彩色多普勒超声对多发性大动脉炎病变的诊断价值

目的：探讨多发性大动脉炎的彩色多普勒超声图像特点，提高超声对本病诊断的准确性。方法：对20例多发性大动脉炎患者病变段血管进行二维及彩色多普勒超声检查，分析病变段血管的声像图改变。结果：20便多发性大动脉炎患者中头臂型13例，混合型4例，肾动脉型2例，胸腹主动脉型1例，病变段动脉正常结构消失，血管内壁增厚，致管腔不同程度狭窄，并发锁骨下动脉盗血综合征2例，颈总动脉闭塞4例。结论：彩色多普勒超声可较准确、直观地显示病变段血管的形态学及血流动力学改变，是诊断多发性大动脉炎的有效方法之一，可为临床提供有价值的诊断信息。     

翁维良教授活血通络法治疗多发性大动脉炎长时医案举隅

大动脉炎是一种慢性进行性非特异性炎性疾病，以抗炎、抑制免疫、扩血管等内科治疗为主，由于大动脉炎具有反复发作的特点，因此如何控制病情发展、减少受累血管、控制血压、缓解症状成为治疗的重点与难点。文章系统分析翁维良教授应用活血通络法治疗大动脉炎长时医案1则（治疗33 a），通过对其诊治思路、用药经验进行分析，总结出翁教授在活血通络基础上，根据疾病不同发展阶段本虚标实的偏盛，运用搜风通络、清热解毒、清肝平肝治疗大动脉炎的临证经验。     

Mixed connective tissue disease: a disease entity?

OBJECTIVE: To explicate whether mixed connective tissue disease (MCTD) is a distinct disease and evaluate the reliability of three different diagnostic criteria proposed by Sharp, Alarcon-Segovia and Kasukawa respectively. METHODS: Clinical follow-up of 50 MCTD patients lasted 2-8 years (80% > 5 years). HLA-A, -B as well as -DR typing was performed by complemently dependent cytotocity assay. Autoantibody profile was detected by counterimmune electrophoresis (CIE). RESULTS: Thirteen (26.0%) of the 50 MCTD patients subsequently developed other connective tissue disease (OCTD), including 7 systemic lupus erythematosis (SLE), and 6 progressive systemic scleroderma (PSS). Among 23 of the MCTD patients fulfilling Sharp's criteria, 1 (4.3%) developed PSS, but among 23 of the patients fulfilling Kasukawa's, not Sharp's, 7 (30.4%) developed OCTD and among 27 of the patients fulfilling Alarcon-Segovia's, not Sharp's, 12 (44.4%) developed OCTD. In the frequencies of DR4 and DR5, there were significant differences between patients fulfilling Sharp's (60.9%, 56.5%) and controls (24.3%, P < 0.005, RR = 4.7 and 21.4%, P < 0.005, RR = 4.6%), but there were no significant differences between the patients not fulfilling Sharp's and normal control (P > 0.05). CONCLUSIONS: MCTD is a distict rheumatic disease. Sharp's criteria is the most reliable for diagnosis of MCTD.     

Huntington's disease: prenatal screening for late onset disease.

This article presents a set of moral arguments regarding the selective abortion of fetuses on the basis of prenatal screening for late onset genetic diseases only, and for Huntington's Disease* in particular. After discussion of human suffering, human perfection and the distinctive features of the lives of people confronting late onset genetic disease, the author concludes that selective abortion is difficult to justify ethically, although it must remain a matter of personal choice.     

Management of neurodegenerative disorders: Parkinson's disease and Alzheimer's disease

Neurodegenerative disorders result from premature progressive degeneration of specific neurons, and manifest as diseases or syndromes with varied combinations of cognitive, motor, sensory and autonomic dysfunctions. The management involves pharmacotherapy as well as non-pharmacological measures and also to lessen the burden of the care-givers. The medications available for medical treatment are: Levodopa, dopamine agonists, amantadine, anticholinergics, enzyme inhibitors, etc. Advanced Parkinson's disease is concerned with management of motor complications and non-motor complications. Recently surgical treatment is a great option for managing motor complication. Orthostatic hypotension, gait distiurbances, emotional and psychiatric problems, sleep disturbances can be managed and had been discussed in brief. Currently there is no medication available for the cure of Alzheimer's disease. The specific medications claimed to improve patient's well being and cognition include cholinesterase inhibitors, N-methyl-D-aspartate receptor antagonist, anti-oxidants, and anti-amyloid therapy. While medical and surgical treatments for Parkinson's disease have revolutionised the management, still drug therapy for Alzheimer's disease is dismal.     

Parkinson’s disease therapy: treatment of early and late disease

Purpose To summarize the current strategies for the treatment of early and late Parkin son’s disease (PD).Data sources The presented guidelines are based on the review of the literature as well as th e author’s extensive experience with the treatment of 7000 patients with PD over the past 25 years.Results An analysis of reported data as well as personal experience suggest that while y oung patients seem to have a slower progression of the disease, they are at a hi gher risk for developing levodopa induced complications, such as motor fluctuati ons and dyskinesias. It is, therefore, prudent practice to delay levodopa thera py, particularly in younger patients, until the PD symptoms become troublesome a nd interfere with social or occupational functioning. Other strategies, such as the use of deprenyl, amantadine, trihexyphenidyl and dopamine agonists, should be employed before instituting levodopa therapy. Entacopone and dopamine agonis ts are useful in smoothing out levodopa related motor fluctuations. Surgical in terventions, such as pallidotomy and pallidal or subthalamic deep brain stimulat ion, are effective therapeutic strategies, but should be reserved only for patie nts in whom optimal medical therapy fails to provide satisfactory control of sym ptoms.Conclusion The medical and surgical treatment of patients with PD must be individualized an d tailored to the needs of the individual patient.     

Erdheim-Chester disease: a rare cause of interstitial lung disease

Erdheim-Chester disease is a non-Langerhans cell histiocytosis that is progressive and may lead on to multi-organ involvement. Pulmonary involvement is rare, its presentation is nonspecific, and it carries an adverse outcome. Several radiological features, when considered together, may point to the diagnosis. This condition should be considered in the differential diagnosis of interstitial lung disease. We describe a 39-year-old woman who presented with dry cough, malaise and progressive dyspnoea. She was diagnosed to have late stage interstitial lung disease due to Erdheim-Chester disease.