包涵体肌炎的临床和病理分析

目的探讨包涵体肌炎的临床和病理特点。方法分析20例包涵体肌炎患者的临床、骨骼肌活检组织化学染色及电镜病理特点。结果骨骼肌活检组织化学染色病理特点：肌周膜、肌内膜、血管周围炎性细胞浸润,肌纤维大小不等,散在变性、坏死和再生肌纤维,结缔组织增生,肌纤维内可见边缘空泡,空泡周边淀粉样物质沉积,可伴破碎红纤维、肌纤维类型或分布异常。电镜：肌原纤维结构紊乱,Z线排列不整或消失,肌浆包涵体内可见大量髓磷脂小体和吞噬空泡聚集,周边可见脂滴、糖原颗粒。结论包涵体肌炎临床诊断、鉴别诊断困难,骨骼肌活检病理分析是确诊本病的可靠标准。     

酷似运动神经元性疾病的包涵体肌炎

肌萎缩性侧索硬化(ALS)和包涵体肌炎(IBM)是具有不同临床和肌电图表现的纯运动障碍性疾病,因此其鉴别诊断通常比较明了。但有时不典型的特征可能引起混淆。本文旨在找出误诊的根源。     

散发性包涵体肌炎一例报告

目的 探讨散发性包涵体肌炎临床与病理特点.方法 对1例散发性包涵体肌炎的临床表现,肌电图,肌肉活检等资料进行分析.结果 该病例根据临床表现,实验室检查,肌电图表现,肌肉病理改变确诊为散发性包涵体肌炎.结论 散发性包涵体肌炎发病机制尚未明确,临床表现缺乏特异性,肌肉病理学检查可以作为诊断散发性包涵体肌炎的金标准.     

特发性炎性肌病119例肌肉活检病理分析

目的：探讨特发性炎性肌病（IM）的肌肉活检病理类型及其临床病理联系。方法：回顾性分析IM的病理改变，归纳出各病理类型的特点，随访并评估IM患者的预后情况。结果：在119例IM中，重、中、轻度坏死性肌炎分别为11例、19例和27例，束周坏死／萎缩性肌炎20例，无炎细胞浸润的坏死性肌炎22例，间质性肌炎11例，肌筋膜炎3例，包涵体肌炎4例，肉芽肿性肌炎和增生性肌炎各1例，其中72例获得随访资料，轻度坏死性肌炎和束周坏死／萎缩性肌炎患者的好转和治愈的百分比高于中，重度坏死性肌炎患者，间质性肌炎和无炎细胞浸润的坏死性肌炎的好转和治愈百分比高于重度坏死性肌炎，结论：IM的病理类型对判断预后具有重要的参考价值。     

包涵体肌炎的运动干预及其作用机制研究进展

包涵体肌炎是老年人最常见的炎性肌肉疾病,其对激素或免疫调节治疗的反应均较差。目前,运动训练是包涵体肌炎治疗的重要组成部分。本文综述了运动训练治疗在包涵体肌炎临床治疗中的应用,以及运动训练的作用机制的研究进展。     

多发性肌炎临床诊治体会

多发性肌炎 (ＰＭ )是主要影响四肢近端骨骼肌、颈肌和咽肌的一种慢性炎症性疾病。为更好地诊断和治疗ＰＭ ,现将我们收治的 14例ＰＭ病例资料分析如下。临床资料1.一般资料 :本组 14例ＰＭ患者均符合Ｂｏｈａｎ和Ｐｅｔｅｒ于1975年制定的诊断标准 ,年龄 17～ 5 3岁 ,平均年龄 ( 33.4±11.1)岁 ,女性 9例 ,男性 5例。其中 1例合并类风湿性关节炎(ＲＡ) ,2例合并系统性红斑狼疮 (ＳＬＥ)。首发症状到诊断日期为 1～ 42个月 ,平均 ( 2 .9± 2 .9)个月。2 .临床表现 :( 1)治疗前临床表现 :抬头困难 6例 ;上肢近端肌 (前臂屈肌 )力Ⅴ°4例、Ⅳ…     

外源性变应性肺泡炎的临床病理特征和影像学表现

目的探讨外源性变应性肺泡炎(EAA)的临床病理特征和影像学表现。方法分析5例外源性变应性肺泡炎病例的临床特点、影像学表现、肺活检的病理特征。结果 EAA常见的临床表现为咳嗽、呼吸困难、咳痰、发热;主要阳性体征为轻度紫绀、肺部听诊湿啰音或Velcro啰音;肺功能检查显示限制性通气功能障碍和弥散功能障碍。HRCT表现为磨玻璃影、小叶间隔增厚、小叶中心性结节、网格影和蜂窝肺等。支气管肺泡灌洗液显示淋巴细胞增多。肺活检组织病理学示淋巴细胞性间质性肺炎,细支气管周围可见小的不典型肉芽肿和多核巨细胞。患者对糖皮质激素治疗有效。结论临床表现结合影像学特点可提示EAA临床诊断,肺活检是诊断EAA有效的检查方法。     

以扩张型心肌病为临床特征的多发性肌炎一例

患者 ,男 ,16岁 ,因活动后气短、乏力 4年 ,加重并发热 1周入院。既往有发作性肌肉疼痛 ,蹲下、起立困难史。多次以“扩张型心肌病”在外院治疗 ,效果不佳 ,外院心肌酶谱简摘如表 1。 1周前气短、乏力明显加重 ,疑为“多发性肌炎”收住院。查体 :体温 38 8℃ ,呼吸 2 2次 /ｍｉｎ ,脉搏 110次 /ｍｉｎ ,血压 95 / 75ｍｍＨｇ (1ｍｍＨｇ =0 133ｋＰａ) ,平卧位 ,双肺底闻及湿音。心界向两侧扩大 ,心率 110次 /ｍｉｎ ,Ｓ1低钝 ,心尖部闻及舒张期奔马律及收缩期Ⅱ / 6级吹风样杂音。四肢肌肉不同程度萎缩 ,局部有压痛 ,双下肢略浮肿。实验…     

包涵体肌炎累及颈部肌肉一例

患者：男，６２岁。因双下肢无力进行性加重半年，抬头无力１月，于１９９８年５月１６日收住入院。患者半年前无明显诱因渐感双下肢无力，行走、下蹲后站立及上下楼梯均感困难。近一月来抬头无力，头常低垂，食欲明显下降，消瘦。无肌痛及吞咽困难。曾在外院查肌酶谱正常...     

ISCHEMIC HEPATITIS: CLINICAL FEATURES, DIAGNOSIS AND PROGNOSIS

Nineteen episodes of ischemic hepatitis were diagnosed by hepatitic liver function tests and characteristic liver pathology in 17 patients. All patients had an acute illness associated with a likely fall in cardiac output although only five episodes were associated with documented hypotension. Right ventricular failure was severe in only four, mild in six, and absent in nine whilst left ventricular failure was clinically apparent in 16. The hepatitic illness was usually mild. No patient died as a direct result of hepatic damage, prognosis depending on the underlying cardiac or systemic disease. Liver function tests were characterised by a marked rise in serum transaminase levels with a parallel increase in serum lactic dehydrogenase of hepatic origin and a short time course of the enzyme elevation lasting 3 to 11 days. It is concluded that ischemic hepatitis (a) is' caused by poor hepatic perfusion associated with an acute fall in cardiac output; (b) is usually a subclinical illness with little influence on prognosis, and (c) may be accurately differentiated from viral hepatitis on clinical and biochemical criteria alone.     

CLINICAL AND ENDOCRINE FEATURES OF HYPERPROLACTINAEMIC AMENORRHOEA

The clinical, radiological and endocrine findings in thirty-five women with hyperprolactinaemia and amenorrhoea are described. Twelve patients had radiological evidence of a pituitary tumour and six were tested after pituitary ablation. Seventeen patients with hyperprolactinaemia and normal pituitary X-rays were also studied. None was on any drug known to increase prolactin secretion and all patients were euthyroid when tested. Basal serum prolactin concentrations were high in the group with untreated pituitary tumours and in those with normal X-rays. The levels were variable in the post-ablation cases. The increase of prolactin after TRH was subnormal in all of the groups. Serum oestradiol concentrations were low in most patients and nineteen of twenty-one patients tested had no withdrawal bleeding after treatment with a progestogen. Mean serum gonadotrophin concentrations (basal and after LHRH) were normal in twenty-nine patients but subnormal in four post-ablative cases. Anovulatory responses to clomiphene were obtained in nineteen of twenty patients tested. Fifteen patients were treated with bromocriptine; twelve ovulated and eight became pregnant; two not responding had impaired LH and FSH production. Hyperprolactinaemic amenorrhoea is a common disorder with characteristic endocrine features. Galactorrhoea is unusual (30%). Treatment with bromocriptine lowers prolactin concentrations and rapidly repairs the reproductive defect.     

WEGENER'S GRANULOMATOSIS: CLINICAL FEATURES AND OUTCOME IN SEVENTEEN PATIENTS

Seventeen patients with Wegener's granulomatosis are reviewed. Eleven males and six females, with a mean age of 46.9± 4.5 years, were followed for 35.7 ± 9.0 months. Mean duration from time of onset of symptoms to diagnosis was 8.5 ±3.1 months. Constitutional symptoms (100%), lower respiratory tract involvement (93%), renal involvement (87%), and upper respiratory tract involvement (80%) were the most frequent clinical manifestations. Arthritis (60%), dermal vasculitis (60%), and inflammatory ocular disease (40%) were also common. Elevated ESR (94%), anemia (70%), and lymphopenia (77%) were frequent laboratory findings prior to treatment. Five patients had renal failure at presentation and two patients progressed from no renal involvement at presentation to renal failure at diagnosis, while five patients progressed from renal involvement without impairment at diagnosis to end-stage renal failure. Seven patients died; six of these deaths were related to active Wegener's granulomatosis. The patients with a severe systemic vasculitis, and renal involvement had a poor outcome while predominant respiratory disease had a good prognosis.     

17例庚型肝炎临床病理分析

对17例经逆转录聚合酶链反应(RT-PCR)法检测HGV RNA阳性的庚型肝炎患者进行临床和/或肝脏组织分析。结果发现经输血感染者8例(47.1％),散发性病例9例(52.9％)。单纯HGV感染者8例(47.1％),HGV和HBV或HCV二重、三重感染者9例(52.9％)。临床病理分型发现急性肝炎2例,慢性肝炎12例,重型肝炎2例和肝硬变1例。单纯HGV感染多呈隐匿发病,症状轻,,无黄疸,ALT轻度升高,但慢性化程度高。本文结果提示HGV感染易致病程慢性化,病理损伤轻,HGV与HBV重叠感染时,HGV有可能干扰HBV的复制。     

LEPROSY NEPHROPATHY: A REVIEW OF CLINICAL AND HISTOPATHOLOGICAL FEATURES

Leprosy is a chronic disease caused by Mycobacterium leprae, highly incapacitating, and with systemic involvement in some cases. Renal involvement has been reported in all forms of the disease, and it is more frequent in multibacillary forms. The clinical presentation is variable and is determined by the host immunologic system reaction to the bacilli. During the course of the disease there are the so called reactional states, in which the immune system reacts against the bacilli, exacerbating the clinical manifestations. Different renal lesions have been described in leprosy, including acute and chronic glomerulonephritis, interstitial nephritis, secondary amyloidosis and pyelonephritis. The exact mechanism that leads to glomerulonephritis in leprosy is not completely understood. Leprosy treatment includes rifampicin, dapsone and clofazimine. Prednisone and non-steroidal anti-inflammatory drugs may be used to control acute immunological episodes.     

ACUTE SARCOID ARTHRITIS: OCCURRENCE, SEASONAL ONSET, CLINICAL FEATURES AND OUTCOME

In a 2-yr prospective follow-up study of patients presentingclinically with possible reactive arthritis (ReA), 17 (9%) ofthe patients turned out to have acute sarcoid arthritis (SA).The number of new cases of SA per year was 2.9/100000 personsin the city of Oslo between 18 and 60 yr of age. The onset ofSA clustered in the spring. All the SA patients presented withbilateral anlde joint involvement and bilateral hilar lymphadenopathy,and ten (59%) presented with the triad of erythema nodosum,arthritis and lung involvement. A prospective follow-up after104 weeks showed complete remission of arthritis in all 17 casesof SA. The total duration of arthritis [median (range)] was11 (2–107) weeks. Erythema nodosum was mild and transientin all cases. At week 104, the lung and hilar manifestationshad resolved. We conclude that the outcome of SA appeared favourable.Bilateral ankle joint involvement, erythema nodosum and bilateralhilar lymphadenopathy found at the routine chest X-ray examinationare important clues for the diagnosis of SA. KEY WORDS: Sarcoidosis, Arthritis, Erythema nodosum, Hilar lymphadenopathy, Incidence, Seasonal onset, Outcome     

老年和青年急性心肌梗死临床特点和心理分析

目的分析103例青年和老年急性心肌梗死(AMI)患者的临床特点和心理状态.方法将103例AMI患者按年龄分为老年组和青年组,并对二组的临床特点和心理状态进行分析.结果①青年组AMI均为男性,吸烟占95.5%,饮酒占90.9%,均明显高于老年组(P＜0.01).老年组AMI有高血压病占48.1%,高脂血症占60.5%,糖尿病占34.6%,均明显高于青年组(P＜0.05).②青年组AMI患者以焦虑为主,占59.1%,与老年组比较差异有统计学意义,老年组患者以抑郁为主,占45.7%,二组比较差异有统计学意义(P＜0.05).③青年组有典型心绞痛19例,占86.3%,与老年组比较差异有统计学意义.④二组比较,老年组病死率高于青年组(P＜0.05).结论男性、大量吸烟、大量饮酒是青年AMI的主要危险因素;而高血压病、糖尿病等并发症是老年AMI的主要危险因素.青年AMI以焦虑为主,症状典型;而老年AMI以抑郁为主,症状不典型,并发症多,病死率高.     

CLINICAL FEATURES OF MITOCHONDRIAL MYOPATHY

Five patients with mitochondrial myopathy are discussed. Two presented with progressive external ophthalmoplegia (CPEO), one with CPEO and retinitis pigmentosa, and two with Kearns-Sayre syndrome. Ragged red fibres and intra-mitochondrial para-crystalline inclusions were found in each case. The clinical heterogeneity of the mitochondrial myopathy syndrome in the presence of identical pathological changes in skeletal muscle is emphasised.