Hyaluronic acid in dermatomyositis and polymyositis: relationship with disease and cutaneous lesions

Abstract: Background: There are scarce studies in the literature about hyaluronic acid in systemic autoimmune myopathies. Objectives: To analyze the serum level of hyaluronic acid in patients with dermatomyositis and polymyositis. Methods: Cross-sectional study, single-center, that evaluated hyaluronic acid in 18 dermatomyositis and 15 polymyositis (Bohan and Peter criteria), newly diagnosed, with clinical and laboratory activity, with no previous drug treatment. The patients were also age-, gender- and ethnicity-matched to 36 healthy individuals. The hyaluronic acid was analyzed by ELISA/EIA kit anti-hyaluronic acid. Results: There was a higher serum level of hyaluronic acid in patients with autoimmune myopathies, in relation to control group (P<0.05). Moreover, the serum level of this glycosaminoglycan was higher in dermatomyositis, when compared to polymyositis. Both groups were comparable with regard to demographic, clinical and laboratory data, except for the presence of skin lesions in the first group. Study limitations. The presence of hyaluronic acid in cutaneous lesions, particularly of patients with dermatomyositis, was not analyzed neither quantified. In addition, due to disease rarity and the establishment of strict inclusion and exclusion criteria, there was a small sample in the present study. Conclusions: As an example of others systemic autoimmune diseases, it is possible that the hyaluronic acid is involved in the pathogenesis of autoimmune myopathies, and particularly when associated with cutaneous lesions.

Spontaneous pneumomediastinum in dermatomyositis: a case series and literature review / Pneumomediastino espontâneo na dermatomiosite: uma série de casos e revisão de literatura

OBJECTIVES: To describe a case series of spontaneous pneumomediastinum in dermatomyositis and to review the literature. METHODS: This was a retrospective single-center case series, reporting 9 patients with pneumomediastinum and defined dermatomyositis, followed from 2005 to 2017. RESULTS: Median age of patients: 33 years; cutaneous and pulmonary involvement in all cases; constitutional symptoms in 88.8% of patients; involvement of the joints in 11.1%, gastrointestinal tract in 44.4%, and muscles in 77.7%; subcutaneous emphysema was observed in 55.5% and pneumothorax in 11.1%, respectively. Muscle weakness was observed in 77.7% of cases and with a median level of serum creatine phosphokinase of 124U/L. Drawing on results for our literature review, the overall analysis showed that the risk factors associated with spontaneous pneumomediastinum were: (a) a history of interstitial pneumopathy; (b) normal or low levels of muscle enzymes; (c) previous use of systemic glucocorticoid; (d) over 50% of patients had subcutaneous emphysema; (e) high mortality as a consequence of severity of the interstitial lung disease. CONCLUSIONS: Our case series revealed that pneumomediastinum is a rare complication in dermatomyositis that occurs in patients with a history of interstitial pneumopathy and may be accompanied by subcutaneous emphysema and pneumothorax.

OBJETIVOS: Descrever série de casos de pneumomediastino espontâneo em portadores de dermatomiosite e revisar a literatura. MÉTODOS: Trata-se de série de casos, único centro, relatando 9 pacientes com pneumomediastino e dermatomiosite definida, acompanhados de 2005 a 2017. RESULTADOS: A mediana da idade dos pacientes foi de 33 anos. Sintomas constitucionais estavam presentes em 88,8% dos pacientes. Houve acometimento cutâneo e pulmonar em todos os casos, acometimento das articulações em 11,1%, trato gastrointestinal em 44,4% e musculatura em 77,7% dos pacientes. Enfisema subcutâneo foi observado em 55,5% e pneumotórax em 11,1%, respectivamente. A fraqueza muscular foi observada em 77,7% dos casos, com um nível médio de creatinofosfoquinase sérica de 124U/L. Com base nos resultados da revisão da literatura, a análise geral mostrou que: os fatores de risco associados ao pneumomediastino espontâneo foram: história de pneumopatia intersticial, níveis normais ou baixos de enzimas musculares, uso prévio de glicocorticoide sistêmico; >50% dos pacientes tiveram enfisema subcutâneo; houve alta mortalidade como consequência da gravidade da doença pulmonar intersticial. CONCLUSÕES: Nossa série de casos revelou que o pneumomediastino é uma complicação rara na dermatomiosite e que ocorre em pacientes com história de pneumopatia intersticial e pode ser acompanhada por enfisema subcutâneo e pneumotórax.

Insulin resistance is increased in adult patients with dermatomyositis / Resistência insulínica aumentada em pacientes com dermatomiosite

OBJECTIVE: To evaluate insulinemia in glucocorticoid naïve patients with dermatomyositis and to evaluate insulin resistance using the homeostatic model assessment of insulin resistance (HOMA2-IR). METHODS: This cross-sectional study included 25 dermatomyositis, non-diabetic glucocorticoid naïve patients. The control group consisted of 50 volunteers matched for age, gender, ethnicity, weight and height. The HOMA2-IR index was calculated from baseline insulin and glucose data. The International Myositis Assessment & Clinical Studies Group (IMACS) parameters were used to evaluate disease status. RESULTS: Mean age of the patients was 43.5 years and these were predominantly females. Patients had low disease activity according to IMACS parameters. Higher body mass index and waist circumference were observed in the dermatomyositis group compared to the control group. Insulin level and HOMA2-IR were also higher in patients with dermatomyositis. Moreover, analyzing dermatomyositis alone, the HOMA2-IR index correlated positively with weight, body mass index and waist circumference and was independent on disease status parameters. CONCLUSIONS: Patients with dermatomyositis had higher values for basal insulinemia, insulin resistance, body mass index and waist circumference. Moreover, HOMA2-IR moderately correlated with these anthropometric parameters. These metabolic abnormalities are related to the development of metabolic syndrome, one of the main comorbidities observed in dermatomyositis.

OBJETIVO: Avaliar a insulinemia em pacientes com dermatomiosite virgens de glicocorticoide e avaliar a resistência insulínica, utilizando o modelo de avaliação da homeostase de resistência insulínica (HOMA2-IR). MÉTODOS: Este estudo transversal incluiu 25 pacientes com dermatomiosites, não-diabéticos e sem uso prévio de glicocorticoides. Para o grupo de controle, 50 voluntários foram pareados por idade, gênero, etnia, peso e estatura. O índice HOMA2-IR foi calculado a partir de dados basais de insulina e glicose. Os parâmetros do International Myositis Assessment & Clinical Studies Group (IMACS) foram utilizados para avaliar o status da doença. RESULTADOS: A méda de idade dos pacientes foi de 43,5 anos, predominantemente do sexo feminino. Os pacientes apresentaram baixa atividade de doença de acordo com os parâmetros do IMACS. O índice de massa corporal e a circunferência da cintura foram maiores no grupo da dermatomiosite em comparação com o grupo controle. O nível de insulina e o HOMA2-IR também foram maiores em pacientes com dermatomiosite. Além disso, analisando a dermatomiosite isoladamente, o índice HOMA2-IR correlacionou-se positivamente com o peso, o índice de massa corporal e a circunferência da cintura e foi independente dos parâmetros de status da doença. CONCLUSÕES: Pacientes com dermatomiosite apresentam valores mais elevados de insulinemia basal, resistência à insulina, índice de massa corporal e circunferência da cintura. Além disso, o HOMA2-IR está moderadamente correlacionado com esses parâmetros antropométricos. Essas anormalidades metabólicas estão relacionadas ao desenvolvimento da síndrome metabólica, uma das principais comorbidades observadas na dermatomiosite.

Quantitative Analysis of Tissue Injury after Minimally Invasive Total Hip Arthroplasty

BACKGROUND: We intended to clarify the hypothesis that minimally invasive total hip arthroplasty (MI-THA) leads to less tissue damage and inflammatory response than does conventional total hip arthroplasty (C-THA). METHODS: We performed 30 cases of THA between September 2005 and May 2006 and evaluated these cases prospectively. We chose 15 MI-THA cases for the study group and another 15 C-THA cases for the control group. We checked skeletal muscle marker enzymes, such as serum creatinine kinase and aldolase, the pro-inflammatory cytokines, interleukin (IL)-6 and 8, and the anti-inflammatory cytokines, IL-10 and IL-1 receptor antagonist (ra) the day before surgery and at postoperative days 1, 7, and 14. RESULTS: On postoperative days 1 and 3, the study group showed significantly lower serum creatinine kinase, IL-6, IL-10, and IL-1ra values than those in the control group. Additionally, IL-8 was significantly lower on day 7 after surgery. CONCLUSIONS: These data show that MI-THA decreased the release of muscle marker enzymes due to tissue damage immediately after surgery and minimized the inflammatory response related to the surgery during the early postoperative period.

Evaluation of some biochemical changes in diabetic patients

The present study consisted of 50 subjects were classified into three groups; Group [GI] Control group consisted of 10 clinically healthy adult subjects of both sexes free from any liver, kidney or cardiovascular diseases. Group [GII] diabetes mellitus type 1 consisted of 20 patients of both sexes. Group [GIII] diabetes mellitus type 1 with nephropathy consisted of 20 patients of both sexes. All subjects were undergo to the following investigated parameters; Ascorbic acid [vitamin C], Catalase, Total antioxidant capacity, Aldolase and Pyruvate kinase enzyme. vitamins C, catalase, total antioxidant capacity enzymes were highly significant decreased [P < 0. 01] in diabetes mellitus type 1 [GII] and diabetes mellitus type 1 with nephropathy [GIII] when compared to the control group. Adolase activity was highly significant increased [P < 0. 01] in diabetes mellitus type 1 with nephropathy [GIII] when compared to the control group. Pyruvate Kinase activity was highly significant increased [P < 0. 01] in diabetes mellitus type 1 [GII] when compared to the control group. The antioxidant and enzymes can be used for follow up in patients suffering from diabetes mellitus type 1 and predict other complications

Variabilidad biológica de aldolasa sérica: su utilidad en la interpretación de los resultados / Biological variation of serum aldolase: its use for interpretation of the results / Variabilidade biológica de aldolase sérica: sua utilidade na interpretagáo dos resultados

El objetivo de este trabajo fue determinar la variabilidad biológica (VB) de Aldolasa (ALD) en individuos sanos, el índice de individualidad (II) del analito y evaluar la utilidad del intervalo de referencia poblacional (IRP) considerando al IRP de los analitos útil cuando el II es > 1,4 y de poca utilidad si es <0,6. También se evaluó el valor de referencia para el cambio (VRC) en la interpretación de dos determinaciones seriadas del mismo individuo, especialmente en el seguimiento de enfermedades músculo-esqueléticas en las que los niveles de creatinquinasa (CK) permanecen dentro del intervalo de referencia, mientras que los de ALD están elevados. Se obtuvieron 8 muestras de sangre de 10 individuos aparentemente sanos en días diferentes según protocolo preestablecido. Se determinó la actividad de ALD por método enzimático UV y se calcularon los parámetros de VB, II y VRC. Se obtuvo una VB intraindividual: 31%; VB interindividual: 21%, II 1.47 y VRC 90%. De acuerdo a los coeficientes de VB y el II obtenidos, los IRP son útiles para la interpretación de los resultados de ALD. Un resultado seriado será estadísticamente diferente del dato previo cuando el mismo tenga una diferencia mayor al 90% en relación al valor previo. El VRC entre dos determinaciones seriadas debe ser calculado en cada laboratorio con su propia variabilidad analítica para una correcta interpretación de los resultados.

The aim of this study was to determine the Biological Variation (BV) of Aldolase (ALD) in healthy patients, the individuality index (II) of the analyte and to assess how useful the population reference interval (PRI) is for the interpretation of the results, considering PRI useful when the II is >1.40 and useless when it is <0.6. Reference change values (RCV) were also assessed in the interpretation of two serial measurements of the same individual, specially in the monitoring of patients in which the serum level of Creatine Kinase (CK) remain within the reference range while ALD values are increased. Eight bloodsam-ples were obtained from ten apparently healthy subjects in different days according to a predetermined protocol. ALD activity was measured by an enzymatic UV method and BV, II and RCV were calculated. Intraindividual BV was 31 %, interindividual BV 21 %, II 1.47 and RCV 90%. Based on the results obtained for BV and II, PRIs are useful for the interpretation of ADL results. A serial result will be statistically different from the previous one when the variability between both results is higher than 90%. RCV must be determined in each laboratory using their own analytical variability in order to obtain a correct interpretation of the results.

O objetivo deste trabalho foi determinar a variabilidade biológica (VB) de Aldolase (ALD) em individuos saudáveis, o índice de individualidade (II) do analito e avaliar a utilidade do intervalo de referencia populacional (IRP) considerando o IRP dos analitos útil quando o II é > 1,4 e de pouca utilidade se é <0,6. Também foi avaliado o valor de referencia da variagáo (VRV) na interpretagáo de duas determinagóes seriadas do mesmo individuo, especialmente no seguimento de doengas músculo-esqueléticas nas quais os níveis de creatinoquinase (CK) permanecem dentro do intervalo de referencia, enquanto que os de ALD estáo elevados. Foram obtidas 8 amostras de sangue de 10 individuos aparentemente saudáveis em dias diferentes conforme protocolo preestabelecido. Determinou-se a atividade de ALD por método enzimático UV e se calcularam os parámetros de VB, II e VRC. Foi obtida uma VB intra-individual: 31 %; VB interindividual: 21 %, II 1.47 e VRV 90%. De acordo com os coeficientes de VB e o II obtidos, os IRP sáo úteis para a interpretagáo dos resultados de ALD. Um resultado seriado será estatisticamente diferente do dado prévio quando o mesmo tenha uma diferenga maior a 90% em relagáo ao valor prévio. O VRV entre duas determinagóes seriadas deve ser calculado em cada laboratório com sua própria variabilidade analítica para uma correta interpretagáo dos resultados.

MELAS: clinical features, muscle biopsy and molecular genetics / MELAS: manifestações clínicas, biópsia muscular e estudo molecular

OBJECTIVE: The aim of the study was to analyze a series of Brazilian patients suffering from MELAS. METHOD: Ten patients with MELAS were studied with correlation between clinical findings, laboratorial data, electrophysiology, histochemical and molecular features. RESULTS: Blood lactate was increased in eight patients. Brain image studies revealed a stroke-like pattern in all patients. Muscle biopsy showed ralled-red fibers (RRF) in 90 percent of patients on modified Gomori-trichrome and in 100 percent on succinate dehydrogenase stains. Cytochrome c oxidase stain analysis indicated deficient activity in one patient and subsarcolemmal accumulation in seven patients. Strongly succinate dehydrogenase-reactive blood vessels (SSV) occurred in six patients. The molecular analysis of tRNA Leu(UUR) gene by PCR/RLFP and direct sequencing showed the A3243G mutation on mtDNA in 4 patients. CONCLUSION: The muscle biopsy often confirmed the MELAS diagnosis by presence of RRF and SSV. Molecular analysis of tRNA Leu(UUR) gene should not be the only diagnostic criteria for MELAS.

OBJETIVO: O objetivo deste estudo foi analisar uma série de pacientes brasileiros portadores de MELAS. MÉTODO: Dez pacientes com MELAS foram estudados com correlação entre manifestações clínicas, alterações laboratoriais, estudo eletrofisiológico, histoquímico e molecular. RESULTADOS: O nível de lactato sérico estava aumentado em 8 pacientes. O estudo das imagens do crânio revelou padrão semelhante ao de AVC isquêmico em todos os pacientes. A biópsia muscular mostrou fibras rajadas vermelhas (RRF) em 90 por cento dos pacientes na coloração pelo tricrômio de Gomori modificado e em 100 por cento na reação histoquímica pela desidrogenase succicínica (SDH). A análise da coloração pela citocromo c oxidase indicou atividade deficiente em um paciente e acúmulo subsarcolemal em sete pacientes. Vasos com forte reação para SDH (SSV) ocorreram em seis pacientes. O estudo molecular do gene tRNA Leu(UUR) por PCR/RLFP e seqüenciamento direto mostrou a mutação A3243G no DNAmt de 4 pacientes. CONCLUSÃO: A biópsia muscular frequentemente confirma o diagnóstico de MELAS pela presença de RRF e SSV. O estudo molecular do gene tRNA Leu(UUR) não deve ser o único critério diagnóstico para MELAS.

Toxic myopathies: muscle biopsy features

Several drugs and toxic substances can cause muscular abnormalities and are frequent causes of acquired myopathies. We present a series of 32 patients, predominance of young adult patients, diagnosed with toxic myopathy. The most common substances inducing myopathy were corticosteroids (56.2 percent) followed by the propoxyphene, neuroleptics, zidovudine and drug-induced hypokalemia. The investigation showed normal serum creatine kinase levels in 65.4 percent, myopathic pattern of the needle electromyography in 40 percent and the more frequent histological diagnosis of the muscle biopsy was type 2 fiber atrophy (59.3 percent). Clinical features, etiology, course of the disease, serum levels of muscular enzymes, electromyographic features and, especially, muscle biopsy features are discussed.

Diversos medicamentos e substâncias tóxicas podem causar alterações musculares e são causas freqüentes de miopatia adquirida. Apresentamos uma série de 32 pacientes, predomínio de pacientes adulto jovens, com miopatia tóxica. As substâncias mais relacionadas com a miopatia foram os corticosteróides (56,2 por cento) seguidos pelo propoxifeno, neurolépticos, zidovudina e drogas indutoras de hipocalemia. A investigação mostrou níveis normais de creatino quinase sérica em 65,4 por cento, eletromiografia de agulha com padrão miopático em 40 por cento e o mais freqüente diagnóstico histológico da biópsia muscular foi atrofia de fibras do tipo 2 (59,3 por cento). As manifestações clínicas, etiologia, tempo de evolução, nível sérico das enzimas musculares, alterações da eletroneuromiografia e, especialmente, da biópsia muscular são discutidos.

Prednisolone in Duchenne muscular dystrophy.

This Study was carried out in the department of Neuromedicine, Bangabandbu Sheikh Mujib Medical University (BSMMU), Dhaka, Bangladesh. A total of 19 Duchenne muscular dystrophy (DMD) patients were recruited for randomized controlled study to see the effect of prednisolone in the natural course of the disease process. Prednisolone was given in 10 patients (study group) in a dose of 0.75 mg/kg for six months. Vitamin was given in 8 patients (control group) for the same duration. One patient was dropped from the study. Patients were assessed for average muscle strength, timed function test and functional grades (pelvic and pectoral). It was found that at the end of the study, almost all the parameters were improved significantly in the prednisolone treated group (P < 0.05).

Effect of trypanosomes infection on blood ascorbic acid and serum aldolase levels on the fresh water fishes, Clarias batrachus and Heteropneustus fossilis.

The trypanosomes presence has been found to decrease blood ascorbic acid levels in fishes, Clarias batrachus and Heteropneustus fossilis by 55.7% and 54.70% respectively. The infection also showed significant increase in serum aldolase level of three fishes by 50.19% and 48.06% respectively.

Enzimas séricas e letromiografia em doenças neuromusculares: estudo comparativo de 817 casos / Serum enzymes and electromyography in neuro-muscular diseases: comparative study of 817 cases

Foram estudadas as enzymas séricas (creatinaquinase, desidrogenase láctica, aldolase, aspartato e alamina aminotransferase) de 817 pacientes e eletromiografias (EMG) de 588 casos de portadores de doenças neuromusculares. As enzimas e as EMG foram relacionadas utilizando testes de estatística descritiva e do qui-quadrado. Foi encontrado aumento importante das enzimas séricas relacioandas com EMG de padréo miopático (mais importante com a creatinaquinase, seguido da aldolase, desidrogenase láctica, aspartado aminotransferase e alamina aminotransferase), e, inversamente, normalidade ou discreto aumento nas EMG normais ou com padräo de desinervaçäo näo têm qualquer relaçäo com a elevaçäo das enzimas séricas

Some serum enzymes in burns and their probable correlation with surgical shock--a prospective combined surgicobiochemical study.

Serum enzymes such as phosphohexoisomerase, aldolase and amylase were estimated in serum of 100 patients with thermal burn of different degrees. These enzymes were estimated at the time of admission, at 12th hour, 24th hour, 36th hour, 72nd hour, 7th day and 14th day. The patients with high serum levels of these enzymes till 72nd hour showed grave prognosis and serial measurements of these enzymes might help in predicting the outcome.

Distrofia muscular progresiva tipo Duchenne; características de la casuística del Instituto de Neurología y Neurocirugía de La Habana / Duchenne type progresive muscular distrophy: characteristics of the casuistic of the Institute of Neurology and Neurosurgery, La Habana

Se estudió, desde el punto de vista clínico, genético y bioquímico, un grupo de pacientes con el diagnóstico de distrofia muscular progresiva, el cual fue atendido en el Instituto de Neurología y Neurocirugía en un período de 20 años. El 56% de los pacientes representó casos esporádicos. Del análisis de las diferentes variables clìnicas estudiadas en ambos grupos de pacientes (esporádicos y familiares) no se obtuvo diferencias entre éstas. El 74% de los pacientes mostró en su desarrollo motor, y el 63,3%, déficit mental. Se destaca que la edad promedio de aparición de los síntomas fue de 3,1 años para los esporádicos, y 4,4 para los familiares, mientras que la edad promedio a la consulta neurológica fue a los 7,5 años para los primeros y de 8,4 para el otro grupo. El 77% de los pacientes destacó hipertrofia gemelar, y macroglosia sólo el 6% Todos los enfermos presentaron alteraciones electrocardiográficas. Se mostró elevada la actividad sérica de las enzimas aldolasa transaminasa glutamicooxalacética, transaminasa glutamicopirúvica y de la creatinina fosfoquinasa; en esta última se obtuvieron los valores màs elevados

Valores de aldolasa, glucosa-6-fosfatasa y lactato dehidrogenasa en trabajadores expuestos a plaguicidas / Values of aldolase, glucose-6-phosphatase and lactate dehydrogenase in workers expose

Se realizó el estudio en 62 operadores y ayudantes de fumigación de diferentes Empresas de Cultivos Varios de la Provincia La Habana, expuestos a distintos tipos de plaguicidas durante 1 a 20 años, y como control en 37 trabajadores no expuestos a plaguicidas. Se les realizó exámenes clínicos y análisis de laboratorio clínico. Se determinaron las enzimas aldolasa, lactato dehidrogenasa y glucosa-6-fosfatasa en suero. No se encontraron diferencias significativas en los valores de lactato dehidrogenasa y glucosa-6-fosfatasa entre la muestra y el control. Los valores de aldolasa de la muestra fueron: 4.02 ñ 2,48 mUI y en el grupo control fueron de 6,85 ñ 2,54 mUI, los cuales fueron significativos para p<0,05

Serum aldolase in tetanus

This study included fifty-two patients with tetanus of different age and sex and seventeen normal subjects as control group.Serum aldolase activity was found to be related to the severity of the disease. Muscle spasm was a good criterion of the severity of the disease. In this work patients with rigidity but no spasm usually recovered [Grade I]. On the other hand the mortality rate was very high in grade Ill. So we can depend on serum aldolase activity and muscle spasm in evaluating patients with tetanus