Autosomal dominant centronuclear myopathy]

In a family 6 members in 3 generations were affected by centronuclear myopathy (CNM) of autosomal dominant inheritance. The apparent onset was in the early forties and the disease progressed slowly. Limb weakness was predominant. Strabismus was present in 5 cases and calves hypertrophy in 3. Serum creatinine kinase was always within the normal range. In one case myotonic bursts were found at electromyography. In 2 cases brain stem auditory evoked potential studies demonstrated abnormal prolongation of interpeak latencies I-III and favoured subclinical nervous system involvement. Muscular biopsies showed typical features of centronuclear myopathy with 50 to 80% central nuclei. In two cases immunocytochemical labelling of dystrophin showed staining in the sarcoplasm in favour of an arrest in the morphogenesis of developing myofiber. Others families with autosomal dominant CNM in the literature and also some sporadic adult cases had similar clinical features.     

Patient attitude towards waiting in an outpatient clinic and its applications.

Patient waiting time in outpatient clinics is often the major reason for patients' complaints about their experiences of visiting outpatient clinics. Therefore, patient satisfaction with waiting time plays a crucial role in the process of health quality assurance or quality management. This paper reports the results of a survey on patient attitude towards waiting in an outpatient surgery clinic. Generally patients appear reasonably satisfied if they wait no more than 37 minutes when arriving on time, and no more than 63 minutes when late for appointments. Patients coming up to 15 minutes early are prepared to take full responsibility for the extra waiting caused, but the patients coming even earlier intend to be seen earlier and are only prepared to wait 15 minutes longer than otherwise. The potential applications of patient attitude towards waiting in the quality assurance process, ie setting up waiting time limits and designing patient dispatching rules, are also discussed.     

恶性肿瘤患者的皮肤转移（附50例报告）

恶性肿瘤的皮肤转移确与患者的病种、病理类型、术前后淋巴结转移情况、临床分期、手术类型、放疗及化疗的剂量、遗传因素有关。其出现于任何部位均提示预后不佳。     

Treatment of hemophilia A by living mother-to-son splenic transplantation. First case report in the world.

A case of severe hemophilia A was treated by living mother-to-son splenic transplantation. The recipient was a 9-year-old boy. After splenic transplantation, the level of blood factor VIII:C was increased, and no spontaneous hemorrhage occurred. The boy was followed up for over two years. This is a case of spleen allotransplantation with the longest function in the world.