重症药疹74例临床分析

目的探讨重症药疹的发生规律、临床特点和治疗措施。方法对1998～2011年我科收治的74例重症药疹患者的临床资料进行回顾性分析。结果重症药疹以重症多形红斑型药疹(SJS)多见,占48.6%(36/74)。别嘌呤醇在致敏药物中居首位,占21.6%(16/74),其次是卡马西平,占18.9%(14/74)。别嘌呤醇的潜伏期最长,且肝肾损害率高,分别为87.5%和50.0%。四种类型中SJS与中毒性表皮坏死松解型药疹(TEN)的黏膜损害率均为100%,药物超敏反应综合征(DRESS)的黏膜损害率最小为28.6%。DRESS的发病年龄比剥脱性皮炎型药疹(ED)小,DRESS的潜伏期较TEN与SJS长。TEN的日最大糖皮质激素用量平均值为4.13mg/kg,均高于其他三组,其住院时间较SJS长,平均为23.09d。TEN的并发症为85.7%,高于其他三组,死亡率高。结论致敏药物的种类、药疹类型与病情的严重程度密切相关,别嘌呤醇与卡马西平应用需谨慎,激素联合免疫球蛋白(IVIG)治疗有效。     

62例皮肤口腔黏膜综合征及中毒性表皮坏死松解型药疹的护理

[目的]总结62例皮肤口腔黏膜综合征(SJS)及中毒性表皮坏死松解型药物疹(TEN)患者的护理经验.[方法]本院确诊为SJS或TEN的患者62例,给予糖皮质激素、静脉滴注免疫球蛋白(IVIG)、支持治疗及规范的专科护理,根据SCORTEN评分系统评价病情严重程度及预后.[结果]引起SJS/TEN最常见的药物为非甾体类抗炎药(26例)、抗癫痫药(15例)、抗生素类(13例)、中药(3例)、别嘌呤醇(2例),乙型肝炎疫苗(1例),不详2例.患者SCORTEN评分平均(0.97士1.15)分,预期病死率9.13％(6/62),实际病死率3.23％(2/62).1例TEN皮损愈合后死于艾滋病(SCORTEN评分3分),1例TEN患者死于大肠埃希菌败血症(SCORTEN评分5分).[结论]对TEN/SJS标准规范的专科护理能明显降低病死率.     

糖皮质激素联合人免疫球蛋白治疗Stevens-Johnson综合征和中毒性表皮松解症

  目的  探讨Stevens-Johnson综合征(Stevens-Johnson syndrome, SJS)和中毒性表皮松解症(toxic epidermal necrolysis, TEN)的临床特征及治疗方法, 以提高对SJS/TEN的认识。  方法  回顾性分析北京协和医院7例SJS/TEN患者的临床表现、疾病严重程度、眼部受累程度、致敏药物、治疗方案及临床结局。  结果  患者年龄26~81岁, 均伴有黏膜损害, 致敏药物以非甾体类抗炎药、抗惊厥药及别嘌醇为主, 7例均使用大剂量糖皮质激素联合人免疫球蛋白治疗, 治愈6例, 死亡1例。  结论  SJS/TEN是一组累及全身皮肤黏膜严重威胁患者生命的疾病, 病死率高, 早期足量使用大剂量糖皮质激素联合人免疫球蛋白为主要的治疗手段, 伴有系统疾病的患者预后较差。眼部损伤与疾病严重程度具有不同步性, 需及早给予针对性治疗。     

基于NRS2002 TEN和SJS患者的营养风险筛查

目的 基于欧洲营养风险筛查工具(NRS2002)对中毒性表皮坏死松解症(TEN)和Stevens-Johnson综合征(SJS)患者进行营养风险筛查,研究TEN和SJS患者营养风险的发生率,探讨此类患者发生营养风险的危险因素。方法 回顾性调查2011年1月至2021年3月在本院住院的临床资料完整的TEN和SJS患者215例,按受累皮肤的体表面积(BSA)予以区分TEN、SJS、SJS/TEN重叠综合征。同时基于NRS2002进行营养风险筛查评分,探讨TEN和SJS患者存在营养风险的危险因素。结果 TEN和SJS患者入院时营养风险的发生率为64.19%(138/215)。其中,年龄(χ²=7.198,P<0.01)、SCORTEN((χ²=18.843,P<0.01)、致敏药物((χ²=13.169,P<0.01)、皮损面积(χ²=119.245,P<0.01)、黏膜受累部位(χ²=8.241,P<0.01)对于TEN和SJS患者发生营养风险有统计学...     

别嘌呤醇引起皮肤不良反应与HLA-B~* 5801基因相关性研究

目的探讨别嘌呤醇引起的皮肤不良反应与HLA-B*5801基因基因的相关性。方法通过收集本院服用别嘌呤醇患者及因服用别嘌呤醇引起不同程度皮肤不良反应的的病例,检测其HLA-B*5801基因的携带情况,分析别嘌呤醇引起的皮肤不良反应与HLA-B*5801基因的相关性。结果服用别嘌呤醇患者的HLA-B*5801基因携带率为11.9%,发生严重皮肤不良反应患者的该基因携带率高达50%(χ2=20.51,P0.05),出现Stevens-Johnson综合征(SJS)和中毒性表皮坏死松解(TEN)的患者100%携带有HLA-B*5801基因。结论别嘌呤醇引起的严重皮肤不良反应与HLA-B*5801基因有密切相关,建议首次服用别嘌呤醇患者检测HLA-B*5801基因。     

中毒性表皮坏死松解症和Stevens-Johnson综合征

中毒性表皮坏死松解症(toxic epidermal necrolysis,TEN)和Stevens-Johnson综合征(Stevens-Johnson syndrome,SJS)是累及皮肤和黏膜的严重药物不良反应,由大范围的角质形成细胞坏死引起,导致表皮-真皮交界处发生皮肤剥脱,外观如烫伤。TEN和SJS因为潜在的致命性被归为医疗急症范畴。现就流行病学、临床特征、治疗以及预后等方面对TEN和SJS作一综述。     

中国南方汉族人群中卡马西平导致SJS/TEN与HLA-B基因的相关性

目的:探讨中国南方汉族人群中卡马西平(CBZ)所致Stevens-Johnson综合征/中毒性表皮坏死松解症(SJS/TEN)与HLA-B基因的相关性。方法:采用病例对照研究,收集11例CBZ-SJS/TEN患者(CBZ-SJS/TEN组)、93例CBZ耐受患者(CBZ耐受组)及93例未服用抗癫痫药物的正常人(正常对照组)。PCR和测序法检测其HLA-B*1502基因型;χ2检验分析HLA-B*1502及其他HLA-B位点与CBZ-SJS/TEN的相关性。结果:CBZ-SJS/TEN组的HLA-B*1502基因阳性率为72.7%,显著高于CBZ耐受组及正常对照组。3例HLA-B*1502阴性CBZ-SJS/TEN患者的基因型分别为HLA-B*1511/1511、5401/5401及4001/4601。除HLA-B*1502之外,HLA-B*1511基因频率在CBZ-SJS/TEN患者与中国南方汉族正常人群之间具有显著的统计学差异,其他位点差异均无显著性。结论:在我国南方汉族人群中HLA-B*1502与CBZ-SJS/TEN具有相关性,建议汉族人群在服用CBZ前应该进行基因型检测。同时,对HLA-B*1502阴性个体使用CBZ治疗时,需密切监视,避免SJS/TEN的出现。     

重症多形红斑及中毒性表皮坏死松解症治疗的研究进展

<正>Stevens-Johnson综合征(SJS)和中毒性表皮松解症(TEN)是一组主要由药物引起的,少见的累及皮肤黏膜的严重疾病谱,目前普遍认为SJS和TEN是这一疾病谱表现的两个极端。临床上主要根据皮损形态学特点以及表皮剥脱的程度来进行区分。目前常见的治疗手段包括:及早、足量使用糖皮质激素,防止继发感染,加强支持疗法,静脉注射人血丙种免疫球蛋白,必要时行血浆置换。其中,最重     

1例Stevens-Johnson综合征合并糖尿病酮症酸中毒患者的护理体会

正Stevens-Johnson综合征(SJS)又称重症多型红斑,与皮肤松解症(TEN)是一组严重的皮肤黏膜反应,临床特点为水疱、表皮剥脱和多部位的黏膜炎症,常伴有系统功能的紊乱[1],引起SJS/TEN的致敏药物以抗癫痫药(卡马西平)、解热镇痛药、抗生素、中药、抗痛风药等为常见[2]。糖尿病酮症酸中毒(DKA)是糖尿病最常见的急性并发症之一,主要生化表现为血糖异常升高,血、尿酮体升高以及代谢性酸中毒。DKA常见易感因素是感染,漏注胰岛素     

苯溴马隆联合别嘌呤醇治疗痛风稳定期的临床疗效分析

目的分析苯溴马隆联合别嘌呤醇治疗痛风稳定期的临床疗效。方法回顾性选取沈阳245医院2015-03—2016-04收治的痛风稳定期113例患者临床资料,根据治疗时所用不同方法分成两组,对照组51例单一采用别嘌呤醇治疗,观察组62例采用苯溴马隆联合别嘌呤醇治疗,对两组临床疗效、血尿酸检测值及不良反应发生情况进行对比。结果治疗后观察组血尿酸水平为(306.98±30.65)μmol/L,对照组为(351.62±34.25)μmol/L,观察组血尿酸水平下降幅度优于对照组(P0.05);治疗后观察组血小板、白细胞分别为(236.96±36.45)×109/L、(6.12±0.48)×109/L,对照组分别为(115.65±30.98)×109/L、(3.83±0.59)×109/L,观察组血小板、白细胞数值较对照组更接近正常值,两组下降幅度比较差异具有统计学意义(P0.05);两组均未出现严重不良反应,两组不良反应发生率4.84%、7.84%比较差异无统计学意义(P0.05)。结论痛风稳定期患者采用苯溴马隆联合别嘌呤醇治疗效果显著,降低血尿酸水平,且较为安全可靠。     

Morbidity and mortality of mucocutaneous diseases in the pediatric population at a tertiary care center.

Toxic epidermal necrolysis (TEN) is a life threatening desquamating disease that is often an adverse reaction to drugs. Because mortality is so high, up to 30% nationally, and the morbidity significant, these cases are managed in burn centers. This study was conducted to evaluate what drugs were given to children who developed exfoliating skin disease and to identify the complications that these patients suffered. Thirty-two pediatric cases of erythema multiforme, Stevens-Johnson syndrome (SJS), and TEN were identified during a period of 8 years in which the average number of admissions to the burn center was 200 per year. Age, sex, drug history before admission, drug treatment during hospital stay, and clinical outcomes were noted. Several drugs were identified as probable causative agents. The most common cause of exfoliating disease was a combination of azithromycin and ibuprofen, followed by ibuprofen alone. Notably, the combination of ibuprofen and another drug was responsible for four additional cases, making the total percentage of pediatric cases involving ibuprofen 47%. Although no children died, several children with TEN and SJS suffered severe ocular involvement, sepsis, pneumonia, and genitourinary complications. All of the children who experienced complications had received ibuprofen. Chi-square analysis showed the correlation between ibuprofen and complications to be statistically significant (<0.05). This association was not observed with any other drug administered. Not only is ibuprofen a potential etiologic agent of exfoliating skin disease in children, it also may contribute to the development of complications in pediatric patients with the disease. Although this association does not prove that ibuprofen is the definitive cause of complications in these cases, caution is advised when giving this drug to children with suspected erythema multiforme, SJS, and TEN.     

HLA‐B*1502 Strongly Predicts Carbamazepine‐Induced Stevens–Johnson Syndrome and Toxic Epidermal Necrolysis in Thai Patients with Neuropathic Pain

Background: Carbamazepine (CBZ) is one of the standard pharmacological treatments for neuropathic pain. However, its serious adverse drug reactions include Stevens–Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN). Recently, HLA‐B*1502 allele was implicated as a genetic marker of CBZ‐induced SJS/TEN in some Asian epilepsy populations. Methods: This is a case control study to describe the clinical characteristics of SJS/TEN in Thai patients with neuropathic pain who were treated with CBZ, and to determine the association of HLA‐B*1502 in these patients, comparing with those who exposed to CBZ for at least 6 months without any cutaneous reactions. Results: Thirty‐four SJS/TEN patients and 40 control patients were included in this study. Mean age of SJS/TEN patients was 47 years. SJS/TEN was developed in 10.8 ± 1.4 days after initiation of CBZ. HLA‐B*1502 allele was found in 32 of 34 SJS/TEN patients (94.1%) but it was found only in 7 of 40 control patients (17.5%). The association was very strong with an odds ratio of 75.4. Sensitivity and specificity of this HLA‐B*1502 genotype test were 94.1% and 82.5%, respectively, while the positive predictive value and negative predictive value were 1.43% and 99.98%, respectively. Positive and negative likelihood ratios were 5.37 and 0.07, respectively. Conclusions: HLA‐B*1502 is a strong genetic marker for CBZ‐induced SJS/TEN in Thai patients with neuropathic pain. The screening for this marker should be performed prior to initiation of CBZ treatment to assess the risk of this serious side effect.     

Atteintes bronchopulmonaires au cours des toxidermies graves

Skin is one of the most common targets of adverse drug reactions. Life-threatening skin adverse reactions include Stevens-Johnson syndrome (SJS), toxic epidermal necrolysis (TEN), and drug rash with eosinophilia and systemic symptoms (DRESS). SJS and TEN are characterized by skin and mucous membrane detachments. Respiratory complications occur in about 40% of the cases, and can be related to specific bronchial epithelial injuries as well as to nonspecific manifestations like infection, pulmonary edema, and atelectasis. Mechanical ventilation is required in about 25% of cases. Bronchoscopy is warranted in mechanically ventilated patients to remove detached mucous membranes and prevent airway obstruction. Pulmonary involvement in DRESS is rare and related to lung infiltration by eosinophils, which rarely leads to the acute respiratory distress syndrome. The most severe cases of DRESS usually present other organ involvement like cytolytic hepatitis and are usually reversible after steroid treatment. Removal of the involved drug is warranted.     

CHOP与CHOP—L方案化疗对45例外周T细胞淋巴瘤非特异型疗效及预后分析

目的：探讨外周T细胞淋巴瘤非特异型CHOP与CHOP-L方案化疗疗效比较及预后因素分析。方法对我院2008年1月至2012年6月经病理及免疫组化诊断明确外周T细胞淋巴瘤非特异型45例患者的临床资料进行回顾性分析。研究近期化疗疗效及影响疗效相关因素及预后分析。结果45例4疗程化疗后疗效：11例CR（16%）,20例PR（54%）,5例SD（12%）,8例PD（18%）,总反应率（ORR）为70%。原发耐药（2疗程后未缓解）4例。CHOP方案Ⅰ～Ⅱ度骨髓抑制11例（68.7%）,Ⅲ～Ⅳ度5例（31.3%）;CHOP-L方案Ⅰ～Ⅱ度骨髓抑制16例（55%）,Ⅲ～Ⅳ度13例（45%）。CHOP-L方案及CHOP方案均有少数患者出现肝功能异常、心脏毒性及胰腺酶异常,但 CHOP-L 方案副反应发生率无明显高于CHOP方案组。化疗疗效与年龄、LDH水平、ECOG、IPI评分、化疗方案及KI-67相关,有统计学意义（P＜0.05）。临床分期、B组症状、LDH水平及IPI评分与患者PFS有关（P＜0.05）。完全缓解患者1年和3年总生存率分别为84.6%、16.1%;部分缓解患者1年OS为50.2%,3年OS为4.4%。多因素分析临床分期（P＜0.05）是本组病例的独立预后因素。结论 PTCL-U 患者常规化疗近期疗效较好,CHOP-L 方案化疗有效率高于CHOP方案,两组不良反应率差异不明显。临床分期是PTCL-U的独立预后因素。     

Overlap Stevens Johnson Syndrome/Toxic Epidermal Necrolysis developed due to the use of toxic-dose vinblastine in case of Langerhans Cell Histiocytosis(Letterer-Siwe)

Except for side effects expected standart dose use of the chemotherapeutics agents, toxic effects (poisoning) may occur if high doses of are mistakenly used in the treatment of haemato-oncological diseases and these toxic doses are usually fatal. Here, we report a case of Stevens Johnson Syndrome (SJS) / Toxic Epidermal Necrolysis (TEN) following administration of toxic dose of vinblastine by mistake. A 20-month-old male patient with a diagnosis of Langerhans Cell Histiocytosis (Letterer-Siwe) at the pediatric oncology department was admitted to intensive care unit, after having received treatment protocol consisting of vinblastine, etoposide and prednisolone, with fever, altered consciousness and decompensated shock findings. Skin biopsy which performed from bullous lesions in the perianal, neck and axillary regions was resulted compatible with SJS / TEN in the patient with multiple organ failure, at 48 h of admission. It was later determined that the patient has been mistakenly given 10 times the normal dose of vinblastine he needed (60 mg/m²), which was 6 mg/m². Plasma exchange was performed 3 times for vinblastine toxicity, intravenous immunoglobulin was administered for SJS / TEN therapy and phenobarbital was initiated to increase drug metabolism. The patient whose clinical picture fully improved, was transferred to the oncology department on the 30th day of intensive care hospitalization. Vinblastine toxicity is a life-threatening condition that can cause multiple organ failure, SJS / TEN. Plasma exchange is an effective treatment method for the removal of vinblastine from the body and in these cases of toxicity.     

儿童肺炎支原体肺炎200例临床特征分析

【目的】探讨儿童肺炎支原体肺炎（MPP）的临床特点以及肺外并发症的表现,为MPP患儿的-临床诊断及治疗提供依据。【方法】选取本院2010年1月至2013年1月收治的MPP患儿200侧为研究对象,回顾性分析患儿的临床资料、并发症发生情况及转归情况,选取普通上呼吸道感染患儿100例为对照1组,同时选取100例正常体检儿童为对照2组,对比分析三组儿童血清白细胞介素-6（IL-6）、白细胞介素-17（IL-17）、肿瘤坏死因子-α（TNF-α）的变化情况。【结果】MPP患儿患病年龄0-2岁32例（16．00％）,3～6岁88例（44．00％）,7～10岁48例（24．00％）,11～13岁32例（16．00％）。MPP患儿肺外并发症主要为心肌缺血45例（22．5％）、神经系统疾病22例（11．0％）、肝肾损害15例（7．5％）、皮肤黏膜损害10例（5．0％）以及消化系统损害9例（4．50％）。MPP组患儿血清中IL6、IL-17、TNF-α水平显著高于对照1组及对照2组,两两对比差异有显著性（PdO．05）。MPP患儿药敏实验结果证实对阿奇霉素敏感性好,未出现耐药情况,所有患儿均治愈出院。【结论】MPP主要以学龄儿童为主,患儿临床表现为多系统性器官损害。IL-6、IL-17、TNF-α等炎症因子水平的上升可能是导致MPP发生的重要因素,通过测定患儿炎症因子水平能有效对患儿病情做出诊断。     

经皮穿针与弹性髓内钉固定治疗青少年肱骨近端骨折的疗效比较

目的比较经皮穿针与弹性钉髓内固定治疗青少年肱骨近端骨折的临床疗效及安全性。方法对我院2006年5月至2013年6月采用钛制弹性钉髓内固定(TEN组)或经皮穿针固定(PP组)治疗并获得随访的113例青少年肱骨近端骨折患者的临床资料进行回顾性分析。其中采用钛制弹性钉髓内固定65例,经皮穿针固定48例。比较两组患者的手术资料、影像学结果、临床疗效及并发症情况。结果 TEN组患者手术时间(33.1±6.3)min、透视(8.2±2.7)次、骨折愈合时间(6.2±1.2)周;PP组患者手术时间(36.3±8.2)min、透视(11.6±4.1)次、骨折愈合时间(6.1±1.1)周。TEN组透视次数少于PP组,差异有统计学意义(t=2.198,P=0.029);两组患者手术时间、骨折愈合时间差异无统计学意义(t=1.418、0.527,P=0.230、0.668)。TEN组与PP组术后6周随访时Constant-Murley评分分别为(90.4±9.6)、(81.8±9.3)分,差异有统计学意义(t=3.016,P=0.003)。两组患者术后3个月Constant-Murley评分分别为(95.9±5.1)、(96.3±4.8)分,差异无统计学意义(t=0.364,P=0.815)。末次随访时,两组Neer-Horwitz分型及最大成角差异无统计学意义(χ2=0.372,P=0.830;t=0.672,P=0.504)。PP组总体并发症发生率高于TEN组,差异有统计学意义(χ2=21.660,P=0.000)。结论 TEN与PP固定均是治疗青少年肱骨近端骨折的有效方法。与PP相比,TEN固定具有透视次数少,功能恢复早,并发症少等优点。应对术者的经验以及患者的因素等综合考虑,选择合适的固定方式。     

Warfarin‐induced Stevens–Johnson syndrome with severe liver injury

Stevens–Johnson syndrome/toxic epidermal necrolysis (SJS/TEN) is a life-threatening mucocutaneous disease that is predominantly drug-induced. Warfarin is the most commonly used drug for long-term anti-coagulant therapy; however, warfarin-induced SJS/TEN is seldom reported. In this study, we presented the case of a 61-year-old man who developed SJS after receiving multiple-drug therapy following aortic valve replacement surgery. The patient was diagnosed with drug-induced liver injury (DILI) based on significantly abnormal liver function test results. Warfarin was identified as the culprit drug using the algorithm of drug causality for epidermal necrolysis (ALDEN) score, enzyme-linked immunospot (ELISPOT) assay, and Roussel Uclaf Causality Assessment Method (RUCAM). After warfarin discontinuation and corticosteroid therapy, the lesions and liver function test findings improved. Human leukocyte antigen typing was conducted to detect the risk allele. To our knowledge, this is the first reported case of warfarin-induced SJS/TEN with DILI. This case suggests that commonly used and safe pharmaceutical agents such as warfarin can potentially cause serious adverse events, including SJS/TEN and DILI. The application of ALDEN, the ELISPOT assay, and RUCAM could be useful in identifying culprit drugs.