伴Kasabach-Merritt现象卡波西形血管内皮瘤和丛状血管瘤的临床及病理特征

Objective To investigate the clinical and pathological features of Kasabach-Merritt phenomenon(KMP)associated with Kaposiform hemangioendothelioma(KHE)and tufted angioma(TA).Methods Clinical presentations and pathological features of seven patients with KHE or TA complicated by KMP were analyzed retrospectively and a literature review was made.Results Five patients were diagnosed with KHE and two with TA,and all of them exhibited a decrease in platelet count(less than 100×109/L).In all patients,lesions were present or developed on the trunk or limbs at birth or wimin one month after birth.The lesions showed a rapid growth in two patients,slow expansion in two patients,kept stable or regressed gradually in three patients.Lesion appearance varied in KHE and TA,and characteristic manifestation was dark-erythematous or prunosus tumors or patches involving the skin or subcutis.Histopathologically.both KHE and TA were composed of numerous fusiform endothellal cell clumps arranged in a lobular fashion.The nodules of KHE had an indistinct boundary usually with erythrocyte stasis or hemosiderin deposition,and TA was characterized by rounded,well-defined nodules distributed in a crater-like fashion within the dermis.A satisfactory outcome was achieved in two patients treated with oral dehydrocortisone and one patient with surgical resection plus skin grafting;the other four patients remained untreated and no progress was observed during follow-up.Conclusions KMP is pathologically associated with KHE or TA.KHE is an intermediate tumor with local aggressiveness while TA is a benign tumor.Both of them can regress spontaneously and should be difierentiated from other types of vascular tumors.     

利用皮肤成纤维细胞重建兔角膜基质组织的初步研究

Objective To explore whether skin fibroblasts could be used as a cell source for reconstruction of the corneal stroma. Methods It was an experimental study. Skin fibroblast cells were isolated from newborn rabbits, cultured and expanded in vitro. Cells were labeled with green fluorescence protein (GFP) gene by retro-viral infection. Fibroblasts at passage 3 were seeded on polyglicolic acid (PGA) non-woven fibers to form a cell-scaffold construct. Constructs were then implanted into the adult rabbit corneal strema layer after being cultured in vitro for 1 week. Engineered stroma were observed continuously and harvested after 8 weeks of transplantation for gross, histological evaluation and Keratocan examination. PGA alone was used as control. Results The engineered tissue in the cornea became transparent gradually over a period of 8 weeks. Histological analysis showed that engineered stromal lamellar was relatively regular and the orientation of fibers was parallel to the surface of cornea, which is similar to normal cornea. The implanted cells were confirmed by GFP expression under fluorescent microscope, which also express Keratocan. By transmission electron microscopy examination, no significant difference in the diameter of collagen fiber was observed between engineered stroma (33.08 ± 2.47 ) nm and normal stroma ( t = 1.80, P = 0.0771 ). Conclusion Skin fibroblast cells could be used as seed cells for reconstruction of the corneal stroma.     

长脉冲激光治疗下睑静脉性黑眼圈的临床研究

目的 探讨长脉冲激光治疗下睑静脉性黑眼圈的临床疗效和安全性.方法 采用长脉冲1064nm Nd:YAG激光,光斑直径6mm,双脉宽6～10ms,脉冲间隔20ms,能量130～140J/cm2,接触式冷却治疗,治疗间隔1个月;治疗后6个月时随访,并评价病灶的清除率,共分为五级:Ⅰ级(0～24%),Ⅱ级(25%～49%),Ⅲ级(50%～74%),Ⅳ级(75%～99%),Ⅴ级(100%).结果 本组30例患者,治疗1～3次,均达到下睑静脉完全清除.获随访6个月,清除率为100%,未见复发.治疗时,患者仅有中度疼痛(评分均值为3.6分),皮肤红斑均在24h内消退,治疗部位无一例发生紫癜、色素改变和瘢痕形成.结论 长脉冲1064nm Nd:YAG激光能有效、安全地治疗黑眼圈,并能选择性地清除显露的下睑静脉,对下睑皮肤无损伤,可达到理想的美容效果.     

长脉冲Nd：YAG激光治疗龟头静脉畸形

目的评价长脉冲Nd：YAG激光治疗龟头静脉畸形的疗效及安全性。方法 10例龟头静脉畸形患者接受长脉冲1064nmNd：YAG激光治疗,7～8ms双脉宽、脉冲间隔20ms、120～140J/cm2能量密度、接触式冷却。治疗5～7次,治疗间隔1个月,疗程结束后随访12个月,观察并发症,并分五级评价病灶清除率。结果 1例患者治疗时有轻度疼痛;所有患者治疗部位均出现暂时性红斑和水肿,无出血、感染、色素改变、瘢痕形成和勃起功能障碍等。随访12个月,全部患者龟头静脉畸形病灶完全清除,未见复发。结论长脉冲Nd：YAG激光能安全有效地治疗龟头静脉畸形,并发症少,可作为龟头静脉畸形的首选治疗方法之一。     

葡萄酒色斑增厚及结节形成的病理基础和临床意义

目的 通过分析葡萄酒色斑增厚和结节病灶的组织学特点,探讨其可能的扩张机制.方法 观察3例葡萄酒色斑平坦病灶、11例增厚病灶、9例结节病灶的HE切片,分析比较不同病灶的组织学特征.结果 普通葡萄酒色斑主要表现为真皮浅层轻度血管的扩张,不伴其他皮肤组织的增生.葡萄酒色斑增厚病灶主要表现为真皮层血管进一步扩张,伴皮脂腺增多、毛囊肥大、皮脂腺分泌物淤积等错构现象.葡萄酒色斑结节可分为3种不同表现类型:Ⅰ类似葡萄酒色斑增厚病灶,表现为真皮层血管扩张,伴皮脂腺增多、毛囊肥大、皮脂腺分泌物淤积的错构现象;Ⅱ类表现为真皮层大量扩张血管,呈蜂窝状,问质少,但未观察到其他组织的错构现象;Ⅲ类表现为扩张血管少,组织弥漫胶原化,间质疏松,淋巴细胞灶性浸润,呈淋巴水肿样改变.结论 葡萄酒色斑增厚病灶、增牛结节组织中,部分病灶完伞以扩张血管为主要构成成分,另一部分则不仅观察到不同程度的扩张血管,同时表现出外分泌腺增多、毛囊肥大,并且葡萄酒色斑结节可分成3种不同的表现类型.提示畸形血管的进行性扩张是葡萄酒色斑增生的重要基础,但在部分病例中,增厚与结节可能源于不明原因的组织增生.     

病毒介导的基因转染在组织工程中的应用

目的构建重组病毒载体,将较多细胞因子通过病毒载体转入种子细胞,提高细胞增殖及分泌基质能力,维持表型,延缓老化.方法应用基因工程技术,构建缺陷型逆转录病毒重组载体和缺陷性腺病毒重组载体.分别通过PT67包装细胞筛选克隆及293细胞扩增,获得假病毒上清液,经浓缩纯化后转染靶细胞.结果成功构建胶质细胞源性神经营养因子(GDNF)、Fas 配体(Fas-Ligand)、绿色荧光蛋白(GFP)等逆转录病毒载体以及TGFβ1、人骨形成蛋白因子-7(hBMP7)、端粒酶、Smad3、Smad7等腺病毒载体,其中GFP-逆转录病毒重组载体在软骨细胞表达至2月.结论通过有效的含目的基因重组载体转染种子细胞,延缓体外培养细胞的老化及促进细胞的增殖与基质分泌,获得组织工程所需要的大量种子细胞,为组织工程构建奠定了基础.     

骨髓基质干细胞-藻酸钙复合物构建骨组织的实验研究

目的研究犬骨髓基质干细胞(BMSCs)体外成骨活性和在犬体内异位成骨能力,探讨其作为骨组织工程种子细胞的可能性. 方法抽取成年犬骨髓,梯度离心法获取单个核细胞,条件培养液体外培养、诱导、扩增后,进行组织化学、免疫细胞化学染色,透射电镜下观察其成骨活性.将培养的第3代细胞与藻酸钙所形成的复合物植于犬皮下,3月后行组织学检测. 结果体外培养的BMSCs Von Kossa染色可见钙结节,AKP染色及I型胶原、Osf2/Cbfα1、骨钙素免疫细胞化学染色先后呈现阳性;透射电镜下见基质钙化;组织学检测提示细胞-藻酸钙复合物体内培养3月后形成骨样组织. 结论BMSCs在大量扩增的同时,在一定条件下能向成骨细胞分化,细胞与藻酸钙复合物能在具免疫力的哺乳动物皮下形成骨样组织.BMSCs可作为骨组织工程较理想的种子细胞.     

新生儿红斑的自然病程及其与葡萄酒色斑的鉴别研究诊断

目的探索新生儿红斑的自然病程,及其与葡萄酒色斑的鉴别要点。方法对2007年3月至2011年5月,来我科就诊的36例面颈部红斑患儿进行随访,观察红斑的发生特点及转归,分析并总结新生儿红斑与葡萄酒色斑的鉴别诊断依据。结果本组36例患儿中,诊断为新生儿红斑21例,多部位累及者15例(71.4%),其中累及前额及眉间者有16例(38.1%)、项部有13例(31.0%)、眼睑有9例(21.4%)、人中有3例(7.1%)。随访至2岁时,18例消退或减淡(85.7%);其余3例随访至5岁,红斑无减退倾向,遂诊断为葡萄酒色斑。本组中另15例患者诊断为葡萄酒色斑,其中颞区2例(13.3%),面颊区8例(53.3%),下颌区3例(20%),多区累及者2例(13.3%)。随访至2岁时,90%患儿无明显改变,其余2例有轻微加深。结论新生儿红斑与葡萄酒色斑在形态学上具有相似性,但两者发生部位及自然病程存在明显差异。当难以鉴别时,加强随访是减少误诊及防止错误治疗的重要手段。     

脉冲染料激光治疗溃疡性血管瘤的临床研究

目的探讨脉冲染料激光(Pulsed dye laser,PDL)治疗溃疡性血管瘤的临床疗效和安全性。方法创面清洁的溃疡性血管瘤患儿,采用脉冲染料激光治疗(波长585 nm,脉宽0.45 ms,光斑5 mm,能量密度6.0～6.5 J/cm2),治疗间隔时间2～3周。治疗后随访观察溃疡完全再上皮化时间、疼痛缓解时间,以及溃疡创面的出血、感染等情况。结果本组共15例溃疡性血管瘤患儿,治疗前及治疗过程中均未接受其他治疗。14例(93.3%)仅接受1次PDL治疗,1～2周内溃疡创面完全再上皮化;1例(6.7%)接受2次PDL治疗后,溃疡创面再上皮化。溃疡的平均完全再上皮化时间为(1.68±0.23)周。所有患儿均在治疗后1～2 d内疼痛得到缓解。治疗后无一例出现溃疡创面扩大,未发生出血、感染等并发症。结论PDL能加快溃疡性血管瘤的创面愈合,缓解疼痛,治疗后无出血、感染及溃疡加重等并发症,可作为溃疡性血管瘤早期治疗的安全有效的方法。     

微静脉畸形伴唇肥大畸形的病理特点及临床意义

目的 分析微静脉畸形伴唇肥大畸形的组织学特点,探讨微静脉畸形可能的扩张机制.方法 通过观察21例微静脉畸形伴唇肥大畸形标本的HE切片,分析其组织学特点.结果微静脉畸形伴唇肥大畸形的组织学表现为广泛的扩张血管,血管平均管径(39.8±15.7)μm.扩张血管与横纹肌、唇腺、脂肪交错排列,呈典型的组织错构表现,错构程度:轻度1例、中度7例、重度13例.结论 微静脉畸形伴唇肥大畸形的组织学表现以轻度扩张血管、横纹肌、唇腺、脂肪的组织错构为主要特点,属于先天性软组织的错构和发育异常.