瘤内注射平阳霉素治疗囊性淋巴管瘤18例分析

目的 了解瘤内注射平阳霉素治疗囊性淋巴管瘤的疗效及不良反应。方法 对2001—2005年收治的18例囊性淋巴管瘤患儿进行了瘤内注射平阳霉素的治疗。注射前通过超声或CT定位,平阳霉素用注射用水溶解成1mg／ml的溶液,总剂量1～18mg（平均4,2mg／次）,注射1—4次。结果 18例中16例（88．9％）肿块明显缩小,其中11例（61．1％）肿块完全消退;2例肿块未缩小。3例进行了手术。瘤内注射治疗后个别病例出现短暂发热和局部肿痛的炎症反应,未出现严重的并发症。结论 瘤内注射平阳霉素对治疗囊性淋巴管瘤有效。     

低浓度平阳霉素注射治疗1110例淋巴管瘤单中心临床分析

目的 探讨镇静下B型超声引导下低浓度平阳霉素注射微创治疗软组织淋巴管瘤的安全性及有效性.方法 1 110例软组织淋巴管瘤患儿,年龄1个月～17岁8个月.病变类型:大囊型淋巴管瘤(囊状水瘤)233例、小囊型淋巴管瘤391例、海绵状淋巴管瘤247例、混合型淋巴管瘤224例、弥漫性淋巴管瘤15例.基础麻醉下行B型超声引导下低浓度平阳霉素注射微创治疗,大囊及小囊性淋巴管瘤浓度为0.4 mg/ml囊内注入,海绵状、混合性及弥漫性淋巴管瘤为0.33 mg/ml浓度瘤内多点注入.单次治疗最大剂量:学龄儿及以上(≥5岁)不超过4 mg;3～5岁不超过2～3 mg,幼儿(＜3岁)不超过2mg;婴儿(＜1岁)不超过1mg;年龄小于6月龄的婴儿用量＜0.5 mg,1月龄内不采用平阳霉素治疗,1～2月龄慎用.每隔2～3个月一次,共1～10余次不等.结果 1 110例软组织淋巴管瘤患儿总的完全缓解率为51.62％,非常好的部分缓解率为41.53％,部分缓解率为4.59％,微缓解率为1.17％,疾病稳定率为1.08％.其中淋巴管瘤各类型中大囊型疗效最好,CR为100％;其次为小囊型,CR为86.96％;CR仅发生在大囊型及小囊型两组.海绵状VGPR 83.40％;混合性VGPR91.07％;弥漫型疗效最差,80％为SD,20％为MR.迟发瘤内出血及迟发感染等并发症发生率为3.42％,无急性感染、皮肤破溃、坏死、硬变、肺纤维化等严重并发症.结论 镇静下B超引导下低浓度平阳霉素注射精准微创治疗软组织淋巴管瘤为安全、有效的治疗方法.     

平阳霉素加确炎舒松-A局部注射治疗小儿淋巴管瘤

目的总结平阳霉素加确炎舒松-A局部注射治疗小儿淋巴管瘤的临床经验，为局部注射治疗淋巴管瘤的推广运用提供依据。方法回顾性分析157例淋巴管瘤患儿的临床资料，对其临床特点、治疗方法及疗效进行评估。结果157例中，囊性120例，海绵状25例，混合性12例。肿瘤直径〉5cm者达91例，其中〉10cm者40例。采用平阳霉素加确炎舒松-A瘤内注射治疗1～5次，痊愈137例，好转19例，无效2例，有效率达98．7％。随访3～7年，疗效巩固。结论药物局部注射治疗小儿淋巴管瘤安全，经济，简便，疗效好。在诊治小儿淋巴管瘤的过程中，应注意鉴别后天性或继发性淋巴管瘤。     

平阳霉素联合地塞米松瘤体内注射治疗小儿特殊部位血管瘤的疗效

目的总结和评价平阳霉素联合地塞米松瘤体内注射治疗小儿特殊部位血管瘤的疗效。方法回顾性分析本院2005年1月-2010年12月应用平阳霉素联合地塞米松瘤体内注射治疗小儿特殊部位血管瘤27例临床资料。将平阳霉素8 mg+地塞米松5 mg+20 g.L-1利多卡因1～2 mL+9 g.L-1盐水稀释至8 mL,配制成1 g.L-1平阳霉素注射液。按体表瘤体面积每1.0 cm×1.0 cm注射1 mL平阳霉素注射液计算注射量。根据瘤体大小的不同,治疗时可在同一刺入点向不同方向注射药物,注射后以血管瘤体表面皮肤或黏膜肿胀变白为限,局部施压延长药物在瘤体内的作用时间。对面积稍大的血管瘤,可采用多处进针注射的方法,一般以2、3个点为宜。1次平阳霉素用量不超过8 mg。观察2周,根据瘤体颜色及硬度决定是否继续用药,直至瘤体开始变硬萎缩为止,平阳霉素注射总量不超过40 mg。结果 27例小儿特殊部位血管瘤行瘤体内注射治疗,2例出现发热,1例出现轻微胃肠道反应,均未予任何处理,1～2 d恢复正常,另1例患儿在注射2次后出现唇部黏膜溃疡,经换药愈合。治愈22例(81.5%),显效5例(18.5%),有效率达100%。随访1～2 a未见复发。结论平阳霉素联合地塞米松瘤体内注射治疗小儿特殊部位血管瘤能减少对组织结构的破坏,最大程度保持形态功能的完整,是安全、简便易行、有效的治疗方法。     

B超引导下注射平阳霉素治疗婴幼儿囊性淋巴管瘤22例

目的总结B超引导下注射平阳霉素治疗囊性淋巴管瘤的经验与疗效。方法对我科2002年～2006年收治的22例囊性淋巴管瘤患儿在B超引导下于瘤内注射平阳霉素。注射前先行B超定位,将平阳霉素用注射用水溶解成1mg/ml的溶液,一次最大用量不超过4mg,每2周注射1次,共注射1～4次。结果本组18例肿块完全消退,4例肿块明显缩小,个别患儿有短暂发热、局部红肿、疼痛等炎症反应。结论B超引导下注射平阳霉素治疗婴幼儿囊性淋巴管瘤安全、有效、经济,值得推广。     

聚桂醇瘤腔内注射治疗小儿囊性淋巴管瘤

目的 探索聚桂醇瘤腔内注射治疗小儿囊性淋巴管瘤的方法和经验.方法 回顾性分析2010年9月至2014年8月采用聚桂醇瘤腔内注射治疗小儿囊性淋巴管瘤39例的临床资料.39例术前均经彩色超声、CT或MRI检查确诊,治疗时在彩色超声引导下经皮穿刺抽尽囊液后注入药物,对多囊性患儿由深到浅依次抽液后分别注入药物.根据瘤腔位置和大小不同,对眼眶周、口腔、颌下及瘤体容积超过100 ml的18例采用聚桂醇泡沫进行注射,其余21例采用聚桂醇原液进行注射.观察瘤体大小变化、治疗次数和并发症.疗效以瘤体消失,1年后无复发为显效;瘤体缩小50％以上,1年后无增大为有效;瘤体缩小50％以下或治疗后1年内复发为无效.结果 39例治疗1～5次,平均(2.85±0.87)次.显效36例(92.3％),有效2例(5.1％),无效1例(2.6％),总有效率为97.4％(38/39).其中,采用聚桂醇泡沫治疗的次数为(2.72±0.75)次,采用聚桂醇原液治疗的次数为(2.95±0.97)次,两组比较,差异无统计学意义(t=0.26,P＞0.05).有3例第一次注射治疗后当天轻度发热,经物理降温后缓解;5例治疗后局部肿胀,轻微疼痛,观察1～3 d后消失.其余未发现明显并发症.结论 聚桂醇瘤腔内注射治疗小儿囊性淋巴管瘤是一种微创、安全、有效的方法.     

空肠闭锁和相应位置的囊状淋巴管瘤

肠系膜囊状淋巴管瘤并不常见。肠闭锁合并相应位置肠系膜的囊状淋巴管瘤此前从未见报道。作者发现1例2.63kg的早产女婴空肠闭锁和相应位置肠系膜囊状淋巴管瘤,同时合并有多跟肋骨畸形。该联合畸形的病例报道尚属首次。该患儿孕35周出生,出生后因胆汁样呕吐腹胀诊断为十二指肠闭锁。体格检查:心率128次/min,呼吸48次/min,胸廓不对称,心前区有杂音及震颤。心脏B超显示有隔膜缺失。腹胀明显,肠鸣音消失。腹部未扪及包块。实验室检查:红细胞压积36%,白细胞计数7100ul/L,低钠血症,胸片显示第六、七、八、九肋骨缺失,第十肋分裂成两部分。腹部平…     

平阳霉素局部注射治疗小儿阴茎头海绵状血管瘤五例

自 1996年 11月～ 2 0 0 0年 9月我院采用平阳霉素局部注射治疗小儿阴茎头海绵状血管瘤 5例 ,效果满意 ,现报告如下 :本组 5例患儿 ,年龄 4～ 8岁 ,平均6 .7岁 ,均以发现龟头红斑就诊 ,病程6个月～ 4年 ,术前体检均可见龟头有大小不等的暗红色肿块 ,压之褪色 ,松压后迅速充盈 ,大小约为 0 .4～ 1.0ｃｍ ,平均为 0 .6ｃｍ ,距尿道外口约 0 .4～ 1.2ｃｍ ,平均为 0 .7ｃｍ。所有患儿术前检查胸片、心电图、血、尿常规均无异常。手术方法 :平阳霉素 8ｍｇ用生理盐水 3～ 5ｍｌ溶解备用 ,麻醉满意后 ,直接用皮试针头刺入肿块基底部注药 ,一般穿…     

先天性巨大囊性淋巴管瘤一例

患儿女,出生15 min,因"胎龄36周,发现胸腹部肿物15 min"入院。系第1胎第1产,阴道分娩,无窒息,羊水、脐带、胎盘未见异常,出生体重2850 g。生后无明显气促、发绀,无呻吟、吐沫。查体:HR 136次/min,R 40次/min。神清,皮肤欠红     

沙培林囊内注射治疗儿童淋巴管瘤的疗效观察

目的：探讨沙培林在儿童淋巴管瘤治疗中的疗效及安全性。方法回顾性分析2009年6月至2013年10月我们收治的61例淋巴管瘤患儿临床资料,均采取沙培林囊内注射治疗。结果61例患儿沙培林注射治疗后经过1个月、2个月、3个月、18个月的随访,总有效率达96．7％,无一例复发。结论沙培林注射治疗儿童淋巴管瘤是一种起效快、疗效可靠、安全、创伤小的方法。     

Retro-peritoneal cystic lymphangioma in association with fetal hydantoin syndrome

Antiepileptic drugs are known to be teratogenic. Use of phenytoin during pregnancy can cause various congenital malformations leading to ‘fetal hydantoin syndrome’. One such case reported is unique in the sense that it occurred with retroperitoneal cystic lymphangioma, itself a rare condition. Such an association is not described elsewhere.     

Intra-abdominal cystic lymphangioma,in a newborn infant

We report a newborn infant presenting with an intra-abdominal cystic lymphangioma, in which necrosis and infection led to an unusual combination of solid and liquid areas observed by ultrasonography.Abbreviations KUB Kidney Ureter Bladder - BUN blood urea nitrogen - MCT median chain triglycerides     

Abdominal cystic lymphangioma in childhood: report of eight cases

Lymphangioma is a common pathology in children, however intra-abdominal cystic lymphangioma is rare. Morphology and clinical symptoms are variable and can be mixed up with other etiology. Ultrasound can make the diagnosis in the majority of the cases. Surgery is the best choice of treatment and prognosis is in general good. Authors present eight cases in a period of fifteen years, all of them treated surgically. Results were good in seven cases. One late death was observed.     

Improving nutritional status of children with cystic fibrosis at Red Cross War Memorial Children's Hospital

Aim: To determine the nutritional status of children attending a cystic fibrosis clinic in a tertiary hospital in South Africa and compare it to previously reported 10‐year rates. Methods: Weights and heights were measured of 69 (37 male and 32 female) children aged between 1 year and 18 years. Expected weight‐for‐age, expected height‐for‐age, expected weight‐for‐height and body mass index (BMI) were compared with international standards for underweight, stunting, wasting and BMI goal. Results: The nutritional status of the patients has improved over the last 10 years, most significantly for wasting, which decreased from 58.3% in 1996 to 15.9% in 2006 (95% confidence interval (CI), 1.315–14.09, P < 0.05). Fifty‐two percent of the children were underweight in 2006, compared with 66.7% in 1996 (95% CI, 0.044–13.96, P < 0.05). Stunting was found in 31.9% of the current sample. Females over 15 years had expected weight‐for‐age 25.9% lower than those between 10 years and 15 years, while no difference was found between the male age groups. Female height‐for‐age was 7.06 percentage points greater than males between 10 years and 15 years (95% CI, 2.16–11.96, P < 0.01). Males between 10 years and 15 years had significantly lower BMIs than the corresponding female group. Coloured patients had significantly lower BMIs than white patients in all age groups. Conclusions: These children demonstrated continuing improvement in nutritional status, although deficits remain. The normalisation of mean weight‐for‐age and weight‐for‐height with far fewer wasted patients is encouraging. Interventions are needed in some areas to ensure that all children show progress.     

应用关节镜治疗儿童膝关节滑膜皱襞综合征30例临床分析

目的探讨关节镜对儿童滑膜皱襞综合征的诊断价值和治疗效果。方法对30例38膝膝关节滑膜皱襞综合征患儿进行关节镜检查,同时行滑膜皱襞松解切除术;采用Lysholm评分标准评价术前及术后膝关节功能的变化。结果该组平均随访时间3.8a（10个月～5年4个月）;术前平均Lysholm评分（52.57±5.26）分,术后平均Lysholm评分（93.00±4.04）分,平均改善41分,术前与术后功能评分差异有统计学意义（t=15.393,P〈0.01）。结论关节镜技术是治疗儿童滑膜皱襞综合征的一项安全、可靠、有效的措施。     

Liver and intestinal transplantation in a child with cystic fibrosis: a case report

Cystic fibrosis (CF) is an inherited disorder that presents as a multisystem disease with meconium ileus being the presenting symptom in 20% of patients. Approximately half of these patients present with complicated meconium ileus mandating early surgical intervention, potentially resulting in short gut syndrome. Although liver transplantation in children with CF has been described, this is the first report of a combined liver and small bowel transplant in a recipient with CF. A 7-month-old boy with CF presented with short bowel syndrome following extensive small bowel resection for meconium ileus and progressive cholestatic liver failure from intravenous hyperalimentation. He underwent combined liver and small intestinal transplant. He was discharged home three weeks post-transplant on enteral feeds with supplemental intravenous fluid. He has had routine protocol small bowel allograft biopsies with no documented rejection episodes. He has been treated for minor respiratory infections without major sequelae. Improvements in pulmonary therapy have impacted on the survival in the CF population to the point where the need for multiorgan transplantation will be increased in the future. Extrapolating from the excellent experience of liver transplantation in children with CF, early liver and small intestinal multivisceral transplantation, if indicated, can be performed safely in children with CF.