotiroidismo freqüentemente apresentam sintomas músculo-esqueléticos e aumento de enzimas musculares, como a creatinaquinase (CK). Com o tratamento hormonal adequado é incomum ocorrer grandes aumentos de CK e manutenção de queixas musculares. Este trabalho relata o caso de uma paciente com hipotiroidismo, queixa de fraqueza muscular proximal e aumento de CK, sem melhora clínica com uso de levotiroxina. O diagnóstico de polimiosite foi confirmado por eletroneuromiografia e biópsia muscular. Após tratamento com prednisona e metotrexato, além de adequação da dose de levotiroxina, houve melhora clínica significativa e normalização da CK.

Juvenile dermatomyositis: clinical, laboratorial, histological, therapeutical and evolutive parameters of 35 patients.

This study was based on a prospective and a retrospective analysis of 35 patients who met Bohan and Peter criteria for juvenile dermatomyositis diagnosis. The mean follow-up time was three years ten months. Calcinosis was present in five (14.28 %) patients, cutaneous ulcers in four (11.42%), and systemic involvement in nine (27.71%) patients. All patients presented alterations in the serum levels of muscle enzymes, and all of them were submitted to muscle biopsy as a diagnostic procedure. Nine (25.71%) patients received corticotherapy prior to and 26 (74.28%) after the muscle biopsy. Chloroquine, methotrexate, cyclosporine, cyclophosphamide and intravenous immunoglobulin were used in patients with poor response to corticotherapy. Continuation of cutaneous manifestations was observed in 4 (11.43%) patients, laboratorial activity in 1 (2.85%), cutaneous and laboratorial activities in 3 (8.57%). Ten (28.57%) patients were out of activity, and 17 (48.57%) in remission at study end-point, on March 2002. Two (5.71%) patients died.

A case of hemophagocytic syndrome associated with active dermatomyositis.

We report a case of dermatomyositis (DM) with hemophagocytic syndrome (HPS). The patient is a 60 year old male admitted to our hospital with muscle weakness, high fever, weight loss and pancytopenia. On physical examination, proximal muscle weakness and skin rash on the back were noted. Laboratory data revealed elevated serum levels of muscle enzymes, lactate dehydrogenase and ferritin. Serum levels of M-CSF, TNF-alpha, soluble IL 2 receptor were remarkably increased. Bone marrow aspiration showed histiocytosis of 7 to 10% with prominent hemophagocytosis. Gastroendoscopic examination revealed II-a gastric cancer. He was treated with methylprednisolone (m-PSL) pulse theraphy (1 g/ day x 3 days) followed by 60 mg/day oral prednisolone. He quickly responded to the treatment and laboratory data returned to normal in 20 days despite the remaining gastric cancer which was removed successfully 3 months later. HPS is a rare complication of DM and only three cases have been documented so far in the literature. Augmented cytokine in his serum is considered to be closely related to HPS in this case.

Polyomyositis and myocarditis associated with acquired toxoplasmosis in an immunocompetent girl

BackgroundAcquired toxoplasmosis more frequently goes unrecognized. Immunocompetent adults and adolescents with primary infection are generally asymptomatic, but symptoms may include malaise, fever, and lymphadenopathy. By contrast, immunocompromised patients may experience severe manifestations including encephalitis and multisystem organ failure.Case presentationWe report a case of polymyositis and myocarditis in a 13-year old immunocompetent girl with toxoplasmosis. The patient presented with proximal muscle weakness, dysphagia, palms and soles rash and elevated serum levels of muscle enzymes, with liver and myocardial involvement. The diagnosis of toxoplasmosis was confirmed by serology. The patient was treated with prednisolone and had an excellent outcome. During a follow-up period of four years no relapses occurred and antibody levels to the T. gondii significantly decreased.ConclusionsAlthough several previous cases of toxoplasmosis occuring in association with polymyositis have been described in the literature such a wide spectrum of acute toxoplasmosis is rather unusual in immunocompetent adolescents. The relationship between T. gondii and polymyositis remains obscure. Appropriate investigation should be performed in every case of polymyositis not only for the appropriate treatment but also for further elucidation of this relationship.

A Case of Amyopathic Dermatomyositis

We report a 33-year-old woman with a 1-year history of typical skin features of dermatomyositis without any evidence of muscle involvement.Her skin eruption of the face (heliotrope erythema) and hands (Gottron's papules) and skin biopsy findings were typical of dermatomyositis. Levels of serum muscle enzymes were within normal ranges and electromyography showed no signs of muscle involvement. During a 1-year follow-up, she had no signs of muscle weakness. Based on these clinical and laboratory findings, our case can be diagnosed as amyopathic dermatomyositis. (Ann Dermatol 12(2) 126~129, 2000).

Progressive muscular dystrophy in alpha-sarcoglycan-deficient mice.

Limb-girdle muscular dystrophy type 2D (LGMD 2D) is an autosomal recessive disorder caused by mutations in the alpha-sarcoglycan gene. To determine how alpha-sarcoglycan deficiency leads to muscle fiber degeneration, we generated and analyzed alpha-sarcoglycan- deficient mice. Sgca-null mice developed progressive muscular dystrophy and, in contrast to other animal models for muscular dystrophy, showed ongoing muscle necrosis with age, a hallmark of the human disease. Sgca-null mice also revealed loss of sarcolemmal integrity, elevated serum levels of muscle enzymes, increased muscle masses, and changes in the generation of absolute force. Molecular analysis of Sgca-null mice demonstrated that the absence of alpha-sarcoglycan resulted in the complete loss of the sarcoglycan complex, sarcospan, and a disruption of alpha-dystroglycan association with membranes. In contrast, no change in the expression of epsilon-sarcoglycan (alpha-sarcoglycan homologue) was observed. Recombinant alpha-sarcoglycan adenovirus injection into Sgca-deficient muscles restored the sarcoglycan complex and sarcospan to the membrane. We propose that the sarcoglycan-sarcospan complex is requisite for stable association of alpha-dystroglycan with the sarcolemma. The Sgca-deficient mice will be a valuable model for elucidating the pathogenesis of sarcoglycan deficient limb-girdle muscular dystrophies and for the development of therapeutic strategies for this disease.

A Pediatric Case of Polymyositis Associated with Mycoplasma pneumoniae Infection

We describe the case of a 10-year-old girl who developed polymyositis associated with a Mycoplasma pneumoniae infection. She exhibited mild muscle weakness without skin rash, markedly elevated serum levels of muscle enzymes, and infiltration of mononuclear cells in a muscle biopsy specimen. Efficacy of both oral prednisolone and of intravenous methylprednisolone pulse therapy for her disease was limited. However, other immunosuppressive drugs, cyclophosphamide and methotrexate, proved clinically effective. This is the first report of steroid-resistant polymyositis associated with M. pneumoniae infection in a child.

Dermatomyositis with normal muscle enzymes: a single blind study of the diagnostic value of thallium‐201 scanning

AbstractBackground It is well documented that the classic cutaneous lesions of dermatomyositis can precede the onset of muscle involvement or may occur in the absence of any detectable muscle disease by current diagnostic criteria. Thallium scanning has been reported to be a useful test in the evaluation of muscle disease in patients with dermatomyositis.Objective Our purpose was to examine the utility of thallium scanning in patients with dermatomyositis with normal muscle enzymes.Methods In this single blind study we used thallium‐201 scanning to evaluate 5 patients who presented with classical clinicopathologic dermatomyositis but with normal levels of serum muscle enzymes. A positive control patient with active myositis and a negative control patient were also examined.Results All patients had muscle groups with decreased signal intensity on thallium scanning. Even the negative control patient demonstrated marked asymmetry of several different muscle groups. These results are very different from those previously reported.Conclusions Non‐invasive radiologic examinations clearly have a role in the evaluation of patients with dermatomyositis sine myositis or dermatomyositis. We have previously reported that magnetic resonance imaging and ultrasound are useful adjunctive tests. However, thallium scanning does not seem to be useful in the evaluation of patients with dermatomyositis with normal muscle enzymes. Thallium scanning appears to be a very sensitive test. However, the abnormal findings on every patient, including the negative control patient, suggest a very low specificity.

Dermatomyositis with normal muscle enzymes: a single blind study of the diagnostic value of thallium-201 scanning

Background It is well documented that the classic cutaneous lesions of dermatomyositis can precede the onset of muscle involvement or may occur in the absence of any detectable muscle disease by current diagnostic criteria. Thallium scanning has been reported to be a useful test in the evaluation of muscle disease in patients with dermatomyositis.Objective Our purpose was to examine the utility of thallium scanning in patients with dermatomyositis with normal muscle enzymes.Methods In this single blind study we used thallium-201 scanning to evaluate 5 patients who presented with classical clinicopathologic dermatomyositis but with normal levels of serum muscle enzymes. A positive control patient with active myositis and a negative control patient were also examined.Results All patients had muscle groups with decreased signal intensity on thallium scanning. Even the negative control patient demonstrated marked asymmetry of several different muscle groups. These results are very different from those previously reported.Conclusions Non-invasive radiologic examinations clearly have a role in the evaluation of patients with dermatomyositis sine myositis or dermatomyositis. We have previously reported that magnetic resonance imaging and ultrasound are useful adjunctive tests. However, thallium scanning does not seem to be useful in the evaluation of patients with dermatomyositis with normal muscle enzymes. Thallium scanning appears to be a very sensitive test. However, the abnormal findings on every patient, including the negative control patient, suggest a very low specificity.

Study of neuromyopathy in amyloid kidney transplant patients.

Neuromuscular status of amyloid and control groups of kidney transplant recipients was assessed through complete neurological examination, assay for serum levels of muscle enzymes (CPK and LDH), electromyography and nerve conduction velocity studies. Neuromyopathic findings were detected in both groups but without severe disabling clinical manifestations. These findings were more prominent in the amyloid group, evidenced by a more significant increase in polyphasicity detected by electromyography and longer prolongation of terminal latency measured in the median nerve. From this study, we concluded that amyloid kidney transplant recipients are more prone to neuromyopathy than the general kidney transplant population, which is mostly due to the amyloidosis itself and/or the colchicine therapy.