Our aim for this narrative review was to undertake a search of studies into exercise for people living with Idiopathic Inflammatory myopathies (IIM). We explored the strength of existing evidence with a particular consideration for the implications for people living with IIM and what is important to them. The search strategy from the 2021 Cochrane Physical Activity review in neuromuscular disease was used, and we selected articles that included people with IIM, including Dermatomyositis (DM), Inclusion Body Myositis (IBM), Immune Mediated Necrotising Myopathy (IMNM) [also known as necrotizing autoimmune myopathy (NAM)], and Polymyositis (PM). 2967 records were screened and 16 were included in this review. Safety of exercise was demonstrated in nine articles, using a range of measures of disease activity, serum creatine kinase, indicators of inflammation, pain, or fatigue. Two studies that took muscle biopsies showed no evidence of increased inflammation. Aerobic exercise protocols were used in 8 studies across conditions and demonstrated improvements in cardiorespiratory fitness or exercise capacity. Six studies of strength training observed improvements in muscle function, with two studies reporting muscle biopsy results of amplified immune response and up regulation of genes related to recycling of damaged proteins. Nine of 13 studies that measures functional outcomes showed significant improvements, and evidence for behaviour change was observed in a study of a self-management intervention. The evidence of safety and effect of training is reassuring and welcome, and we now need to explore how we support people to incorporate exercise and physical activity longer term into active lifestyles. 相似文献
HYPOTHESIS: Labyrinthitis ossificans results in part from the intense inflammatory response to Streptococcus pneumoniae cell wall components. Depletion of complement in Mongolian gerbils following induction of meningitis will reduce the degree of inflammation and subsequent cochlear fibrosis. STUDY DESIGN: Random prospective study. Histological evaluations were performed with the researcher blinded to the experimental group METHODS: S. pneumoniae meningitis was induced in 10 control and 18 experimental Mongolian gerbils with an intrathecal injection of the bacteria. Both groups of animals received treatment with penicillin. The experimental group was also treated with cobra venom factor to deplete complement in the animals. Three months after the induction of meningitis, the animals' temporal bones were harvested for histological evaluation. RESULTS: The decomplemented animals developed significantly less intracochlear fibrosis (P < .01). The mortality rate for the experimental group was 11% compared with 40% in the control group (P = .14). CONCLUSIONS: Reduction of the intense inflammatory response to the S. pneumoniae cell wall components in suppurative labyrinthitis secondary to bacterial meningitis reduced the degree of labyrinthitis ossificans. 相似文献
Fibrodysplasia ossificans progressiva (FOP) is a rare and debilitating genetic disorder of skeletal malformations and progressive heterotopic ossification. Flare‐ups are episodic, with bone formation in skeletal muscle and connective tissue leading to ankylosis of major joints of the axial and appendicular skeleton. This report outlines the management of a patient with FOP who had ankylosis of the temporomandibular joint and progressive ossification of the neck structures. The patient underwent two different surgical and anesthetic procedures within a 10‐year period to manage his oral pain. The authors compare the surgical techniques, osteotomy versus the more conservative buccal approach, anesthesia techniques, and conventional intubation versus sedated fiberoptic intubation. This report emphasizes the importance of a less invasive surgical technique and an appropriate anesthetic management that reduces the risks, cost, and morbidity associated with routine surgical management of patients with FOP. 相似文献
While increased serum troponin levels are often due to myocardial infarction, increased levels may also be found in a variety of other clinical scenarios. Although these causes of troponin elevation have been characterized in several studies in older adults, they have not been well characterized in younger individuals.
Methods
We conducted a retrospective review of patients 50 years of age or younger who presented with elevated serum troponin levels to 2 large tertiary care centers between January 2000 and April 2016. Patients with prior known coronary artery disease were excluded. The cause of troponin elevation was adjudicated via review of electronic medical records. All-cause death was determined using the Social Security Administration's death master file.
Results
Of the 6081 cases meeting inclusion criteria, 3574 (58.8%) patients had a myocardial infarction, while 2507 (41.2%) had another cause of troponin elevation. Over a median follow-up of 8.7 years, all-cause mortality was higher in patients with nonmyocardial infarction causes of troponin elevation compared with those with myocardial infarction (adjusted hazard ratio [HR] 1.30; 95% confidence interval [CI], 1.15-1.46; P < .001). Specifically, mortality was higher in those with central nervous system pathologies (adjusted HR 2.21; 95% CI, 1.85-2.63; P < .001), nonischemic cardiomyopathies (adjusted HR 1.66; 95% CI, 1.37-2.02; P < .001), and end-stage renal disease (adjusted HR 1.36; 95% CI, 1.07-1.73; P = .013). However, mortality was lower in patients with myocarditis compared with those with an acute myocardial infarction (adjusted HR 0.43; 95% CI:, 0.31-0.59; P < .001).
Conclusion
There is a broad differential for troponin elevation in young patients, which differs based on demographic features. Most nonmyocardial infarction causes of troponin elevation are associated with higher all-cause mortality compared with acute myocardial infarction. 相似文献
Idiopathic focal myositis is a rare clinical entity and is mostly localized in the neck and thigh muscles presenting as a
pseudotumor. We describe a 49-year-old patient with inflammation restricted to the small muscles of one forefoot. Systemic
disease was not present and progression to polymyositis did not occur within 2 years of follow-up. The myositis improved promptly
after initiation of immunosuppressive therapy with corticosteroids and azathioprine. This unusual location of focal myositis
has not been previously reported.
Received: 28 May 1998 / Accepted: 15 June 1998 相似文献
The pathogenesis of the idiopathic inflammatory myopathies has been postulated to be an environmental trigger causing the expression of the disease in a genetically predisposed patient. We report a case of anti-Jo1 antibody-positive myositis which was associated with pleural effusions, pericardial effusion with tamponade, and mechanics hands, probably related to the consumption of a fermented Kombucha beverage. Kombucha mushroom, a symbiosis of yeast and bacteria, is postulated to be the trigger for our patients disease owing to the proximity of his symptoms to the consumption of the Kombucha beverage.Abbreviations ALT Alanine transferase - AST Aspartate aminotransferase - CPK Creatinine phosphokinase 相似文献
Sickle cell disease (SCD) is associated with sensorineural hearing loss (SNHL). Although the hearing loss is usually mild, some develop severe‐to‐profound hearing loss, in whom cochlear implants (CI) may be an option. We present the cases of two children with SCD who developed bilateral severe‐to‐profound SNHL and underwent cochlear implantation. One patient became profoundly deaf after an acute episode of dizziness. Imaging indicated bilateral cochlear ossification, making subsequent cochlear implant surgery challenging. The second patient developed bilateral severe‐to‐profound SNHL following acute vaso‐occlusive crises. She went on to have uncomplicated cochlear implant surgery. These cases illustrate the variable manner in which children with SCD may develop SNHL, and the difficulties associated with managing such cases. We recommend that children with SCD should undergo regular audiological assessment. Furthermore, clinicians should be aware of the risk of cochlear fibrosis and ossification and ensure prompt assessment following an acute vaso‐occlusive crisis or unexplained vestibulocochlear event. 相似文献
Background: Trismus is a problem commonly encountered by the dental practitioner. It has a number of potential causes, and its treatment will depend on the cause. However, there are very few reports of trismus due to fibrodysplasia ossificans progressiva (FOP) following third molar surgery.
Clinical Presentation: FOP is a rare human genetic disorder with characteristic clinical features like progressive formation of extraskeletal bone or heterotopic ossification and congenital malformation of the great toes.
Clinical Significance: It is troublesome to the maxillofacial surgeon, that minimal manipulation and minor surgery can induce bone formation in soft tissues of the head and neck region, particularly the masticatory muscles and the temporomandibular joint. This paper presents a case of severe trismus following third molar extraction, intractable by routine treatment methods, which was later diagnosed as FOP. 相似文献