先天性肌性斜頸病變組織中細胞外間質(zhì)的變化特點
發(fā)布時間：2018-08-21 08:11
【摘要】：目的：觀察先天性肌性斜頸(Congenital Muscular Torticollis, CMT)病變組織中脂肪增生現(xiàn)象及分布特點,分析脂肪增生程度與纖維化和年齡的關系。 方法：對手術治療,切除的病理組織,常規(guī)石蠟包埋,并取部分新鮮標本行液氮冰凍處理保存。石蠟標本行常規(guī)HE染色,觀察先天性肌性斜頸病變胸鎖乳突肌基本病理改變和脂肪增生現(xiàn)象。Masson染色,觀察胞外基質(zhì)的纖維化情況。冰凍標本行蘇丹Ⅲ脂肪染色,定性病變肌組織中增生的脂肪組織。 結(jié)果：188例CMT患兒中,男123例,女65例,男女比約2：1；左側(cè)75例,右側(cè)113例,左右比約2：3；冰凍標本31例；年齡最小2月24天,最大16歲,平均年齡2.9歲。所有標本均符合CMT診斷標準及排除其它疾病。①HE染色下CMT病變胸鎖乳突肌肌纖維大小不等,呈萎縮、肥大、壞死等多種改變,部分肌細胞有空泡變性和核中心化表現(xiàn),間質(zhì)增生纖維化明顯。②Masson染色顯示肌內(nèi)膜、肌束膜輕重不一的纖維化增生,包繞肌纖維。隨著年齡的增長,纖維化程度呈現(xiàn)兩頭高中間低的特點,0-3歲纖維化與年齡負相關(r=-0.168,P=0.049),3歲后纖維化與年齡正相關(r=0.281,P=0.001)。③蘇丹Ⅲ脂肪染色可見橙紅色陽性分布區(qū)域,與HE染色下脂肪分布相對應。④存在脂肪增生的標本占總標本數(shù)的69.68%(131/188),增生程度輕重不一,標本中可見少量的脂肪細胞,也可見大量脂肪增生；增生的脂肪組織多呈團塊狀或條索狀；大多為纖維化膠原所包繞,也可見穿插于肌纖維間。脂肪增生隨纖維化加重和年齡增大呈增多趨勢,且三者間相互具有正相關關系(P0.05)。 結(jié)論：CMT的基本病變是病變的胸鎖乳突肌纖維化,纖維化程度隨年齡增長呈現(xiàn)兩頭高中間低的特點。本研究在常規(guī)觀察基本病變組織中,發(fā)現(xiàn)了脂肪增生現(xiàn)象。脂肪增生參與CMT的基本病理變化過程,隨病變胸鎖乳突肌纖維化進程而加重,并可能對肌纖維的病理改變與組織纖維化產(chǎn)生影響。 目的：觀察先天性肌性斜頸(congenital muscular torticollis, CMT)病變組織中肌纖維基底膜的變化特點,分析基底膜改變程度與對應肌纖維形態(tài)改變和間質(zhì)纖維化程度的關系,推測基底膜的改變與先天性肌性斜頸病情發(fā)展與改變的內(nèi)在聯(lián)系。 方法：通過手術治療,切除40例先天性肌性斜頸患兒病變胸鎖乳突肌(sternocleidomastoid muscle, SCM)組織及5例發(fā)育性髖關節(jié)脫位(developmental dysplasia of the hip, DDH)患兒的內(nèi)收肌組織,常規(guī)石蠟包埋,切片后行HE染色、Masson染色和針對Ⅳ型膠原和層粘連蛋白的免疫組織化學染色,以發(fā)育性髖關節(jié)脫位患兒手術切除的內(nèi)收肌作正常對照。觀察Ⅳ型膠原和層粘連蛋白的表達分布特點,并根據(jù)Ⅳ型膠原和(或)層粘連蛋白的免疫組織化學染色所顯示的肌纖維基底膜形態(tài),分析同一標本不同病變區(qū)域的基底膜改變與對應肌纖維形態(tài)變化的關系,分析不周標本中基底膜改變與間質(zhì)纖維化程度的關系。 結(jié)果：HE染色下,CMT病變組織鏡下觀察可見明顯的間質(zhì)增生纖維化,殘存肌纖維被增生的纖維化組織包繞并分離,肌纖維退行性變明顯；但同一標本的不同病變區(qū)域纖維化程度與肌纖維殘存數(shù)量不一,部分標本的病變組織可分為纖維化區(qū)、肌細胞纖維混合區(qū)和肌細胞密集區(qū)。Masson染色顯示,對照組DDH內(nèi)收肌,平均纖維化為8.74±1.54(%),先天性肌性斜頸病變組織中纖維化程度明顯,為55.06±17.82(%),經(jīng)兩獨立樣本T檢驗分析,t=15.97,P0.01。在CMT中,藍色的纖維化區(qū)域呈彌漫性增生,肌內(nèi)膜、肌束膜均有輕重不一的纖維化,包繞殘存肌纖維。免疫組織化學染色結(jié)果顯示Ⅳ型膠原和層粘連蛋白二者的組織內(nèi)表達部位基本一致,即表達在肌纖維基底膜和血管壁上,而在CMT病變組織間質(zhì)表達不明顯。對照組中肌纖維形態(tài)規(guī)則,大小均一,基底膜邊緣光滑,薄且均勻,并緊貼與肌纖維胞膜上,不能區(qū)分。同一CMT標本不同病變區(qū)域肌纖維及其基底膜變化特點是,①纖維化區(qū),肌纖維零星,Ⅳ型膠原和層粘連蛋白的陽性表達部位僅存在于視野中的血管壁上,而其他區(qū)域的染色結(jié)果為陰性；僅有零星肌纖維的基底膜上,未見表達,完全缺失。②肌細胞纖維混合區(qū),殘存肌纖維被增生的纖維化組織分割,呈孤島狀態(tài),肌細胞形態(tài)不規(guī)整,常見肥大、萎縮的表現(xiàn),部分肌纖維有明顯的核中心化、空泡變性等退行性變,并且這種變化在基底膜缺損嚴重的肌細胞表現(xiàn)更為突出。相應的肌纖維基底膜在部分肌纖維周圍有極少量的陽性表達,缺損明顯,且不連續(xù)：一些部位基底膜顯像彌漫性減弱,表達不連續(xù),有局部缺失。③肌細胞密集區(qū),肌纖維數(shù)量較豐富,基底膜表達較好,連續(xù)但不平整,不均一。不同CMT標本間肌纖維基底膜缺損程度與間質(zhì)纖維化程度對比,經(jīng)單因素方差分析結(jié)果提示基底膜缺損越重的標本中,間質(zhì)纖維化程度越高(P0.05)。 結(jié)論：間質(zhì)增生纖維化是先天性肌性斜頸的基本病理改變,在纖維化的基礎上病變組織中肌纖維基底膜表現(xiàn)出輕重不一的缺損特點。同一標本的不同病變區(qū)域基底膜及其對應肌纖維的形態(tài)改變存在差異,肌細胞密集區(qū),Ⅳ型膠原和層粘連蛋白染色出較為正常的基底膜。基底膜染色缺損程度與間質(zhì)纖維化程度相關,即纖維化越重,缺損越明顯。推測肌纖維基底膜的變化特點,反應了生后CMT-SCM的病理演變過程。在肌細胞密集區(qū)較為正常的表達,支持肌細胞較為正常成�。辉诨旌蠀^(qū)和纖維化區(qū)其完整性破壞的表現(xiàn),可能是CMT-SCM內(nèi)肌纖維的胞體不穩(wěn)定性的表現(xiàn),肌纖維退行性變不斷加重,有效肌纖維數(shù)量減少,纖維化加重。并且缺失的基底膜對SCM內(nèi)肌衛(wèi)星細胞和(或)成肌細胞的保護作用降低,致使生后SCM的肌形成和再生能力降低,SCM新生肌纖維得不到補充,間質(zhì)纖維程度繼續(xù)加重,可導致CMT病情繼續(xù)發(fā)展；反之,如果基底膜完整性相對正常,是肌細胞相對正常的表現(xiàn),可能是CMT-SCM內(nèi)肌纖維生命力維持的原因之一,也可能為CMT的臨床自愈的表現(xiàn)。
[Abstract]:Objective: To observe the fatty hyperplasia and its distribution in congenital muscular torticollis (CMT) and analyze the relationship between the degree of fatty hyperplasia and fibrosis and age.
Methods: Surgical treatment, excision of pathological tissue, routine paraffin embedding, and some fresh specimens were frozen and preserved in liquid nitrogen. Paraffin specimens were stained with routine HE to observe the basic pathological changes and fat hyperplasia of sternocleidomastoid muscle in congenital muscular torticollis. Masson staining was used to observe the fibrosis of extracellular matrix. Sultan fatty acid staining was used to identify adipose tissue in pathological muscle tissue.
Results: Among 188 children with CMT, 123 were males and 65 were females, the ratio of male to female was about 2:1; 75 were left, 113 were right, the ratio of left to right was about 2:3; 31 were frozen specimens; the minimum age was 24 days, the maximum age was 16 years, and the average age was 2.9 years. (2) Masson staining showed the fibrosis and hyperplasia of endomyocardium and myofibrillar membrane with different degrees, wrapping the myofibrils. With the increase of age, the degree of fibrosis showed the characteristics of both ends, high school and low, fibrosis and fibrosis in 0-3 years old. Age was negatively correlated (r = - 0.168, P = 0.049), and fibrosis was positively correlated with age after 3 years old (r = 0.281, P = 0.001). 3 Sudan III fat staining showed an orange-red positive distribution, which corresponded to the distribution of fat under HE staining. Cells, but also a large number of adipose hyperplasia; hyperplastic adipose tissue is mostly lumpy or banded; most of them are wrapped by fibrotic collagen, but also can be seen interpenetrating between muscle fibers.
Conclusion: The basic pathological changes of CMT are fibrosis of the sternocleidomastoid muscle, and the degree of fibrosis is lower in both ends of the sternocleidomastoid muscle with age. It may also affect the pathological changes of muscle fibers and tissue fibrosis.
Objective: To observe the changes of myofibrillar basement membrane (MBM) in congenital muscular torticollis (CMT) lesions and to analyze the relationship between the changes of MBM and the morphological changes of corresponding myofibrils and the degree of interstitial fibrosis.
Methods: Forty children with congenital muscular torticollis were treated with surgical excision of sternocleidomastoid muscle (SCM) and five children with developmental dysplasia of the hip (DDH). The adductor muscles were embedded in paraffin and stained with HE, Masson and for type IV collagen and DDH. Immunohistochemical staining of laminin was performed in adductor muscles of children with developmental dislocation of the hip. The expression and distribution of type IV collagen and laminin were observed. The same marker was analyzed according to the morphology of basement membrane of myofibrils revealed by immunohistochemical staining of type IV collagen and/or laminin. The relationship between the changes of basement membrane and the morphological changes of myofibrils in different lesion areas was analyzed.
Results: Under HE staining, there were obvious interstitial hyperplasia and fibrosis in CMT lesions, and the residual muscle fibers were surrounded and separated by hyperplastic fibrosis tissues, and the degeneration of muscle fibers was obvious. Masson staining showed that the average fibrosis of adductor muscles in the control group was 8.74 (%) and that of congenital torticollis was 55.06 (%). Immunohistochemical staining showed that type IV collagen and laminin were expressed in the basement membrane and vascular wall of myofibrils, but not in the interstitial tissue of CMT lesions. The changes of myofibrils and their basement membranes in different lesions of the same CMT specimen were characterized by: (1) the positive expression of myofibrils, sporadic myofibrils, type IV collagen and laminin only existed in the vascular wall of the visual field, but not in the same CMT specimen. The staining results of other areas were negative; only sporadic myofibrils were found on the basement membrane, but no expression was found and completely absent. The corresponding basement membrane of myofibrils had a few positive expression around some myofibrils, and the defect was obvious and discontinuous. The basement membrane imaging of some parts of the myofibrils was diffuse weakening, expression was discontinuous, and there were local deletions. The number of fibers was abundant, the expression of basement membrane was good, continuous but uneven, and uneven. The degree of fibrosis and defect of myofibrillar basement membrane in different CMT specimens were compared. The results of one-way ANOVA analysis showed that the more serious the defect of basement membrane, the higher the degree of interstitial fibrosis (P 0.05).
Conclusion: Interstitial hyperplasia and fibrosis are the basic pathological changes of congenital muscular torticollis. The basement membrane of myofibrillar basement membrane in the pathological tissues of congenital muscular torticollis shows different characteristics on the basis of fibrosis. The degree of defect of basement membrane staining is related to the degree of interstitial fibrosis, that is, the more fibrosis, the more obvious the defect. It is speculated that the changes of basement membrane of muscle fibers reflect the pathological evolution of postnatal CMT-SCM. In the mixed and fibrotic regions, the integrity of the myofibrils may be destroyed by the instability of the cytoplasm, the degeneration of myofibrils, the decrease of the number of effective myofibrils and the aggravation of fibrosis, and the decrease of the protective effect of the missing basement membrane on the satellite cells and/or myoblasts in the SCM, resulting in the decrease of postnatal SC. If the integrity of basement membrane is relatively normal, it is a relatively normal expression of myocytes, which may be one of the reasons for maintaining the viability of myofibers in CMT-SCM, and it may also be the clinical spontaneity of CMT. The expression of healing.
【學位授予單位】：遵義醫(yī)學院
【學位級別】：碩士
【學位授予年份】：2012
【分類號】：R726.8
本文編號：2195094
[Abstract]:Objective: To observe the fatty hyperplasia and its distribution in congenital muscular torticollis (CMT) and analyze the relationship between the degree of fatty hyperplasia and fibrosis and age.
Methods: Surgical treatment, excision of pathological tissue, routine paraffin embedding, and some fresh specimens were frozen and preserved in liquid nitrogen. Paraffin specimens were stained with routine HE to observe the basic pathological changes and fat hyperplasia of sternocleidomastoid muscle in congenital muscular torticollis. Masson staining was used to observe the fibrosis of extracellular matrix. Sultan fatty acid staining was used to identify adipose tissue in pathological muscle tissue.
Results: Among 188 children with CMT, 123 were males and 65 were females, the ratio of male to female was about 2:1; 75 were left, 113 were right, the ratio of left to right was about 2:3; 31 were frozen specimens; the minimum age was 24 days, the maximum age was 16 years, and the average age was 2.9 years. (2) Masson staining showed the fibrosis and hyperplasia of endomyocardium and myofibrillar membrane with different degrees, wrapping the myofibrils. With the increase of age, the degree of fibrosis showed the characteristics of both ends, high school and low, fibrosis and fibrosis in 0-3 years old. Age was negatively correlated (r = - 0.168, P = 0.049), and fibrosis was positively correlated with age after 3 years old (r = 0.281, P = 0.001). 3 Sudan III fat staining showed an orange-red positive distribution, which corresponded to the distribution of fat under HE staining. Cells, but also a large number of adipose hyperplasia; hyperplastic adipose tissue is mostly lumpy or banded; most of them are wrapped by fibrotic collagen, but also can be seen interpenetrating between muscle fibers.
Conclusion: The basic pathological changes of CMT are fibrosis of the sternocleidomastoid muscle, and the degree of fibrosis is lower in both ends of the sternocleidomastoid muscle with age. It may also affect the pathological changes of muscle fibers and tissue fibrosis.
Objective: To observe the changes of myofibrillar basement membrane (MBM) in congenital muscular torticollis (CMT) lesions and to analyze the relationship between the changes of MBM and the morphological changes of corresponding myofibrils and the degree of interstitial fibrosis.
Methods: Forty children with congenital muscular torticollis were treated with surgical excision of sternocleidomastoid muscle (SCM) and five children with developmental dysplasia of the hip (DDH). The adductor muscles were embedded in paraffin and stained with HE, Masson and for type IV collagen and DDH. Immunohistochemical staining of laminin was performed in adductor muscles of children with developmental dislocation of the hip. The expression and distribution of type IV collagen and laminin were observed. The same marker was analyzed according to the morphology of basement membrane of myofibrils revealed by immunohistochemical staining of type IV collagen and/or laminin. The relationship between the changes of basement membrane and the morphological changes of myofibrils in different lesion areas was analyzed.
Results: Under HE staining, there were obvious interstitial hyperplasia and fibrosis in CMT lesions, and the residual muscle fibers were surrounded and separated by hyperplastic fibrosis tissues, and the degeneration of muscle fibers was obvious. Masson staining showed that the average fibrosis of adductor muscles in the control group was 8.74 (%) and that of congenital torticollis was 55.06 (%). Immunohistochemical staining showed that type IV collagen and laminin were expressed in the basement membrane and vascular wall of myofibrils, but not in the interstitial tissue of CMT lesions. The changes of myofibrils and their basement membranes in different lesions of the same CMT specimen were characterized by: (1) the positive expression of myofibrils, sporadic myofibrils, type IV collagen and laminin only existed in the vascular wall of the visual field, but not in the same CMT specimen. The staining results of other areas were negative; only sporadic myofibrils were found on the basement membrane, but no expression was found and completely absent. The corresponding basement membrane of myofibrils had a few positive expression around some myofibrils, and the defect was obvious and discontinuous. The basement membrane imaging of some parts of the myofibrils was diffuse weakening, expression was discontinuous, and there were local deletions. The number of fibers was abundant, the expression of basement membrane was good, continuous but uneven, and uneven. The degree of fibrosis and defect of myofibrillar basement membrane in different CMT specimens were compared. The results of one-way ANOVA analysis showed that the more serious the defect of basement membrane, the higher the degree of interstitial fibrosis (P 0.05).
Conclusion: Interstitial hyperplasia and fibrosis are the basic pathological changes of congenital muscular torticollis. The basement membrane of myofibrillar basement membrane in the pathological tissues of congenital muscular torticollis shows different characteristics on the basis of fibrosis. The degree of defect of basement membrane staining is related to the degree of interstitial fibrosis, that is, the more fibrosis, the more obvious the defect. It is speculated that the changes of basement membrane of muscle fibers reflect the pathological evolution of postnatal CMT-SCM. In the mixed and fibrotic regions, the integrity of the myofibrils may be destroyed by the instability of the cytoplasm, the degeneration of myofibrils, the decrease of the number of effective myofibrils and the aggravation of fibrosis, and the decrease of the protective effect of the missing basement membrane on the satellite cells and/or myoblasts in the SCM, resulting in the decrease of postnatal SC. If the integrity of basement membrane is relatively normal, it is a relatively normal expression of myocytes, which may be one of the reasons for maintaining the viability of myofibers in CMT-SCM, and it may also be the clinical spontaneity of CMT. The expression of healing.
【學位授予單位】：遵義醫(yī)學院
【學位級別】：碩士
【學位授予年份】：2012
【分類號】：R726.8
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