面肩肱型肌营养不良的肌肉病理特点及形态计量分析  

Muscular pathological features and morphological analysis of facioscapulohumeral muscular dystrphy

作　　者：张艳丽[1] 吴雪梅 杨立明[1] 于雪凡[3] Zhang Yanli;Wu Xuemei;Yang Liming;Yu Xuefan(Department of NICU、General Hospitail of Tisco,the Sixth Hospital of Shanxi Medical University,Taiyuan 030003,China;Department of Neurology,General Hospitail of Tisco,the Sixth Hospital of Shanxi Medical University,Taiyuan 030003,China;Department of Neurology,the First Hospital of Jilin University,Changchun 130012,China)

机构地区：[1]太原钢铁(集团)有限公司总医院(山西医科大学第六医院)NICU,太原030003 [2]太原钢铁(集团)有限公司总医院(山西医科大学第六医院)神经内科,太原030003 [3]吉林大学第一医院神经内科,长春130012

出　　处：《中国医师杂志》2020年第10期1524-1528,共5页Journal of Chinese Physician

摘　　要：目的分析面肩肱型肌营养不良(FSHD)患者肌肉活检病理学特点,对出现炎细胞浸润者进行形态计量分析并与多发性肌炎(PM)患者进行比较,以鉴别诊断。方法回顾性分析2006年1月至2019年1月期间山西医科大学第六医院及吉林大学第一医院门诊就诊及住院的30例FSHD患者的临床及肌肉病理学资料,按有无炎细胞浸润分为非炎性浸润组(16例)和炎性浸润组(14例);应用Image-Pro Plus生物分析软件对FSHD患者炎性浸润组与多发性肌炎患者肌肉病理进行形态计量分析比较。结果 30例FSHD中14例可见肌膜内及肌间质炎细胞浸润,尤其以血管周围明显,免疫组织化学染色证实浸润的炎细胞主要是CD4+T淋巴细胞。形态计量分析显示:炎细胞浸润FSHD组肌纤维表面积、密度、直径、炎细胞浸润、再生和坏死肌纤维与PM组比较差异无统计学意义(P>0.05)。炎细胞浸润FSHD组肌间质总面积明显大于PM患者,差异有统计学意义(P=0.03)。结论肌肉病理形态计量分析示FSHD患者炎细胞浸润组肌间质结缔组织增生明显多于PM组,从病理上鉴别二者,可为临床提供帮助。Objective To analyze the pathological features of facioscapulohumeral muscular dystrophy(FSHD).For better characterization of inflammatory response in FSHD,we performed histochemical moiphological analysis for FSHD and polymyositis(PM)muscle biopsies.In order to provide a reference for guide targeted therapeutic interventions.Methods The clinical and mvopathological data of 30 patients with FSHD from January 2006 to January 2019 in the Sixth Hospital of Shanxi Medical University and the first hospital of Jilin University were retrospectively analyzed.The patients were divided into non-inflammatory infiltration group(16 cases)and inflammatory infiltration group(14 cases)according to the presence or absence of inflammatory cell infiltration.For better characterization of inflammatoiy response in FSHD,we performed histochemical morphological analysis for two groups of muscles:FSHD and PM muscle biopsies,using Image-Pro plus bioanalytical software.Results In 30 cases of FSHD,14 cases showed intramuscular and interstitial inflammatory cell infiltration,especially around the blood vessels.Immunohistochemical staining confirmed that the infiltration of inflammatory cells was mainly CD4^+T lymphocytes.Morphometric analysis showed that there were no significant differences in muscle fiber surface area,density,diameter,inflammatory cell infiltration,regeneration and necrosis between FSHD patients and PM patients(P>0.05).The total area of myointerstitium in FSHD group was significantly larger than that in PM group(P=0.03).Conclusions The pathological morphometric ciinilysis showed that the proliferation of interstitial connective tissue in FSHDinflammatory cell infi It ration group was significantly more than that in PM group.Clinicians can identify the two from pathology and provide help fur clinical practice.

关 键 词：肌营养不良 面肩肱型 多发性肌炎 肌肉病理 形态计量 

分 类 号：R746.2[医药卫生—神经病学与精神病学]