婴幼儿血管瘤病理结构变化与临床演变过程的联系

目的探讨婴幼儿血管瘤患者临床演变过程和病理结构变化的内在联系。方法选取52例婴幼儿血管瘤标本,年龄2个月至11岁。采用HE染色观察各阶段血管瘤的病理特点,使用图像分析系统分析血管瘤组织中的细胞总数量、微血管总数量和总面积。结果按患儿出生后时间:1~6个月,血管瘤中细胞增殖速度快、排列紧密,微血管数量迅速增加;7~12个月,血管瘤中细胞数量迅速减少,微血管数量减少,但总面积继续扩大;1~3岁,大部分血管瘤组织呈微血管团样,微血管面积最大;3~5岁,微血管数量和总面积均迅速减少;5岁之后,大部分血管瘤消退完成,被纤维脂肪组织代替。结论婴幼儿血管瘤患者临床演变过程和病理结构变化有密切联系,病理结构变化主导临床演变过程。     

婴幼儿血管瘤病理演变过程的免疫机制

血管瘤是儿童期常见的良性肿瘤，以血管瘤内皮细胞增殖为主要特征，发病率1％～3％，好发于头面部。生后2～4周出现肿物，增长迅速，约6个月生长逐渐减慢，约至1岁左右进入消退期，80％患者7～12岁左右就基本或完全消退。目前，血管瘤的退化演变机制仍不完全清楚。     

婴幼儿血管瘤的起源及其病理演变机制

婴幼儿血管瘤是儿童期最常见的肿瘤，在白种人儿童中的发病率高达8％～10％，好发于头面部，在开始6个月中，增长非常迅速，此后，增长速度逐渐减慢，到1岁左右，开始进入消退期。增殖期血管瘤可能发生各种并发症，如溃疡、出血、感染，常可导致毁容后果，位于眼睑、结膜的血管瘤将影响视力，位于呼吸道则会导致呼吸障碍，     

不消退型先天性血管瘤与婴幼儿血管瘤的病理比较

目的 比较研究不消退型先天性血管瘤(noninvoluting congenital hemangioma,NICH)与婴幼儿血管瘤(infantile hemangioma,IH)的病理特征,检测葡萄糖转运蛋白-1(Glut-1)的表达,探讨NICH的病理结构与病程发展的关系.方法 2005年1月至2010年8月,收集13例NICH,13例增殖期IH和13例消退期IH临床标本,采用HE染色和免疫组化染色方法,比较NICH、增殖期IH和消退期IH的病理结构以及Glut-1的表达.结果 HE染色显示,NICH瘤体呈规则的小叶结构,小叶被丰富的纤维组织包绕,小叶内微血管管径较大,小叶内血管壁完整,内皮细胞罕有增殖和凋亡.IH病变呈现从增殖到消退改变,微血管管径较小,血管壁不完整,周细胞扁平,血管内皮细胞和间质细胞可见增殖和凋亡.NICH中不表达Glut-1,增殖期IH肿瘤内皮细胞中Glut-1表达阳性,消退期IH中Glut-1表达阴性或弱阳性.结论 NICH是一种具有低增殖性的血管瘤,病理结构稳定,与增生期IH血管内皮细胞的增殖性具有明显的不同;Glut-1可以作为鉴别NICH和增殖期IH的可靠抗原标记.

Abstract：

Objective To distinguish noninvoluting congenital hemangioma (NICH) and infantile hemangioma ( IH ) by comparing the pathological structure and marker antigen expression. Methods From Jan. 2005 to Aug. 2010,39 paraffin-embedded samples, including 13 cases of NICH, 13 cases of proliferating IH and 13 cases of involuting IH, were collected from operation. Hematoxylin-eosin staining was used to observe the pathological structure. Immunohistochemical analysis was also performed to investigate the expression of Glut-1. Results The lobules of capillaries were well-defined in NICH. The lobules were surrounded by abundant fibrous tissue. The capillaries were often large and integrity in NICH.There were few mitosis and apoptosis in endothelial cells and stromal cells in NICH. While in IH, the pathologic findings were totally different. Immunochemistry revealed that the Glut-1 was expressed in endothelial cells of IH, but not in NICH. Conclusions NICH has a steady histologic structure and low proliferation, while the endothelial cells in proliferative IH has a high proliferation. Glut-1 can be used as the reliable marker antigen for differential diagnosis of NICH and proliferative infantile hemangiomas.     

婴幼儿血管瘤中周细胞的分布及其演变

目的观察婴幼儿血管瘤中周细胞的分布、表型及其演变。方法52例婴幼儿血管瘤标本,应用α-SMA作为标记抗原观察血管瘤中周细胞的分布,应用透射电镜和TUNEL染色观察周细胞的凋亡状况。结果增生早期和中期,血管瘤细胞团中有大量周细胞,周细胞与内皮细胞共同进行微血管发生;增生晚期,血管瘤周细胞大量凋亡;消退早期,微血管周围有少数周细胞;此后微血管逐渐闭塞,血管瘤周细胞也逐渐消失。结论周细胞是血管瘤的主要组成细胞之一,血管瘤周细胞和内皮细胞共同进行微血管发生和发展而主导婴幼儿血管瘤的病理演变过程。     

小剂量普萘洛尔治疗婴幼儿血管瘤的临床观察

目的：探讨小剂量普萘洛尔治疗婴幼儿血管瘤的疗效及安全性。方法：收集增生期婴幼儿血管瘤23例（男6例,女17例）,口服普萘洛尔0.5～0.75 mg/（kg.d）,疗程1～9个月,并进行疗效评定和安全性评价。结果：疗效评定：优6例（26.1%）,良9例（39.1%）,中等8例（34.8%）;不良反应包括：心率轻度减慢8例（34.78%）,睡眠障碍2例（8.7%）,腹泻1例（4.3%）。不良反应轻,均1周内自行消失;安全性评定：15例为安全,8例为比较安全。结论：小剂量普萘洛尔治疗婴幼儿血管瘤,疗效良好,不良反应轻,安全性较好。     

口服普萘洛尔治疗严重婴幼儿血管瘤的临床观察

目的:评估普萘洛尔治疗严重婴幼儿血管瘤的临床疗效和安全性。方法:2010年9月～2012年9月,笔者科室对34例严重婴幼儿血管瘤患儿进行口服普萘洛治疗,服药剂量为第1天1.0mg/kg,第2天1.5mg/kg,第3天2mg/kg,12h 1次、分2次服用。服药后1周、1个月和停药时进行疗效评价,并进行随访。结果:所有患儿在口服普萘洛尔1周后瘤体得到控制,服药1月和停药时,97.1%(33/34)的患儿表现为促进消退。3例伴发溃疡的血管瘤患儿,溃疡在服药后1个月内均愈合。8.8%(3/34)患儿有腹泻、食欲减退的不良反应。26.5%(9/34)患儿停药后有复发倾向。结论:普萘洛尔治疗婴幼儿血管瘤疗效明显,不良反应小,可作为严重婴幼儿血管瘤的治疗方法之一。     

口服普萘洛尔治疗婴幼儿血管瘤的临床效果观察

目的:评价普萘洛尔治疗婴幼儿血管瘤的疗效。方法:按照严格的纳入、排除标准,成功筛选30例血管瘤患儿作为口服普萘洛尔的治疗对象,年龄为28天～10月,平均年龄4.8月。<3个月患儿,剂量1.5mg.kg-1.d-1;>3个月患儿,剂量2.5mg.kg-1.d-1,每天分2次服用。一般住院治疗5～7天,复查心电图、三大常规、肝肾功能、血糖、血脂及电解质等。如无异常予以出院带药。嘱患者出院后继续服药,用药前3个月,每2周复诊1次;3个月后每月复诊一次。结果:按四级评分法对药物疗效进行评估。治疗30例患儿,其中I级疗效有2例(差);Ⅱ级4例(中);Ⅲ级14例(好);Ⅳ级10例(优)。结论:口服普萘洛尔治疗婴幼儿血管瘤效果明显,副作用小,用药安全范围广,今后在临床上有望推广应用。     

大剂量普萘洛尔治疗严重婴幼儿血管瘤的初步临床观察

目的 初步探讨口服大剂量普萘洛尔治疗严重婴幼儿血管瘤的临床疗效和安全性.方法 2010年4月至2011年2月为56例严重的婴幼儿血管瘤患儿行口服普萘洛尔治疗,治疗前行全面的临床评估、心电图、血常规、肝功能、心肌酶和肌钙蛋白检查,治疗时口服普萘洛尔剂量逐渐增加,前3 d的剂量分别为每天1 ms/kg、1.5 mg/kg、2 mg/kg,每天按12 h 1次,分2次,喂奶后半小时服用,住院6 d,患儿无异常表现后出院;出院后每周复测心率,每个月复诊,6个月为1个治疗疗程,治疗结束时逐渐减量后停药.结果 56例患儿在服药后第2～4天就可观察到血管瘤的颜色改变.治疗后1个月复诊时观察血管瘤明显改善,瘤体体积不同程度变小、质地变软,伴有溃疡的患儿除1例溃疡扩大外其余均愈合.56例中10例血管瘤完全消退,46例明显改善;1例治疗3个月后血管瘤消退停止;3例出现不良反应:1例在治疗1个月后出现肝功能轻度异常,1例治疗过程中CKMB升高,给予继续观察,1例CK-MB、LDH、ALT、GGT持续升高,给予停药.结论 大剂量口服普萘洛尔治疗严重的婴幼儿血管瘤显效快,疗效明显,患儿耐受性好,不良反应少,可显著缩短血管瘤的病程,可能成为治疗严重婴幼儿血管瘤的首选方法.

Abstract：

Objective To investigate the clinical results of the treatment of severe infantile hemangioma with high-dose propranolol in Chinese. Methods 56 cases with severe infantile hemangioma were treated with propranolol. Clinical evaluation, electrocardiography, and experimental examination of liver function and heart function were performed before treatment. The daily dose of propranolol was increased from 1 mg/kg at the first day to 1. 5 mg/kg at the second day, and to 2 mg/kg at the third day.The propranolol was given twice a day. The treatment was lasted for six months. The patients were visited every month. Results The lesion color was changed after 2-4 days of treatment in all the cases. All the lesions were dramatically improved after one month of treatment. The ulceration were healed, except one case. Until now, complete regression was achieved in 10 cases and marked improvement in 46 cases. Side effects were happened in 3 cases, including one case of abnormal liver function, one case of CK-MB increase and one case of continuous increase of CK-MB, LDH, ALT, GGT. Conclusions High-dose Propranolol is very effective in the treatment of infantile hemangioma with minor side effects and short disease period. It might be used as the first-line treatment for infantile hemangioma.     

口服普萘洛尔治疗婴幼儿血管瘤的临床研究

目的:观察口服普萘洛尔治疗婴幼儿血管瘤的疗效。方法:2009年1月～2011年6月,笔者采用口服普萘洛尔治疗11例婴幼儿血管瘤,2mg/kg/天,分2次口服,3个月为1个疗程。如观察瘤体有复发表现,则重复1个疗程,至患儿1岁停药。结果:口服普萘洛尔后1周,瘤体颜色开始变淡、萎缩变软。治疗3个月后,大部分瘤体明显萎缩。至1岁时,瘤体基本消退,表面遗留毛细血管扩张。部分患儿出现心率减慢和腹泻,均在停药1周后恢复,继续治疗。结论:口服小剂量普萘洛尔治疗婴幼儿血管瘤具有良好疗效。     

口服普萘洛尔治疗眶周部增生期婴幼儿血管瘤

目的 探讨口服普萘洛尔治疗眶周部增生期婴幼儿血管瘤的疗效及安全性.方法 2009年9月至2010年10月,临床治疗12例眶周部增牛期婴幼儿血管瘤患儿,女9例,男3例,年龄1.5～8.5个月,平均3.3个月,采用口服普萘洛尔治疗.服药剂量每日2 mg/kg,分3次给药,治疗时间为4～41周(平均16周),依据服药前后瘤体大小、颜色变化进行疗效评价,通过服药疗程及期间出现的并发症情况进行安全性评估.结果 12例患儿中,9例服药后瘤体明显消退;2例瘤体生长明显受抑制;1例服药后,因药物不良反应而终止治疗.服药期间,除少数患儿出现轻度的心率、血压暂时性降低及胃返流外,未出现其他较为严重的并发症.结论 口服普萘洛尔治疗眶周部增生期婴幼儿血管瘤安全有效,随着临床研究的不断深入,有望成为非手术治疗婴幼儿血管瘤的重要方法之一.

Abstract：

Objective To observe the efficacy and safety of oral propranolol in the treatment of periorbital proliferating phase infantile hemangioma. Methods A retrospective review of patient medical records was performed. 12 patients (9 female, 3 male; 1.5-8.5 months, average 3.3 months) with periorbital proliferating phase infantile hemangioma underwent oral propranolol therapy. The dosage was slowly increased to 2 mg/kg daily in divided doses for a mean duration of 16 weeks ( range 4 weeks41 weeks). Therapeutic outcomes and safety were established by evaluating colour, size of lesion, duration of treatment and side-effects of treatment before and after treatment. Results Of these, 9 had a signification reduction in colour and size of the lesions, 2 had no further growth. 1 is stopped therapy due to hypotension after drug administration. 11 other patients, although mild adverse effects were noted, no symptoms were severe enough to discontinue treatment. Conclusions Propranolol appears to be a safe and effective treatment in the management of periorbital proliferating phase infantile hemangioma.     

Endangering cutaneous infantile hemangioma treated with vincristine: a case report

Infantile hemangiomas are the most common tumors of childhood. They have a well-defined self-limiting natural history. In about 10% of patients, the hemangioma threatens or potentially threatens life or function during the proliferative phase. The most common treatments for such endangering hemangiomas are intralesional and/or oral steroids. Although steroids are well known to slow growth and promote involution in hemangiomas, there may be no effect on proliferation in up to 30% of patients. There are several reports of the use of intravenous vincristine being successfully used for steroid-resistant hemangiomas, although most of these have been described as hemangioendotheliomas. The degree of improvement in such cases is somewhat subjective. We describe a patient with a large steroid-resistant cervicofacial infantile hemangioma causing stridor. The use of intravenous vincristine rapidly improved the clinical state so much that immediate tracheostomy was avoided, and adrenaline nebulizers could be stopped within 1 day of starting vincristine. We believe that this is the first report of objective improvement in the symptom associated with an infantile hemangioma as a result of treatment with vincristine.     

葡萄糖转运蛋白-1在血管瘤和血管畸形中的表达及其意义

目的检测葡萄糖转运蛋白-1(glucose transporter-1,Glut1)在血管瘤和血管畸形组织中的表达并探讨其意义。方法各阶段婴幼儿血管瘤标本52例、海绵状静脉畸形25例、动静脉畸形9例、毛细血管畸形2例、正常皮肤软组织5例。应用En Vision法免疫组化染色检测Glut1在上述标本中的表达。结果增生早期,较多血管瘤内皮细胞表达Glut1;增生中期,绝大部分微血管内皮细胞和散在分布的内皮细胞表达Glut1;增生晚期,Glut1表达迅速减弱;消退期血管瘤微血管内皮细胞不表达Glut1。所有海绵状静脉畸形、动静脉畸形、毛细血管畸形、正常皮肤软组织中的小动静脉和微血管均不表达Glut1。结论Glut1是血管瘤内皮细胞发展过程中的一种表型,而不是其固有特征,Glut1表达是血管瘤内皮细胞适应代谢需要而产生的。     

普萘洛尔作为严重婴幼儿血管瘤一线治疗的前瞻性研究

目的 前瞻性评价普萘洛尔作为一线方案治疗严重婴幼儿血管瘤的疗效和安全性.方法 2009年3月至2010年2月对78例严重婴幼儿血管瘤患儿口服普萘洛进行治疗,用药剂量为每天2 mg/kg.患儿性别、年龄、肿物部位、并发症以及患儿入选该治疗的指征、不良反应、停药后有无复发等均被详细记录.针对服药后1周、1个月和停药时疗效分别评价.平均随访时间为16.7个月(12.1～23.6个月).结果 初始服药平均年龄为3.7个月(1.1～9.2个月),停止服药的平均年龄为11.2个月(5.2～22.3个月).疗程平均7.6个月(2.1～18.3个月).所有患儿口服普萘洛尔1周后肿物生长有效控制,其中88.5%(69/78)的患儿表现为促进消退.服药1个月和停药时,表现为促进消退的患儿达98.7%(77/78).14例伴发溃疡的血管瘤患儿,溃疡在服药后2个月内均愈合.15.4%(12/78)患儿有轻微不良反应.35.9%(28/78)患儿停药后有复发倾向.结论 普萘洛尔治疗婴幼儿血管瘤疗效明显,不良反应小,可作为严重婴幼儿血管瘤的一线治疗.

Abstract：

Objective To prospectively assess the efficacy and safety of propranolol as a first-line treatment for problematic infantile haemangioma in China. Methods From Mar. 2009 to Feb. 2010, 78 patients with problematic infantile hemangioma were included in the prospective study. The characteristics of the tumor, including sex, age, site, complications, were recorded. The response to treatment at 1 week, at 1 month and at the end of treatment was evaluated. The efficacy of treatment was graded as no response, stabilization, or accelerated regression. The indications for treatment, side effects and relapse after treatment were documented. The mean follow-up period was 16.7 months (range, 12.1-23.6 months). Results Oral therapy was initiated at mean age of 3. 7 months (range, 1.1-9.2 months) as first-line therapy. The mean age at the end of treatment was 11.2 months ( range, 5. 2-22. 3 months) . The treatment was lasted for 7. 6 months (range, 2. 1-18. 3 months). One week after treatment beginning, the hemangioma growth was controlled in all the patients. The accelerated regression was achieved in 88. 5% (69/78) of patients after one week of treatment, and 98.7% (77/78) of patients after 1 month of treatment and at the end of treatment. Ulceration was occurred in 14 cases before treatment, which was healed after treatment for 2 months. Minor side effects were happened in 15.4% ( 12/78) of patients.Rebound growth of lesion was noticed in 35. 9% (28/78 ) of patients. Conclusions Propranolol is effective in the treatment of infantile hemangioma with minor side effect. We suggest it should be used as the first-line treatment.     

β-受体在婴幼儿血管瘤组织中的表达及意义

目的 探讨β受体在婴幼儿血管瘤组织中的表达及意义.方法 采用免疫组织化学SP法,检测40例婴幼儿血管瘤(实验组)、20例静脉畸形及10例正常皮肤(阴性对照组)标本组织中β受体的表达.结果 40例婴幼儿血管瘤中仅在28例标本中见β2受体呈阳性表达,阳性表达率70％(28/40),β1受体未见表达;28例阳性标本中24例为增殖期,4例为非增殖期,婴幼儿血管瘤与静脉畸形、正常皮肤组织β2受体阳性表达比较,差异有统计学意义,β2受体在增殖期与消退期的阳性表达比较,差异有统计学意义(P＜0.05).20例静脉畸形及10例正常皮肤组织中未见β1受体表达.结论 β2受体特异表达于婴幼儿血管瘤,且高表达于增殖期.     

咪喹莫特治疗婴幼儿血管瘤

目的 探讨咪喹莫特治疗婴幼儿血管瘤( infantile hemangioma,IH)的临床适应证.方法 320例婴幼儿血管瘤,浅表型250例,深在型20例,混合型50例,年龄3～24周,隔日夜间睡前外用5％咪喹莫特乳青涂擦16周,随访至1岁.疗效评价分为6级,1级:完全消退;2级:75％≤消退≤99％;3级:50％≤消退＜75％;4级:25％≤消退＜50％;5级:消退＜25％;6级:未消退或增生.记录局部皮肤反应及全身不良反应.结果 浅表型、深在型、混合型IH的有效率分别为61.2％(153/250)、10.0％ (2/20)、60.0％( 30/50).浅表型和混合型之间比较差异无统计学意义(P=0.874),浅表型与深在型之间比较差异有统计学意义(P＜0.01),深在型与混合型之间比较差异有统计学意义(P＜0.01).咪喹莫特外用后4～8周,IH开始生长控制并出现消退现象.56.0％ (28/50)混合型IH出现深部病灶的增生.皮肤反应主要为红斑、表皮剥落、结痂,程度轻微.结论 浅表型IH和混合型IH的浅表病灶是外用咪喹莫特治疗的适应证,治疗后局部皮肤反应轻微,不引起局部组织萎缩和身体发育迟滞,对腔穴部位及皮肤皱褶处应避免使用,用药方式简单便捷,可作为IH浅表病灶的安全有效的治疗方法.