Neuromuscular features in Marfan syndrome

Marfan syndrome is a clinically and allelic heterogeneous, heritable connective tissue disorder with infrequently reported neuromuscular features. This study is the first to delineate these symptoms in a non-selected population. Neuromuscular involvement was evaluated in 10 Marfan patients through a standardized questionnaire, physical examination, nerve conduction study (NCS), needle electromyography (EMG), muscle ultrasound, laboratory investigation, and muscle biopsy. Existing neuroimages were screened for dural ectasia and spinal meningeal cysts. Twenty healthy controls with similar age distribution completed the questionnaire.
The results showed that various neuromuscular symptoms occur more frequently in the patients. Four older patients reported muscle weakness, five patients had a mild-to-moderate reduction in vibration sense, and all older patients mentioned mild functional impairments. NCS showed axonal polyneuropathy in four and EMG myopathic and neurogenic changes in all patients. Increased echo intensity and atrophy on muscle ultrasound was found in more than half of the patients. Muscle biopsies obtained in two patients showed myopathic changes in the older, female patient.
In conclusion, the majority of Marfan patients exhibited neuromuscular symptoms characterized as myopathy or polyneuropathy or both, and signs of lumbosacral radiculopathy, with symptoms being most pronounced in the older patients. Although meriting corroboration, these findings indicate a need to further the awareness of neuromuscular involvement in this population.     

Education and the metabolic syndrome in women

OBJECTIVE: The main objective was to examine the association between the metabolic syndrome and socioeconomic position (as indicated by education) among women. RESEARCH DESIGN AND METHODS: The study sample comprised healthy women (aged 30-65 years) in Sweden who were representative of the general population in a metropolitan area. Socioeconomic position was measured by educational level (mandatory [< or = 9 years], high school, or college/university). The metabolic syndrome was defined as the presence of two or more of the following components: 1) fasting plasma glucose level > or = 7.0 mmol/l; 2) arterial blood pressure > or = 160/90 mmHg; 3) fasting plasma triglycerides > or = 1.7 mmol/l and/or HDL cholesterol < 1.0 mmol/l; and 4) central obesity (waist-to-hip ratio > 0.85 and/or BMI > 30 kg/m2). RESULTS: After adjustment for age, the risk ratio for the presence of the metabolic syndrome comparing the lowest (< or = 9 years) with the highest (college/university) education was 2.7 (95% CI 1.1-6.8). This association persisted after controlling for menopausal status, family history of diabetes, and behavioral risk factors. CONCLUSIONS: Low education is associated with increased risk for metabolic syndrome in middle-aged women. These findings show that not only are women with low socioeconomic position at increased risk for individual risk factors that are associated with cardiovascular disease and type 2 diabetes, they are also at increased risk for the metabolic clustering of risk factors.     

蒽环类药对白血病及淋巴瘤患儿的心脏毒性

蒽环类药对白血病及淋巴瘤患儿的心脏毒性翟力平吴吴长根马伴吟陈伟达蒽环类化疗药（包括阿霉素、柔红霉素）引起心脏毒性已经是众所周知，但由于在肿瘤治疗中的重要作用，至今尚无满意的药物可以替代。据认为对儿童心毒性更敏感。如何掌握儿童治疗与心毒性之间的关系，...     

A portable target controlled propofol infusion system

A portable target controlled infusion system for propofol has been developed based on a Psion hand-held microcomputer and the Ohmeda 9000 syringe pump. The system uses a pharmacokinetic model which describes the distribution and elimination of propofol to achieve and maintain any selected target blood concentration. Target blood concentrations of 1 g/ml, 3 g/ml and 5 g/ml were selected in laboratory trials and the cumulative volumes delivered by the Psion system each minute were compared with the theoretical output calculated by the pharmacokinetic model. The results obtained showed that the computer system delivered volumes which were always within 2% of the theoretical values. This system offers a convenient and simple method of maintaining anaesthesia using propofol.