RESUMO
Rhabdomyolysis is a myopathy characterized by severe acute myonecrosis with lysis of muscle cells and extravasation of its content into the bloodstream, causing a secondary renal failure and myoglobinuria. Case-reports have been documented in a wide range of wild or domestic animal species, but to our knowledge, no reports in water buffaloes (Bubalus bubalis) have been done. Three animals had signs of paralysis, muscle tremors and myoglobinuria while others died spontaneously. Samples of blood from affected animals showed increased levels of creatin kinase (CK), potassium (K), aspartate transaminase (AST), and lactate dehydrogenase (LDH). In addition, Selenium (Se) levels of those animals were low. Necropsy findings consisted of severe gelatinous and yellowish edema and pallor of the skeletal muscles of the hind limbs, lumbar, cervical and scapular region. Microscopically, acute and severe segmental monophasic coagulative necrosis of skeletal muscles and acute pigmentary tubular nephrosis was observed. We suspect that selenium deficiency was a predisposing factor of rhabdomyolysis in this particular case.
Assuntos
Animais , Búfalos , Deficiência de Minerais , Rabdomiólise/patologia , Rabdomiólise/veterinária , Selênio/análise , Doenças Musculares/veterináriaRESUMO
Rhabdomyolysis is a myopathy characterized by severe acute myonecrosis with lysis of muscle cells and extravasation of its content into the bloodstream, causing a secondary renal failure and myoglobinuria. Case-reports have been documented in a wide range of wild or domestic animal species, but to our knowledge, no reports in water buffaloes (Bubalus bubalis) have been done. Three animals had signs of paralysis, muscle tremors and myoglobinuria while others died spontaneously. Samples of blood from affected animals showed increased levels of creatin kinase (CK), potassium (K), aspartate transaminase (AST), and lactate dehydrogenase (LDH). In addition, Selenium (Se) levels of those animals were low. Necropsy findings consisted of severe gelatinous and yellowish edema and pallor of the skeletal muscles of the hind limbs, lumbar, cervical and scapular region. Microscopically, acute and severe segmental monophasic coagulative necrosis of skeletal muscles and acute pigmentary tubular nephrosis was observed. We suspect that selenium deficiency was a predisposing factor of rhabdomyolysis in this particular case.(AU)
Assuntos
Animais , Rabdomiólise/patologia , Rabdomiólise/veterinária , Búfalos , Selênio/análise , Deficiência de Minerais , Doenças Musculares/veterináriaRESUMO
Snakebite envenoming is a neglected public health issue in many tropical and subtropical countries. To diagnosis and treat snakebites may be challenging to health care personnel since sufficient information has not been yet provided. This review presents the clinical, therapeutic, and laboratory aspects of Crotalus durissus (South American rattlesnakes) victims. The clinical setting may show local effects such as little or no pain, mild edema, and recurrent erythema. In contrast, the systemic effects may be quite remarkable, such as changes due to neurological damage, intense rhabdomyolysis, incoagulability of the blood, and variations in the peripheral blood elements. The main complication is acute kidney injury. The appropriate treatment depends mainly on the correct recognition of the aggressor snake and the symptoms expressed by the victim. Rattlesnake venom can cause irreparable damage and lead to death. Therefore, a prompt diagnosis allows the immediate onset of proper serotherapy.
Assuntos
Injúria Renal Aguda/diagnóstico , Antivenenos/uso terapêutico , Crotalus , Mordeduras de Serpentes/diagnóstico , Injúria Renal Aguda/tratamento farmacológico , Injúria Renal Aguda/patologia , Injúria Renal Aguda/prevenção & controle , Animais , Transtornos da Coagulação Sanguínea/diagnóstico , Transtornos da Coagulação Sanguínea/tratamento farmacológico , Transtornos da Coagulação Sanguínea/patologia , Venenos de Crotalídeos/efeitos adversos , Humanos , Imunização Passiva , Rabdomiólise/diagnóstico , Rabdomiólise/tratamento farmacológico , Rabdomiólise/patologia , Mordeduras de Serpentes/tratamento farmacológico , Mordeduras de Serpentes/patologia , Mordeduras de Serpentes/prevenção & controleRESUMO
BACKGROUND: The myopathic form of carnitine palmitoyltransferase type II deficiency is an inherited autosomal recessive metabolic myopathy usually starting in childhood. Most reports have been on European and Japanese populations, and no Native South American patients have been reported to date. The p.Ser113Leu mutation is the most frequent in the European population. Only lower-leg magnetic resonance imaging findings have been reported, with gluteus maximus involvement in one case and normal imaging in other patients. CASE PRESENTATION: Two Native South American siblings, a boy and a girl, presented to our neuromuscular clinic with recurrent rhabdomyolysis associated with transient muscle weakness after prolonged exercise. During episodes, their creatine kinase concentrations were markedly increased, up to 148,000 (1.48 × 105) IU/L in the boy and 18,000 (1.8 × 104) IU/L in the girl. The results of electroneuromyography and histopathology suggested a nonspecific myopathy. CPT2 gene sequencing showed two heterozygous mutations: the p.Ser113Leu variant and a novel one (predicted to be deleterious by in silico analysis), the p.Ser373Pro variant. The patients' parents were asymptomatic carriers. Whole-body magnetic resonance imaging showed mild selective involvement in the thoracic extensors and pelvic girdle in both siblings, and in the thighs and lower legs in one of them. Dietary and bezafibrate treatment was started, and symptomatic relief was observed. CONCLUSIONS: To the best of our knowledge, this is the first reported Native South American family with a CPT2 deficiency carrying a novel mutation and particular features visualized by whole-body magnetic resonance imaging.
Assuntos
Carnitina O-Palmitoiltransferase/deficiência , Doenças Musculares/genética , Adolescente , Carnitina O-Palmitoiltransferase/genética , Feminino , Humanos , Imageamento por Ressonância Magnética , Masculino , Doenças Musculares/diagnóstico , Doenças Musculares/patologia , Mutação , Estudos Retrospectivos , Rabdomiólise/diagnóstico , Rabdomiólise/genética , Rabdomiólise/patologia , Adulto JovemRESUMO
Acute kidney injury (AKI) from leptospirosis is frequently nonoliguric with hypo- or normokalemia. Higher serum potassium levels are observed in non-survivor patients and may have been caused by more severe AKI, metabolic disarrangement, or rhabdomyolysis. An association between the creatine phosphokinase (CPK) level and maximum serum creatinine level has been observed in these patients, which suggests that rhabdomyolysis contributes to severe AKI and hyperkalemia. LipL32 and Lp25 are conserved proteins in pathogenic strains of Leptospira spp., but these proteins have no known function. This study evaluated the effect of these proteins on renal function in guinea pigs. Lp25 is an outer membrane protein that appears responsible for the development of oliguric AKI associated with hyperkalemia induced by rhabdomyolysis (e.g., elevated CPK, uric acid and serum phosphate). This study is the first characterization of a leptospiral outer membrane protein that is associated with severe manifestations of leptospirosis. Therapeutic methods to attenuate this protein and inhibit rhabdomyolysis-induced AKI could protect animals and patients from severe forms of this disease and decrease mortality.
Assuntos
Injúria Renal Aguda/patologia , Proteínas da Membrana Bacteriana Externa/metabolismo , Leptospirose/complicações , Lipoproteínas/metabolismo , Rabdomiólise/patologia , Injúria Renal Aguda/microbiologia , Animais , Creatina Quinase/sangue , Creatinina/sangue , Modelos Animais de Doenças , Cobaias , Leptospira , Músculos/patologia , Potássio/sangue , Rabdomiólise/microbiologiaRESUMO
BACKGROUND: Rhabdomyolysis is defined as skeletal muscle necrosis. Ultrasound assessment has recently become a useful tool for the diagnosis and monitoring of muscle diseases, including rhabdomyolysis. A case is presented on the ultrasound findings in a patient with rhabdomyolysis. OBJECTIVE: To highlight the importance of ultrasound as an essential part in the diagnosis in rhabdomyolysis, to describe the ultrasound findings, and review the literature. CLINICAL CASE: A 30 year-old with post-traumatic rhabdomyolysis of both thighs. Ultrasound was performed using a Philips Sparq model with a high-frequency linear transducer (5-10MHz), in low-dimensional scanning mode (2D), in longitudinal and transverse sections at the level of both thighs. The images obtained showed disorganisation of the orientation of the muscle fibres, ground glass image, thickening of the muscular fascia, and the presence of anechoic areas. CONCLUSIONS: Ultrasound is a useful tool in the evaluation of rhabdomyolysis.
Assuntos
Rabdomiólise/diagnóstico por imagem , Ultrassonografia/métodos , Injúria Renal Aguda/etiologia , Adulto , Síndromes Compartimentais/diagnóstico , Diagnóstico Diferencial , Humanos , Imobilização/efeitos adversos , Isquemia/etiologia , Perna (Membro)/irrigação sanguínea , Masculino , Montanhismo/lesões , Fibras Musculares Esqueléticas/patologia , Rabdomiólise/etiologia , Rabdomiólise/patologia , Coxa da PernaRESUMO
Patients with long-chain 3-hydroxy-acyl-CoA dehydrogenase (LCHAD) deficiency commonly present liver dysfunction whose pathogenesis is unknown. We studied the effects of long-chain 3-hydroxylated fatty acids (LCHFA) that accumulate in LCHAD deficiency on liver bioenergetics using mitochondrial preparations from young rats. We provide strong evidence that 3-hydroxytetradecanoic (3HTA) and 3-hydroxypalmitic (3HPA) acids, the monocarboxylic acids that are found at the highest tissue concentrations in this disorder, act as metabolic inhibitors and uncouplers of oxidative phosphorylation. These conclusions are based on the findings that these fatty acids decreased ADP-stimulated (state 3) and uncoupled respiration, mitochondrial membrane potential and NAD(P)H content, and, in contrast, increased resting (state 4) respiration. We also verified that 3HTA and 3HPA markedly reduced Ca2+ retention capacity and induced swelling in Ca2+-loaded mitochondria. These effects were mediated by mitochondrial permeability transition (MPT) induction since they were totally prevented by the classical MPT inhibitors cyclosporin A and ADP, as well as by ruthenium red, a Ca2+ uptake blocker. Taken together, our data demonstrate that the major monocarboxylic LCHFA accumulating in LCHAD deficiency disrupt energy mitochondrial homeostasis in the liver. It is proposed that this pathomechanism may explain at least in part the hepatic alterations characteristic of the affected patients.
Assuntos
3-Hidroxiacil-CoA Desidrogenases/deficiência , Cardiomiopatias/patologia , Permeabilidade da Membrana Celular/efeitos dos fármacos , Metabolismo Energético/efeitos dos fármacos , Ácidos Graxos/farmacologia , Erros Inatos do Metabolismo Lipídico/patologia , Potencial da Membrana Mitocondrial/efeitos dos fármacos , Mitocôndrias Hepáticas/efeitos dos fármacos , Miopatias Mitocondriais/patologia , Dilatação Mitocondrial/efeitos dos fármacos , Doenças do Sistema Nervoso/patologia , Rabdomiólise/patologia , 3-Hidroxiacil-CoA Desidrogenases/metabolismo , Animais , Transporte Biológico , Cálcio/metabolismo , Cardiomiopatias/metabolismo , Erros Inatos do Metabolismo Lipídico/metabolismo , Mitocôndrias Hepáticas/metabolismo , Membranas Mitocondriais/metabolismo , Miopatias Mitocondriais/metabolismo , Proteína Mitocondrial Trifuncional/deficiência , NADP/metabolismo , Doenças do Sistema Nervoso/metabolismo , Fosforilação Oxidativa/efeitos dos fármacos , Consumo de Oxigênio/efeitos dos fármacos , Ratos , Ratos Wistar , Rabdomiólise/metabolismoRESUMO
Injuries caused by freshwater stingrays are characterized by intense pain and pathological changes at the lesion site, including oedema, erythema and, in most cases, necrosis. In this study, the systemic myotoxic activity induced by mucus extracts from the dorsal region and stinger of the stingrays Plesiotrygon iwamae and Potamotrygon motoro was described, analysed and quantified. Twenty-four hours after injection of 400 µg of the extracts into the gastrocnemius muscle of mice, the following effects were observed: coagulative necrosis of the muscle tissue, muscle fibre regeneration and the presence of inflammatory infiltrates, including neutrophils, macrophages, and a reduced number of eosinophils and lymphocytes. These changes were also observed, although to a lesser extent, in the gastrocnemius muscles of the contralateral limbs, demonstrating that the extracts from the two species could induce systemic rhabdomyolysis. Based on morphometric analysis, it was observed that the stinger extract of P. motoro was more potent in inducing local and systemic myotoxic activity, followed by the dorsal extract from P. motoro and stinger and dorsal extracts from P. iwamae, which induced similar effects.