雷帕霉素在卡波西样血管内皮瘤及丛状血管瘤治疗中的应用

目的探讨应用雷帕霉素治疗卡波西样血管内皮瘤(Kaposiform hemangio-endothelioma,KHE)及丛状血管瘤(tufted angioma,TA)的有效性和安全性。方法2014年8月至2015年5月,共5例KHE及TA患儿纳入研究,病变累及颈部、躯干及四肢。4例曾经传统药物治疗或其他治疗方法无效。雷帕霉素单个疗程为3个月,具体治疗方案为:每天口服0.1 mg/Kg。在服药期间及停药后,观察随访患儿一般情况、可能的副作用,以及病灶改变和血小板情况。结果雷帕霉素治疗后,5例患儿均出现了明显的病灶颜色减退,瘤体缩小,质地变软等消退迹象,血小板计数稳定在正常范围内。药物使用过程中,仅出现轻度的副反应。停药3个月后,所有患儿均未出现症状反复。结论雷帕霉素单药口服治疗KHE及TA能获得较好的疗效,短期服用安全性尚可。     

成年人后腹膜卡波西样血管内皮瘤一例

本文报道一例57岁女性后腹膜卡波西样血管内皮瘤病例, 患者经手术完整切除瘤体, 术后随访4个月, 未见复发与远处转移。卡波西样血管内皮瘤生物学行为为中间型肿瘤, 手术完整切除是其首选治疗方式, 术后患者可以适当给予药物治疗和/或放射治疗, 同时术后需长期随访监测。     

小儿卡波西型血管内皮瘤的个体化治疗分析

目的总结分析小儿卡波西型血管内皮瘤（KHE）的临床特点,探讨其个体化治疗策略。 方法回顾性分析2016年1月至2020年3月江西省儿童医院收治的20例KHE患儿的临床资料,总结病例的临床特征、肿瘤性状、是否合并卡萨巴赫-梅里特现象（KMP）、治疗策略及转归等特点。 结果20例KHE患儿中女6例,男14例。年龄<1岁的患儿16例（80.0%）,其中13例（65.0%）出生时即发现。合并KMP的患儿有8例,合并KMP与不合并KMP患儿在瘤体大小（P=0.009）、是否侵犯深部肌肉（P=0.003）的差异有统计学意义。合并KMP患儿中3例病灶行网状缝扎治疗,5例口服西罗莫司治疗;1例网状缝扎治疗无效后联合口服西罗莫司治疗。不合并KMP的12例患儿中,7例行肿瘤完全切除,2例行肿瘤部分切除后联合口服激素,2例病灶行网状缝扎治疗,1例口服西罗莫司;1例网状缝扎治疗无效后联合口服西罗莫司治疗。随访2~5年,合并KMP的患儿中1例实现瘤体接近消失,7例带瘤生存;不合并KMP的患儿中7例肿瘤完全切除,未见复发,5例无症状带瘤生存。 结论大多数KHE为婴儿期发病,肿瘤大小、病灶累及深部肌肉情况与患儿是否合并KMP相关。对于合并KMP的患儿,初步予以激素冲击治疗或输注血液制品稳定病情,病灶合适病例可先单纯进行网状缝扎治疗;如病灶不适合网状缝扎术或治疗无效,可单用或联合口服西罗莫司治疗。     

增殖期血管瘤和卡波西样血管内皮瘤的病理比较研究

目的正确鉴别体表软组织深部的增殖期婴幼儿血管瘤(Infantile hemangioma,IH)和卡波西样血管内皮瘤(Kaporsiform Hemangioendothelioma,KHE)是选择合适治疗方案的重要条件。从多方面比较增殖期IH和KHE的病理特征,为更准确地鉴别诊断这两种疾病提供依据。方法在2001年1月至2009年6月期间,收集21例增殖期IH和12例KHE标本。采用HE染色、透射电镜和免疫组化染色等方法,比较增殖期IH和KHE的病理结构和抗原标记(D2-40和Glut1)的表达。结果 HE染色显示,增殖期IH有众多毛细血管丛,新生毛细血管壁内可见扁平状周细胞;KHE由肿瘤结节组成,结节中心是大量狭缝状管腔,结节边缘可见毛细血管。在电镜下观察,增殖期IH的毛细血管壁基底膜呈多层板状结构,基底膜内有周细胞;KHE的狭缝状管腔和毛细血管壁基底膜仅有数层,且不连续,基底膜内也可见周细胞。增殖期IH肿瘤内皮细胞不表达D2-40,强烈表达Glut1;而KHE仅表达D2-40,不表达Glut1。结论增殖期IH的病理结构与KHE有明显差别。D2-40和Glut1可以作为鉴别增殖期IH和KHE的可靠抗原标记。     

对“卡-梅现象”的认识

卡梅现象(Kasabach-Merritt Phenomenon,KMP)的概念是在卡梅综合征Kasabach-Merritt Syndrome,KMS)的基础上提出来的,在近两年逐渐被广大学者所接受,成为主流     

肝上皮样血管内皮瘤伴转移1例

肝上皮样血管内皮瘤（hepatic epithelioid haemangioendothelioma,HEHE）是一种罕见的以上皮样细胞为特征的血管内皮肿瘤,其恶性程度介于血管瘤与血管肉瘤之间,属于中度或低度恶性肿瘤,生长缓慢,转移率不高,约在27％的患者中可出现肿瘤的转移,且多出现在肺部或肝内。     

肝脏多发上皮样血管内皮瘤1例报告

肝脏上皮样血管内皮瘤（hepatic epithelioid hemangioendothelioma，HEH）较为罕见一我科收治1例。报告如下.     

肝脏上皮样血管内皮瘤1例分析并文献回顾

目的探讨肝脏上皮样血管内皮瘤的临床病理、影像学表现及治疗。方法分析我院一例经病理证实的肝脏上皮样血管内皮瘤的临床表现、影像表现、病理组织特征,并进行文献相关回顾,探讨该罕见肿瘤的临床、影像、病理特征及治疗方案。结果肝脏上皮样血管内皮瘤的临床表现特异性差,影像表现为低密度,文献病例存在"包膜回缩征"和"棒棒糖征",强化特征随肿瘤大小变化,病理表现为上皮样细胞等细胞组成,呈条索状、巢状或腺样排列,免疫组化CD31、CD34阳性,治疗以肝肿物切除与肝移植手术为主。结论肝脏上皮样血管内皮瘤临床、影像表现特异性差,病理组化是确诊的方法,以手术治疗为主,在临床诊治中应予以重视。     

骶骨上皮样血管内皮瘤1例

<正>患者女,42岁。3个月前无明显诱因出现右侧臀后部皮肤烧灼感,局部疼痛,外用膏药后烧灼感症状加重。CT示S2~3椎体右侧骨质破坏,右侧第2骶孔膨大,边缘轻度硬化,内见软组织肿块,考虑神经源性病变。MRI示患者S2~4椎体右侧可见团块状稍长T1混杂稍长T2信号(图1A),内见圆形长T1短T2信号及点片状长T2信号,肿瘤与骨质交界处呈线形长T1     

肝上皮样血管内皮瘤的诊治进展

肝上皮样血管内皮瘤是一种低度恶性源于血管的少见肿瘤,临床症状和实验室检查无特异性,最终诊断需要病理检查.根治性的肝切除术或肝移植是可选择的主要治疗方法,其他各种辅助治疗有待进一步评价.     

Kaposiform hemangioendothelioma: a rare cause of spontaneous hemothorax in infancy. Review of the literature

Kaposiform hemangioendothelioma is a rare vascular tumor found almost exclusively in early childhood often associated with a life-threatening thrombocytopenia and consumptive coagulopathy known as Kasabach-Merritt phenomenon. The natural history of these lesions and treatment remains controversial. We portray a case of a spontaneous hemothorax in a 2-month-old female infant and review of 153 reported cases in attempts to better delineate the available treatment options.     

Kaposiform hemangioendothelioma of the mediastinum in a 7-month-old boy: a case report

Kaposiform hemangioendothelioma is an aggressive vascular proliferation that has been recognized as a separate entity from other childhood vascular tumors. The authors present an unusual case of hemangioendothelioma arising in the mediastinum of a 7-month-old male infant with acute respiratory distress and Kasabach-Merritt syndrome. This patient with life-threatening lesion showed a dramatic response to interferon alfa-2a. This case had many histologic and clinical features consistent with kaposiform hemangioendothelioma.     

Multivertebral Kaposiform hemangioendothelioma presenting as scoliosis - A case report and review of literature

Kaposiform hemangioendothelioma (KHE) is a rare, locally invasive vascular tumour of childhood that may occur in soft tissue or bones, and is associated with cutaneous plaques and Kasabach-Merritt phenomenon (KMP). We present an instance of a 9-year-old girl with primary vertebral involvement of KHE, whose clinical presentation was with painless, progressive scoliosis alone, sans cutaneous markers. We highlight the imaging features of this rare manifestation and importance of histopathological diagnosis for optimal management.     

经导管动脉硬化栓塞术治疗Kasabach-Merritt综合征

目的探讨经导管动脉硬化栓塞术治疗Kasabach-Merritt综合征(KMS)的疗效。方法收集经超声、CT、生化检查结合临床表现确诊的KMS患儿58例,纠正血小板减少及凝血功能异常后,给予经导管动脉硬化栓塞术进行治疗。结果对所有患儿均顺利完成经导管动脉硬化栓塞术,术后动脉造影见瘤体基本消失;术后1周血小板[(267.66±61.50)×109/L]和纤维蛋白原[(2.64±0.40)g/L]水平均明显高于术前[血小板:(35.24±12.12)×109/L,纤维蛋白原:(1.47±0.33)g/L],差异有统计学意义(P均0.01);44例患儿接受1次经导管动脉硬化栓塞术、11例接受2次经导管动脉硬化栓塞术、3例接受3次经导管动脉硬化栓塞术;所有患儿均达治愈标准。结论经导管动脉硬化栓塞术治疗KMS安全、有效,值得临床推广应用。     

Capillary Hemangioma of the Liver with Kasabach-Merritt Syndrome in a Neonate: Report of a Case

(Received for publication on Sept. 28, 1998; accepted on July 13, 1999)     

Living donor liver transplantation in a patient with giant hepatic hemangioma complicated by Kasabach-Merritt syndrome: Report of a case

We herein present a case of unresectable giant hepatic hemangiomas with Kasabach-Merritt syndrome which was successfully treated by living donor liver transplantation using a left lobe graft. The patient was a 45-year-old woman who complained of abdominal distension. Two sessions of transarterial embolization were performed, but failed to reduce the size of the tumor. The hepatic tumors were thus judged untreatable and the only option for a cure was to offer living donor liver transplantation, because of the tumor size, its location, and the association with Kasabach-Merritt syndrome. A left lobe graft with the middle hepatic vein donated by her 47-year-old brother was transplanted under venovenous bypass. The postoperative course of the recipient was complicated by small-for-size graft syndrome, which developed after episodes of acute cellular rejection on postoperative day 8 and sepsis on day 31. The patient successfully recovered from the complications and was discharged on day 72, and she remains well at 10 months after transplantation. In conclusion, living donor liver transplantation was found to be an effective option for the treatment of a patient with unresectable giant hepatic hemangiomas complicated by Kasabach-Merritt syndrome.     

儿童股骨干骨折1004例临床分析

目的　对 10 0 4例儿童股骨干骨折进行回顾性总结 ,讨论其临床特点和治疗问题。方法　高处坠落伤 35 8例 ,车祸伤2 0 3例 ,平地跌伤 2 90例 ,重物压砸伤 6 3例 ,产伤 6 1例 ,其他外伤 2 9例 ;上段骨折 2 41例 ,中段 5 82例 ,下段 181例 ;横骨折 5 37例 ,斜骨折 34 0例 ,螺旋骨折 38例 ,粉碎骨折 89例。 973例采用牵引治疗 ,31例采用手术切开复位加内固定。结果　全部病例均获短期随访 (4～ 8周 ) ,约半数病例获长期随访 (5～ 15年 )。除 5例因过早下地负重而使成角畸形 >2 0°,其余均获满意疗效。结论　根据不同年龄和不同骨折部位而采用不同的牵引方式应为治疗儿童股骨干骨折的一个原则 ,只有少数病例需手术治疗。     

慢性肾脏病3期中医证候分布与实验室指标的相关性研究

目的分析慢性肾脏病（CKD）3期患者中医证候与实验室指标相关性。方法将60例慢性原发性肾小球疾病、CKD3期（肾小球滤过率30-59ml／min）、中医辨证均符合气阴（血）两虚证、肾络瘀痹、风湿内扰证或湿热内蕴证的患者为研究对象,按照统一的标准对患者进行询问调查,并详细填写凋查表。结果60例患者中医证候主要以肾气阴（血）两虚为主,所占比例达60％,湿热内蕴证占2%。风湿内扰证患者的24h尿蛋白定量明显高于非风湿内扰证患者。肾络瘀痹较非肾络瘀痹肾功能下降明显。各证型组间血常规、血尿素氮、血尿酸比较无显著差异。中医证候总积分与24h尿蛋白定量、三酰甘油（TG）和总胆同醇（TC）水平呈正相关,与肾小球滤过率水平呈负相关。结论CKD3期中医证候分布和实验室指标有一定的相关性,对于评估预后、指导临床治疗有一定意义。     

Fracture of the Carpal Scaphoid in Children: A Clinical and Roentgenological Study of 108 Cases

A series of 108 carpal scaphoid fractures in children was analysed with respect to the site, type and treatment of the fracture. the patients were followed until radiological fusion occurred. All fractures united and healed completely with conservative treatment. Ninety—four fractures, 41 of which were avulsions, were located in the distal third of the scaphoid. There were 13 waist fractures, and one avulsion fracture was located in the proximal pole of the scaphoid. the great number of avulsion fractures and the location of the fractures in the distal third of the scaphoid is very typical in children. in three neglected cases marked bone resorption was seen on the fracture surface 3 weeks after the injury. Because non—union is possible especially in neglected cases of waist fractures, control radiographs should be taken 1 or 2 weeks after the injury if the fracture is not visible on the primary radiographs but is suspected clinically. Immobilization with an adequate plaster cast is mandatory in all transverse fractures of the carpal scaphoid in children.