甲泼尼龙冲击疗法联合环磷酰胺治疗SLE患者严重血小板减少19例疗效观察

系统性红斑狼疮(SLE)是常见的累及多系统的自身免疫性疾病.血小板减少是SLE患者最常见的血液系统改变之一.我科于2008-2010年应用甲泼尼龙联合环磷酰胺(CTX)治疗SLE引起的顽固性血小板减少取得较好疗效,现报告如下.     

SLE患者外周血内皮祖细胞数量及生物学功能的研究

目的 探讨SLE患者外周血内皮祖细胞数量和功能的变化。方法 女性SLE患者及正常人对照各20例,取外周血20ml,密度梯度离心法分离单一核细胞,采用CD133/CD34、CD133/VEGFR2双荧光标记鉴定细胞,CD34/CD133/VEGFR2三荧光标记流式检测EPC细胞数量,采用MTT比色法、改良的millicell小室和黏附能力测定实验观察EPC的增殖、迁移和黏附能力。结果 SLE患者外周血内皮祖细胞数量较正常人对照明显下降（4.49% ± 1.66%比20.81% ± 4.14%,P < 0.01）,其增殖（23.11 ± 3.16比35.65 ± 1.74）、迁移（12.00 ± 2.12个细胞/视野比23.60 ± 3.0个细胞/视野）、黏附能力（22.43 ± 4.43个细胞/视野36.43 ± 3.69个细胞/视野）均有所下降（P < 0.01）。结论 SLE患者外周血内皮祖细胞数量减少、功能受损。     

血小板/淋巴细胞比值与系统性红斑狼疮脏器受累及疾病活动的相关性分析

目的观察血小板淋巴细胞比值(PLR)与系统性红斑狼疮(SLE)脏器受累及疾病活动的相关性。方法选取2014年1月—2018年12月于我院就诊的初治SLE患者120例作为SLE组,同时选取同期于哈尔滨医科大学附属第二医院进行体检的健康者80例作为健康对照组,依据有无肾脏受累或者浆膜炎将SLE患者分为狼疮肾炎(LN)组和无LN组,浆膜炎组和无浆膜炎组,采用SLE疾病活动指数2000(SLEDAI-2000)评估疾病活动度,SLE-DAI<15分为轻中度疾病活动组,SLEDAI>15分为重度疾病活动组。分别比较以上各组的PLR值。同时利用Spearman相关分析PLR与SLE患者实验室指标的相关性,采用Logisitic回归模型分析PLR与SLE疾病活动度之间的关系,通过绘制受试者工作特征曲线(ROC)评价PLR在SLE诊断及活动性评估中的价值。结果SLE组PLR明显高于健康对照组,差异具有统计学意义(P<0.05);SLE组白细胞计数、淋巴细胞计数以及血小板计数明显低于健康对照组,而红细胞分布宽度、尿酸水平明显高于健康对照组,差异具有统计学意义(P<0.05);LN组PLR明显高于无LN组,合并有浆膜炎组PLR明显高于无浆膜炎组,差异具有统计学意义(P<0.05);重度疾病活动(SLEDA≥15分)的SLE患者有78例,中重度疾病活动(SLEDAI<15分)的SLE患者有42例,重度疾病活动组PLR值明显高于轻中度疾病活动组,差异具有统计学意义(P<0.05);Spearman相关分析结果显示,PLR分别与CRP(r=0.567,P=0.015),SLEDAI评分(r=0.875,P=0.001)呈正相关,与血清总蛋白(=-0.502,P=0.002),血清白蛋白(r=-0.611,P<0.001)呈负相关;Logisitic回归分析结果显示PLR是SLE患者疾病活动的独立危险因素(P<0.05);ROC结果显示PLR用于诊断SLE的最佳截断值为174.78,敏感性为70.89%,特异性为87.14%,AUC=0.735,95%CI 0.681~0.813。PLR用于诊断LN的最佳截断值为178.97,敏感性为64.12%,特异性为83.09%,AUC=0.713,95%CI 0.667~0.794。PLR用于诊断SLE患者重度疾病活动的最佳截断值为187.04,敏感性为56.19%,特异性为76.09%,AUC=0.702,95%CI 0.613~0.737。结论PLR与SLE患者的肾脏受累浆膜炎及疾病活动密切相关,提示其作为一种新的炎症指标,对评估SLE疾病活动及脏器受累具有重要意义。     

系统性红斑狼疮和银屑病患者外周血细胞黏附功能与红细胞表面及血小板CD35的活性研究

为研究银屑病和系统性红斑狼疮血细胞的天然免疫功能状况,比较这两种疾病红细胞表面CD35及血小板CD35定量活性变化,探讨两种疾病的发病机制,我们采用分离新鲜红细胞、淋巴细胞、粒细胞三系,分别测定其对肿瘤细胞的快速天然免疫黏附反应。应用流式细胞仪测定红细胞表面及血小板CD35的表达水平。银屑病患者红细胞、淋巴细胞以及粒细胞对肿瘤细胞黏附率均明显高于正常对照组（P〈0.001）。SLE患者血细胞对肿瘤细胞黏附率又明显低于正常对照组（P〈0.001）;银屑病患者的血细胞对肿瘤细胞黏附率,进展期最高,静止期其次,消退期再次。银屑病患者红细胞及血小板CD35的表达明显高于正常对照组和SLE组（P〈0.001）;而SLE红细胞及血小板CD35明显低于正常对照组（P〈0.001）;银屑病红细胞CD35分子与红细胞黏附率呈显著正相关（γ=0.806,P〈0.05）。SLE和银屑病患者红细胞天然免疫功能存在紊乱。并可能与病情的活动性有关。     

自体造血干细胞移植对SLE患者生活质量的影响

目的探讨自体外周血造血干细胞移植(APBSCT)对系统性红斑狼疮(SLE)患者生活质量的影响。方法对21例住院的SLE患者进行APBSCT治疗。动员方案为环磷酰胺(CTX)4 g/m2,粒细胞集落刺激因子(G-CSF)5μg/(kg.d),4~5天。预处理方案为CTX 50 mg/(kg.d),4天;抗胸腺球蛋白(ATG)15~25 mg/(kg.d),3天。预处理结束后回输采集到的CD34+造血干细胞。采用WHOQOL-BREF中文版,观察并评价移植前后患者的生活质量评分。结果21例患者造血干细胞均成功植入。移植相关的死亡率为0%。WHOQOL-BREF表的五个方面,包括生理、心理、社会关系、环境、总体感觉等在移植后均有明显改善(P<0.05或P<0.01)。结论APBSCT治疗SLE有良好的疗效,患者在移植后生活质量明显改善。     

IL-12对SLE患者PBMC分泌IL-10、IFN-γ的影响

目的　了解白细胞介素 12 (IL 12 )对SLE患者外周血单一核细胞 (PBMC)分泌白细胞介素 10 (IL 10 )、γ 干扰素 (IFN γ)的影响。方法　在培养体系中加入或不加入IL 12 ,观察IL 12对SLE患者及正常对照PBMC分泌IL 10 ,IFN γ的水平变化。结果　IL 12可使SLE患者PBMC分泌IL 10水平降低而使IFN γ水平升高。结论　在体外 ,加入适当的IL 12可部分纠正与SLE发病有关的主要的异常细胞因子格局     

两种中药方剂对SLE患者PBMC凋亡率的影响

目的探讨中药方1(生药总量共计195g,组分:水牛角、黄芩、生地黄、丹皮、紫草、白花蛇舌草、半枝莲、忍冬藤、防风、秦艽、威灵仙、鸡血藤、甘草)和中药方2(生药总量共计270g,组分:熟地黄、山茱萸、山药、北沙参、麦冬、玄参、生地黄、双花、丹皮、丹参、益母草、丝瓜络、菟丝子、白芍、党参、甘草)对系统性红斑狼疮(SLE)患者外周血单个核细胞(PBMC)的凋亡率水平的影响及意义。方法根据血清药理学制备含药大鼠血清,采用密度离心法分离SLE患者的PBMC,分离的PBMC分别与含有高、中和低剂量的两种中药方剂大鼠血清培养48小时,用AnnexinV-FITC流式细胞仪技术检测PBMC凋亡率的变化。结果与空白对照组比较,中药方1高剂量组PBMC的凋亡率无明显差异(P0.05),而中药方2高剂量组PBMC的凋亡率显著降低(P0.01)。结论中药方1对体外培养的SLE进行期患者PBMC水平无明显影响。中药方2能使体外培养的SLE缓解期患者PBMC的凋亡率降低。     

补骨胶囊对SLE患者激素治疗后骨质疏松的作用及细胞因子表达的影响

目的探讨中成药补骨胶囊对系统性红斑狼疮(SLE)患者激素治疗后骨质代谢及细胞因子表达的影响。方法66例SLE患者随机分为两组,治疗组34例,采用糖皮质激素和补骨胶囊治疗;对照组32例,单纯采用糖皮质激素治疗,另设健康组30例。观察治疗前后两组病人IL-1,IL-6,TNF-α表达水平和血钙、磷、碱性磷酸酶、甲状旁腺素的变化以及三角区骨密度的改变,并与健康组对照比较。结果治疗前两组病人的各项指标差异均无显著性;治疗后两组患者的IL-1,IL-6,TNF-α表达水平和血钙、甲状旁腺素的变化以及三角区骨密度的改变,差异均有显著性(P<0.01或P<0.05)。结论中成药补骨胶囊能有效防治由糖皮质激素致患者骨质疏松的发生,其作用机制可能与调节患者体内细胞因子IL-1,IL-6,TNF-α表达水平和甲状旁腺素的分泌有关。     

SLE患者T细胞PKA活性及血清因子对PKA活性的影响

目的:研究SLE患者T细胞功能异常是否与T细胞CA/cAMP/PKA信号传导途径存在异常有关,探讨SLE的发病机理。方法:用尼龙柱分离肝素化的静脉血得到T细胞悬液,用流式细胞术测定CD_3阳性细胞90％以上,用CD_3单抗与羊抗鼠二抗IgG相交联和rIL-1α共同刺激T细胞24 小时,用RT-PCR检测IL-2和IL-2R mRNA的表达,证实T细胞是否活化。用PKA检测试剂盒检测T细胞PKA总体活性变化,用SLE血清或含不同浓度IFN-α的完全培养基和正常人T细胞培养24小时,然后用上述方法激活T细胞,分别测量T细胞PKA的总体活性。结果:SLE患者T细胞活化后PKA总体活性是下降的,SLE患者血清免疫复合物及不同浓度的IFN-α都不能使正常人T细胞的PKA总体活性下降。SLE患者T细胞活化后总体PKA活性下降和血清中的免疫复合物及血中高浓度的IFN-α无关。结论:1,SLE患者T细胞CA/cAMP/PKA信号传导途径存在异常。2,SLE患者T细胞功能异常是原发的,是由于细胞内生物化学信号途径异常所致。     

糖皮质激素对SLE患者Th1/Th2类细胞因子mRNA表达及产生的影响

目的 探讨糖皮质激素治疗对系统性红斑狼疮(SLE)患者外周血单一核细胞(PBMC)中Th1/Th2类细胞因子mRNA表达及产生的影响.方法 采用逆转录-聚合酶链反应和酶联免疫吸附试验检测糖皮质激素治疗前后SLE患者PBMC中Th1类细胞因子干扰素γ(IFN-γ)和Th2类细胞因子白介素4(IL-4)、白介素10(IL-10)mRNA表达和产生的变化.结果 经糖皮质激素治疗后SLE患者PBMC产生IFN-γ,IL-4和IL-10水平分别为(74.08±15.85)pg/mL,(246.43±45.25)pg/mL,(549.52±67.00)pg/mL;mRNA平均表达水平分别为0.3298±0.2181,0.4951±0.2938,0.8671±0.6620.治疗前IFN-γ,IL-4和IL-10水平分别为(113.5±27.6)pg/mL,(272.97±33.78)pg/mL,(625.32±104.61)pg/mL;mRNA平均表达水平分别为1.4094±0.6317,0.6872±0.4713,1.1758±0.6255;治疗后各细胞因子水平均较治疗前显著降低(P<0.01);但IFN-γ较IL-4,IL-10降低更为显著.结论 糖皮质激素可通过影响SLE患者细胞因子mRNA表达和产生,调节Th1/Th2类细胞因子网络平衡,可能为糖皮质激素治疗SLE的机制之一.     

New fillers for the new man

ABSTRACT:  Two new collagen-based lidocaine-containing dermal fillers, ArteSense™/ArteFill™ (Artes Medical, San Diego, CA) and Evolence® (Colbar LifeScience Ltd., Herzliya, Israel), have proved to be of particular interest to men, many of whom seek a long-lasting or permanent correction. ArteFill™ has been available in the United States since 2006, and it is expected that Evolence® will reach the American market in 2008. The properties of the two products will be described, and experience based on the administration of many hundreds of syringes of both products by a Canadian dermatologist will be detailed here, with tips and precautions to optimize patient outcomes.     

Outcomes and adverse effects of ablative vs nonablative lasers for skin resurfacing: A systematic review of 1093 patients

It is generally believed that ablative laser therapies result in prolonged healing and greater adverse events when compared with nonablative lasers for skin resurfacing. To evaluate the efficacy of ablative laser use for skin resurfacing and adverse events as a consequence of treatment in comparison to other modalities, a PRISMA‐compliant systematic review (Systematic Review Registration Number: 204016) of twelve electronic databases was conducted for the terms “ablative laser” and “skin resurfacing” from March 2002 until July 2020. Studies included meta‐analyses, randomized control trials, cohort studies, and case reports to facilitate evaluation of the data. All articles were evaluated for bias. The search strategy produced 34 studies. Of 1093 patients included in the studies of interest, adverse events were reported in a total of 106 patients (9.7%). Higher rates of adverse events were described in nonablative therapies (12.2% ± 2.19%, 31 events) when compared with ablative therapy (8.28% ± 2.46%, 81 events). 147 patients (13.4%) reported no side effects, 68 (6.22%) reported expected, transient self‐resolving events, and five (0.046%) presented with hypertrophic scarring. Excluding transient events, ablative lasers had fewer complications overall when compared with nonablative lasers (2.56% ± 2.19% vs 7.48% ± 3.29%). This systematic review suggests ablative laser use for skin resurfacing is a safe and effective modality to treat a range of pathologies from photodamage and acne scars to hidradenitis suppurativa and posttraumatic scarring from basal cell carcinoma excision. Further studies are needed, but these results suggest that ablative lasers are a superior, safe, and effective modality to treat damaged skin.     

Occupational allergic contact dermatitis caused by decorative plants

12 cases of occupational allergic contact dermatitis caused by decorative plants were diagnosed in a 14-year period. The patients were middle-aged, and their average exposure time was 13 years. The plant families and plants causing occupational contact dermatitis were Compositae (5 patients: chrysanthemum, elecampane, gerbera, feverfew), Alstroemeriaceae (5 patients, Alstroemeria ), Liliaceae (4 patients; tulip, hyacinth). Amaryllidaceae (2 patients: narcissus) and Caryophyllaceae (2 patients; carnation, cauzeflower). The known chemical allergens causing dermatitis were tuliposide-A and sesquiterepene lactones, such as alantolactones and parthenolide, in the Liliaceae and Compositae families. 7 of the 12 patients were able to continue their work; 5 were not because of severe relapses of skin symptoms. The plant allergen and extract series currently available are of great help in the diagnosis.     

Deutliche Einschränkung der Lebensqualität bei Patienten mit Pemphigus vulgaris: Ergebnisse der deutschen Bullous Skin Disease (BSD)‐Studiengruppe

Background: Pemphigus vulgaris is a potentially life‐threatening autoimmune disorder of the skin and mucous membranes characterized by antibodies against epidermal adhesion molecules. Clinically characteristic are painful chronic blisters or erosions of mucous membranes and skin. There are no published studies on the impact o this disease on quality of life. Patients and methods: This registration was performed within the scope of the German BSD (Bullous Skin Disease) study group, from November 1997 until January 2002. A total of 36 patients with the first diagnosis of pemphigus vulgaris were registered at the university hospitals of Dresden, Erlangen, Kiel, Mannheim, München and Würzburg. Thirty of the 36 (83 %) patients participated in the quality of life questionnaire utilizing the German version of ‘Dermatology Life Quality Index’ (DLQI) provided by A. Y. Finlay. The DLQI varies from 0 to 30 with an increased DLQI score indicating a decrease in quality of quality. Results: The overall DLQI total score of 10 ± 6,7 in the investigated pemphigus patients was significantly increased in comparison to other skin diseases. Conclusions: These results suggest that the DLQI can be a very useful additional outcome criteria for clinical studies with pemphigus vulgaris and in the treatment of these patients.     

Mutations in mevalonate pathway genes in patients with familial or sporadic porokeratosis

Porokeratosis comprises heterogeneous keratinization disorders that are characterized by one or more atrophic patches surrounded by a ridge‐like cornoid lamella. In this study, we evaluated seven families affected by porokeratosis and five sporadic patients of the disease in a Chinese population. We performed Sanger sequencing of exons and flanking intron–exon boundaries of mevalonate pathway genes (MVD, MVK, PMVK and FDPS) and of SLC17A9. In five familial and three sporadic patients, we detected six variations, including four novel mutations (MVD c.1A>G; p.Met1?, c.916G>A; p.Ala306Thr, c.1013+1G>A, and PMVK c.65A>G; p.Lys22Arg) and two recurrent mutations (MVD c.746T>C; p.Phe249Ser, and MVK c.1028T>C; p.Leu343Pro). We then applied I‐TASSER and iGEMDOCK to assess these variants for probable functional impacts. The findings of this study extend the mutation spectrum of porokeratosis and provide further evidence for the genetic basis of this disease.     

[Translated article] Pruritus in Dermatology: Part 1—General Concepts and Pruritogens

Pruritus is the most common symptom of dermatologic and systemic diseases. The diagnosis of pruritus is clinical, although additional tests may be necessary to identify or confirm the cause. Translational medicine has led to the discovery of new mediators of itch, or pruritogens, as well as new receptors. Knowing how to properly recognize the main pathway that mediates itch in each patient is the key to successful treatment. Although the histaminergic pathway predominates in conditions like urticaria or drug-induced pruritus, it is the nonhistaminergic pathway that predominates in nearly all other skin diseases covered in this review. Part 1 of this 2-part review discusses the classification of pruritus, additional testing, the pathophysiology of itch and the pruritogens implicated (including cytokines and other molecules), and central sensitization to itch.     

Anästhesieverfahren in der Dermatologie

Zusammenfassung Die Verfahren der Lokalanästhesie sind integraler Bestandteil der operativen Dermatologie. Sie gewährleisten eine effiziente und sichere Analgesie in umschriebenen Haut- und Weichteilregionen und ermöglichen, einen sonst schmerzhaften diagnostischen oder therapeutischen Eingriff bei erhaltenem Bewusstsein zu tolerieren. Einzelne Methoden der Applikation sind "konkurrenzlos", wie die topische Applikation von EMLA^® oder die Kryoanästhesie, andere bieten alternative Optionen zur Allgemeinanästhesie. Die Tumeszenzlokalanästhesie wurde—jenseits der kosmetischen Liposuktion—zu einer effizienten Anästhesieform für größere Operationen bei Tumoren der Haut, plastische Rekonstruktionen und in der Phlebochirurgie weiterentwickelt. Die Wahl des Verfahrens im Einzelfall wird vom Alter, der Kooperationsfähigkeit und der Komorbidität des Patienten bestimmt. Für Infiltrationsanästhesien werden heute vorwiegend Lokalanästhetika vom Amidtyp eingesetzt. Fundierte Kenntnisse über die Anatomie der sensiblen Nerven sind Voraussetzung für erfolgreiche operationsfeldnahe periphere Blockaden. Wenn die Wirkungsweise der Lokalanästhetika, ihre toxischen Effekte und potenzielle Arzneimittelinteraktionen bei ihrem Metabolismus in der Praxis beachtet werden, dann ist das Risiko von Komplikationen relativ gering. Es sollte dennoch nicht unterschätzt, und adäquate Notfallmaßnahmen im Operationsteam sollten regelmäßig trainiert werden.