支气管镜下肺介入治疗在儿童获得性声门下狭窄中的应用

目的:探讨电子支气管镜下肺介入治疗儿童获得性声门下狭窄的价值。方法:回顾性分析2019年湖南省儿童医院重症医学科收治的8例获得性声门下狭窄患儿的临床资料,病例均在支气管镜下诊断,均在全麻喉罩、支气管镜下行肺介入治疗(激光、钳夹、冷冻、球囊扩张)。所有患儿接受6-12个月的随访。结果:8例患儿术后即刻均顺利拔管撤机,1例因吸气困难反复插管,染色体结果提示猫叫综合征,家长放弃治疗;1例因再次肺部感染家长放弃治疗。另6例患儿呼吸困难、声音嘶哑、喉鸣等症状明显改善,内镜下可见狭窄处明显增宽,4.0 mm内镜均能顺利通过;4例已达临床治愈,治疗次数约3.25次/例(13/4);2例症状消失,目前尚在随诊中;患儿均未见严重出血、窒息、呼吸衰竭等并发症。结论:支气管镜下肺介入治疗是解决儿童声门下狭窄的安全有效方法。     

支气管镜下冷冻治疗儿童肉芽及瘢痕组织导致的下气道狭窄及阻塞22例

目的 探讨支气管镜下冷冻治疗儿童肉芽及瘢痕组织致下气道阻塞和狭窄的疗效及安全性.方法 22例包括:肺炎支原体肺炎18例,支气管内膜结核2例,支气管异物导致的肉芽组织增生,阻塞气道,引起阻塞远端肺不张1例,先天性心脏病手术气管插管后气管瘢痕引起气管重度狭窄1例.对患儿实施支气管镜下病变部位多次冷冻治疗.气管插管后导致气管狭窄的患儿,配合电凝治疗.分别于术前和最后一次冷冻治疗后,评价临床症状及支气管镜下病变部位情况,肺不张病例根据胸部CT肺不张的面积、气管重度狭窄的病例根据潮气量进行评估.结果 22例分别接受冷冻治疗1～4次.显效16例:临床症状完全改善,支气管镜下肉芽及瘢痕组织消失,灌洗治疗病变远端管腔通畅,CT见肺不张消失或基本消失.气管重度狭窄病例管腔直径由2 mm增宽为约5 mm,潮气量由3.0 ml/kg提高至8.8 ml/kg.有效5例:临床症状完全改善,支气管镜下肉芽及瘢痕组织消失,病变部位远端的部分气道变形、狭窄及闭锁,灌洗治疗不畅,CT见肺不张面积缩小1/3～ 2/3.无效1例(1/22):临床症状完全改善,支气管镜下管腔肉芽及瘢痕组织消失,但病变部位远端大部分气道管腔闭锁,灌洗治疗管腔不通畅,CT见肺不张面积未改变.总有效率95.5％.冷冻治疗术中及术后无并发症,经l～12个月随访未见病情复发.结论 支气管镜下冷冻治疗肉芽及瘢痕组织导致的儿童下气道狭窄及阻塞有效、安全.     

纤维支气管镜下带囊支架置入术治疗儿童气管狭窄2例

目的:通过纤维支气管镜下带囊支架置入术治疗儿童气管狭窄,观察近期疗效和并发症。方法:例1,女,4月龄,因“发现心脏杂音3个月余,咳嗽10 d渐加重,伴发热、喘息3 d”入院,诊断为膜周部+肌部室间隔缺损,房间隔缺损（继发孔型）,肺动脉高压,主气道下段及左右主支气管变形狭窄。行肺动脉环缩术,房间隔、室间隔缺损修补术+主动脉悬吊术成功后,术后2个月内3次撤离呼吸机均未成功。例2,女,1岁10个月,因“咳嗽1个月余”入院,诊断为左主支气管狭窄,EBV相关性噬血细胞淋巴组织细胞增生症。左侧支气管狭窄致使排痰困难,肺部反复感染,左肺气肿。2例患儿家长在充分了解纤维支气管镜下带囊支架置入术可能的风险后签署知情同意书。采用“边麻边进”方法行气道黏膜表面麻醉,例1和例2 选择带囊支架长度分别为24和29 mm,直径均为4 mm（均经雷帕霉素处理）,由支架导入器送入纤维支气管镜,在纤维支气管镜直视下释放带囊支架,然后退出支架导入器。结果:2例患儿置入带囊支架后狭窄段气管扩张良好,带囊支架放置2~3个月随访,未见肉芽组织增生等并发症。结论:纤维支气管镜下带囊支架置入术治疗气管狭窄近期疗效较好,远期疗效尚待观察。     

The role of bronchoscopy in slide tracheoplasty in children北大核心CSCD

目的 总结支气管镜在儿童Slide气管成形术中的作用。方法 回顾性分析2017—2020年湖南省人民医院收治的4例气管狭窄患儿的诊疗经过,总结支气管镜在Slide气管成形术术前评估、术中定位与测量及术后创面评估和治疗中的作用。结果 Slide气管成形术术前支气管镜评估显示,4例患儿中3例存在完全气管环,2例合并肺动脉吊带,2例存在多处狭窄。3例在该院完成Slide气管成形术,术中支气管镜下判断狭窄段中点及测量狭窄段长度,协助离断气管狭窄段,术后灌洗明确病原;1例外院Slide气管成形术术后9个月出现瘢痕牵拉,经支气管镜下介入治疗好转。2例术后第4天支气管镜下发现气管黏膜改变,调整治疗方案;2例术后1个月出现肉芽增生,经支气管镜下冷冻治疗好转。1例因吻合口坏死放弃治疗死亡,存活的3例随访6个月以上,预后可,但均存在气管支气管软化。结论 支气管镜可用于气管狭窄患儿Slide气管成形术的管理,有助于术后康复及随访。     

先天性心脏病合并气管狭窄气管支架置入后取出指征探讨

方法 比较分析CHD合并气管狭窄气管支架置入后尚留和取出病例的临床资料。结果 9例置入气管支架患儿均为重度气道狭窄,置入气管支架为乐普（北京）公司生产的血管金属支架（PARTNER),其中尚留气管支架的4例患儿,平均年龄13.75个月,平均体重8.70 kg,随访支气管镜检查均未发现肉芽组织增生,镜下气管支架支撑良好;5例患儿于气管支架置入68～96 d 后取出,平均年龄48个月,平均体重4.96 kg。气管支架取出后发现支架支撑处并未塌陷,不影响患儿通气功能,取出气管支架完整,随访10～11个月,气道未见再阻塞及其他并发症,未出现需要再次置入气管支架的情况和死亡病例。在复习文献的基础上提出气管支架取出指征：①在良好的随访体系下气管支架置入2~3个月;②经支气管镜证实无肉芽组织增生,CT及其三维气道重建显示气道通畅;③临床无呼吸困难表现及感染征象,血气分析示无肺通、换气功能异常,X线胸片正常,超声心动图检查示心脏功能正常;④支气管镜下能清楚显示气管支架边界,且整个张开的支架位于气道表面并未上皮化;⑤支气管镜下钳夹住气管支架后,轻轻上下小幅推拉,镜下证实可以松动与气道紧密粘贴的支架。结论 对于小婴儿气管支架取出从长远角度来看是最佳选择,在良好的随访体系下,在不影响患儿生命质量的前提下,CHD合并气管狭窄气管支架置入2～3个月后取出安全可行。     

小儿先天性气管支气管畸形46例诊治分析

目的 探讨先天性气管支气管畸形的临床特点,以提高对该病的认识,减少误诊漏诊.方法 2004年2月至2008年6月在温州育英儿童医院呼吸科诊断为先天性气管支气管畸形的患儿46例,其中男29例,女17例.分析其临床、影像学、纤维支气管镜等特点.结果 46例患儿年龄3d至14岁,其中<3个月8例;3个月至1岁20例,>1～3岁15例,>3～14岁3例.均诊断为先天性气管支气管畸形,其中气管支气管软化10例,支气管起源异常15例,支气管缺如5例,气管支气管狭窄21例.复合畸形10例.发病年龄3d至14岁,均表现为反复咳嗽、反复或持续喘息,喘息多在吃奶、哭闹或运动后加重.X线胸片和CT平扫可见弥漫性或局限性透明度增强或肺不张,10例气管支气管软化通过纤维支气管镜确诊,螺旋CT三维重建诊断气管支气管狭窄21例,诊断支气管起源异常14例.结论 (1)对有下列临床特点者应该考虑到气管支气管畸形的可能:持续咳嗽经常规脸查不能明确病因;反复或持续喘息,经抗炎或支气管扩张剂治疗无效;反复或持续喉鸣;X线胸片同一部位反复或持续肺炎或肺不张;X线胸片提示局限性肺气肿、肺不张、纵隔移位而原因不明.(2)纤支镜对先天性气管支气管软化的诊断具有重要价值.(3)螺旋CT三维重建对诊断气管支气管起源异常和狭窄的诊断价值和纤支镜相当,尤其适用于年幼儿和危重儿.     

电子支气管镜在小儿不明原因呼吸困难疾病诊断中的应用

目的 探讨电子支气管镜在小儿呼吸困难疾病中的诊断价值.方法对53例临床表现为呼吸困难且常规内科治疗无明显好转的患儿行电子支气管镜检查,并对临床资料进行回顾性分析.结果 53例不明原因呼吸困难患儿中喉-气管-支气管软化22例,气管支气管畸形15例,支气管异物4例,肿瘤或支气管赘生物4例,肺结核1例,肺出血1例,未发现异常6例(考虑炎症所致),总诊断率为88.6%.结论电子支气管镜在小儿呼吸系统疾病中具有诊断和治疗双重作用,值得推广应用.     

氩气刀治疗儿童声门和声门下新生物3例

摘要 目的 探讨小儿声门及声门下疾病治疗的临床经验。方法 回顾性分析本院2011年引进氩气刀技术后,收治的4例小儿声门及声门下疾病患儿的治疗情况及转归。结果 3例声门下病变病例在手术后均未出现肉芽组织的再次增生,未发现气道内的疤痕和狭窄,顺利拔出气管切开的套管及气管插管,恢复正常呼吸。1例声门的喉乳头状瘤的患儿五个月后复查,未发现乳头状瘤复发,也顺利拔除气管切开套管。结论 电子气管镜图像清晰,镜身细小柔软,可随意弯曲,转换角度,定位准确。氩气刀喷头直径小,能顺利通过小儿的声门,操作精确,手术时间短,对周围正常组织无损伤,在小儿声门及声门下疾病的治疗中,值得推广应用。     

支气管镜在儿童气管食管瘘全程手术管理中的应用研究

目的 探讨支气管镜在儿童气管食管瘘术前、术中和术后的临床应用价值.方法 对14例疑似气管食管瘘患儿(其中1例支气管食管瘘)尝试应用支气管镜进行手术全程管理.结果 术前对4例患儿应用支气管镜检查明确诊断为气管食管瘘;运用相同方法确诊1例患儿为气管窦道,排除了气管食管瘘;利用支气管镜引导下气管插管技术,成功封堵1例因误食烧碱造成左主支气管-食管瘘.在术中应用"支气管镜透射照明"法和"导丝环"法精确定位3例气管食管瘘,减少了手术创伤和探查瘘管的时间.在术后应用支气管镜发现2例气管食管瘘复发和2例存在气管软化;支气管镜还充当了一次"超细胃镜",将导丝引入食道,然后进行食管狭窄的球囊扩张.结论 支气管镜在儿童气管食管瘘诊治中有很大的临床应用价值,可应用于气管食管瘘的全程管理.     

气管插管治疗急性喉气管支气管炎和急性喉炎

目的探讨气管插管在治疗小儿急性喉气管支气管炎和急性喉炎中的应用价值。方法2000年10月～2006年10月北京儿童医院儿童加强监护病房（PICU）收治小儿急性喉气管支气管炎和急性喉炎45例。气管插管机械通气治疗12例（27%）;其中喉气管支气管炎10例,喉炎2例。患儿均选用较正常年龄对应管号小0.5～1.0号导管行经口气管插管。插管指征：Ⅲ度喉梗阻经雾化及口服或静脉肾上腺皮质激素治疗后短期症状不缓解或进行性加重,存在威胁生命的呼吸衰竭9例;Ⅳ度喉梗阻3例,其中1例在院外行心肺复苏。结果除1例为院外心肺复苏时插管外,余患儿插管过程均顺利,操作时无窒息。患儿插管后均行呼吸机机械通气,带管时间平均3.4d（24h～9d）。所有患儿拔管后常规使用经鼻持续呼吸道正压（NCPAP）呼吸支持,平均1.5d（2h～4d）。初次拔管失败者2例,其中1例拔管后呼吸道梗阻改用气管切开治疗。患儿均治愈出院。平均住院天数11.2d（4～22d）。结论气管插管治疗可用于急性喉气管支气管炎和急性喉炎所致严重喉梗阻,多数情况下可取代气管切开成为主要治疗手段。     

Resilience in Families with a Child with Cancer

The aim of this study was to identify and explore resilience factors associated with family adaption after a child had been diagnosed with cancer. Using a cross-sectional survey research design, parents (n = 26), and children (n = 25) from the same families independently completed six self-report questionnaires, as well as responded to an open-ended question about those qualities that helped their family through the period following the diagnosis. The most significant results came from the children's data. According to these results, connectedness within the family, the experience of control over life events, family routines, positive, and supportive communication, redefinition of crisis situations, and lastly, a passive appraisal of crisis situations, were positively linked to better family adaptation. The identified factors should be strengthened and developed in families finding themselves in a similar situation.     

Therapeutic experience with hepatoblastoma associated with trisomy 18

Trisomy 18 is often fatal, but patients with this disease can now have longer survival due to proactive treatment intervention. However, hepatoblastomas may develop in these patients. In this study, we report four cases of hepatoblastoma associated with trisomy 18. All of the patients had congenital heart disease and three had undergone intracardiac surgical repair. Tumor growth was relatively slow in all cases, and there were no problems with chemotherapy tolerability and surgical resection. Three of the patients are currently disease‐free and the fourth is alive with remaining of the tumor. These cases suggest that combined chemotherapy and surgical resection may be an option to treat hepatoblastoma associated with trisomy 18 when cardiac pulmonary function is relatively stable.     

Colonization with Clostridium difficile in Children with Cancer

Objective

Clostridium difficile is a gram-positive, anaerobic, spore-forming bacillus. Usually it does not cause disease unless a patient who is colonized with toxin-producing strains has been treated with antibiotics, particularly those that change the anaerobic flora of the large intestine.

Methods

We investigated in a prospective study intestinal colonization of C. difficile and its toxins in children with malignancy that used different antibiotics and cytotoxic drugs.

Findings

One hundred fifty-two patients were included in this prospective study. Stool samples were obtained within the first 48 hours after admission and cultured for C. difficile; cytopathic effect of C. difficile was detected on HELA cells, also ELISA test was performed for detection of toxins A and B. 25% of patients had positive culture for C. difficile; 36/38 (92%) revealed positive cytopathic effect on HELA cells. No significant relation was found between age, gender, history of antibiotic consumption and C. difficile positive culture and cytopathic effect on HELA cells. The only relation was seen between cotrimoxazol usage and cytopathic effect on HELA cells (P=0.03).

Conclusion

Although the rate of C. difficile colonization (25.6%) and toxigenic strains (23.7%) in admitted children in hematologic ward is high, the rate of ELISA positive test for toxin A+B was not correspond with culture and cytopatic effect on HELA cell. With respect to sensitivity and specificity of ELISA test, possibility for existence of toxin C with cytopathic effect is high in this type of patients.     

Tyrosinemia associated with perinatal infection with cytomegalovirus.

A premature infant presented with elevated concentrations of tyrosine in blood and urine, evidence of hepatocellular damage, demineralization of the bones, and a renal Fanconi syndrome. This is the clinical picture found in hereditary tyrosinemia. The infant also had a perinatal infection with cytomegalovirus.     

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.     

Juvenile rheumatoid arthritis with myelofibrosis with myeloid metaplasia

Myelofibrosis with myeloid metaplasia is defined as a myeloproliferative disorder characterized by leukoerythroblastosis, tear drop erythrocytes, extramedullary hematopoesis and varying degree of myelofibrosis. It may be idiopathic or secondary to a large number of conditions. Here is a rare case of myelofibrosis occurring in a patient with juvenile rheumatoid arthritis.