腹腔镜胃造口术联合Forley尿管球囊扩张治疗小儿瘢痕性食管狭窄

目的 探讨腹腔镜胃造口术联合Forley尿管球囊扩张治疗小儿瘢痕性食管狭窄的可行性和疗效.方法 自2006年7月至2014年2月间河北医科大学第二医院小儿外科收治瘢痕性食管狭窄患儿11例,男8例,女3例,年龄(39.27±29.32)个月.均于全身麻醉下行腹腔镜胃造口术,术后第7天利用简易Forley尿管球囊进行狭窄食管扩张.于术后1个月、3个月、6个月、1年、2年回院复查,食管钡餐检查,测量对比术后食管扩张不同时间段狭窄段食管直径变化.结果 所有11例患儿均顺利完成手术,手术时间(24.叭±5.02)min,出血量(15.91±4.37)ml,术后住院(8.18±0.98)d.术后第7天开始扩张食管,操作顺利.扩张1个月后,4例可进少量固体食物;3个月时,9例可进普通饮食;6个月时,患儿几乎全部恢复正常饮食.术后1～3个月期间食管扩张效果最明显,与其他时间段比较均有显著型差异(F=13.407,P=0.000＜0.01).术后造瘘口周围皮炎或湿疹8例,造瘘口脱垂1例,扩张后食管黏膜出血2例,无造瘘口回缩、食管破裂穿孔、导丝或尿管断裂、腹膜炎等发生.结论 腹腔镜胃造口术联合Forley尿管球囊扩张治疗小儿瘢痕性食管狭窄,损伤轻、出血少、恢复快,采用器械简单,操作方法简易,疗效明显.     

胃镜辅助下探条扩张在小儿食道狭窄中的应用

目的总结胃镜辅助下探条扩张技术在小儿食道狭窄性疾病中的应用,探讨影响探条扩张效果的因素。方法对2003年12月～2005年5月收治的11例食道狭窄患儿采用探条扩张法进行治疗,按扩张次数将患儿分为3组,分析各组间病因、食道狭窄长度和狭窄食管直径对扩张效果的影响。结果11例患儿共接受了36次扩张,平均每例扩张3.3次(1～10次),无1例发生并发症。1例患儿已进行手术治疗,1例等待手术,总有效率为81.8%(9/11)。A组4例,均为先天性食道闭锁术后吻合口狭窄患儿;B组4例,主要为先天性食道狭窄和胃食管返流术后仍合并返流患儿;C组3例,以食道化学性烧灼伤为主。A、B、C三组患儿食道扩张前狭窄段平均直径分别为4.8mm(3.0～6.0mm)、5.0mm(4.0～6.0mm)、4.3mm(4.0～5.0mm);平均狭窄段长度分别为1.8cm(1.5～2.0cm)、2.4cm(2.0～3.0cm)和6.8cm(4.5cm～10.0cm)。扩张有效率分别为100%(4/4)、100%(4/4)和33.3%(1/3)。三组间病因不同,C组患儿食道狭窄段平均直径虽小于其他两组,但不具有显著性差异;其食道狭窄段长度显著大于其他两组,P<0.05。C组患儿扩张有效率显著低于A、B组(P<0.05)。结论胃镜辅助下食道探条扩张方法安全、有效。食道术后吻合口狭窄患儿扩张有效率高;化学性烧灼伤患儿存在反复多次扩张和再手术问题。食道狭窄段长度比狭窄段直径更能影响扩张的效果。     

胃镜直视下球囊扩张术治疗小儿食管狭窄

目的 探讨胃镜直视下球囊扩张治疗小儿食管狭窄的安全性和有效性.方法 12例食管狭窄患儿,其中食管闭锁术后吻合口狭窄7例、先天性食管狭窄3例、腐蚀性炎性狭窄2例,年龄5～59个月,在静脉复合麻醉和气管插管下,通过胃镜直视用控制辐射状扩张(CRE)三级扩张球囊行食管狭窄扩张.观察术后腹痛、黑便、呕吐的发生,同时随访术后3～12个月恢复饮食种类,狭窄口大小、营养情况.结果 12例共进行22次扩张,19次成功,3次术后出现并发症,扩张成功率为86%.12例中,3例扩张失败,9例扩张成功、症状改善,有效率为75%.扩张前狭窄口直径2～8 nun,3～12个月后复查胃镜和随访,狭窄口直径9～13mm,8例可进食固体食物、营养状况改善.结论 CRE三级食管球囊行食管狭窄扩张治疗,操作简单、效果确切,食道闭锁术后吻合口狭窄的扩张效果较好.     

胃镜下球囊扩张治疗小儿溃疡性幽门狭窄

目的探讨溃疡性幽门狭窄形成持久性(外科性)幽门梗阻的新的治疗方法。方法对10例幽门口(管)溃疡并狭窄患儿,在静脉复合麻醉下,通过胃镜直视用CRE三级扩张食管球囊行幽门扩张,观察术后腹痛、黑便、呕吐的发生,同时随访术后3、6、9、12个月恢复饮食种类、幽门大小、体重、身高情况。结果总有效率达100%,6～9个月复查胃镜和随访,幽门直径≥12mm,5例可进食普食,4例可进食软饭;8例患儿生长发育赶上同龄儿,占80%。结论CRE三级食管球囊行幽门扩张治疗溃疡性幽门性狭窄,该方法操作简单、效果确切、安全性好。     

单中心儿童食管狭窄外科综合治疗经验北大核心CSCD

目的总结儿童食管狭窄的外科诊治经验,探讨儿童食管狭窄的处理策略。方法回顾性分析2012年12月至2023年1月重庆医科大学附属儿童医院胸心外科收治的297例食管狭窄患儿的临床资料,其中男196例,女101例;首诊年龄中位数为3.8(1.7,32.8)个月;首诊食管手术后食管狭窄164例,创伤后食管狭窄59例,食管壁内气管支气管软骨残留30例,纤维肌性食管狭窄10例,贲门失弛缓症16例,食管裂孔疝术后食管狭窄5例,反流性食管炎伴食管狭窄7例(包含Barrett食管2例),外压性食管狭窄(伴血管环)5例,大疱性表皮松解症伴食管狭窄1例。诊治患儿定期随访,收集患儿治疗方式及治疗结局等信息。结果进行食管注射治疗3例,食管扩张230例,食管支架5例,手术治疗59例。治疗结果为治愈120例,治疗有效177例,无死亡病例。总体治愈率40.4%(120/297),有效率59.6%(177/297)。结论儿童食管狭窄需多因素评估后制定个体化综合处理方案,可取得良好治疗效果。多学科治疗模式和微创精确手术可能是未来小儿食管狭窄的治疗方向。     

胃镜辅助下儿童食管贲门狭窄的扩张治疗

目的探讨胃镜辅助下儿童食管贲门狭窄的扩张治疗。方法对21例食管贲门狭窄患儿进行胃镜辅助下扩张治疗,其中探条扩张18例,球囊扩张3例。结果 21例患儿经1～4次间断扩张治疗后基本消除梗阻,吞咽困难明显改善。结论胃镜辅助下食管扩张术是一种简单、安全、有效、并发症少的治疗方法,值得临床推广应用。     

胃镜下球囊扩张治疗食管闭锁术后吻合口狭窄的疗效分析

目的探讨胃镜下球囊扩张治疗食管闭锁术后吻合口狭窄的安全性及有效性。方法本研究回顾性收集2017年11月1日至2018年10月31日由首都医科大学附属北京儿童医院收治的61例先天性食管闭锁术后吻合口狭窄病例作为研究对象,其中12例为本院食管闭锁术后患儿。根据患儿辅食添加情况,将首次扩张年龄分为3个月(n=19),3～6个月(n=12),6～12个月(n=9)和≥12个月(n=21)4组。症状缓解持续1个月以上,不需要扩张者被认为短期成功;症状缓解持续3个月以上,不需要扩张者被认为中期成功。结果 61例患儿共扩张331次,单人扩张次数1～13次,扩张次数的中位数和四分位间距为5(IQR:3～7)次。首次扩张年龄3个月的患儿,扩张次数的中位数和四分位间距为4(IQR:3～7)次,扩张终点球囊直径为(8.3±1.2)mm。首次扩张年龄为3～6个月者扩张次数的中位数和四分位间距为5(IQR:3.25～7.0)次,扩张终点球囊直径为(10.1±2.0)mm。首次扩张年龄为6～12个月者扩张次数的中位数和四分位间距为9(IQR:6.5～11.5)次,扩张终点球囊直径为(12.3±2.3)mm。≥12个月的患儿扩张次数的中位数和四分位间距为4(IQR:2～6)次,扩张终点的球囊直径为(13.6±1.7)mm。不同年龄组扩张次数存在统计学差异(H=94.258,P0.001)。不同年龄组扩张终点球囊直径存在统计学差异(F=33.147,P0.001)。61例患儿中,短期扩张成功率为72.1%,中期扩张成功率为59.0%。1例发生穿孔,经保守治疗后痊愈,穿孔率为0.3%(1/331)。结论胃镜下球囊扩张治疗食管闭锁术后吻合口狭窄,疗效满意,并发症少,可作为治疗的第一选择。     

儿童食管狭窄治疗面临的挑战北大核心CSCD

食管狭窄是目前临床上常见的儿童食管疾病,主要包括先天性食管狭窄和后天性食管狭窄,后者主要包括食管术后吻合口狭窄、腐蚀性食管狭窄等[1,2]。临床上可出现吞咽困难、体重减轻、营养失衡和误吸引起肺炎等风险,严重影响患儿的生活质量。食管狭窄的治疗需要被重视,目前食管狭窄首选的治疗方式是球囊扩张术,此外还有支架置入术、食管黏膜下注射药物、食管重建术等。狭窄复发是临床上的一个挑战,发生率约为10%[3]。食管狭窄的复发将加重医疗经济负担,反复的内镜下治疗也会加大术后并发症的发生概率,并降低患儿的生活质量[4]。自1983年开始,应用金属支架治疗开创了食管狭窄的治疗新局面,但术后存在的并发症,如胸痛、呕吐、肉芽组织增生、再狭窄等,很大程度上限制了其在儿童食管狭窄中的应用。本文就外科医生对球囊扩张的困惑、食管支架的使用及材料选择、外科技术面临的困惑等进行探讨。     

球囊扩张治疗先天性食管闭锁术后食管狭窄的时机选择

目的 探讨新生儿先天性食管闭锁术后吻合口狭窄采用球囊扩张治疗的时机.方法 回顾性分析15例先天性食管闭锁术后吻合口狭窄患儿的临床资料,均在纤维胃镜或电子胃镜下实施球囊扩张术.结果 球囊扩张后直径为0.3～1.2 cm.单个患儿扩张次数为2～6次不等.以呼吸道症状逐渐减轻、消失,饮食由流体状态向固态改善,体重逐渐增加为治疗有效.48次球囊扩张术有43次扩张完成,完成率为89.6%.有效14例,1例扩张术后无好转行胃造瘘术.结论 食管闭锁术后1～3个月是食管狭窄治疗的最佳时机,早期发现并通过球囊扩张治疗新生儿先天性食管闭锁术后食管狭窄是有效而安全的治疗手段.     

Cheatham-Platinum支架置入术治疗儿童先天性心脏病血管狭窄的疗效评价

目的 评价NuMED Cheatham-Platinum(CP)支架置入术治疗儿童先天性心脏病(CHD)血管狭窄的即刻和早中期疗效.方法 2005年8月至2007年5月,采用CP支架置入治疗5例CHD血管狭窄(先天性主动脉缩窄1例,肺动脉狭窄4例).男3例,女2例,年龄4～15岁(中位年龄12岁),体重20～51 kg(中位体重24 kg).根据数字减影血管造影结果选择CP支架和NuMED双球囊,支架准确定位后先后充盈内外球囊扩张支架.结果 5例CHD血管狭窄进行了6次支架置入操作,共置入8只支架,均为8-zig CP支架,支架长度22～39 mm.除1例右肺动脉狭窄支架置入术中移位而于术后11个月重新置入第2只CP支架外,余4例6只支架即刻置入成功.跨狭窄段收缩压差由术前(43.43±25.61)mm Hg(1 mm Hg=0.133 kPa)降至术后(3.29±3.09)mm Hg(t=4.320,P<0.01),最窄处血管直径由术前(6.86±2.04)mm增加至术后(13.44±4.02)mm(t=-4.508,P<0.01).2例单侧肺动脉分支狭窄术后狭窄侧肺血流量占全肺血流量百分比分别由11.0%和13.0%增加至47.5%和52.2%,2例双侧肺动脉分支狭窄的右心室/主动脉收缩压比分别由术前62.3%和72.2%降至术后27.0%和33.3%.1例主动脉缩窄术后上肢血压由206/133 mm Hg降至156/95mm Hg.随访13～34个月(中位时间20个月),2只CP支架于术后6个月发生支架内再狭窄,余结果稳定无并发症发生.结论 CP支架置入术治疗儿童CHD血管狭窄安全可行,即刻和早中期疗效较好,但远期疗效有待进一步随访和更多病例的研究.     

Treatment with Probucol of Children with Familial Hypercholesterolemia

ABSTRACT. Nine children with familial hypercholesterolaemia, age range 2 to 12 years, were treated with a low cholesterol diet and probucol (10 mg/kg/day). The year before, the children received, as only treatment, a low fat-cholesterol diet. During this period their mean plasma total cholesterol level fell from 8.2±1.45 mmol/l to 7.17±0.84 mmol/l (12.6%). This level was further reduced to 5.92±0.63 mmol/l (17.1%) after the addition of probucol. Plasma high density lipoprotein cholesterol levels were lowered in absolute terms but not in relation to total cholesterol. No apparent side effects were observed. However, the use of probucol should be restricted for the moment to severe cases of hypercholesterolaemia as the long-term excretion of the drug in children is not yet known.     

Treatment with probucol of children with familial hypercholesterolaemia

Nine children with familial hypercholesterolaemia, age range 2 to 12 years, were treated with a low cholesterol diet and probucol (10 mg/kg/day). The year before, the children received, as only treatment, a low fat-cholesterol diet. During this period their mean plasma total cholesterol level fell from 8.2 +/- 1.45 mmol/l to 7.17 +/- 0.84 mmol/l (12.6%). This level was further reduced to 5.92 +/- 0.63 mmol/l (17.1%) after the addition of probucol. Plasma high density lipoprotein cholesterol levels were lowered in absolute terms but not in relation to total cholesterol. No apparent side effects were observed. However, the use of probucol should be restricted for the moment to severe cases of hypercholesterolaemia as the long-term excretion of the drug in children is not yet known.     

Growth of infants with osteogenesis imperfecta treated with bisphosphonate

Background:  Osteogenesis imperfecta (OI) is a heritable bone disease characterized by bone brittleness and various degrees of growth disorder. Cyclic pamidronate therapy is reportedly useful to prevent bone fracture in OI and in infants with OI, but, it remains unclear how infants with OI grow during bisphosphonate therapy.
Methods:  Height and weight measurements of OI infants treated with cyclic pamidronate therapy were taken before and every 6 months during therapy until 18 months. Vertebral morphometry and the concavity index were analyzed using X-ray films taken simultaneously.
Results:  Among OI patients, those in the group for which the height z- score decreased tended to have more femur fractures than those of the group for which the height z- score increased. Morphometry of the lumbar spine showed that compression fractures occurred less during cyclic pamidronate therapy, by which the lumbar bone mineral density increased.
Conclusions:  Bisphosphonate preserved vertebral morphometry during 18 months after starting therapy in infants. Prevention of femur fracture during the infantile period might help prevent short stature; therapeutic strategies during infancy must better emphasize prevention of long bone fracture before the beginning of gait.     

Duration of isolation of children with influenza A treated with oseltamivir

The duration of fervescence (24 hours in children or 30 hours in adults) was shorter in the oseltamivir group than in the placebo group. According to a Japanese law enacted before oseltamivir therapy became available, schoolchildren with influenza must be isolated 48 hours after defervescence. Our data suggest isolation should be at least 84 hours for children with influenza A treated with oseltamivir and 108 hours for preschool children.     

Comparison of patients with Kawasaki disease with retropharyngeal edema and patients with retropharyngeal abscess

Kawasaki disease with retropharyngeal edema (KD with RPE) is a rare complication, and it is diagnosed by neck CT. Most reported cases had a delayed diagnosis because those patients' conditions were misdiagnosed as retropharyngeal abscess (RPA). The purpose of this study was to differentiate KD with RPE from RPA. We performed a retrospective case–control study comparing children with KD with RPE to those with RPA hospitalized at the tertiary pediatric hospital in Tokyo between 2005 and 2011. The 39 patients revealing RPE on neck CT were divided into two groups: group A was classified as KD (n?=?21) and group B was classified as non-KD (n?=?18). Patients in group B were finally evaluated as having RPA clinically and were treated with antibiotic therapy. A significantly higher proportion of patients in group B complained of dysphagia (11 patients vs. 5 patients; p?=?0.0170) and neck pain (17 patients vs. 12 patients; p?=?0.0106). Neck CT revealed a ring enhancement (16 patients vs. no patients; p?<?0.0001) and mass effect in a greater proportion of patients in group B (11 patients vs. 1 patient; p?<?0.0003). Conclusion: Careful attention to manifestations and close analyses of CT imaging may allow clinicians to differentiate KD with RPE from RPA.     

Treatment of Children with Advanced-Stage Lymphoblastic Lymphoma with Pegaspargase

Objective: To evaluate the feasibility of Pegaspargase instead of L-asparaginase to treat children with advanced-stage lymphoblastic lymphoma (LBL) on the Berlin-Frankfurt-Munster (BFM)-95 protocol. Methods: Fifty-four newly diagnosed patients with stage III or IV LBL and without any treatment were enrolled in this study. Pegaspargase took place of L-asparaginase in BFM-95. The complications and treatment responses of patients treated on the BFM-95 protocol and modified BFM-95 protocol were then evaluated respectively. Findings : For LBL patients treated with BFM-95 protocol or modified BFM-95 protocol, the complete response, event-free survival, overall survival were similar. Stage 4 myelosuppression was the most common complication in both groups. Besides that, among 31 patients receiving modified BFM-95 protocol, coagulation defects were the most common complication. In contrast, anaphylactic reaction was the most common complication in the other 23 patients receiving BFM-95 protocol. Conclusion: Modified BFM-95 protocol is available to children with advanced-stage LBL with an equal outcome and enhances its compliance and decreases the incidence of anaphylactic reaction, compared to BFM-95 protocol. Coagulation defects are the major complication and tolerable in modified one.Key Words: Pegaspargase, L-Asparaginase, Lymphoblastic Lymphoma, Chemotherapy     

Treatment of refractory hemorrhage with factor XIII in a patient with hemophilia A with inhibitor

An 11‐year‐old male with hemophilia A and a known high‐titer Factor VIII inhibitor was admitted with retroperitoneal hemorrhage. The patient was receiving infusions of recombinant activated Factor VII (rFVIIa) for a recent elbow hemorrhage when retroperitoneal bleeding commenced. Despite increased dosing of rFVIIa and a dose of activated prothrombin complex concentrate (aPCC), he continued to hemorrhage and required several blood transfusions. Factor XIII was administered 1 hour after rFVIIa and the patient demonstrated cessation of bleeding and normalization of clot strength. Factor XIII may act as an adjuvant in effective clot stabilization in patients with hemophilia and inhibitory antibodies. Pediatr Blood Cancer 2013; 60: E23–E25. © 2013 Wiley Periodicals, Inc.