海姆泊芬光动力疗法对15例Sturge-Weber综合征患儿面部鲜红斑痣的疗效观察  

Efficacy of hematoporphyrin monomethyl ether-mediated photodynamic therapy in the treatment of facial port-wine stains in 15 children with Sturge-Weber syndrome

作　　者：张升[1] 王秀伟[2] 陈见友 邓维 张海花 张高磊[1] 刘晓雁[1] 苏伟[1] Zhang Sheng;Wang Xiuwei;Chen Jianyou;Deng Wei;Zhang Haihua;Zhang Gaolei;Liu Xiaoyan;Su Wei(Department of Dermatology,Children's Hospital,Capital Institute of Pediatrics,Beijing 100020,China;Beijing Municipal Key Laboratory of Child Development and Nutriomics,Translational Medicine Laboratory,Capital Institute of Pediatrics,Beijing 100020,China)

机构地区：[1]首都儿科研究所附属儿童医院皮肤科,北京100020 [2]北京市儿童发育与营养学重点研究室首都儿科研究所转化医学研究室,北京100020

出　　处：《中华皮肤科杂志》2024年第7期616-622,共7页Chinese Journal of Dermatology

基　　金：国家自然科学基金(82073461)。

摘　　要：目的观察海姆泊芬光动力疗法(HMME-PDT)治疗Sturge-Weber综合征(SWS)患儿面部鲜红斑痣的临床疗效及不良反应。方法回顾性分析2020年12月至2022年1月在首都儿科研究所附属儿童医院皮肤科行HMME-PDT治疗的SWS患儿临床资料。以5 mg/kg静脉注射HMME,采用532 nm LED光源照射治疗SWS皮损,治疗间隔8周。根据治疗前后红斑消退情况及皮肤镜视野内血管数量和密度变化情况,评价HMME-PDT治疗SWS的疗效;记录治疗后不良反应事件。采用Fisher精确检验法分析疗效差异。结果纳入15例SWS患儿,男7例,女8例,平均年龄4.74岁(1~14岁)。临床表型Ⅰ型10例,Ⅱ型5例;10例合并青光眼,6例合并癫痫,10例颅脑影像学检查异常。HMME-PDT治疗SWS皮损,15例中4例痊愈,3例显效,5例好转。8例接受2次治疗,1例显效,4例好转;7例接受≥3次治疗,4例痊愈,2例显效,1例好转;治疗≥3次患者痊愈和显效比例高于治疗2次的患者(均P<0.05)。7例为粉红型皮损,1例痊愈,2例显效,4例有效;4例紫红型,3例痊愈,1例显效;4例增厚型中1例有效;3种类型患者的显效和有效比例差异有统计学意义(P<0.05)。皮损累及面正中区14例,4例显效,2例有效;累及面外侧区15例,5例痊愈,3例显效,4例有效;面外侧区痊愈比例高于面正中区(P<0.05)。皮损皮肤镜下呈4种血管模式,3例为短棒状血管,4例为线状血管,5例网状血管,3例为混合型血管。短棒状血管痊愈3例;线状血管显效2例,好转2例;网状血管痊愈1例,显效1例,好转3例;混合型血管无效3例。4种血管模式的痊愈和有效患者比例差异有统计学意义(P<0.05)。所有患儿治疗后出现不同程度的疼痛、肿胀、紫癜和结痂反应,均未出现眼部及神经系统合并症加重情况。结论HMME-PDT治疗SWS患儿鲜红斑痣安全有效,疗效与治疗次数、皮损分型和部位相关。Objective To evaluate the clinical efficacy and safety of hematoporphyrin monomethyl ether(HMME)-mediated photodynamic therapy(PDT)in the treatment of facial port-wine stains(PWS)in children with Sturge-Weber syndrome(SWS).Methods A retrospective analysis was conducted based on the clinical data from SWS children treated with HMME-PDT at the Department of Dermatology,Children's Hospital,Capital Institute of Pediatrics from December 2020 to January 2022.HMME was intravenously injected at a dose of 5 mg/kg,followed by the irradiation of SWS lesions with a 532-nm light-emitting diode light source,and the treatment interval was 8 weeks.The efficacy of HMME-PDT for SWS was evaluated based on the subsidence of erythema and changes in the number and density of blood vessels under a dermoscope before and after treatment;adverse events after treatment were recorded.Fisher's exact test was used to analyze differences in efficacy.Results A total of 15 children with SWS were included,comprising 7 males and 8 females,with an average age of 4.74 years(range,1-14 years).There were 10 cases of clinical phenotypeⅠand 5 cases of typeⅡ;10 patients were accompanied by glaucoma,6 by epilepsy,and 10 showed abnormalities on craniocerebral imaging.After HMME-PDT treatment,4 out of 15 patients achieved complete remission of SWS lesions,3 showed marked improvement,and 5 achieved improvement.Among 8 cases receiving 2 sessions of treatment,1 achieved marked improvement and 4 showed improvement;among 7 cases receiving 3 or more sessions of treatment,4 achieved complete remission,2 achieved marked improvement,and 1 showed improvement;the proportions of patients achieving complete remission and marked improvement were significantly higher among those receiving 3 or more sessions of treatment than those receiving 2 sessions(both P<0.05).Among 7 patients with pink-type PWS,1 recovered completely,2 achieved marked improvement,and 4 showed improvement;among 4 patients with purplish-red-type PWS,3 recovered completely and 1 showed marked improv

关 键 词：STURGE-WEBER综合征 葡萄酒色痣 血卟啉光照疗法 儿童 海姆泊芬 光动力 疗效 安全性 

分 类 号：R758.61[医药卫生—皮肤病学与性病学]