Serum Muscle Enzymes Research Articles

An Unusual Complication: Prolonged Myopathy Due to an Alternative Medical Therapy with Heat and Massage

A 66-year-old male presented with swelling of the neck and arms, which was limiting his daily activities. Serum muscle enzymes were increased. A detailed history revealed that the patient received 10 cycles of infrared heat and massage therapy approximately 1 month before his first visit to the outpatient clinic. The swelling of the extremities began on day 11 of therapy, and the pain became unbearable. He was followed up with analgesics. There was a significant decrease in the muscle enzymes and a subjective improvement of 60-70% one month after discharge. Alternative therapies may have serious complications, and patients usually do not report them unless asked specifically.

Autosomal dominant HMSN with proximal involvement: new Brazilian cases

We report four Brazilian siblings with Autosomal Dominant Hereditary Motor Sensory Neuropathy with Proximal Dominant Involvement (HMSN-P), a rare form of HMSN, that was characterized by proximal dominant muscle weakness and atrophy onset after the age of 30 years, fasciculation, arreflexia and sensory disturbances with autosomal dominant inheritance. Electrophysiological study and sural nerve biopsy were in the accordance with axonal sensory motor polyneuropathy and laboratorial analysis disclosed serum lipids and muscle enzymes abnormalities. Our report is the first done by a group outside Japan, where the disease initially seemed to be restricted and stressed the phenotypic variability from the original report.

Elevated serum interferon‐α activity in juvenile dermatomyositis: Associations with disease activity at diagnosis and after thirty‐six months of therapy

Interferon-alpha (IFNalpha) has been implicated in the pathogenesis of juvenile dermatomyositis (DM). The aim of this study was to examine serum IFNalpha activity in a cohort of children with juvenile DM to determine relationships between IFNalpha and indicators of disease activity and severity. Thirty-nine children with definite/probable juvenile DM were included in the study. Serum samples were obtained at the time of diagnosis from 18 untreated patients with juvenile DM. Second samples from 11 of these patients were obtained at 24 months, while they were receiving treatment, and third samples were obtained from 7 of these patients at 36 months. The remaining 21 children were studied 36 months after their initial diagnosis. Serum IFNalpha activity was measured using a functional reporter cell assay. Patients with juvenile DM had higher serum IFNalpha activity than both pediatric and adult healthy control subjects. In untreated patients, serum IFNalpha activity was positively correlated with serum muscle enzyme levels (P<0.05 for creatine kinase, aspartate aminotransferase, and aldolase) and inversely correlated with the duration of untreated disease (P=0.017). The tumor necrosis factor alpha -308A allele was associated with higher serum IFNalpha levels only in untreated patients (P=0.030). At 36 months, serum IFNalpha levels were inversely correlated with muscle enzyme levels in those patients still requiring therapy and with the skin Disease Activity Score in those patients who had completed therapy (P=0.002). Serum IFNalpha activity was associated with higher serum levels of muscle-derived enzymes and a shorter duration of untreated disease in patients with newly diagnosed juvenile DM and was inversely correlated with measures of chronic disease activity at 36 months postdiagnosis. These data suggest that IFNalpha could play a role in disease initiation in juvenile DM.

Malignant Hyperthermia Associated with Ryanodine Receptor 1 (C7360G) Mutation in Quarter Horses

Anesthetic-induced malignant hyperthermia (MH) has been documented in Quarter Horses with a single point mutation in the ryanodine receptor 1 gene (RyR1) at nucleotide C7360G, generating a R2454G amino acid substitution. However, there have been no reports of nonanesthetic manifestations of MH in horses with the C7360G mutation. To describe clinical manifestations of Quarter Horses with the C7360G mutation. Eleven Quarter Horses with the RyR1 C7360G mutation. This prospective study included horses with suspected MH, undetermined etiology of sudden death, death within hours of onset of rhabdomyolysis, muscle rigidity, stiffness, intermittent sweating, and persistent increases in serum muscle enzyme activities. Whole blood in EDTA and skeletal muscle were processed for genetic and histochemical analysis. Medical records and pedigrees were collected when available. Both anesthetic- and non-anesthetic-associated myopathic manifestations of MH occurred in halter Quarter Horses with mutation of RyR1. The disease is inherited as an autosomal dominant trait. Clinical and laboratory abnormalities were similar in both forms. Rhabdomyolysis was a common finding in both groups of horses. Skeletal muscle histochemical findings were nonspecific and compatible with a noninflammatory myopathic process. MH is a potentially fatal disease of Quarter Horses that could be triggered by halogenated anesthetics and other nonanesthetic factors that may include exercise, stress, breeding, illnesses, and concurrent myopathies.

En kvinne med muskelsvakhet og utslett

A 44-year-old woman complained about weak proximal muscles in arms and legs. Levels of muscle enzymes in serum and findings in muscle biopsies were both normal. She then developed slight dyspnoea on exertion and high resolution CT showed ground glass opacities. Later on, dermatological manifestations such as V-sign and Shawl-sign were observed and the patient complained of Raynaud's phenomenon. Capillaroscopy revealed slight dearrangement of capillary architecture and reduced capillary density. The diagnosis was hypomyopathic dermatomyositis. Clinical aspects of amyopathic and hypomyopathic dermatomyositis are discussed.

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.

Myotoxicity of Lipid-Lowering Agents in a Teenager With MELAS Mutation

The use of lipid-lowering statins has been associated with raised serum muscle enzymes and, occasionally, with rhabdomyolysis, especially in patients with pre-existing metabolic myopathies. The A3243G mutation is one of the most common mutations associated with mitochondrial disorders. A teenager harboring the A3243G mutation had the unusual association of hereditary glomerulopathy and recurrent episodes of raised creatine kinase levels with the use of lipid-lowering agents. Muscle biopsy showed both normal respiratory chain enzyme activities and normal coenzyme Q(10) levels, although decreased muscle coenzyme Q(10) concentration had been postulated to have a pathogenic role in statin-related myopathies. The close temporal relationship of statin administration and raised creatine kinase levels in this patient suggests caution in the use of statins in children and teenagers with mitochondrial myopathies.

Effects of whole-body cryotherapy on serum mediators of inflammation and serum muscle enzymes in athletes

Whole-body cryotherapy (WBC) covers a wide range of therapeutic applications and consists of briefly exposing the body to extremely cold air. In sports medicine, WBC is used to improve recovery from muscle injury; however, empirical studies on its application to this area are lacking. To fill this gap, we compared changes in immunological parameters (C3, IgA, IgM, IgG, C-reactive protein, PGE2), cytokines (IL-2, IL-8, IL-10), adhesion molecules (sICAM-1), and muscle enzymes (creatine kinase [CK], lactate dehydrogenase [LAD]) before and after WBC in 10 top-level Italian National team rugby players. The subjects underwent five sessions on alternate days once daily for 1 week. During the study period, the training workload was the same as that of the previous weeks. Compared to baseline values, immunological parameters remained unchanged, while CK and LAD levels significantly decreased after treatment. No alterations in immunological function were observed but there is a decrease in pro-inflammatory cytokine/chemokine and an increase in anti-inflammatory cytokine. As measured by changes in serum CK and LAD concentrations, and cytokines pathway, short-term cold air exposure was found to improve recovery from exercise-induced muscle injury and/or damage associated with intense physical training.

Effects of a MAPK p38 inhibitor on lung function and airway inflammation in equine recurrent airway obstruction

It has been suggested that many of the beneficial effects of corticosteroids are mediated through mitogen-activated protein kinase (MAPK) p38 inhibition. To investigate the efficacy of the MAPK p38 inhibitor compound MRL-EQ1 to either prevent (Phase 1) or treat (Phase 2) recurrent airway obstruction (RAO) in horses. MRL-EQ1 was administered i.v. at a dosage of 0.75-1.5 mg/kg bwt q. 12 h. In Phase 1, susceptible horses in clinical remission were divided into 2 groups (n = 5/group), based on historical values of respiratory mechanics. All horses were entered in the study in pairs (one control, one treated horse) and exposed to the same environmental challenge (stabling, mouldy hay and dusty conditions). The treatment group received MRL-EQ1 for 14 days while the control horses were untreated during the same period. In Phase 2, affected horses were ranked by severity of respiratory dysfunction and split randomly into either dexamethasone or MRL-EQ1 treatment groups (n = 5/group). Bronchoalveolar lavage fluid, respiratory mechanic measurements, MRL-EQ1 plasma concentration and tumour necrosis factor (TNF) whole blood activity were evaluated sequentially. In Phase 1, MRL-EQ1 did not prevent the occurrence of clinical signs and pulmonary inflammation. However, treatment was associated with a reduction in severity and a delay in the onset of signs and a reduction in pulmonary neutrophilia. In Phase 2, plasma concentrations achieved resulted in ex vivo suppression of lipopolysaccharide-induced TNF production in equine blood. MRL-EQ1 did not improve airway inflammation or lung function and was associated in a dose dependent manner with behavioural (depression, excitability) and blood changes (neutrophilia, increased serum muscle enzyme concentrations). Inhibition of p38 in the horse was partially effective in reducing clinical signs and airway inflammation when administered prior to, but not during clinical exacerbation in RAO. Inhibitors of p38 MAPK with a better toxicity profile may be effective in the prevention or treatment of RAO.

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.

Clinical, neurological and clinicopathological signs, treatment and outcome of metaldehyde intoxication in 18 dogs

To describe the clinical signs, clinicopathological abnormalities and outcome of metaldehyde intoxication in dogs. Medical records of dogs presenting between 1989 and 2005 with a diagnosis of metaldehyde toxicity were reviewed retrospectively. Data obtained from the medical record included signalment, history, clinical signs, laboratory tests results, hospitalisation period length, treatments and outcome. Eighteen dogs fulfilled the inclusion criteria. The most prevalent clinical signs were seizures, hyperthermia, tachycardia and muscle tremors. Serum biochemistry abnormalities included increased serum muscle enzymes activities, acidaemia (six dogs) and decreased blood bicarbonate (eight dogs). Treatment was symptomatic and supportive. Hyperbilirubinaemia was observed in two dogs. Diazepam was the most commonly used anticonvulsant followed by phenobarbitone and pentobarbital. General inhalant anaesthesia was required in nine of 18 dogs with seizures unresponsive to anticonvulsants. The survival was 83 per cent (15 of 18 dogs). This clinical study recorded, for the first time in the veterinary literature, several clinicopathological abnormalities from severely intoxicated dogs. Metabolic acidosis was common, while acute or delayed hepatotoxicity was an uncommon complication.

Calcinose tumorale d’Inclan

Tumoral calcinosis is a rare disease seen in adolescents and young adults and consisting of calcifications in periarticular soft tissue. Mutations in fibroblast growth factor 23 and GalNAc transferase 3 have been identified in the familiar forms of tumoral calcinosis.A 10 year-old boy of light phototype presented multiple calcified periarticular masses since the age of seven years as well as sciatica secondary to nerve compression. Laboratory examinations revealed hyperphosphoraemia. Serum calcium levels, kidney tests, parathormone assay and muscle enzymes were all normal. Histology of a cutaneous biopsy sample revealed dermal calcifications. Inclan's tumoral calcinosis was diagnosed. Surgical resection was performed and drug treatment comprising phosphate chelators (aluminium hydroxide) was administered. At five months, other sites were apparent.This case of tumoral calcinosis is notable in terms of its sporadic onset in a child of light phototype and by the presence of compression of the external sciatic nerve. The diagnosis was made after ruling out other causes of soft tissue calcification.

A clinical analysis of juvenile dermatomyositis; focus on clinical manifestations at diagnosis

Purpose : Juvenile dermatomyositis (JDM) is the most common of the idiopathic inflammatory myopathies in children. The purpose of this study is to observe demographic, initial presentations, duration of time between disease onset and diagnosis, clinical manifestations and laboratory findings at diagnosis of patients with JDM. Methods : Forty seven patients identified at Seoul National University Children's Hospital from January 1986 to May 2007. Medical records were reviewed retrospectively focusing on initial presentations, clinical manifestations and laboratory findings at the time of diagnosis of patients with JDM. Results : Male and female patients were 25 and 22, respectively and sex ratio was 1.14:1. The average age at the time of diagnosis was 6.51 years. Skin rash (94%) was the most common symptom, followed by the proximal muscle weakness (89%). The disease activity score was 10.8. The duration between the onset of the skin rash and the muscle weakness and diagnosis was 7.18 and 4.70 months, respectively. The serum muscle enzymes, LDH, AST, CK and aldolase, were elevated in the patient with JDM. Autoimmune antibodies, antinuclear antibody, anti SSA antibody and anti SSB antibody, were negative findings. Electromyography findings were consistent with JDM in 88% of the patients, the muscle biopsy was in 91% and all MRI findings were compatible with those of patients with JDM. The most common symptom besides musculocutaneous lesions was the calcinosis (62.5%). The most common site of calcinosis was the pelvic area and buttocks. Conclusion : This study shows that the major symptoms are proximal muscle weakness and cutaneous lesion, and they are important to diagnose JDM.

A Case of Hypercapnic Respiratory Failure in a Patient with Eosinophilic Polymyositis

Eosinophilic infiltration into skeletal muscles has been rarely reported in a variety of conditions such as parasite infection, sarcoidosis, rheumatoid arthritis, eosinophilia-myalgia syndrome, and idiopathic hypereosinophilic syndrome. Eosinophilic myositis (EM) is one of idiopathic inflammatory muscle diseases associated with muscle and/or blood eosiophilia. The case of EM complicated with hypercapnic respiratory failure has been extremely rarely reported. A 61-year-old woman was admitted with sudden-onset pain in both calves. She had elevated serum muscle enzymes and peripheral eosinophil count. Findings of electromyography were consistent with inflammatory myopathy. MRI showed diffuse hyperintensity of calf muscles on T2-weighted and enhanced T1 images. Muscle biopsy showed eosinophils' infiltration in the endomysium and perivascular area. During the diagnostic work-up, she presented with hypercapnic respiratory failure. She was successfully treated with mechanical ventilation and high doses of prednisolone. This case suggests EM can cause respiratory failure secondary to respiratory muscle involvement.

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.

Quail poisoning in a child

It has been well known since ancient times that the consumption of quail may be deleterious to health. The European quail sub-species (Coturnix coturnix coturnix), common to Europe, North Africa, and western Asia is indeed potentially poisonous to humans, but only during the autumn migration (not during their return flight in spring). Quail poisoning is an acute dietary-toxicological syndrome. In this case report, we describe a rare, non-fatal case of quail poisoning in a 12-year-old boy. The victim was admitted to hospital 5 hours after the consumption of quail because of the signs and symptoms, as well as laboratory findings (elevation of serum muscle enzymes, myoglobinuria) significant of acute rhabdomyolysis. He was treated symptomatically with bed rest, forced continuous diuresis, urine alkalization, and maintenance of fluid and electrolyte balance. The victim recovered completely without sequelae after 8 days hospitalization. The aetiology of this syndrome is still unclear. It has been suggested that a toxic effect or a genetic sensitivity plays a major role. Many cases of quail poisoning in adults have been reported previously on the island of Lesbos during autumn. To our knowledge, this is the first case reported in a child.

Effect of a Marathon Run on Serum Lipoproteins, Creatine Kinase, and Lactate Dehydrogenase in Recreational Runners

Abstract The objective of this study was to determine the effect of a marathon run on serum lipid and lipoprotein concentrations and serum muscle enzyme activities and follow their recovery after the run. These blood concentrations were measured before, immediately after, and serially after a marathon run in 15 male recreational runners. The triglyceride level was significantly elevated postrace, then fell 30% below baseline 1 day after the run, and returned to baseline after 1 week. Total cholesterol responded less dramatically but with a similar pattern. High-density lipoprotein cholesterol remained significantly elevated and low-density lipoprotein cholesterol was transiently reduced for 3 days after the run. The total cholesterol/high-density cholesterol ratio was significantly lowered for 3 days. Serum lactate dehydrogenase activity significantly doubled postrace and then declined but remained elevated for 2 weeks. Serum creatine kinase activity peaked 24 hr after the run, with a 15-fold rise, and returned to baseline after 1 week. The rise of these enzymes reflects mechanically damaged muscle cells leaking contents into the interstitial fluid. It is concluded that a prolonged strenuous exercise bout in recreational runners, such as a marathon, produces beneficial changes in lipid blood profiles that are significant for only 3 days. However, muscle damage is also evident for 1 week or more from the dramatic and longlasting effect on enzyme levels. Laboratory values for these runners were outside normal ranges for some days after the race.

Effect of a Marathon Run on Serum Lipoproteins, Creatine Kinase, and Lactate Dehydrogenase in Recreational Runners

The objective of this study was to determine the effect of a marathon run on serum lipid and lipoprotein concentrations and serum muscle enzyme activities and follow their recovery after the run. These blood concentrations were measured before, immediately after, and serially after a marathon run in 15 male recreational runners. The triglyceride level was significantly elevated postrace, then fell 30% below baseline 1 day after the run, and returned to baseline after 1 week. Total cholesterol responded less dramatically but with a similar pattern. High-density lipoprotein cholesterol remained significantly elevated and low-density lipoprotein cholesterol was transiently reduced for 3 days after the run. The total cholesterol/high-density cholesterol ratio was significantly lowered for 3 days. Serum lactate dehydrogenase activity significantly doubled postrace and then declined but remained elevated for 2 weeks. Serum creatine kinase activity peaked 24 hr after the run, with a 15-fold rise, and returned to baseline after 1 week. The rise of these enzymes reflects mechanically damaged muscle cells leaking contents into the interstitial fluid. It is concluded that a prolonged strenuous exercise bout in recreational runners, such as a marathon, produces beneficial changes in lipid blood profiles that are significant for only 3 days. However, muscle damage is also evident for 1 week or more from the dramatic and longlasting effect on enzyme levels. Laboratory values for these runners were outside normal ranges for some days after the race.

Periodicidade e escolha de exames laboratoriais na terapia hipolipemiante

Pharmacologic lipid-lowering interventions should be monitored periodically to assess efficacy and safety parameters. Statins are usually well-tolerated drugs and major side effects include increased serum liver and muscle enzymes (AST, ALT, CK). Treatment should be stopped or diminished in case of significant increase of AST or ALT (> 3x ULN), or CK (> 10x ULN). Other lipid lowering agents may also produce hepatotoxicity or myositis, especially in association with statins (fibrates and nicotinic acid) or in presence of metabolic abnormalities (thyroid, liver or renal disorders). Nicotinic acid can also increase glucose and uric acid plasma levels. Laboratory tests might be performed prior to hypolipidemic drug treatment and should be repeated every three months during the first year and then at 6-mo intervals. Shorter intervals should be recommended in individual cases.