体外分离与培养组织工程皮肤附属物：发育标记物检测及调控和增殖

背景：现有各类组织工程皮肤面临着共同的难题,突出表现在没有完整的皮肤功能,尤其是缺乏皮肤附属物如毛囊、汗腺、皮脂腺等。如何培养具有皮肤附属物的“功能性组织工程皮肤”即完整的皮肤器官,成为皮肤组织工程迫在眉睫的研究方向。 目的：文章通过综述皮肤附属物相关干细胞的分离与培养以及向皮肤附属物诱导分化的研究进展,为解决当前人工复合皮肤中难以形成皮肤附属物的难题提供帮助。 方法：应用计算机检索Pubmed数据库(1999-01/2009-12),以“skin tissue engineering,skin appendages”为检索词;应用计算机检索中国期刊全文数据库(2004-01/2009-12),以“皮肤组织工程、皮肤附属物、毛囊、汗腺、皮脂腺”为检索词。纳入标准：文章所述内容应与利用组织工程技术使干细胞分化、增殖形成毛囊、汗腺、皮脂腺等皮肤附属物研究相关。排除标准：重复研究或荟萃分析类研究。 结果与结论：计算机初检得到97篇文献,排除因研究目的与此文无关的35篇,内容重复性的研究26篇,共保留36篇文献进行综述。人体多种细胞都有向皮肤附属物分化增殖的能力,进一步阐明胚胎发育的机制,深入研究干细胞定向分化的调控机制,在皮肤修复的同时能够诱导毛囊、皮脂腺和汗腺的发生,无疑是构建理想组织工程皮肤的重要途径。将生长因子、干细胞生物技术与生物材料工程学相结合,才可能研制成功真正意义上的皮肤永久替代物,但体外研制成功的皮肤永久替代物应用于人体的安全性及效果还有待进一步分析。     

Knockdown of Parhyale Ultrabithorax recapitulates evolutionary changes in crustacean appendage morphology

Crustaceans possess remarkably diverse appendages, both between segments of a single individual as well as between species. Previous studies in a wide range of crustaceans have demonstrated a correlation between the anterior expression boundary of the homeotic (Hox) gene Ultrabithorax (Ubx) and the location and number of specialized thoracic feeding appendages, called maxillipeds. Given that Hox genes regulate regional identity in organisms as diverse as mice and flies, these observations in crustaceans led to the hypothesis that Ubx expression regulates the number of maxillipeds and that evolutionary changes in Ubx expression have generated various aspects of crustacean appendage diversity. Specifically, evolutionary changes in the expression boundary of Ubx have resulted in crustacean species with either 0, 1, 2, or 3 pairs of thoracic maxillipeds. Here we test this hypothesis by altering the expression of Ubx in Parhyale hawaiensis, a crustacean that normally possesses a single pair of maxillipeds. By reducing Ubx expression, we can generate Parhyale with additional maxillipeds in a pattern reminiscent of that seen in other crustacean species, and these morphological alterations are maintained as the animals molt and mature. These results provide critical evidence supporting the proposition that changes in Ubx expression have played a role in generating crustacean appendage diversity and lend general insights into the mechanisms of morphological evolution.     

ES细胞源性表皮干细胞与胶原海绵构建组织工程皮肤

【目的】以胚胎干细胞(ES细胞)源性表皮干细胞为种子细胞与胶原海绵构建组织工程皮肤,探讨其对皮肤缺损修复的作用。【方法】胎鼠皮肤成纤维细胞与胶原海绵构建类真皮,植入小鼠全层皮肤缺损创面,以生物膜为载体,把羊膜诱导后带有核标记的ES细胞源性表皮干细胞覆盖在类真皮上,术后1～8周连续取材,HE染色,β1整合素、CK15、CK19、CK10和CEA免疫荧光双标和免疫组化观察。【结果】植入后3周,创面完全长合,较厚新生皮完全覆盖创面,基底层细胞增生,形成许多大小不一的细胞柱伸向真皮层,新生表皮中可见核标记的细胞呈β1整合素、CK15、CK19阳性,真皮中的管腔样结构呈核荧光和β1整合素、CEA免疫组化双标阳性,4～8周新生表皮基底层细胞呈CK19、CK10阳性,新生表皮下可见毛囊样、皮脂腺样结构。【结论】ES细胞源性表皮干细胞为种子细胞与胶原海绵构建的组织工程皮肤可以修复缺损皮肤,在其下真皮层有分化为毛囊样、皮脂腺样和汗腺样的结构,但是是否来源于ES细胞源性表皮干细胞仍需进一步证实。     

Distribution of keratin 8-containing cell clusters in mouse embryonic tongue: evidence for a prepattern for taste bud development

The initiation of the morphogenesis of gustatory papillae is independent of innervation. To address the question of whether taste bud formation is associated with gustatory papilla morphogenesis, we examined developing tongues in mouse embryos from embryonic day 11 to birth. Despite the smooth morphological appearance of the lingual dorsal surface at 13 days of gestation, we observed embryonic taste bud primordia as discrete collections of cytokeratin 8-positive and elongated cells in epithelial placodes in the anterior tongue. In subsequent stages until birth, cytokeratin 8 continues to be expressed in embryonic taste buds distributed in punctuate patterns at regular intervals along rows that are symmetrically located on both sides of the median sulcus in the dorsal anterior developing tongue. Embryonic taste buds were observed in the developing circumvallate papillae from 15.5 days of gestation until birth. The dorsal epithelium of the anterior tongue is not innervated when embryonic taste buds first occur. The increased numbers of embryonic taste buds in developing fungiform papillae until birth are not correlated with the neural invasion of the epithelium. Thus, taste buds occur prenatally more likely independently of the innervation.     

Ultrasound appearances of the testicular appendages: pictorial review

Five testicular appendages are formed during development of the male genito-urinary tract, which are the remnants of the degenerating mesonephric and paramesonephric ducts. The testicular and epididymal appendages, found at the upper pole of the testis and at the head of the epididymis respectively, are the most common and have a range of appearances on ultrasound. These appendages have the ability to undergo torsion, an important differential diagnosis in the child who presents with an acute scrotum. The varying ultrasound appearances of the testicular appendages are described and illustrated. Ultrasound features of appendiceal torsion are also demonstrated. Electronic Publication     

Echocardiographic features of left juxtaposed atrial appendages associated with dextro-transposition of the great arteries

We found that echocardiography in cases of left juxtaposed atrial appendage (JAA) consistently featured a malpositioned right atrial appendage, abnormal spatial orientation of the atrial septum, and posterior deviation of the septum secundum toward the left atrium. The qualitative displacement of the septum secundum together with the posterior and leftward displacement of the right atrial appendage (with respect to the great arteries) prompted us to pose questions concerning the morphogenesis of left JAA and the implications for surgery. The importance of precatheterization and preoperative recognition of left JAA in dextro-transposition of the great arteries by echocardiography cannot be overemphasized when planning a balloon atrial septostomy or a biventricular repair.     

ES细胞源性表皮干细胞在皮肤缺损创面的分化

目的探讨ES细胞源性表皮干细胞移植入小鼠全层皮肤缺损后对创面的修复作用,为组织工程皮肤的研究提供新的思路。方法将羊膜诱导后的带有核荧光标记的ES细胞源性表皮干细胞以生物膜为载体,覆盖小鼠全层皮肤缺损创面。术后1～8周连续取材,HE染色观察,苏丹Ⅲ染色,β1整合素、CK15、CK19、CEA免疫组化荧光双标观察。结果术后2周,创面完全长合,较厚的新生皮完全覆盖创面,基底层细胞增生,形成细胞柱伸向真皮层,真皮层中可见管腔样结构,4周后新生表皮开始变薄,新生表皮下可见毛囊样、汗腺样、皮脂腺样等结构,皮脂腺样结构苏丹Ⅲ染色阳性。免疫双标结果显示,1～4周新生表皮基底层细胞呈β1整合素、CK19阳性,真皮层中带有核标记的管状或泡状结构呈CK15、CEA阳性。结论ES细胞源性表皮干细胞在小鼠全层皮肤缺损创面可分化为表皮样、汗腺样、毛囊样和皮脂腺样等结构的潜能。     

Multiple pilomatricomas in twins with Rubinstein-Taybi syndrome

Pilomatricomas are benign tumors originating from the capillary matrix, which may present as solitary lesions or, less commonly, multiple. Myotonic dystrophy and familial adenomatous polyposis are the most frequently associated disorders with multiple pilomatricomas. There are few reports relating these tumors to other genetic syndromes. Rubinstein-Taybi syndrome is a rare autosomal dominant disorder characterized by intellectual disability and typical dysmorphic characteristics. There are five case reports relating to multiple pilomatricoma to Rubinstein-Taybi syndrome, an association that needs to be clarified. For this reason, we report the first case of multiple pilomatricoma in monozygotic twins with typical Rubinstein-Taybi syndrome.     

2q37.3 Deletion with Complex Heart Defects Suggesting Interruption of Early Ventricular Looping

A maternally inherited 828 kb microdeletion of 2q37.3 manifested in a 3-week-old premature boy as left juxtaposition of the atrial appendages associated with tricuspid atresia, double outlet infundibulum, subvalvar pulmonary atresia, large secundum atrial septal defect, and right aortic arch with mirror-image branching, consistent with developmental arrest early in heart looping. To the best of our knowledge, no previous 2q37 deletion syndrome has been reported with such a severe cardiac dysmorphology. Hence, this case adds to the cardiac phenotypes identified in 2q37 deletion syndrome.     

卵巢小细胞神经内分泌癌一例

卵巢小细胞神经内分泌癌是一种发病率极低的卵巢恶性肿瘤,临床表现不明显,诊断需在病理基础上结合神经内分泌指标,并明确其原发性。治疗以手术联合以铂类为基础的化疗为主,生存期与临床分期及治疗方案有关。本例患者因下腹胀痛半年加重2个月,发现左侧附件区占位2 d入院。予附件肿块穿刺活检后经病理学确诊。患者术前行3个周期EP方案(依托泊苷+顺铂)新辅助化疗结束后,行经腹"全子宫双附件切除术+大网膜切除术+阑尾切除术+右侧盆壁腹膜活检术+肠系膜活检术",术后继予3个周期EP方案化疗,术后随访至今无瘤生存15个月。