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Idiopathic inflammatory myopathies [keywords]
- [Kirschner wires and tension-band fixation through posterolaterla minimal incision combined with plaster fixation at supinated position for the treatment of Garland type III supracondylar humeral fractures in children]. [English Abstract, Journal Article]
- Zhongguo Gu Shang 2013 Feb; 26(2):92-4.
To evaluate the efficacy of Kirschner wires and tension-band fixation through posterolateral minimal incision for the treatment of displaced supracondylar humeral fractures in children.From January 2005 to December 2010, there were 62 children (38 males and 24 females, ranging in age from 2 to 14 years, averaged 6.8 years) with Gartland type III supracondylar humeral fractures. All the injuries were caused by falling, and all the fractures were fresh injuries. The duration from injury to surgery ranged from 5 to 20 hours. All the children were treated with open reduction through a posterolateral minimal approach, Kirschnere wires and tension-band fixation were fixed with plaster at 90 degrees of elbow flexion, forearm supination, and palms facing upwards. The kirschner pins and wires were removed after fractures healing. The Flynn's criterion was used to evaluate therapeutic effects.The operation time ranged from 30 to 50 min (averaged 45 min). All the patients achieved solid union. Sixty patients were followed up, and the mean follow-up time was 15 months (ranged from 6 to 24 months). At the 6th month after operation, 48 patients got an excellent result, 9 good, 3 bad (light cubitus varus with varus angle about 6 degree, without infection on function) according to Flynn's criteria. There were no complications such as procedure-related pin tract infection, iatrogenic nerve and vascular injuries and myositis ossificans.The Kirschner wires and tension-band fixation through posterolateral minimal incision approach can obtain clearer surgical field, simple in operation, and few wound complications. Therefore, this modified treatment is an effective and reliable method for pediatric displaced Gartland type III supracondylar humeral fractures.
- Inflammatory and toxic myopathy. [Journal Article]
- Semin Neurol 2012 Nov; 32(5):491-9.
Although muscle diseases are relatively rare, several treatable myopathies must be recognized by the clinician to maximize the possibility of restoring strength in affected patients. The inflammatory myopathies, including polymyositis, dermatomyositis, inflammatory necrotizing myopathy, and myositis in association with mixed connective tissue disease, typically respond well to immunosuppressive treatment. Inclusion body myositis, a myopathy that has features of both inflammation and primary degeneration, may not be treatable at this time, but treatments are actively being sought. Muscle dysfunction caused by toxins must also be recognized because removal of the offending toxin usually results in restoration of normal muscle function. Important muscle toxins include cholesterol-lowering medications, colchicine, zidovudine, corticosteroids, emetine, and ethanol.
- Imaging of benign soft tissue tumors. [Journal Article]
- Semin Musculoskelet Radiol 2013 Apr; 17(2):156-67.
The evaluation of soft tissue tumors should be approached systematically, with careful assessment of the patient's age, clinical presentation, anatomical location of the mass, and MRI characteristics. The imaging evaluation of a suspected soft tissue mass begins with conventional radiography to exclude an underlying osseous lesion and assess for any lesional calcification. MRI is particularly useful in evaluating the signal intensity, enhancement pattern, and extent of soft tissue masses that can expand beyond fascial planes and involve the neurovascular bundle, joint, or bone. Among the common benign soft tissue tumors, a fairly definitive imaging diagnosis can be made in cases of lipoma, elastofibroma dorsi, hemangiomas, myositis ossificans, giant cell tumor of tendon sheath, and peripheral nerve sheath tumors. In the remaining cases, the differential diagnosis can be narrowed by knowing the patient's demographics and any associated syndromes, in conjunction with recognizing specific MRI features. Knowledge of the World Health Organization's tumor designations and the incidence of specific tumors based on patient age and anatomical location are vital tools for the interpreting radiologist.
- Beyond the Binding Site: In Vivo Identification of tbx2, smarca5 and wnt5b as Molecular Targets of CNBP during Embryonic Development. [Journal Article]
- PLoS One 2013; 8(5):e63234.
CNBP is a nucleic acid chaperone implicated in vertebrate craniofacial development, as well as in myotonic dystrophy type 2 (DM2) and sporadic inclusion body myositis (sIBM) human muscle diseases. CNBP is highly conserved among vertebrates and has been implicated in transcriptional regulation; however, its DNA binding sites and molecular targets remain elusive. The main goal of this work was to identify CNBP DNA binding sites that might reveal target genes involved in vertebrate embryonic development. To accomplish this, we used a recently described yeast one-hybrid assay to identify DNA sequences bound in vivo by CNBP. Bioinformatic analyses revealed that these sequences are G-enriched and show high frequency of putative G-quadruplex DNA secondary structure. Moreover, an in silico approach enabled us to establish the CNBP DNA-binding site and to predict CNBP putative targets based on gene ontology terms and synexpression with CNBP. The direct interaction between CNBP and candidate genes was proved by EMSA and ChIP assays. Besides, the role of CNBP upon the identified genes was validated in loss-of-function experiments in developing zebrafish. We successfully confirmed that CNBP up-regulates tbx2b and smarca5, and down-regulates wnt5b gene expression. The highly stringent strategy used in this work allowed us to identify new CNBP target genes functionally important in different contexts of vertebrate embryonic development. Furthermore, it represents a novel approach toward understanding the biological function and regulatory networks involving CNBP in the biology of vertebrates.
- Four-year-olds, healthy or recovering from Juvenile Dermatomyositis, do not achieve a full score on the Childhood Myositis Assessment Scale (CMAS). [JOURNAL ARTICLE]
- Arthritis Care Res (Hoboken) 2013 May 10.:NA.
OBJECTIVE:To test 4-year-olds, using the Childhood Myositis Assessment Scale (CMAS) 14 maneuvers, comparing healthy children with those with Juvenile Dermatomyositis (JDM).
METHODS:Healthy 4-year-olds (n=28) completed the CMAS. Their scores were compared with children with JDM (n=18) who had a muscle Disease Activity Score (DAS-M) of zero.
RESULTS:The healthy children achieved a mean CMAS score of 46.6 ±2.3 and an inter-quartile range of (46,47). There were no significant differences between boys and girls; the scores were not significantly associated with height or weight. The greatest variation involved items that assessed endurance. Item 1: neck raise, yielded a mean score of 28.2 ±19.3 seconds, with a CMAS score of 2.5 ±0.9, (maximum score = 5). Item 3: leg lift, yielded a mean score of 55.5 ±37.3 seconds, with a mean CMAS score of 3.1 ±1.1, (maximum score = 5). Item 5: sit ups maneuver, yielded a mean score of 5.3 ±1.1 sit ups. Almost identical data were obtained for the 18 treated children with JDM who had normal strength on DAS-M.
CONCLUSION:Healthy children aged 4 years do not achieve the total CMAS of 52, attained by older children. Both boys and girls were remarkably consistent, with a mean CMAS of 46.6. Children with JDM, age 4 with a DAS-M of zero, also achieved a CMAS of 46.6. We conclude that half of 4-year-old children achieve a CMAS of 46 or 47, not a CMAS of 52, suggesting that weakness may be over diagnosed in 4-year-olds with an inflammatory myopathy. © 2013 by the American College of Rheumatology.
- A further patient with parasitic myositis due to Haycocknema perplexum, a rare entity. [JOURNAL ARTICLE]
- J Clin Neurosci 2013 May 7.
A new genus of nematode, Haycocknema perplexum, causing polymyositis in humans, was first described in two Australian patients from Tasmania in 1998. Three patients with myositis due to the same nematode were reported from northern Queensland in 2008. We report the sixth case from Australia, a 50-year-old man, also from Tasmania. He had a 2-year history of progressive weakness, weight loss of 10kg and dysphagia. Muscle biopsy was initially interpreted as polymyositis with eosinophils. Maximum creatine kinase (CK) level was 5700U/L and full blood examination was normal. He deteriorated after several months of treatment with prednisolone and methotrexate and review of the muscle biopsy showed intramyofibre parasites of H. perplexum. After 3months of treatment with albendazole therapy, he made a very good clinical recovery and his CK decreased to 470U/L. This uniquely Australian parasite can mimic polymyositis and leads to significant irreversible morbidity (two of the previous patients still have weakness and elevated CK after years) and even mortality (one died), if diagnosed late or after corticosteroids. Diagnosis can only be made by histopathology of muscle biopsy.
- The heliotrope sign of dermatomyositis: the correct meaning of the term heliotrope. [Journal Article]
- Arch Dermatol 2012 Oct; 148(10):1178.
- Ultraviolet radiation exposure is associated with clinical and autoantibody phenotypes in Juvenile Myositis. [JOURNAL ARTICLE]
- Arthritis Rheum 2013 May 8.
Objective.Genetic and environmental factors may contribute to the etiology of the juvenile idiopathic inflammatory myopathies (JIIM), systemic autoimmune diseases characterized by muscle and skin inflammation. We investigated the association between ultraviolet radiation (UVR) exposure and the clinical and autoantibody expression of JIIM. Methods. We assessed the relationship between UVR exposure in the month before symptom onset and prevalence of juvenile dermatomyositis (JDM) versus polymyositis (JPM) in 298 patients. Among JDM patients, the association between UVR exposure and myositis autoantibodies was assessed. Regression models were stratified by sex and race. The association between regional UV index in U.S. geoclimatic zones and the clinical and autoantibody subgroups was examined by weighted least squares regression analysis.
Results.We observed increasing odds of JDM compared with JPM per unit increase in the patients' highest UV index in the month before symptom onset in girls (OR = 1.18; 95% CI = 1.00-1.40). The average and highest UV indices were associated with increasing odds of anti-p155/140 autoantibodies, which was strongest in white males (OR 1.30 and 1.23, respectively). No association was observed between the UV index and anti-MJ autoantibodies or patients without myositis autoantibodies. Across US geoclimatic regions, the average UV index was associated with increasing odds of JDM and anti-p155/140 autoantibodies but decreasing odds of anti-MJ autoantibodies.
Conclusion.Short-term UVR exposure prior to illness onset may have a role in the clinical and serologic expression of juvenile myositis. Research examining mechanisms of UVR in JIIM pathogenesis is suggested from these findings. © 2013 American College of Rheumatology.
- Invasive fibroblasts: Fundamental difference between sporadic inclusion body myositis and polymyositis. [JOURNAL ARTICLE]
- Muscle Nerve 2013 May 6.
Introduction: Sporadic inclusion body myositis (sIBM) is a progressive disease that leads to extensive muscle weakness. The aim of this study was to determine whether the number and distribution of fibroblasts differ in sIBM when compared with polymyositis.
Methods:Immunofluorescence double labelling was performed on 35 biopsies with antibodies directed against perlecan and CD90, procollagen I, CD34, and CD105. In addition, non-serial ultrathin sections were studied from 3 biopsies of each condition.
Results:Fibroblasts expressing CD90 and CD34 accumulated in the endomysial compartment in polymyositis and sIBM. In addition, cells expressing CD90 were found beneath the basal lamina in both conditions. At the ultrastructural level in polymyositis, fibroblasts invaded the myofiber, with focal destruction of the basement membrane. In sIBM, by contrast, invasive fibroblasts were ensheathed by the intact myofiber basement membrane. Discussion: The impact of intruding fibroblasts on satellite cells remains to be established. © 2013 Wiley Periodicals, Inc.
- Subcutaneous immunoglobulin treatment of inclusion body myositis stabilizes dysphagia. [LETTER]
- Muscle Nerve 2013 May 4.