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1.
Nat Commun ; 11(1): 1025, 2020 02 24.
Artigo em Inglês | MEDLINE | ID: mdl-32094341

RESUMO

A bioengineered skeletal muscle construct that mimics structural and functional characteristics of native skeletal muscle is a promising therapeutic option to treat extensive muscle defect injuries. We previously showed that bioprinted human skeletal muscle constructs were able to form multi-layered bundles with aligned myofibers. In this study, we investigate the effects of neural cell integration into the bioprinted skeletal muscle construct to accelerate functional muscle regeneration in vivo. Neural input into this bioprinted skeletal muscle construct shows the improvement of myofiber formation, long-term survival, and neuromuscular junction formation in vitro. More importantly, the bioprinted constructs with neural cell integration facilitate rapid innervation and mature into organized muscle tissue that restores normal muscle weight and function in a rodent model of muscle defect injury. These results suggest that the 3D bioprinted human neural-skeletal muscle constructs can be rapidly integrated with the host neural network, resulting in accelerated muscle function restoration.


Assuntos
Bioimpressão/métodos , Regeneração Tecidual Guiada/métodos , Doenças Musculares/terapia , Mioblastos Esqueléticos/fisiologia , Neurônios/fisiologia , Animais , Materiais Biomiméticos/química , Materiais Biomiméticos/uso terapêutico , Diferenciação Celular/fisiologia , Proliferação de Células/fisiologia , Sobrevivência Celular/fisiologia , Modelos Animais de Doenças , Estudos de Viabilidade , Humanos , Hidrogéis/química , Masculino , Músculo Esquelético/citologia , Músculo Esquelético/lesões , Músculo Esquelético/fisiologia , Doenças Musculares/fisiopatologia , Rede Nervosa/fisiologia , Junção Neuromuscular/citologia , Junção Neuromuscular/fisiologia , Impressão Tridimensional , Ratos , Fatores de Tempo
3.
Prog Cardiovasc Dis ; 62(5): 390-394, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31669768

RESUMO

Symptoms during statin therapy are common and often attributed to statin intolerance. Recent data suggest few patients are truly intolerant to statins. Muscle symptoms are similar in statin and control groups in blinded treatment periods of clinical trials. The "nocebo" effect may occur during open-label statin treatment, when previously asymptomatic study participants report symptoms attributed to statin therapy, or during placebo-controlled trials. Most patients reporting statin intolerance can tolerate blinded moderate intensity statin therapy. In clinical practice the large majority of patients are willing to retry a statin, and of those who do, >80-90% successfully remain on statin therapy long-term. Emerging evidence from brain imaging studies and contemporary approaches to pain management suggests that building trust and managing patient expectations can minimize the "nocebo" effect in statin-treated patients.


Assuntos
Dislipidemias/tratamento farmacológico , Inibidores de Hidroximetilglutaril-CoA Redutases/efeitos adversos , Lipídeos/sangue , Doenças Musculares/terapia , Biomarcadores/sangue , Ensaios Clínicos como Assunto , Dislipidemias/sangue , Dislipidemias/diagnóstico , Humanos , Doenças Musculares/induzido quimicamente , Doenças Musculares/epidemiologia , Efeito Nocebo , Estudos Observacionais como Assunto , Medição de Risco , Fatores de Risco
4.
Handb Clin Neurol ; 162: 435-448, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31324324

RESUMO

The differential diagnosis of neonatal hypotonia is a complex task, as in newborns hypotonia can be the presenting sign of different underlying causes, including peripheral and central nervous system involvement and genetic and metabolic diseases. This chapter describes how a combined approach, based on the combination of clinical signs and new genetic techniques, can help not only to establish when the hypotonia is related to peripheral involvement but also to achieve an accurate and early diagnosis of the specific neuromuscular diseases with neonatal onset. The early identification of such disorders is important, as this allows early intervention with disease-specific standards of care and, more importantly, because of the possibility to treat some of them, such as spinal muscular atrophy, with therapeutic approaches that have recently become available.


Assuntos
Doenças do Recém-Nascido/terapia , Hipotonia Muscular/congênito , Hipotonia Muscular/terapia , Doenças Musculares/congênito , Doenças Musculares/terapia , Adulto , Feminino , Humanos , Recém-Nascido , Doenças do Recém-Nascido/diagnóstico , Doenças do Recém-Nascido/genética , Doenças do Recém-Nascido/fisiopatologia , Técnicas de Diagnóstico Molecular , Hipotonia Muscular/diagnóstico , Hipotonia Muscular/genética , Doenças Musculares/diagnóstico , Doenças Musculares/genética , Gravidez
5.
Pediatrics ; 144(1)2019 07.
Artigo em Inglês | MEDLINE | ID: mdl-31227563

RESUMO

A 2-day old term male infant was found to be hypotonic and minimally reactive during routine nursing care in the newborn nursery. At 40 hours of life, he was hypoglycemic and had intermittent desaturations to 70%. His mother had an unremarkable pregnancy and spontaneous vaginal delivery. The mother's prenatal serology results were negative for infectious risk factors. Apgar scores were 9 at 1 and 5 minutes of life. On day 1 of life, he fed, stooled, and voided well. Our expert panel discusses the differential diagnosis of hypotonia in a neonate, offers diagnostic and management recommendations, and discusses the final diagnosis.


Assuntos
Acil-CoA Desidrogenase de Cadeia Longa/deficiência , Letargia/etiologia , Erros Inatos do Metabolismo Lipídico/diagnóstico , Doenças Mitocondriais/diagnóstico , Hipotonia Muscular/etiologia , Doenças Musculares/diagnóstico , /terapia , Diagnóstico Diferencial , Humanos , Hipotermia/etiologia , Recém-Nascido , Erros Inatos do Metabolismo Lipídico/terapia , Masculino , Doenças Mitocondriais/terapia , Doenças Musculares/terapia
6.
Rinsho Shinkeigaku ; 59(5): 253-257, 2019 May 28.
Artigo em Japonês | MEDLINE | ID: mdl-31061299

RESUMO

A 69-year-old man was admitted because of subacute development of lower limb weakness from one month ago. He showed central obesity, gynecomastia, dorsal fat pad ("buffalo hump"), and proximal muscle weakness in the lower extremities (manual muscle test 4). Needle EMG, muscle MRI and labolatry screening including CPK were negative for neuromuscular diseases, except for the hypogenitalism accidentally detected in MRI. Although blood corticol was in normal range, the levels of serum ACTH and 24-hour urinary free cortisol excretion were high, and the dexamethasone suppression tests were positive. Brain MRI showed a small pituitary mass with gadolinium enhancement, and ACTH measurement from petrosal sinus sampling after CRH stimulation lead to the diagnosis of definite Cushing disease. Moreover, he also showed low testosterone and elevated LH and FSH. Chromosome banding revealed 47 XXY in 22 in 30 cells, leading to the diagnosis of mosaic Klinefelter syndrome. The supplementation with testosterone was partially effective for his weakness. The surgical resection of pituitary microadenoma resulted in the full recovery. Either Klinefelter syndrome or mild Cushing disease alone was insufficient as a cause of the muscle weakness in this patient. It is plausible that the mild elevation of cortisol accompanied by the lack of tesstelone may underlie the weakness, probably linked to impaired balance between muscle anabolism and catabolism.


Assuntos
Síndrome de Klinefelter/complicações , Síndrome de Klinefelter/diagnóstico , Doenças Musculares/etiologia , Hipersecreção Hipofisária de ACTH/complicações , Hipersecreção Hipofisária de ACTH/diagnóstico , Doença Aguda , Adenoma/complicações , Adenoma/cirurgia , Hormônio Adrenocorticotrópico/sangue , Idoso , Biomarcadores/sangue , Biomarcadores/urina , Bandeamento Cromossômico , Hormônio Foliculoestimulante/sangue , Humanos , Hidrocortisona/urina , Hormônio Luteinizante/sangue , Imagem por Ressonância Magnética , Masculino , Debilidade Muscular/etiologia , Doenças Musculares/diagnóstico , Doenças Musculares/terapia , Neoplasias Hipofisárias/complicações , Neoplasias Hipofisárias/cirurgia , Testosterona/administração & dosagem , Testosterona/deficiência , Resultado do Tratamento
8.
Mol Genet Metab ; 127(1): 64-73, 2019 05.
Artigo em Inglês | MEDLINE | ID: mdl-31031081

RESUMO

BACKGROUND: Very-Long-Chain Acyl-CoA Dehydrogenase (VLCAD) deficiency is a disorder of fatty acid oxidation included in the recommended uniform newborn screening (NBS) panel in the USA. It can have variable clinical severity and there is limited information on the natural history of this condition, clinical presentation according to genotype and effectiveness of newborn screening. METHODS: Retrospective data (growth parameters, morbidity, biochemical and genetic testing results) were collected from patients with VLCAD deficiency, to evaluate biochemical and clinical outcomes. Descriptive statistics was used for qualitative variables, while linear regression analysis was used to correlate continuous variables. RESULTS: VLCAD deficiency (screened by measuring elevated levels of C14:1-carnitine in blood spots) was more frequent in Utah than the national average (1:27,617 versus 1:63,481) in the first ten years of screening. Twenty-six patients had a confirmed diagnosis of VLCAD deficiency using DNA testing or functional studies. The c.848T>C (p.V283A) variant in the ACADVL gene was the most frequent in our population. Novel variants (c.623-21A>G (IVS7-21A>G); c.1052C>T (p.T351I); c.1183-7A>G (IVS11-7A>G); c.1281G>C (p.W427C); c.1923G>C (p.L641F); c.1924G>A (p.V642M)) were identified in this study, with their pathogenicity remaining unclear in most cases. C14:1-carnitine levels decreased with age and significantly correlated with CK levels as index of muscle involvement. There were no cases of HELLP syndrome nor liver disease during pregnancies in the mothers of VLCAD patients. None of our patients developed cardiac involvement after birth and all patients had normal growth parameters while on treatment. Clinical manifestations were related to concomitant infections and altered biochemical parameters. DISCUSSION: VLCAD deficiency can be identified by neonatal screening. Most patients compliant with therapy normalized biochemical parameters and had no major clinical manifestations. Complications were completely prevented with a relatively low number of pre-emptive ER visits or hospital admissions. It remains unclear whether neonatal screening is now identifying less severely affected patient or if complications will arise as subjects become older. Observation beyond puberty is necessary to fully understand the impact of VLCAD deficiency on morbidity in patients with VLCAD deficiency.


Assuntos
Acil-CoA Desidrogenase de Cadeia Longa/deficiência , Variação Genética , Erros Inatos do Metabolismo Lipídico/diagnóstico , Erros Inatos do Metabolismo Lipídico/genética , Doenças Mitocondriais/diagnóstico , Doenças Mitocondriais/genética , Doenças Musculares/diagnóstico , Doenças Musculares/genética , Triagem Neonatal , Acil-CoA Desidrogenase de Cadeia Longa/genética , Adolescente , Carnitina/sangue , Criança , Pré-Escolar , Feminino , Genótipo , Humanos , Lactente , Recém-Nascido , Erros Inatos do Metabolismo Lipídico/terapia , Masculino , Doenças Mitocondriais/terapia , Morbidade , Doenças Musculares/terapia , Estudos Retrospectivos , Resultado do Tratamento , Utah , Adulto Jovem
9.
Neurology ; 92(14): 663-674, 2019 04 02.
Artigo em Inglês | MEDLINE | ID: mdl-30850443

RESUMO

Neuromuscular adverse events following cancer treatment with anti-programmed cell death protein 1 (PD-1) monoclonal antibodies are relatively rare, yet potentially fatal. We performed a systematic review to characterize the clinical presentation, diagnostic workup, and management of neuromuscular disorders (NMDs) in patients treated with nivolumab or pembrolizumab monotherapy or concurrent with other immunologic agents, such as ipilimumab. Sixty-one publications on 85 patients (mean age 66.9 years [range 34-86]; male/female 2.6:1; 59% metastatic melanoma) were identified from selected indexing databases until June 2018. Forty-eight patients had received nivolumab and 39 pembrolizumab. The mean number of PD-1 inhibitor treatment cycles prior to onset of symptoms was 3.6 (range 1-28). Symptoms included oculomotor (47%), respiratory (43%), bulbar (35%), and proximal weakness (35%), as well as muscle pain (28%). Diagnoses were categorized as myasthenia gravis (27%), neuropathy (23%), myopathy (34%), or a combination of these (16%). After a critical review of the data, however, evidence did not support the stated NMD diagnosis in 13% of cases, while up to 25% of patients had signs of additional NMDs. Cardiac complications occurred in more than 30% of patients diagnosed with myasthenia gravis or myositis. Mortality was high in these patients, despite adequate treatment strategies including corticosteroid, IV immunoglobulins, and plasma exchange. The clinical presentation of NMDs associated with PD-1 inhibitors is often atypical, with considerable overlap between myasthenia gravis and myopathy, and cardiac/respiratory complications are common.


Assuntos
Anticorpos Monoclonais Humanizados/efeitos adversos , Antineoplásicos Imunológicos/efeitos adversos , Doenças Neuromusculares/induzido quimicamente , Nivolumabe/efeitos adversos , Receptor de Morte Celular Programada 1/antagonistas & inibidores , Corticosteroides/uso terapêutico , Humanos , Imunoglobulinas Intravenosas/uso terapêutico , Fatores Imunológicos/uso terapêutico , Doenças Musculares/induzido quimicamente , Doenças Musculares/terapia , Miastenia Gravis/induzido quimicamente , Miastenia Gravis/terapia , Miosite/induzido quimicamente , Miosite/terapia , Doenças Neuromusculares/terapia , Doenças do Sistema Nervoso Periférico/induzido quimicamente , Doenças do Sistema Nervoso Periférico/terapia , Troca Plasmática
10.
Ugeskr Laeger ; 181(8)2019 Feb 18.
Artigo em Dinamarquês | MEDLINE | ID: mdl-30821238

RESUMO

This review summarises the treatment of acute muscle injuries. Muscle injuries are frequent traumatic injuries caused by either excessive strain on the muscle tendon unit (strain injury) or a forceful blow to the muscle (contusion). An early start of rehabilitation after acute strain injuries is a key to shortening the time to return to sport. The application of ice, compression and elevation is well tolerated by patients, but there is no evidence that these methods enhance tissue repair. Complications after strain injuries include risk of recurrence, muscle atrophy and development of scar tissue. Ectopic bone formation may, however rarely, occur after severe contusion injuries.


Assuntos
Traumatismos em Atletas , Contusões , Músculo Esquelético , Doenças Musculares , Traumatismos em Atletas/terapia , Contusões/terapia , Humanos , Músculo Esquelético/lesões , Atrofia Muscular , Doenças Musculares/terapia , Cicatrização
11.
Int J Mol Sci ; 20(3)2019 Feb 05.
Artigo em Inglês | MEDLINE | ID: mdl-30764506

RESUMO

The morpho-functional recovery of injured skeletal muscle still represents an unmet need. None of the therapeutic options so far adopted have proved to be resolutive. A current scientific challenge remains the identification of effective strategies improving the endogenous skeletal muscle regenerative program. Indeed, skeletal muscle tissue possesses an intrinsic remarkable regenerative capacity in response to injury, mainly thanks to the activity of a population of resident muscle progenitors called satellite cells, largely influenced by the dynamic interplay established with different molecular and cellular components of the surrounding niche/microenvironment. Other myogenic non-satellite cells, residing within muscle or recruited via circulation may contribute to post-natal muscle regeneration. Unfortunately, in the case of extended damage the tissue repair may become aberrant, giving rise to a maladaptive fibrotic scar or adipose tissue infiltration, mainly due to dysregulated activity of different muscle interstitial cells. In this context, plasma preparations, including Platelet-Rich Plasma (PRP) and more recently Platelet-Poor Plasma (PPP), have shown advantages and promising therapeutic perspectives. This review focuses on the contribution of these blood-derived products on repair/regeneration of damaged skeletal muscle, paying particular attention to the potential cellular targets and molecular mechanisms through which these products may exert their beneficial effects.


Assuntos
Músculo Esquelético/lesões , Músculo Esquelético/fisiologia , Doenças Musculares/terapia , Plasma/metabolismo , Regeneração , Animais , Fibrose , Humanos , Desenvolvimento Muscular , Músculo Esquelético/crescimento & desenvolvimento , Músculo Esquelético/patologia , Doenças Musculares/metabolismo , Doenças Musculares/patologia , Plasma Rico em Plaquetas/metabolismo , Medicina Regenerativa , Células Satélites de Músculo Esquelético/citologia , Células Satélites de Músculo Esquelético/metabolismo , Células Satélites de Músculo Esquelético/patologia , Cicatrização
12.
Neurol Sci ; 40(4): 671-681, 2019 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-30805745

RESUMO

This is the second part of a two-part document intended to discuss recent therapeutic progresses in genetic neuromuscular disorders. The present review is for diseases of motor neuron and skeletal muscle, some of which reached recently the most innovative therapeutic approaches. Nusinersen, an SMN2 mRNA splicing modifier, was approved as first-ever therapy of spinal muscular atrophy (SMA) by FDA in 2016 and by EMA in 2017. The orally administered small-molecule risdiplam, which increases SMN protein levels similarly but also in peripheral organs, is tested in ongoing phase 2 and 3 trials. After positive results with phase 1 treatment with AAV9-SMN, the first gene therapy for SMA, a phase 3 clinical trial is ongoing. Ataluren is the first approved drug for Duchenne muscular dystrophy (DMD) patients with premature stop codon mutations and its indication has been recently extended since the age of 2 years. Exon skipping technology was and is currently tested in many phase 3 trials, and eteplirsen received a conditional approval by FDA for patients amenable to exon 51 skipping, but not by EMA. Many other compounds with different mechanisms of action are now tested in DMD by phase 2 and 3 trials, including phase 1 gene therapy. Other innovative approaches are under investigation, i.e., gene therapy in X-linked myotubular myopathy and Pompe disease, and antisense oligonucleotides in myotonic dystrophy type 1. Positive evidences are discussed about lamotrigine and ranolazine in non-dystrophic myotonias, chaperons in Pompe disease, and nucleosides in mitochondrial DNA depletion induced by thymidine kinase 2 deficiency.


Assuntos
Terapia Genética/métodos , Doença de Depósito de Glicogênio Tipo II/terapia , Doenças Mitocondriais/terapia , Atrofia Muscular Espinal/terapia , Doenças Musculares/terapia , Distrofia Muscular de Duchenne/terapia , Miopatias Congênitas Estruturais/terapia , Distrofia Miotônica/terapia , Fármacos Neuromusculares/uso terapêutico , Oligonucleotídeos/uso terapêutico , Oxidiazóis/uso terapêutico , Proteínas do Complexo SMN , Humanos
13.
J Atheroscler Thromb ; 26(3): 207-215, 2019 Mar 01.
Artigo em Inglês | MEDLINE | ID: mdl-30662020

RESUMO

Statins are the main treatment for hypercholesterolemia and the cornerstone of atherosclerotic cardiovascular disease prevention. Many patients taking statins report muscle-related symptoms, one of the most important causes of statin treatment discontinuation, which is associated with an increased risk of cardiovascular events. Therefore, it is important to identify patients who are truly statin intolerant to avoid unnecessary discontinuation of this beneficial treatment. Some studies indicate that not all muscle complaints are caused by statins, and most patients can tolerate a statin upon re-challenge, down-titration of dose, or switching to another statin. In this paper, we review the definitions of statin intolerance and approaches to reducing cardiovascular risk among individuals reporting statin-associated muscle symptoms.


Assuntos
Doenças Cardiovasculares/tratamento farmacológico , Inibidores de Hidroximetilglutaril-CoA Redutases/efeitos adversos , Hipercolesterolemia/tratamento farmacológico , Hiperlipidemias/tratamento farmacológico , Doenças Musculares/diagnóstico , Doenças Musculares/terapia , Animais , Humanos , Doenças Musculares/induzido quimicamente
14.
Skeletal Radiol ; 48(9): 1315-1321, 2019 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-30617717

RESUMO

Pathology of the fascia lata attachment at the iliac crest (FLAIC) is an under-recognized and often misdiagnosed cause of lateral hip pain. The fascia lata has a broad attachment at the lateral iliac crest with contributions from the tensor fascia lata muscle, the iliotibial band, and the gluteal aponeurosis. The FLAIC is susceptible to overuse injuries, acute traumatic injuries, and degeneration. There is a paucity of literature regarding imaging and image-guided treatment of the FLAIC. We review anatomy and pathology of the FLAIC, presenting novel high-resolution (18-24 MHz) ultrasound images including ultrasound guidance for targeted therapeutic treatment.


Assuntos
Fascia Lata/anatomia & histologia , Fascia Lata/patologia , Ílio/anatomia & histologia , Doenças Musculares/diagnóstico por imagem , Doenças Musculares/terapia , Ultrassonografia/métodos , Fascia Lata/lesões , Humanos , Ultrassonografia de Intervenção/métodos
15.
Radiology ; 291(1): 250-258, 2019 04.
Artigo em Inglês | MEDLINE | ID: mdl-30620252

RESUMO

Purpose To evaluate the safety and efficacy of percutaneous transarterial embolization (PTAE) for the treatment of spontaneous soft-tissue hematomas (SSTHs) and identify variables predictive of short-term outcome. Materials and Methods Between 2011 and 2017, the outcome was retrospectively analyzed for 112 patients (mean age ± standard deviation, 72 years ± 14; range, 28-92 years), including 65 women (mean age, 73 years ± 12.7; range, 39-92 years) and 47 men (mean age, 70 years ± 14.9; range, 28-91 years), with SSTH treated with PTAE. Thirty-day mortality, technical and clinical success, simplified acute physiology score (SAPS) II, anticoagulation, embolic agent, hematoma volume and location, serum hemoglobin level, hemodynamic instability, and presence of active bleeding at CT and/or angiography were recorded. Clinical success was defined as cessation of bleeding as determined by hemodynamic stability and/or serum hemoglobin level stabilization after PTAE. Univariable and multivariable analyses were performed by using a Cox model to identify variables associated with time to death. Results Mortality rate was 26.8% (30 of 112 patients), angiographic success rate was 95.5% (107 of 112 patients), and clinical success rate was 83% (93 of 112 patients). For surviving patients, mean SAPS II was 19.6 ± 7.1 (range, 13-31) and mean hematoma volume was 862 cm3 ± 618 (range, 238-1887 cm3). For deceased patients, mean SAPS II was 42 ± 13.2 (range, 18-63) and mean hematoma volume was 1419 cm3 ± 788 (range, 251-3492 cm3). SAPS II (P < .001), hematoma volume (P = .01), and retroperitoneal location (P = .01) were independently associated with fatal outcome. Conclusion Percutaneous transarterial embolization is effective for the emergency treatment of spontaneous soft-tissue hematomas. Simplified acute physiology score II, hematoma volume, and retroperitoneal location are predictors of short-term outcome. © RSNA, 2019 Online supplemental material is available for this article.


Assuntos
Embolização Terapêutica/métodos , Hematoma/terapia , Adulto , Idoso , Idoso de 80 Anos ou mais , Embolização Terapêutica/mortalidade , Procedimentos Endovasculares/efeitos adversos , Procedimentos Endovasculares/mortalidade , Feminino , Hematoma/mortalidade , Humanos , Masculino , Pessoa de Meia-Idade , Doenças Musculares/mortalidade , Doenças Musculares/terapia , Espaço Retroperitoneal , Estudos Retrospectivos , Resultado do Tratamento
16.
Tissue Eng Part C Methods ; 25(2): 59-70, 2019 02.
Artigo em Inglês | MEDLINE | ID: mdl-30648479

RESUMO

IMPACT STATEMENT: The goal of this study was to determine the threshold for a critically sized, nonhealing muscle defect by characterizing key components in the balance between fibrosis and regeneration as a function of injury size in the mouse quadriceps. There is currently limited understanding of what leads to a critically sized muscle defect and which muscle regenerative components are functionally impaired. With the substantial increase in preclinical VML models as testbeds for tissue engineering therapeutics, defining the critical threshold for VML injuries will be instrumental in characterizing therapeutic efficacy and potential for subsequent translation.


Assuntos
Doenças Musculares/patologia , Doenças Musculares/terapia , Miofibrilas/fisiologia , Junção Neuromuscular/citologia , Músculo Quadríceps/citologia , Músculo Quadríceps/lesões , Engenharia Tecidual , Animais , Feminino , Camundongos , Camundongos Endogâmicos C57BL , Músculo Quadríceps/fisiologia , Tecidos Suporte , Cicatrização
17.
Autoimmun Rev ; 18(3): 223-230, 2019 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-30639649

RESUMO

Over the past few years, myositis-specific autoantibodies played an increasing role in the inflammatory idiopathic myositis definition. They became the critical immunological marker for immune-mediated necrotizing myopathy diagnosis (IMNM) since the paradigm switch from histological to serological criteria. This review is focused on the key role of the anti-signal recognition particle (anti-SRP) and the anti-3-Hydroxy-3-MethylGlutaryl-Coenzyme A Reductase (anti-HMGCR) antibodies in immune-mediated necrotizing myopathy. Anti-SRP and anti-HMGCR antibodies are robust diagnostic tools in case of both the classical subacute form and the slowly progressive form of IMNM that may mimic muscular dystrophy. Anti-SRP and anti-HMGCR patients share clinical, biological and histological features with some antibody-associated specificity. Anti-SRP patients harbour more severe muscle weakness and atrophy with severe muscle damage on magnetic resonance imaging study. Approximately 10-20% of anti-SRP patients develop extramuscular symptoms, especially lung interstitial disease. Conversely, anti-HMGCR patients are often associated with statin exposure. In both cases, patients have a poor outcome with frequent relapse and the use of combined immunotherapy. Of note, various data suggest a direct pathogenic role of these antibodies reinforcing the interest in targeted therapeutic strategy.


Assuntos
Autoanticorpos/imunologia , Doenças Musculares/imunologia , Animais , Biomarcadores , Humanos , Doenças Musculares/diagnóstico , Doenças Musculares/patologia , Doenças Musculares/terapia , Necrose , Prognóstico
18.
Cardiovasc Intervent Radiol ; 42(3): 335-343, 2019 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-30327927

RESUMO

BACKGROUND: Severe spontaneous soft tissue hematomas (SSTH) are usually treated with transcatheter arterial embolization (TAE) although only limited retrospective studies exist evaluating this treatment option. The aim of this study was to systematically assess the efficacy and safety of TAE for the management of SSTH. METHODS: Medline, EMBASE, PubMed and Cochrane Library were searched from inception to July 2017 using MeSH headings and a combination of keywords. Eligibility was restricted to original studies with patients suffering from SSTH treated with TAE. Patients with traumatic hematomas or who were treated with solely conservative or surgical management were excluded. For each publication, clinical success based on the control of the bleed, rebleeding rates and complications (including mortality) was collected, as well as technical details. RESULTS: Sixty-three studies met the inclusion criteria, with an aggregate total of 267 patients. Follow-up extended from 1 day to 10 years. Bleeding was mainly localized to the iliopsoas (n = 113/267, 42.3%) and anterior abdominal wall (n = 145/266, 54.7%). When information was available, 81.0% (n = 158/195) of patients were on anticoagulant therapy prior to the bleeding episode. Initial stabilization with control of the bleed was obtained in 93.1% (n = 242 patients, n = 60 studies). The most common embolic materials were coils (n = 129, 54.4%). Rebleeding was reported in 25 patients (9.4%). Only two embolization complications were reported (0.7%). The 30-day mortality was 22.7% (n = 42/1857). CONCLUSION: TAE represents a safe and effective procedure in the management of SSTH. We present a management algorithm based on these data, but further studies are needed to address the knowledge gap.


Assuntos
Embolização Terapêutica/métodos , Hematoma/terapia , Doenças Musculares/terapia , Adulto , Idoso , Idoso de 80 Anos ou mais , Angiografia Digital , Angiografia por Tomografia Computadorizada , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Músculo Esquelético/diagnóstico por imagem , Doenças Musculares/diagnóstico por imagem , Estudos Retrospectivos , Resultado do Tratamento , Adulto Jovem
19.
Br J Sports Med ; 53(13): 812, 2019 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-30181323

RESUMO

OBJECTIVE: To optimally target exercise interventions for patients with cancer, it is important to identify which patients benefit from which interventions. DESIGN: We conducted an individual patient data meta-analysis to investigate demographic, clinical, intervention-related and exercise-related moderators of exercise intervention effects on physical fitness in patients with cancer. DATA SOURCES: We identified relevant studies via systematic searches in electronic databases (PubMed, Embase, PsycINFO and CINAHL). ELIGIBILITY CRITERIA: We analysed data from 28 randomised controlled trials investigating the effects of exercise on upper body muscle strength (UBMS) and lower body muscle strength (LBMS), lower body muscle function (LBMF) and aerobic fitness in adult patients with cancer. RESULTS: Exercise significantly improved UBMS (ß=0.20, 95% Confidence Interval (CI) 0.14 to 0.26), LBMS (ß=0.29, 95% CI 0.23 to 0.35), LBMF (ß=0.16, 95% CI 0.08 to 0.24) and aerobic fitness (ß=0.28, 95% CI 0.23 to 0.34), with larger effects for supervised interventions. Exercise effects on UBMS were larger during treatment, when supervised interventions included ≥3 sessions per week, when resistance exercises were included and when session duration was >60 min. Exercise effects on LBMS were larger for patients who were living alone, for supervised interventions including resistance exercise and when session duration was >60 min. Exercise effects on aerobic fitness were larger for younger patients and when supervised interventions included aerobic exercise. CONCLUSION: Exercise interventions during and following cancer treatment had small effects on UBMS, LBMS, LBMF and aerobic fitness. Demographic, intervention-related and exercise-related characteristics including age, marital status, intervention timing, delivery mode and frequency and type and time of exercise sessions moderated the exercise effect on UBMS, LBMS and aerobic fitness.


Assuntos
Terapia por Exercício/métodos , Força Muscular/fisiologia , Doenças Musculares/fisiopatologia , Doenças Musculares/terapia , Neoplasias/fisiopatologia , Aptidão Física/fisiologia , Humanos , Qualidade de Vida
20.
J Formos Med Assoc ; 118(10): 1385-1392, 2019 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-30584005

RESUMO

Statin reduces low-density lipoprotein cholesterol and improves clinical outcomes in high risk patients. In general, statin is a safe and well-tolerated medication. However, varieties of adverse effects are reported in some patients and may interfere long-term drug compliance. Statin-associated muscle events and liver function change account for most of these adverse effects. Patients are regarded as statin intolerance if they need to discontinue statin therapy due to these adverse effects. To date, there is no universal standard definition of statin intolerance. But a pragmatic definition of statin intolerance is essential and helpful for clinicians in daily practice. In this article, after expert consensus meetings and literature review, criteria were recommended to identify patients with statin intolerance in Taiwan. The purpose of this statement is to help health care professionals in Taiwan to diagnose and manage individuals who develop muscular and hepatic side effects after statin therapy.


Assuntos
Doença Hepática Induzida por Substâncias e Drogas/diagnóstico , Inibidores de Hidroximetilglutaril-CoA Redutases/efeitos adversos , Doenças Musculares/induzido quimicamente , Doenças Musculares/diagnóstico , Doença Hepática Induzida por Substâncias e Drogas/sangue , Doença Crônica , Consenso , Humanos , Hepatopatias/complicações , Doenças Musculares/terapia , Fatores de Risco , Taiwan
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