Intravenous immunoglobulin therapy in patients with multiple mucosal involvement in mucous membrane pemphigoid.

Mucous membrane pemphigoid (MMP), also known as cicatricial pemphigoid (CP), is an autoimmune mucocutaneous, blistering disease which can lead to blindness and/or death from sudden asphyxiation, secondary to a scarring process. Conventional therapy for the treatment of MMP consists of high-dose systemic corticosteroids and/or immunosuppressive agents. Some patients do not respond to these treatments and develop multiple serious side effects, which can be potentially fatal. In such patients, alternative treatment modalities are needed. This study presents the use of intravenous immunoglobulin (IVIg) therapy in 15 patients with severe MMP whose disease was nonresponsive to the prolonged use of high-dose systemic corticosteroids and immunosuppressive agents and who developed multiple side effects to them. All 15 patients received an IVIg dose of 1-2 g/kg/cycle. The following objective parameters were used to assess the clinical outcome pre- and post-IVIg therapy: number of side effects, frequencies of recurrences and relapses, duration and total dosage of prednisone therapy, and the quality of life. The differences in these variables between the pre- and post-IVIg data were statistically analyzed using the SAS UNIVARIATE software running the two-sided Wilcoxon signed-rank and sign tests. A statistically significant difference was observed between pre- and post-IVIg therapy data when comparing the aforementioned variables. All 15 patients had an effective clinical response, were able to discontinue previous systemic therapies, and eventually achieved a prolonged clinical remission. IVIg improved the quality of life in all 15 patients and demonstrated a steroid-sparing effect. No serious side effects were observed. IVIg therapy is a safe and effective alternative modality in the treatment of patients with nonresponsive and progressive MMP and can induce a sustained clinical remission.     

局部应用5-氟尿嘧啶预防胆管电凝损伤后瘢痕狭窄的实验研究

目的 探讨早期局部应用5-氟尿嘧啶（5-FU）对预防电凝损伤后胆管瘢痕狭窄的疗效,为临床治疗胆管良性狭窄寻求新的途径。方法 40只SD大鼠,随机分成2组：实验组（5-FU组）与对照组（NS组）,利用电凝损伤胆总管建立胆总管损伤狭窄模型,于胆总管损伤部位分别局部应用5-FU与生理盐水,于术后1周和2周观察肝功能变化、近端胆管扩张程度及胆管狭窄部位病理表现。结果 5-FU组肝功能损害明显低于同时期NS组,差异有统计学意义（P＜0.05）。5-FU组近端胆管扩张程度明显轻于NS组。胆管狭窄病理结果提示：5-FU组损伤部位胆管壁增厚不明显,成纤维细胞散在分布,胶原蛋白分布均匀;NS组损伤部位胆管壁明显增厚,成纤维细胞增生活跃,部分可见肌纤维细胞,胶原蛋白多而不规则。结论 局部应用5-FU对大鼠胆管电凝损伤所致胆管瘢痕狭窄有预防作用,可以为临床治疗胆管良性狭窄提供新的研究方向。     

扩张后颈肩皮瓣修复颈部烧伤瘢痕挛缩

目的寻求在颈部瘢痕挛缩修复中,优于传统手术疗效的方法。方法采用大容量扩张器置入肩部进行常规扩张,扩张6~8周后,行颈肩部皮瓣转移一次性手术完成修复治疗。移植皮瓣的面积为6 cm×11 cm~12cm×21 cm。结果共治疗15例患者,移植皮瓣全部存活。术区的外观、质地佳。结论扩张后颈肩皮瓣血运丰富,供瓣面积大,皮瓣质地佳,供区隐蔽可直接缝合,易为女性及青年患者接受。     

Etiology of cicatricial alopecias: a basic science point of view

ABSTRACT:   This article presents a short summary of our current knowledge of cicatricial alopecia disease pathogenesis and the hypothetical disease mechanisms that may be involved in scarring alopecia development. Several forms of scarring alopecia likely involve targeted cytotoxic action against hair follicle cells mediated by a folliculocentric inflammation. However, the specific nature of the inflammatory interference in hair follicle growth is open to question. A popular hypothesis of lymphocyte-mediated scarring alopecia development involves autoimmune targeting of hair follicle–specific self-antigens, although there is no direct evidence in support of such a view. Alternative hypotheses focus on defects in sebaceous gland function, destruction of hair follicle stem cells, and interference in the communication between hair follicle mesenchyme and epithelium. Many questions arise from these hypotheses, and addressing them with a systematic research approach may enable significant advances in understanding cicatricial alopecia etiology.     

瘢痕性天疱疮35例临床分析

目的:探讨瘢痕性天疱疮临床特点,为临床诊治提供帮助。方法:回顾性分析2002-01/2008-01我科诊治的35例瘢痕性天疱疮临床特点。结果:瘢痕性天疱疮平均发病年龄为62.4岁,男女性别比为20∶15,病史平均3.3a,51.43%以口腔黏膜病变首发,31.43%以眼部病变首发,眼睑内翻倒睫/慢性结膜炎和干眼/角膜病变为5/4/2,17.14%以其它黏膜病变首发。经过治疗,71.43%(25/35)患者症状消失或缓解超过3mo,14.29%(5/35)患者症状反复或无明显缓解,14.29%(5/35)患者中途退出治疗。治疗过程中37.14%(13/35)患者出现全身其它异常,其中6例出现肝功能异常,2例终止治疗,肾功能异常2例,1例终止治疗,血液系统异常3例,1例退出治疗,心肌梗塞1例,退出治疗,明显血压升高1例。结论:瘢痕性天疱疮是一种慢性病,多发于老年人,男性稍多于女性,眼组织经常受累,多表现为干眼症、睑球粘连和内翻倒睫。瘢痕性天疱疮治疗复杂,经常需要多学科协作。     

人性化护理对剖宫产后瘢痕子宫产妇自然分娩的效果

目的:对剖宫产后瘢痕子宫产妇自然分娩采用人性化护理的效果进行研究。方法:选取2017年1月~2018年12月在某院进行分娩的86例剖宫产后瘢痕子宫患者,随机分为对照组和观察组各43例。对照组采用常规护理,观察组采用人性化护理,记录两组的泌乳时间、焦虑度、抑郁度评分,比较两组的分娩方式。结果:观察组顺产率高于对照组,泌乳时间高于对照组,焦虑度、抑郁度自我评分明显低于对照组,两组比较差异具有统计学意义(P<0.05)。结论:人性化护理可以有效促进剖宫产后瘢痕子宫产妇的自然分娩,增加患者的泌乳时间,降低患者的剖宫率,改善孕妇的抑郁情绪,提高新生儿的存活率,值得产科临床上大力推广。     

疤痕子宫中期妊娠引产32例体会

目的寻求一种安全有效的引产方法来终止妊娠,以利于计划生育工作的开展。方法应用利凡诺羊膜腔内注射,部分宫颈管硬、较长,辅以米索前列醇,通过严密观察产程,产后常规及时清宫。结果有效地缩短产程,防止了子宫破裂、胎盘粘连及其引起的一系列并发症。结论利凡诺用于疤痕妊娠子宫引产是安全有效的,虽然米索前列醇有软化宫颈、加强宫缩的作用,但用疤痕妊娠子宫也是安全的,并有缩短产程的作用。     

Emerging drugs for alopecia areata: JAK inhibitors

Introduction: Alopecia Areata is a common form of non-scarring hair loss that usually starts abruptly with a very high psychological impact. Due to the still not completely understood etiopathogenesis, at present there is no treatment that can induce a permanent remission and there is no drug approved for the treatment of this disorder.

Areas covered: Leading existing treatment are briefly overviewed and then ongoing research on Janus Kinases Inhibitors is discussed, reviewing trials with oral and topical formulations so as new opportunities for other forms of alopecia, such as cicatricial alopecia.

Expert opinion: JAK inhibitors represent a promise among alopecia treatments, but further studies are needed on long term safety. There is still no validated dosage for alopecia areata and the vehicles used for topical formulations seem not yet ideal in terms of skin penetration and reduced systemic absorption. Hopefully several studies are ongoing and we hope, in the near future, that JAK inhibitors will become part of the armamentarium to treat alopecia areata patients in terms of safety and costs.