系统性硬化症患者中的可溶性IL-2受体——临床和实验室的关系

作者用酶联免疫吸附试验对79例系统性硬化症(SSc)患者血浆中的可溶性白细胞介素2受体(sIL-2R)水平进行了测定.16名正常人血浆作对照.结果发现,SSc患者的sIL-2R水平(均值±SEM为866.0±63.6U/ml)显著高于正常人对照组(293.0±20.5U.ml,P<0.001),其中68     

系统性硬皮病血浆内皮素水平和肺纤维化的相关性研究

为了解系统性硬皮病(SSc)患者血浆中内皮素水平和肺纤维化之间的关系,对40例SSc患者作了胸部薄层CT扫描,参照CollinsCD评分法评出肺纤维化积分,并测定了血浆内皮素浓度,与40名正常人作了对照。对40例SSc患者的肺纤维化积分和其血浆内皮素值作了相关性分析。结果显示SSc组血浆内皮素浓度(80.09±37.91pg/mL)显著高于正常对照组(55.44±12.21),P<0.001。血内皮素浓度和肺纤维化积分呈显著正相关,r=0.61,P<0.001。结论:SSc患者具有高内皮素血症,与肺纤维化关系密切。     

系统性硬皮病患者血浆肾上腺髓质素及内皮素水平的测定

目的:探讨肾上腺髓质素(ADM)和内皮素(ET)在系统性硬皮病(SSc)发病中的作用。方法:采用放免法测定28例SSc患者和20名健康人的血浆ADM和ET-1水平。结果:SSc患者血浆ADM水平为(222.26±48.64)pg/mL,高于正常对照组(89.07±13.00)pg/mL,两组间比较,差异有显著性(P<0.01);SSc伴肺动脉高压者其血浆中ADM水平(254.73±39.36)pg/mL明显高于不伴肺动脉高压者(206.88±45.64)pg/mL,二者之间差异有显著性(P<0.01)。SSc患者血浆ET-1水平为(196.45±65.20)pg/mL,高于正常对照组(54.17±11.70)pg/mL,两组间比较,差异有显著性(P<0.01);SSc伴肺动脉高压者其血浆中ET-1水平(273.19±48.97)pg/mL明显高于不伴肺动脉高压者(160.11±31.17)pg/mL,二者之间差异有显著性(P<0.01)。血浆中ADM和ET-1水平呈正相关(r=0.5148,P<0.01)。结论:ADM和ET与SSc关系密切,可能在SSc发病和防治中具有重要的生理及病理学意义。     

阿维A联合血管扩张剂治疗6例系统性硬皮病疗效观察

目的：观察阿维A联合血管扩张剂治疗系统性硬皮病（SSc）的疗效和安全性。方法：收集2000-2005年住院和门诊就诊的6例SSc患者,主要使用阿维A0．5～0．6mg／（kg·d）和扩张血管药物等治疗。皮肤受累程度采用改良的Rodnan评分法评估,治疗后评分减少75％以上为临床显效。结果：6例患者中有5例伴有关节疼和雷诺征（83．33％）。另1例伴有吞咽困难（16．67％）,均无其他内脏受累;治疗前皮肤硬化程度评分平均为（22．00±6．96）分（12～30分）。治疗2周后,所有患者的关节疼、雷诺征及吞咽困难逐渐缓解,部分患者面部等处皮肤开始变软;8周后,所有患者的皮肤症状改善,皮肤硬化程度平均评分下降至（7．17±3．92）分（2～12分）,与治疗前比较差异有统计学意义（P〈0．05）。4例患者疗效显著,2例患者在治疗2周后出现肝功能异常、血脂升高。结论：阿维A联合血管扩张剂治疗SSc有效。     

低氧对系统性硬化病患者皮肤成纤维细胞胶原蛋白合成的影响

目的 研究低氧对系统性硬化病(SSc)患者和正常人皮肤成纤维细胞胶原蛋白合成及胶原基因表达的影响。方法 分别以5例SSc患者和5例正常人皮肤成纤维细胞系作为实验对象。将成纤维细胞分别放入20%正常氧和2%低氧分压环境中培养,用分光光度仪测定细胞培养基上清液中羟脯氨酸含量,并折算成胶原蛋白含量。用Trizol提取细胞总RNA,用RT-PCR方法测定其中Ⅰ型和Ⅲ型前胶原mRNA的表达。结果 培养至第6天,低氧分压条件下,SSc患者皮肤成纤维细胞培养基中胶原蛋白含量比正常氧分压条件下显著增加(t=3.97,P=0.0041);正常人皮肤成纤维细胞培养基中胶原蛋白含量此时也显著增加(t=2.63,P=0.0302)。培养至第3天,低氧分压条件下,SSc成纤维细胞Ⅰ型和Ⅲ型前胶原mRNA表达量比正常氧分压条件下均有显著增加(Ⅰ型:t=5.81,P=0.0004;Ⅲ型:t=6.44,P=0.0002);正常人皮肤成纤维细胞此时的Ⅰ型和Ⅲ型前胶原mRNA表达量与SSc患者相似,低氧分压条件下比正常氧分压条件下均有显著增加(Ⅰ型:t=4.40,P=0.0023;Ⅲ型:t=2.24,P=0.0453)。结论 低氧状态下,SSc患者和正常人皮肤成纤维细胞胶原蛋白合成均有增加,Ⅰ型和Ⅲ型前胶原mRNA表达也均增加.低氧可能是皮肤硬化的一个相关因素。     

男性系统性硬皮病的临床分析

我们对我院 1958～ 1997年期间,资料较完整的 22例男性系统性硬皮病 (SSc)的发病情况、临床表现及实验室检查作分析研究,报道如下。 一、临床资料 (一 )一般资料： 131例 SSc均为我院住院患者,均符合 SSc诊断标准。 131例中男 22例 (17％ ),年龄 19～ 72岁,平均 44.1岁;病程 11 d至 19年,平均 3.9年,其中 15例 (68％ )病程 4～ 8个月。 22例中弥漫性 14例 (64％ )、肢端性 8例 (36％ ),女性 SSc 109例,年龄 14～ 72岁,平均 38.8岁;病程 1个月至 33年,平均 5.3年, 1～ 4年为最多,共 58例 (53％ )。弥漫性 14例 (13％ )…     

中波高能紫外线最小红斑量值测定

目的探讨中波高能紫外线最小红斑量值(MED)范围及其与年龄、性别、皮肤日光反应类型的关系。方法以Dualight targeted Phototherapy System UV120-2的UVB作为照射光源,测定73名健康志愿者和35例白癜风患者腹部正常皮肤的MED值范围。结果108名受试者MED值为(189.17±56.156)mJ/cm2,范围为90～330mJ/cm2;Ⅲ型皮肤为(155.88±34.996)mJ/cm2(90～210mJ/cm2),Ⅳ型皮肤为(218.95±54.957)mJ/cm2(120～330mJ/cm2),Ⅲ型显著低于Ⅳ型(P<0.01)。男性受试者中,Ⅲ型为(154.29±39.443)mJ/cm2(90～210mJ/cm2),Ⅳ型为(224.4±54.854)mJ/cm2(150～330mJ/cm2),男性Ⅲ型显著低于Ⅳ型(P<0.01)。女性受试者中,Ⅲ型为(157±32.179)mJ/cm2(90～210mJ/cm2),Ⅳ型为(214.69±55.532)mJ/cm2(120～330mJ/cm2),女性Ⅲ型也显著低于Ⅳ型(P<0.01)。两性别组间和各年龄组间比较无统计学差异(P>0.05)。结论中波高能紫外线的MED值与性别和年龄无直接关系,皮肤日光反应类型是影响其重要因素。     

色素障碍性皮肤病

20 0 30 35 1　白癜风患者血浆与皮肤疱液降钙素基因相关肽测定 /赵达明 (大连医大一院皮肤科 )…∥临床皮肤科杂志 .- 2 0 0 2 ,31(4 ) .- 2 2 8～ 2 2 9通过对 4 0例白癜风患者血浆及其中 2 5例皮肤疱液降钙素基因相关肽 (CGRP)的测定 ,认为寻常型白癜风 CGRP血浆浓度升高 (91.3± 2 4 .7pg/ml) ,其中进展期 (94 .3± 2 2 .2 pg/ml)高于稳定期(78.8± 2 3.7pg/ml) ,而节段型 (78.2± 19.4 pg/ml)则无明显变化。皮肤疱液白斑与非白斑处无明显差异 (P>0 .0 5 )。提示 CGRP可能与白癜风的发病存在一定关系。表 3参 9　 (汤亚娥 )2 0 0 …     

硬皮病肾危象四例

系统性硬化(SSc)是一种以皮肤硬化为主且伴有多脏器损害的自身免疫性结缔组织病。硬皮病肾危象(sclerodermarenalcrisis,SRC)是SSc内脏损害中最危急的并发症之一。我们分析了我院1980～1995年来住院的4例SRC患者。一、临床资料65例SSc住院患者中6例有肾脏损害,4例符合SRC诊断标准[1]:突发性舒张压≥1.33kPa,伴随以下任意两个症状:1高血压眼底或级改变;2癫痫发作;3蛋白尿;4血尿;5微血管病性溶血;6氮质血症;7高肾素血症;或舒张压正常,发生非其它原因的快速进行性肾衰,且伴随以上7项症状中2项者亦可诊为SRC。4例SRC患者中男1例,女3例;…     

老年性瘙痒症患者皮肤功能状态的一些电生理研究

作者对88名(男40名,女48名)年龄60～90岁的老年性瘙痒症患者作了皮肤功能状态电生理研究。疗前检查64名,疗后复查24名。另选15名同龄范围健康正常人(男9名,女6名)作对照,并与文献中同类指标作了比较,后者多从健康年轻人中测定,因此有年龄差别.检查项目及结果如下:皮肤温度(℃):老年人处于界限或略低于正常。对照组为31.2±0.03,比文献资料低。患者疗前31.09±0.59,疗后无显著改变。热觉阈(℃):对照组为34.8±0.9.患者疗前39.53±1.44,明显增高,疗后下降为36.0±1.56,     

Treatment of Systemic Sclerosis

Systemic sclerosis is an extremely variable disease in its manifestations and consequently, treatment needs to be individualized depending on the specific problems that each patient has. Limited scleroderma patients have a prolonged duration of Raynaud’s phenomenon and puffy fingers before they develop any skin thickening, digital ulcers or gastrointestinal symptoms. They are likely to present with all the classic manifestations of scleroderma. Diffuse scleroderma patients have a much more acute systemic onset with marked whole hand swelling and may initially have only subtle skin thickening. A good understanding of the differences between the natural history of limited and diffuse scleroderma will enable the physician to treat present problems and anticipate future ones more effectively. One should determine which major subset and organ systems are involved before deciding on the appropriate therapy. Advances in organ-specific therapy, particularly calcium channel antagonists in Raynaud’s phenomenon, proton pump inhibitors in esophageal reflux, intravenous iloprost and endothelin receptor antagonists in pulmonary hypertension, and ACE inhibitors in renal crisis, have decreased morbidity and mortality in patients with scleroderma. Studies of aggressive therapies to prevent or improve pulmonary fibrosis are in progress. Further clinical experience in wound healing, gastrointestinal malabsorption and physical therapy for loss of motion has helped patients to have a more comfortable life. In recent years, a significant number of controlled clinical trials have been performed and there has been improved understanding of the best way to perform studies and of which patients are most likely to respond to therapy. Penicillamine, methotrexate, photopheresis, relaxin, interferons, and cyclosporine have all been studied in controlled trials with variable outcomes. Although an overall remittive therapy has not yet been determined, new, potentially useful agents are being investigated.     

Esophageal Injection Sclerosis

Hemorrhage from esophageal varices is a serious complication of portal hypertension and cirrhosis, as evidenced by a 50-60% mortality rate and a 40-60% rate of recurrent hemorrhage. Esophageal injection sclerosis (EIS) has emerged as the preferred mode of therapy for esophageal hemorrhage and in most respects is superior to surgical portacaval shunt and medical therapy. EIS controls variceal bleeding acutely in 85-95% of cases and results in the long-term obliteration of varices in 60-80% of patients. EIS decreases the time the patient spends in the hospital, reduces the amount of blood transfused, and may prolong the patient's survival. Complications of ulceration and stenosis can be minimized by proper choice of agent and by manipulating the volume and concentration of sclerosing agent, the interval between sessions, and the pattern of injections within the esophagus. EIS is both effective and reasonably safe for the acute and long-term management of esophageal variceal hemorrhage and can easily be performed at most medical centers in this country.     

结节性硬化症

<正>患者男,14岁,广东惠州人。主诉:颜面、腰骶部渐进性增多皮疹5年。现病史:5年前,患者于左鼻翼部出现一米粒大小肤色丘疹,左侧臀部出现一约1.5 cm×0.5 cm大小长条状淡黄色软斑块,无痛痒,未予相关处理。后逐渐于鼻翼两侧、双面颊及下颌出现散在对称性分布的针尖至粟粒大小暗红色丘疹,伴双侧鼻唇沟出现绿豆至黄豆大小丘疹。原臀部黄色软斑块亦逐渐增至鸡蛋大     

结节性硬化症

图1 鼻翼两侧及双脸颊、下颌、颈部出现散在针尖至粟粒大小暗色毛细血管扩张性坚韧丘疹,伴双侧鼻唇沟绿豆至黄豆大小菜花样丘疹;图2左侧腰骶部见约4cn·×5CIn大小不规则增厚性鲨鱼皮样淡黄色软斑块,周围见沿皮纹走向的散在约l～2cm大小条叶状白色丘疹;图3鼻翼旁丘疹组织病理示：真皮浅层毛细血管增生、扩张,胶原纤维组织明显增生（HE,100×）;图4腰骶部斑块组织病理示：真皮内胶原纤维增生明显,浅层血管周围稀疏淋巴组织细胞浸润（HE,40×）     

结节性硬化症1例

1 临床资料 患者,男,21岁,因全身皮疹20年于2009年8月到本中心门诊部诊治.1岁多时无明显诱因面部出现丘疹,躯干四肢出现皮赘,以后逐渐增多,扩大.面部皮损增至米粒大小,呈淡红或暗红色,躯干、四肢皮疹增至黄豆大小或拇指头样斑块,呈肤色或红黄色;3岁时背腰部出现局限性色素脱失斑,无自觉症状.     

Pseudovitiligo in Systemic Sclerosis

Progressive Systemic Sclerosis is a chronic connective tissue disease characterized by sclerosis of skin with involvement of the internal organs. The cause of PSS is unknown but autoimmune mechanisms and primary damage to blood vessels have been proposed to explain its pathogenesis. We report a case of PSS with widespread depigmentation resembling vitiligo.     

典型结节性硬化症1例

患者男,19岁。从3岁起,面部、四肢、躯干、齿龈、阴囊先后出现皮脂腺瘤、甲周纤维瘤、鲛鱼皮斑、卵圆形或条状叶形白色斑、齿龈纤维瘤、弥漫性白发、阴囊多发性脂囊瘤,以及癫痫、大脑内钙化结节、双侧肾多发性错构瘤。诊断:结节性硬化症。     

Tuberous Sclerosis: Unusual Associations

Uncommon associations of tuberous sclerosis, ichthyosis vulgaris, portwine stain, and spina bifida occulta are reported in a 14-year-old female.