创伤性肘关节骨化性肌炎的预防治疗

目的:探讨如何运用早期复位、止血、血肿穿刺及骨折术后引流等方法预防肘部脱位及骨折时骨化性肌炎的发生。方法:总结分析2001年6月～2006年1月笔者用该方法治疗128例肘部关节脱位、骨折患者的临床治疗资料并与此前76例未采取该治疗方法的病例进行对比评定。结果:128例肘部关节脱位、骨折在1年随访期内,仅出现骨位性炎病例1例。结论:肘部关节脱位、骨折的早期及时良好的复位、患部冰敷、血肿穿刺及骨折术后正确的引流、早中期非抗力性主动功能锻炼能有效降低骨化性肌炎的发生。     

Polymyositis, dermatomyositis, and autoimmune necrotizing myopathy: clinical features

Idiopathic inflammatory myopathies are a heterogeneous group of autoimmune disorders predominantly affecting skeletal muscles, resulting in muscle inflammation and weakness. The 3 most common inflammatory myopathies are polymyositis (PM), dermatomyositis (DM), and inclusion body myositis. This review details the clinical findings noted in PM, DM, and the emerging entity of autoimmune necrotizing myopathy.     

Imaging of skeletal muscle

Various diagnostic imaging techniques such as sonography, computed tomography, scintigraphy, radiography, and magnetic resonance imaging (MRI) have made possible the noninvasive evaluation of skeletal muscle injury and disease. Although these different modalities have roles to play, MRI is especially sensitive in the diagnosis of muscle disorders and injury and has proved to be useful in determining the extent of disease, in directing interventions, and in monitoring the response to therapies. This article describes how magnetic resonance images are formed and how the signal intensities in T1- and T2-weighted images may be used for diagnosis of the above-mentioned conditions and injuries.     

A primer on electrophysiologic studies in myopathy

Electromyography and nerve conduction studies are the primary electrodiagnostic studies employed in the evaluation of patients with weakness and suspected myopathy. This article discusses the physiologic principles that serve as a framework for understanding the purpose, limitations, and interpretation of these tests. In the process the authors also review the differential diagnosis of myopathy.     

Fibrodysplasia ossificans progressiva in a young adult with genetic mutation: Case report

Rationale:Fibrodysplasia ossificans progressiva (FOP) is a rare autosomal dominant disorder characterized by congenital skeletal deformities and soft tissue masses that progress into heterotopic ossification. Deformities of the great toes are distinctive and heterotrophic ossification usually begins in the first decade of the patient''s life. Any invasive procedure could potentially trigger a flare and heterotopic calcification. The diagnosis is difficult and there is no effective treatment for FOP and the approximate life expectancy is 4 decades.Patient concerns:A 22-year-old male patient who had suffered from pain and movement limitations for 14 years. At the early stage of disease, the child underwent an operation on both thighs with a diagnosis of myophagism. He had serious stiffness and multiple bony masses with the characteristic bilateral hallux valgus deformity and microdactyly.Diagnoses:The patient was diagnosed with FOP by the help of characteristic great toe malformations and widespread heterotopic ossification throughout the body. Deoxyribonucleic acid sequencing demonstrated that the patient had a de novo heterozygous mutation (c.617G>A; p.R206H) in activin A receptor/activin-like kinase 2.Interventions:We administered a co-therapy of glucocorticoids, NSAIDs to relieve pain, and montelukast for 2 months. Bisphosphonate (5 mg, intravenous) was used once.Outcomes:At the follow-up 12 months later, the patient still felt low back pain sometimes and need take NSAIDs three times a week.Lessons:Clinicians and radiologists should realize the characteristic features of FOP and early diagnosis can prevent additional invasive harm to the patient.     

Clinical Heterogeneity of Interstitial Lung Disease in Polymyositis and Dermatomyositis Patients With or Without Specific Autoantibodies

Background

The aim of this study was to compare the heterogeneity of interstitial lung disease (ILD) in patients with polymyositis and dermatomyositis (PM/DM) according to serological type.

Spinal arachnoid calcifications

Arachnoid calcifications have been imaged on axial-CT and MRI. These often clinically asymptomatic deposits have been attributed to trauma, myelography (particularly with the use of oil-based contrast), subarachnoid hemorrhage, and spinal anesthesia. This report describes calcified arachnoid plaques in an 86-year-old male cadaver located in the dorsal thoracic level. A laminectomy from C4 to the lumbo-sacral junction was performed and the dura mater was transected and pinned aside. Three plaques between T2-T3, 3 at T7, 2 at T9 were noted. Plaques were 1.0 mm in thickness and ranged from 0.5 cm to 1.0 cm in diameter. The spinal cord or nerve roots were not displaced. The plaques stained positively with Von Kossa and Alizarin Red S staining, confirming that the deposits included calcium phosphate and calcium. Arachnoid calcifications are an incidental finding in cadavers and typically do not compress the spinal cord or nerve roots. However such plaques may be precursors to spinal arachnoid ossificans, a more serious condition that may cause spinal cord or nerve root compression.     

The T Cell Receptor (TCR) in HLA-B27-Restricted T Cell Responses - an Introduction

Summary D-penicillamine, an agent still used in the treatment of rheumatoïd arthritis (RA) may produce inflammatory myopathy or myositis. Some reported cases are documented with muscle biopsy. We report a 34-year old female, receiving the drug for more than 4 years, who consulted us with recently developed proximal muscle pain and weakness. EMG-findings were typical for inflammatory muscle disease; muscle enzymes remained normal. D-penicillamine was stopped and she was started on prednisolone with rapid improvement. The EMG-findings, with follow-up within three months, proved to be a good diagnostic tool, in the absence of laboratory muscle enzymes abnormalities.Correspondence to: Algemeen Ziekenhuis Middelheim Dienst Fysische Geneeskunde en Reumatologie Lindendreef 1 2020 Antwerpen, BELGIUM     

Thoracic insufficiency syndrome in patients with fibrodysplasia ossificans progressiva

Patients with fibrodysplasia ossificans progressiva (FOP) develop thoracic insufficiency syndrome (TIS), which can lead to life-threatening complications. Features contributing to TIS in patients with FOP include costovertebral malformations with orthotopic ankylosis of the costovertebral joints; ossification of intercostal muscles, paravertebral muscles, and aponeuroses; and progressive spinal deformity, including kyphoscoliosis or thoracic lordosis. Pneumonia and right-sided congestive heart failure are the major life-threatening hazards that result from TIS in patients with FOP. Prophylactic measures to maximize pulmonary function, minimize respiratory compromise, and prevent influenza and pneumonia may be helpful in decreasing the morbidity and mortality from TIS in patients with FOP.