进行性骨化性肌炎51例中国文献报道的综合分析

目的：进行性骨化性肌炎（myositis ossificans progressiva,MOP)是一种先天性遗传性疾病，临床较罕见，国内多为个案报道，临床资料不系统，章综合分析了国内51例MOP病例，总结MOP在我国发病的基本情况。方法：光盘检索出自1979－2001年共31篇有关MOP献报道，共51例，综合分析了51例MOP的临床表现、诊断、治疗及预后。结果：我国MOP男性较女性患病率稍高，发病时间主要集中在0－5岁，从发病到明确诊断的间隔为7．88年，33例报道有手指、脚趾异常，首发症状多为颈后软组织包块，脊柱最容易受累。在12岁内，可导致身体大关节僵直，造成患生活能力丧失。我国有关MOP临床治疗资料较少，且缺乏长期随访资料。结论：MOP在我国发病多在0－5岁期间。脊柱是最常被侵犯的部位。     

成人进行性骨化性肌炎1例报告

1病历摘要男,53岁。主因右下肢外伤、疼痛2个月加重3d于2003年7月8日入院。既往史:于30年前着凉后双下肢麻木伴活动受限而就诊,诊断为脊神经炎,经对症治疗,治愈出院。4个月后被人撞击右大腿,出现疼痛,无明显出血及肿胀,能自行行走,未就医。但此后常感右腿活动不灵活,间或烧灼样     

我国进行性骨化性肌炎104例文献分析

目的探讨国内进行性骨化性肌炎的发病情况及临床特点,为临床诊疗提供资料。方法检出1980~2013年进行性骨化性肌炎病例报道70篇（共104例）,对进行性骨化性肌炎的年龄及性别分布、先天指趾畸形、首发症状、最易受累部位、诊断治疗方法等进行统计并分析。结果我国进行性骨化性肌炎男性患病率高于女性,比例为1.36:1。发病年龄为0~38岁,0~4岁年龄段发病率最高。进行性骨化性肌炎呈全身进行性发展,第一指趾畸形的有60例,首发症状多为头颈部肿块,颈部、脊柱最易受累,肿块多由颈部开始逐渐发展至全身各大关节,造成患者的活动能力受限甚至丧失,其诊断主要依靠临床表现与影像学,但疗效确切的治疗方法未见文献报道。结论进行性骨化性肌炎尚无特殊的有效疗法,需提高对该病的早期诊断,探索系统诊疗方案,延缓疾病的进展,提高患者生活质量。     

进行性骨化性肌炎并发骨软骨瘤1例

患儿 ,男 ,1 3岁 ,以扪及双下肢包块进行性增大 5月 ,伴双膝关节活动受限 2月入院。既往史 :无明显外伤史 ;9个月前 ,右膝关节内上方扪及一鸡蛋大小包块 ,质硬 ,Ｘ线照片示右股骨下内份干骺端与长骨垂直的骨性凸起 ,基底窄 ,形成带蒂状的外观 ,经手术切除后 ,病检为 :骨软骨瘤。查体 :一般情况良好 ,全身浅表淋巴结未扪及肿大 ,右膝关节内上方有一长约 4 5ｃｍ手术疤痕 ,在此区及右髂骨外上方均扪及一包块 ,质硬 ,边界清 ,不活动。左股骨中下段外侧及左髋关节后上方各扪及一包块 ,质硬 ,边界清 ,不活动。Ｘ线照片 :沿右侧腰大肌方向有一大小…     

神经源性骨化性肌炎1例影像学表现并文献复习

神经源性骨化性肌炎（neurogenic myositis ossification,NMO）是指因中枢神经系统遭受严重损伤,而在四肢关节周围软组织内出现的异位骨化（helerotopic ossification）。根据形成原因,异位骨化可分为3类：创伤性异位骨化,神经源性异位骨化,原发性异位骨化,NMO是异位骨化的一种类型。异位骨化可发生于身体任何部位,通常在重要关节,如髋、膝关节等关节,尤其是髋关节。     

骨化性肌炎1例

病历摘要 男，21岁。左大腿隐痛不适10a，不能下蹲7a。患者于10a前于同伴玩耍时，被踢中左大腿，至今一直感隐痛不适，但仍能正常行走，当时曾在当地医院拍片检查未见骨质异常，按软组织损伤对症处理，征象未见好转，3a后患者出现左腿不能下蹲，曾服中药（药名不详）治疗，效果不佳，近来上述征象明显加重。于2006—10来我院治疗。查体：脊柱生理弯曲存在，无压痛，四肢生理反射存在，病理反射未引出。髋骨上15cm处测量双大腿周径，右39cm，左35cm。左大腿胭窝上10cm处半腱肌深部可触及一肿物，范围10cm×8cm，界不清，明显触痛，质中活动，局部皮肤外观无异常。CT、X线拍片检查左股骨未见异常。MRI示左大腿下段后侧肌肉深部软组织肿瘤，密度不均，右血凝块存在。手术切除肌肉肿物送病理。     

骨化性肌炎1例

患者 ,男性 ,41岁 ,教师 ,主因左前臂肿胀、疼痛 3年 ,加重伴四肢近端萎缩 3个月 ,于 2 0 0 0年 8月 2 9日入院。 3年前患者无诱因的自觉左腕部变粗 ,戴手表时较紧 ,不伴疼痛 ,未介意 ,后逐渐向前臂发展。 1年半前 ,因患部明显肿胀 ,且每遇感冒肿胀明显加重 ,赴上海华山医院就诊 ,查 :风湿、自身抗体等均正常。经查肌电图示 :肌源性改变。肌肉活检示 :骨化性骨炎。服用强的松 5ｍｇ/ｄ ,肿胀可缓解 ,但后来需递增剂量 ,最大至 60ｍｇ/ｄ。近 3个月来 ,上述剂量不能有效控制病情发展。另外 ,四肢变细 ,以双肩胛 ,臂部为甚 ,经转至北京协和、…     

物理治疗创伤性骨化性肌炎三例

物理治疗创伤性骨化性肌炎三例姜亦虹，徐国宏，朱贞国例１男，１０岁。因右肽骨胶上骨折手法复位石膏固定４周后肘关节疼痛、活动障碍，１９９１年１０月来诊。检查：肘关节肿胀明显，肌肉僵硬，有压痛，肘关节屈曲７０～８０”，可动度为１０”，Ｘ线片示骨折部位软组织...     

儿童进行性骨化性肌炎的X线表现

目的：探讨儿童进行性骨化性肌炎（MOP）的临床特点及主要X线表现。方法：对6例临床表现符合MOP的患儿进行病变部位摄片。结果：结合临床及影像学表现均诊断为MOP。结论：MOP又称进行性骨化性纤维发育不良。X线摄片可以显示病变区肌肉软组织内广泛的多种形态的骨化影,部分骨化影可见到骨皮质、骨松质及髓腔。75％的患儿伴有骨骼先天发育异常,多见于拇趾及拇指。尽管超声、CT、MRI对早期MOP的诊断有一定帮助,但传统X线摄片仍为诊断本病最简单有效的方法。     

局限性骨化性肌炎的超声诊断

局限性骨化性肌炎临床并非罕见,但超声诊断此病仅见少数个案报道。近年来我们用超声诊断局限性骨化性肌炎４例,并为手术和病理所证实。现将其特殊声像图表现报告如下。本文４例中３例为２０～２４岁的青年男性,１例为６５岁的老年女性。均有外伤史,并于外伤后３周～７个月出现局部疼痛性肿块并周围关节活动受限而就诊。其中双侧大腿１例,单侧大腿２例,臀部１例。Ｘ线检查２例未见异常,２例示软组织内钙化性团块。仪器采用ＡＴＬＵＭ９ＤＰ型超声诊断仪,探头频率５ＭＨｚ,于肿块处直接扫查。典型病例例１,男,２０岁。右大腿前方中…     

Temporomandibular joint ankylosis—“Knowing when not to operate”: Case report and qualitative systematic review of literature

Temporomandibular joint ankyloses (TMJA) may manifest in patients with several predisposing systemic conditions. A case of extraarticular TMJA is presented in a patient diagnosed with fibrodysplasia ossificans progressive (FOP) is presented. The features, diagnosis, and management of TMJA superimposed on this condition are presented through a qualitative systematic review of literature.     

Longitudinal study of the activities of daily living and quality of life in Japanese patients with fibrodysplasia ossificans progressiva

Purpose: Fibrodysplasia ossificans progressiva is a rare congenital disorder that causes systemic heterotopic ossification, leading to systemic ankyloses and mobility losses. This study aimed to ascertain the natural history of fibrodysplasia ossificans progressiva.

Methods: In addition to the medical history questionnaire, patients aged 16?years and older were asked to complete activities of daily living and quality of life surveys using the Barthel Index, MOS 36-Item Short-Form Health Survey, and Health Assessment Questionnaire. The surveys were conducted over a 4-years period.

Results: Of the 15 participating patients, 13 reported swelling during the study period. The Barthel Index and Health Assessment Questionnaire surveys indicated a tendency for questionnaire items related to arm function to reflect early decreases in the activities of daily living. Decreases in activities of daily living functioning were closely related to decreases in the quality of life in physical function domains. Activities of daily living and quality of life were maintained at a similar level to baseline values over the study period (Barthel Index: p?=?0.42, MOS 36-Item Short-Form Health Survey: p?=?0.43, Health Assessment Questionnaire: p?=?0.87).

Conclusions: We obtained longitudinal information relating to natural history on fibrodysplasia ossificans progressiva patients.

• Implications for rehabilitation

• Fibrodysplasia ossificans progressiva is a rare congenital disease that causes heterotopic ossification of muscle tissue throughout the body, leading to systemic ankyloses and mobility losses.

• When the Barthel Index was high and the activities of daily living were relatively stable, the items on the Health Assessment Questionnaire that are related to arm function began to show impairment.

• Early focus on upper extremity function that includes the use of assistive devices during the period when a patient is still able to perform many activities of daily living is important.

• Although decreases in activities of daily living functioning were closely related to decreases in the quality of life in the physical function domains, the scores of the domains other than physical function were similar to the national standard score.

     

Bilateral and recurrent myositis ossificans in an athlete: a case report and review of treatment options

An unusual case of recurrent myositis ossificans (MO) bilaterally in the hamstring muscles of a 47-year-old athlete secondary to trauma is presented with a review of the literature of current treatment options. MO is a common condition that occurs among athletes in association with muscle and/or tendon strain or contusion. After an extensive literature review, we believe this to be the first case reported of recurrent and bilateral MO in a nonsurgical setting from recurrent hamstring strains. Plain radiographs and physical examination revealed the appearance and chronology of this pathology. Treatment options to improve flexibility and decrease morbidity are discussed along with prophylaxis for future injury. Treatment of mobility and flexibility, rehabilitation goals and treatment with nonsteroidal anti-inflammatory drugs, bisphosphonates, and magnesium are discussed along with prophylaxis.     

异位骨化预防与治疗的新进展

异位骨化是指在机体骨骼系统之外骨形成,可导致严重的运动功能障碍。本文就异位骨化的定义、发病机制、发生率、临床表现和诊断、预防及治疗的新进展予以概述,旨在探讨异位骨化预防与治疗的有效方法。     

胰腺实性假乳头状癌2例及文献复习

目的 分析胰腺实性假乳头状癌的临床病理特点,提高对该病的认识.方法 对2例胰腺实性假乳头状癌的临床资料、大体观察、镜下观察及免疫组化结果进行分析.结果 组织学:肿瘤有实性区、囊性区及假乳头区,并见大片出血、坏死.假乳头区肿瘤细胞围绕血管周围排列,以纤细的纤维血管为轴心形成假乳头结构.肿瘤细胞中等大小,大小较一致,胞浆嗜酸或空泡状,细胞核圆形、卵圆形,可见核沟,有轻度异形性,核分裂相1～2 个/HP,可见核固缩.并有脾或腹膜受累.免疫组化:抗胰蛋白(α-1-AT)、抗糜蛋白(α-1-AACT)、神经元特异丙烯醇(NSE)、波形蛋白(VIM)阳性,胰酶等阴性,Ki-67 20%～25%阳性.结论 胰腺实性假乳头状癌与胰腺实性假乳头状肿瘤一样有特征性的假乳头结构,免疫组化结果也无特殊.但坏死更显著,有轻度核异形性及核分裂相,有周围组织侵犯,有低度恶性.掌握以上要点有助于病理诊断.     

膀胱癌肉瘤报告结合文献分析

目的　复习膀胱癌肉瘤 (CB)的组织学发生、临床和病理学特征。方法　回顾分析 2例CB患者的临床和病理资料并复习有关文献。结果　肉眼血尿为主要症状。 2例患者 (T2 N0 M0 )均行膀胱部分切除 ,术后随访 9个月和 5年。该肿瘤在显微镜下主要由高分化移行细胞癌和肉瘤成分组成 ,后者包括软骨肉瘤、骨肉瘤、横纹肌肉瘤和未分化梭形细胞成分。免疫组化结果 :在所有的肿瘤中 ,癌成分至少对一种CK反应呈阳性 ;小细胞成分对NSE和CGA反应阳性。肉瘤样成分对Vimentin反应阳性 ;软骨肉瘤病灶区对S 10 0呈持续阳性反应。结论　CB是一种高度恶性肿瘤 ,好发于老年男性 ,对分期较晚的病例 ,根治性切除是最佳治疗方案。CB的预后与肿瘤分期密切相关 ,而与组织学表现似乎没有关系。     

Second-trimester sonographic findings in trisomy 22: report of 3 cases and review of the literature.

OBJECTIVE: To describe cases of trisomy 22 detected prenatally on second-trimester sonography and to review the literature on similar cases, with special emphasis on the prenatal findings and pregnancy outcome. METHODS: We performed follow-up second-trimester sonography and fetal karyotyping on 3 pregnant women who were referred because of abnormal findings on initial second-trimester scans. We also conducted a literature search for other reports of sonographic findings in trisomy 22. RESULTS: Fetal abnormalities shown on sonography included nuchal thickening, mild generalized skin edema, an atrioventricular septal defect, an interventricular septal defect, edema of the scalp, face, and neck, severe left pleural effusion with a marked mediastinal shift, ascites, agenesis of the diaphragm, ambiguous genitalia, a single umbilical artery, bradycardia, a multicystic left kidney, and an absent right kidney. All 3 fetuses had karyotypes indicating trisomy 22. One pregnancy was terminated at the parents' request, and 2 ended in fetal death at 23 and 26 weeks. Our literature search revealed only 1 previous report of second-trimester sonographic diagnosis of trisomy 22. We found 3 other reports describing prenatal diagnosis in the third trimester, but only limited information on the sonographic findings was available. CONCLUSIONS: Second-trimester sonography provides valuable clues for the prenatal diagnosis of several chromosomal disorders, including trisomy 22. Prenatal karyotyping is warranted if fetal growth restriction is detected in the second trimester, especially if associated with congenital defects.     

胰腺实性假乳头状肿瘤8例临床病理分析

目的 分析胰腺实性假乳头状肿瘤(SPTP)的临床病理形态特征及免疫组化特点.方法 对8例SPTP患者的临床资料、大体观察、镜下观察及免疫组化结果进行分析.结果 8例SPTP患者中男性3例,女性4例,年龄11～76岁,平均年龄38.5岁.临床表现为不同程度的上腹痛或无症状.肿块位于胰尾4例,胰头和胰体各2例.肿块大小3cm×3 cm×2.7 cm～12 cm×11 cm×10 cm.8例均有包膜,切面见囊实性区混合.组织学:肿瘤有实性区、囊性区及假乳头区.假乳头区肿瘤细胞围绕血管周围排列,以纤细的纤维血管为轴心形成假乳头结构.免疫组化:抗胰蛋白(α-1-AT)、抗糜蛋白(α-1-AACT)、神经元特异性烯醇化酶(NSE)、波形蛋白(VIM)阳性,胰酶等阴性.结论 SPTP好发于年青女性,有特征性的假乳头结构.α-1-AT、α-1-AACT、VIM阳性,胰酶(-)、Insulin阴性.掌握以上要点有助于病理诊断.     

淀粉样变性心肌病268例临床分析

目的 总结淀粉样变性心肌病(cardiac amyloidosis,CA)的临床特点、诊治方法,为临床进一步认识和诊治该病提供依据.方法 检索中国期刊全文数据库(CNKI)、西文生物医学文献数据库、万方数据库和pubmed,收集2000年1月至2009年10月发表的相关病例报告,进行回顾性调查研究.结果 ①经检索共筛选出相关文献44篇计268例;②CA可发生于任何年龄,多见于中老年,男性发病率明显高于女性(2.67∶1);③临床表现最常见的是进行性的心力衰竭(67.1%),其中双下肢水肿(54.3%),气短(44.5%),肝大(39.0%),颈静脉怒张(34.8%);其次为类似心绞痛的症状--胸闷胸痛(25.6%),心血管系统以外最常受累的是肾脏(37.2%);④临床上易被误诊,其中58.9%误诊为肥厚性心肌病,28.8%误诊为限制性心肌病.⑤心肌病理活检为确诊方法,心电图、心脏彩色超声和磁共振是诊断CA主要的辅助检查,其中磁共振有望成为一种新的无创确诊方法.⑥CA患者合并心力衰竭多预后不良.结论 CA临床表现多样化,易误诊为肥厚性心肌病和限制性心肌病,且合并心力衰竭时多预后不良,应及早发现与诊治.