他汀类药致肌病的临床特点研究

目的:研究他汀类药致肌病的临床特点。方法:收集我院2006-2010年他汀类药致肌病43例为肌病组;同期同一病房原发病相同(均为冠心病或脑梗死患者)、同性别、服用同一类他汀类药的北京地区患者为对照组。对2组在年龄、并发症、合并用药、药物剂量等方面的临床特点进行对照分析。结果:他汀类药致肌病的危险因素主要为高龄、合并甲状腺疾病、嗜酒、服用较大剂量他汀类药、合并其他降脂药或经细胞色素P450(CYP)3A4代谢药。肌病发生时间多为用药2个月之内(86.0%),但预后较好。结论:临床应重视他汀类药致肌病的危险因素,密切关注不良反应的发生,以确保用药安全。     

Myopathy with tubulin-reactive inclusions in two cats

Many types of inclusions have been described in human myopathies including but not limited to nemaline rod bodies, cylindrical spirals, tubular aggregates, cytoplasmic bodies, reducing bodies, and fingerprint bodies, and hyaline inclusions in myofibrillar myopathy and inclusion body myositis. There are very few reports describing inclusions in spontaneously occurring myopathies in cats, and these reports are limited to nemaline rod myopathy. A myopathy with tubulin-reactive crystalline inclusions has recently been reported in a human patient with a clinical presentation of myalgia and fatigue. Similarly, a myopathy with chronic, slowly progressive muscle weakness has been identified here in two unrelated cats. Inclusions were the only pathological change in skeletal muscle biopsies and, ultrastructurally, groups of crystalline structures were evident that had a subsarcolemmal or central location, rhomboid or rectangular shapes, lacked orientation, and were not membrane bound. The crystalline structures reacted positively with an antibody against tubulin. This feline myopathy may be the equivalent of the human myopathy with tubulin-positive crystalline inclusions.     

Progressive myofiber loss with extensive fibro-fatty replacement in a child with mitochondrial DNA depletion syndrome and novel thymidine kinase 2 gene mutations

The mitochondrial DNA depletion syndromes (MDS) are autosomal recessive disorders with a decreased mitochondrial DNA copy number. Mutations in thymidine kinase 2 (TK2) have been responsible for the myopathic form of MDS. We describe a child with congenital muscle weakness who had a progressive mitochondrial myopathy associated with extensive fibro-fatty replacement of myofibers resembling muscular dystrophy. MDS was suspected based upon findings in the initial muscle biopsy. Sequence analysis of the TK2 gene revealed two novel heterozygous mutations: the frame shift mutation, c.255_c.258delAGAA, and the heterozygous missense mutation, c.515G>A, (p.R172Q). This report extends the phenotype and genotype of TK2 defects.     

Clinical and laboratory features of hyponatremia-induced myopathy

Background The present study was undertaken to determine the clinical and laboratory features of hyponatremia-induced myopathy. Methods We collected 14 hyponatremic subjects (six men and eight women) with serum creatine kinase (CK) levels of more than 500 IU/ml during the 5-year period between 2001 and 2005. The mean ± SD patients’ age was 66.5 ± 16.7 years (range, 37 to 88 years). Results The causes of the hyponatremia were: syndrome of inappropriate secretion of antidiuretic hormone (SIADH; n = 4), mineralocorticoid-responsive hyponatremia of the elderly (MRHE; n = 2), hypopituitarism (n = 1), psychogenic polydipsia (n = 3), congestive heart failure (n = 3), and unknown cause (n = 1). The subjects were subgrouped into two groups: acute onset of myopathy and slowly progressive onset. The age at onset was 62.0 ± 5.7 years (mean ± SEM) in the subjects with acute onset, and 77.8 ± 1.5 years in those with slowly progressive onset (P = 0.02). At the onset, there was no difference in serum Na levels between the acute onset and the slowly progressive onset groups, but there was a significant difference in maximal serum CK levels between the groups (7072 ± 2317 vs 722 ± 104 IU/ml; P = 0.02). Maximal serum CK levels were widely distributed among the ages in the subjects with acute onset, whereas maximal serum CK levels were mildly elevated in the elderly subjects with slowly progressive onset. The elevated serum CK levels were normalized at a maximum of 14 days after the onset in all the subjects. Conclusions The present findings indicate that hyponatremia infrequently causes skeletal muscle disruption, and that there are two types of hyponatremia-induced myopathy, acute onset and slowly progressive onset.     

Flexible intramedullary nailing for distal femoral fractures in patients with myopathies

Purpose  

Distal femoral fractures are quite common in nonambulating patients with myopathies, as they present marked osteoporosis. The deterioration of preexisting knee flexion contracture is a known problem, as these fractures are usually angulated posteriorly. The goals of treatment are to reduce immobilization and bed rest to a minimum, prevent function loss, and prevent refracture. The aim of our work was to investigate if these goals can be achieved by an operative treatment with closed reduction and flexible intramedullary nailing (FIN).     

MRI evaluation of amyloid myopathy

Amyloid myopathy is a rare complication of primary amyloidosis. The magnetic resonance imaging (MRI) features of two patients with amyloid myopathy were studied. Slight prolongation of muscle T1 and T2 relaxation times was evident but the striking abnormality was marked reticulation of the subcutaneous fat. The clinical findings of indurated extremities far exceeds the minimal signal intensity alteration seen in the muscles. The MR appearance of amyloid myopathy differs from that of other neuromuscular conditions in the minimal changes found in muscle, but the striking abnormality seen in subcutaneous fat makes it distinct from many neuromuscular conditions.     

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     

Effect of Chronic Alcoholism on Human Muscle Glycogen and Glucose Metabolism

To determine the effect of alcohol on carbohydrate metabolism, 48 human muscle biopsies from chronic alcoholics were studied. The level of glycogen and the activities of the enzymes catalyzing glycogen and glucose metabolism were analyzed. Chronic alcohol intake produced an increase in glycogen concentration and a decrease in pyruvate kinase activity before the first signs of myopathy appeared. When myopathy was present, glycogen decreased. These changes may contribute to the decline in skeletal muscle performance in these patients.     

Myopathology in patients with a Noonan phenotype

Two patients with a Noonan phenotype and progressive hypertrophic obstructive cardiomyopathy are described, in whom abnormal histopathological changes in striated musculature were detected. In both patients an increased density of muscle spindles was found at biopsy. The significance of an increased density of muscle spindles in patients with Noonan phenotype can only be speculated. The question is raised of whether these changes are a distinct feature within the spectrum of patients with Noonan phenotype. Received: 10 July 1995 / Revised, accepted: 3 June 1996