先天性肺囊性疾病52例

目的 探讨儿童先天性肺囊性疾病的诊断、手术治疗方法及预后.方法 回顾性分析2003年1月至2012年6月在复旦大学附属儿科医院住院手术治疗的先天性肺囊性疾病儿童52例.对患儿临床表现、病变部位、影像学、病理学、漏诊误诊、手术治疗方法和短期肺功能进行统计分析.结果 52例先天性肺囊性疾病患儿中,男33例,女19例;年龄1 d～159个月,中位数为3.67个月;新生儿5例.病种:支气管源性肺囊肿42例,肺隔离症6例,大叶性肺气肿3例,先天性囊性腺瘤样畸形1例.临床表现以肺部感染为主,其中61.5％的患儿首次肺部感染后确诊,21.1％的患儿在反复呼吸道感染后确诊,约17.3％的患儿无症状.病变部位以右下肺最多见,约占36.5％.影像学以多发气囊肿最多见,约占42.3％.病理结果均提示支气管源性.漏诊率为17.3％,误诊率为24.9％,其中支气管源性肺囊肿误诊率为19.2％.治疗以肺叶切除术为主,无死亡病例.结论 小儿先天性肺囊性疾病的术前诊断主要依靠影像学检查,临床表现有助于早期识别,术前易漏诊误诊,术后病理有助于确诊,手术疗效肯定,预后好.     

先天性肺囊性疾病52例北大核心CSCD

目的探讨儿童先天性肺囊性疾病的诊断、手术治疗方法及预后。方法回顾性分析2003年1月至2012年6月在复旦大学附属儿科医院住院手术治疗的先天性肺囊性疾病儿童52例。对患儿临床表现、病变部位、影像学、病理学、漏诊误诊、手术治疗方法和短期肺功能进行统计分析。结果52例先天性肺囊性疾病患儿中,男33例,女19例;年龄1d-159个月,中位数为3．67个月;新生儿5例。病种：支气管源性肺囊肿42例,肺隔离症6例,大叶性肺气肿3例,先天性囊性腺瘤样畸形1例。临床表现以肺部感染为主,其中61．5％的患儿首次肺部感染后确诊,21．1％的患儿在反复呼吸道感染后确诊,约17．3％的患儿无症状。病变部位以右下肺最多见,约占36．5％。影像学以多发气囊肿最多见,约占42．3％。病理结果均提示支气管源性。漏诊率为17．3％,误诊率为24．9％,其中支气管源性肺囊肿误诊率为19．2％。治疗以肺叶切除术为主,无死亡病例。结论4,JI,先天性肺囊性疾病的术前诊断主要依靠影像学检查,临床表现有助于早期识别,术前易漏诊误诊,术后病理有助于确诊,手术疗效肯定,预后好。     

小儿支气管肺囊肿影像与临床病理学研究进展

小儿支气管肺囊肿临床上较为少见,由于胚胎发育过程中肺芽分支发育畸形,气管和支气管异常芽造成一段或多段支气管完全或不完全闭锁,远段逐渐扩张形成盲囊,囊内细胞分泌的粘液不能排出而积聚膨胀形成囊肿,临床表现无特异性,影像学上变化多样,易引起误诊误治。本文就小儿支气管肺囊肿的影像学表现与临床组织病理学研究进展综述如下。     

先天性肺囊性疾病

先天性肺囊性疾病是较少见的先天性肺部疾病,是肺组织胚胎发育异常所形成的畸形。囊性病变包括囊性腺瘤样畸形、支气管源性肺囊肿、先天性大叶性肺气肿、肺隔离症。此类疾病的临床表现相似,但发生机制及病理改变有各自的特点。囊性腺瘤样畸形是由于细支气管发育停滞,以肺泡不发育为代价引起的肺间质大量增生,目前根据病理类型分成5型。支气管源性肺囊肿是胚胎发育时期支气管树某段的异常出芽形成的。先天性大叶性肺气肿是支气管内阻外压以及支气管壁的病变造成的气体陷闭,肺泡腔扩张。肺隔离症是部分肺组织与正常的支气管肺组织无交通,供血来自体循环,病变肺无正常功能,分叶内型和叶外型。     

儿童先天性肺囊性疾病诊断和治疗进展

儿童先天性肺囊性疾病属临床少见肺部疾病,是肺组织胚胎发育异常所形成的畸形.根据其起源可以分为支气管源性肺囊肿、囊性腺瘤样畸形、先天性大叶性肺气肿和肺隔离症.该病临床表现无特异性,易与多种疾病相混淆,造成误诊.现对其主要发病机制、临床表现、诊断与鉴别诊断、治疗及干预措施进行阐述.     

先天性肺囊性疾病患儿基因组拷贝数变异分析

目的探讨先天性肺囊性疾病(CCLD)相关基因的拷贝数变异。方法回顾分析16例CCLD患儿的临床资料以及全基因组拷贝数变异检测结果。结果 16例患儿中男12例,女4例,年龄2个月~12岁6个月。10例患儿为支气管源性肺囊肿,其余分别为肺隔离症2例、先天性囊性腺瘤样畸形2例、先天性大叶性肺气肿1例、临床未分型1例。临床表现以发热、咳嗽、咳痰为主,无特异性。全基因组拷贝数变异检测,2例患儿有6个临床意义暂不明确的基因拷贝数变异;4例支气管源性肺囊肿患儿有HDAC8基因部分片段的异常扩增。结论 CCLD与基因拷贝数变异相关的可能性小;HDAC8基因可能为支气管源性肺囊肿相关基因。     

椎管内支气管源性囊肿三例并文献复习

支气管源性囊肿(bronchial cyst或bronchiogenic cyst,BC)为先天性疾患,通常位于纵隔,少数发生于胸骨、心包、皮肤、横膈及脊髓[1].椎管内支气管源性囊肿( spinal bronchial cyst或spinal bronchiogenic cyst,SBC)十分少见,常发生于颈段或上胸段脊髓[1,2].该囊肿发展缓慢,常常存在多年而不被发觉.如出现症状,首选的治疗方法是手术切除,术后大多数患儿的症状会得到缓解.     

罕见儿童巨大肾上腺假性囊肿1例

肾上腺囊肿为罕见病,包括寄生虫性囊肿、上皮性囊肿、内皮性囊肿、假性囊肿四类。上皮性及内皮性囊肿最多见,属于真性囊肿。肾上腺假性囊肿十分罕见,文献报道大多见于成人,发生于儿童者甚少,且临床表现及影像学检查无特异性,易误诊。本文报告儿童巨大肾上腺假性囊肿1例,其诊治体会如下：患儿,女性,年龄3岁4个月,因无明显诱因出现脐周阵发性疼痛1d人院,发病期间伴发热,最高体温达38．3℃,余无特殊不适。     

先天性肺囊性病变28例的诊断与治疗

目的 探讨儿童先天性肺囊性病变的诊断、手术时机和方式.方法 对儿童先天性肺囊性病变28例的临床资料进行回顾性分析.男19例,女9例;年龄1～12岁,平均5.4岁,其中1～5岁11例.先天性肺囊肿21例,其中支气管源性囊肿13例;肺源性囊肿6例;混合型2例.肺隔离症7例.术前诊断主要依靠胸部X线片和CT,术前确诊21例,其中先天性肺囊肿17例,肺隔离症4例.术前误诊7例,误诊率25%,其中误诊为肺脓肿、纵隔占位各2例,气胸、肺大疱、支气管扩张症各1例.行肺囊肿摘除术 3例,肺段切除术2例,肺叶切除17例,其中2例为急诊手术,隔离肺切除4例,双肺叶、肺叶加肺段切除术各1例.结果 本组无死亡病例,术后恢复良好,均痊愈出院,随访1～5 a,患儿生长发育及活动正常.结论 儿童先天性肺囊性病变误诊率高,诊断主要依靠影像学检查,确诊后应尽早手术,手术疗效满意,预后良好.     

电视辅助胸腔镜手术在小儿支气管源性囊肿外科治疗上的应用

目的 探讨电视辅助胸腔镜手术(VATS)在小儿支气管源性肺囊肿治疗中的作用.方法 回顾性分析笔者两院1993年6月至2007年6月经VATS切除并病理证实的41例小儿支气管源性肺囊肿患儿的临床资料.麻醉方式全部采取单腔管插管吸入全麻,手术采取胸腔镜进行,必要辅助小切口协助将病灶以及肺组织取出;气管残端采取间断缝合或者Endo-cutter钉合,统计分析手术时间、切口大小、术后引流时间及总量、术后住院时间.结果 患儿均经VATS手术治疗.年龄从8d～12岁,平均为4.81岁.2例纵隔型支气管囊肿行VATS纵隔囊肿切开+翻转术,14例肺内型行肺楔形切除术,25例肺内单发性囊肿或局限于肺段的多发性肺囊肿行肺叶切除术.全组手术时间为45～265min,平均71.5 min;切口为1.5～7.0 cm.平均4.4 cm;术中失血5～400ml,平均41.5 ml;术后均放置胸管1～2条,引流1～7 d,平均2.0 d;总引流量1 5～770 ml,平均207.2 mt;平均术后住院时间为1～25 d,平均7.0 d.结论 VATS的d,JL支气管源性肺囊肿治疗是可行、安全、彻底、微创的,可成为标准术式.     

Clinical data in children with meningococcal meningitis in a Spanish hospital

Neisseria meningitidis is the main cause of bacterial meningitis in Spain. Of the 213 children included in this study with meningococcal meningitis, 7 died. Mortality was linked to a shorter time from the first symptom to diagnosis (mean time for fatal cases was 9.5 h, mean time for survivors was 19h, p = 0.034), to deteriorated consciousness (DC) (mortality rate (MR) with DC = 6/87, MR without DC = 1/124, p = 0.02) and to shock (MR with shock = 5/7, MR without shock = 2/206, p < 0.0001). Previous treatment reduced the yield from blood culture (36/54 versus 45/137, p < 0.0001). Positivity in both Gram stain (GS) and cerebrospinal fluid (CSF) culture increased with longer duration of symptoms (mean GS+= 25h, GS^-= 16h, p = 0.004; CSF+= 20h, CSF^-= 12h, p = 0.001), and blood culture (BC) gave more positive results when carried out earlier (mean BC+= 14 h, BC = 24 h, p < 0.001). Reduced susceptibility to penicillin was seen in 34% of the strains, and rapidly evolving forms were responsible for most of the deaths; reduced susceptibility was more frequent among strains responsible for death or sequelae (9/15 = 60%) as compared with the more harmless strains (69/ 215 = 32%) ( p = 0.04). The progressive reduction of susceptibility to penicillin indicates that it should be replaced by a third-generation cephalosporine.     

Lingual Bronchogenic Cyst in a Child: An Unusual Site of Presentation

We describe a 4-year-old hispanic male with a bronchogenic cyst (BC) in the base of the tongue. To our knowledge, a BC has not been previously reported in the oral cavity. BC is a developmental anomaly believed to originate from abnormal detachments of accessory lung buds from the ventral foregut. We postulate that the unusual location of the BC in the present case is related to the primitive foregut origin of tongue epithelium posterior to the foramen cecum and the sulcus terminalis. A BC in the base of the tongue appears to represent the most proximal expression of aberrant accessory lung bud detachment from the primitive foregut.     

Bronchogenic cyst of the right hemidiaphragm mimicking a hydatid cyst of the liver: report of the first pediatric case

Bronchogenic cyst (BC), which develops from abnormal budding of the tracheal diverticulum or ventral foregut, is a congenital bronchopulmonary malformation. Localization of the BC varies depending on the level of the abnormal budding. Thoracic or abdominal-sited diaphragmatic lesions are the rarest presentations of the BC. We present a case of BC that originated from the diaphragm and mimicking hydatid cyst of the liver in a 19-month-old girl. Diagnosis of a diaphragmatic lesion was confirmed during laparotomy and complete resection was successful.     

Pulmonary evolution of cystic fibrosis patients colonized by Pseudomonas aeruginosa and/or Burkholderia cepacia

We analysed the pulmonary evolution (radiological scores and pulmonary function) of 81 cystic fibrosis (CF) patients colonized by Pseudomonas aeruginosa (PA), by Burkholderia cepacia (BC) or by both these bacteria, compared to a control group. Pulmonary function was compared in the age bracket 6–13 years. Functional vital capacity (FVC) and forced expiratory volume (FEV₁) values for PA colonized patients were significantly worse than for the control group but better than for children colonized by both organisms. In this last group, the evolution of radiological scores and pulmonary function showed a greater decline 2 years after the first colonization compared to the other groups. FVC and FEV₁ values in patients colonized by BC were not worse than these of patients colonized by PA. Moreover, BC affected older patients with advanced lung disease and often previously colonized with PA. These results suggested that co-colonization by PA and BC could be a more deleterious factor on the pulmonary evolution than the isolated colonization by PA or BC, and that BC could be a severity marker rather than a cause. In addition, after starting the utilization of mouthpieces with filter at single use for spirometry in 1993 (without any other change in preventive measures already taken during hospitalization), incidence of BC decreased from 8.2% to zero, and no new case of BC colonization has been observed over the last 4 years. Conclusion Co-colonization of CF patients by PA and BC is more deleterious for pulmonary evolution than colonization by one of these bacteria alone. Re-inforcement of environmental measures during hospitalization (e.g. use of disposable mouthpieces for spirometry) was sufficient to reduce the transmission of BC. Received: 4 February 1997 and in revised form: 16 September 1997 / Accepted: 23 September 1997     

Using BiliCheck for preterm neonates in a sub-intensive unit: diagnostic usefulness and suitability

BACKGROUND: BiliCheck (BC), a new transcutaneous bilirubinometer is thought to be lacking in the disadvantages of old devices and could be potentially useful for diagnosing jaundice in preterm babies. Although its accuracy is well known in healthy term babies, there is a lack of knowledge about its usefulness in preterm infants. AIMS: To investigate BC usefulness in preterm babies and its suitability in a sub-intensive neonatal unit. STUDY DESIGN: In 340 preterm infants between 30 and 36 weeks of gestational age, transcutaneous and serum bilirubin measurement were performed. Hematocrit, pH, postnatal age, gestational age, and sex were also studied to clarify their influence on BC accuracy. For a subset of 100 neonates transcutaneous measurement, blood collection and serum analysis were timed and costs were considered. RESULTS: Correlation coefficient is 0.795 (p<0.001) and this is not affected by factors previously supposed to be important. Overall sensitivity was 100% and specificity were comprised between 40% and 72%. BC has a tendency to overestimate serum bilirubin, at high values. Considering the whole time for serum bilirubin measurement, transcutaneous bilirubinometry is a faster (p<0.0001), but more expensive technique with a cost of about 5 euro/measurement. Nevertheless, using BC as a screening-device we could safely avoid 58-79% of blood samples, since its positive predictive values is about 21-42%. This would allow to a cost reduction of 1555-2120 euro/year. CONCLUSIONS: BC has a good reliability in preterm infants although not as good as in healthy term babies. BC is a time-sparing tool and can improve the management of neonatal jaundice in preterm infants; however, its tendency to overestimate suggests its use only for screening purposes.     

黄芩甙对新生鼠脑血肿继发脑水肿的治疗研究

目的　探讨黄芩甙对实验性新生大鼠脑血肿继发脑水肿的作用机制和治疗效果。方法　新生 7日龄健康SD大鼠 39只 ,随机分为 3组 :脑血肿组 ,黄芩甙组和对照组。脑血肿组和黄芩甙组采用自体血制作脑出血模型 ,3组大鼠均测定脑系数并用干湿法测脑组织水分 ,同时病理镜检观察脑出血组和黄芩甙组脑组织变化 ,并采用高效液相色谱 荧光法测定兴奋性氨基酸脑组织谷氨酸 (Glu)和抑制性氨基酸γ 氨基丁酸 (GABA)含量变化。结果　脑血肿形成后 ,脑系数和脑组织含水量均明显增加 ,黄芩甙组明显小于脑血肿组 (P <0 0 1) ,而黄芩甙组与对照组比较脑系数和脑组织含水量均变化不大 ;脑血肿组大鼠脑组织Glu含量显著高于对照组和黄芩甙 (P <0 0 1) ,而黄芩甙组和对照组Glu含量变化不大 (P >0 0 5 ) ;在脑血肿组大鼠GABA含量增加 (P <0 0 1) ,而黄芩甙组增加更明显 ,与对照组和脑血肿组比较均有显著性差异 (P <0 0 1)。结论　黄芩甙能减轻实验性脑血肿继发的脑水肿 ,黄芩甙对实验性脑血肿继发性脑水肿的保护作用可能与黄芩甙通过抑制兴奋性氨基酸 (EAA)大量释放 ,同时大量增加抑制性氨基酸以拮抗兴奋性氨基酸Glu的神经毒性作用有关。     

黄芩甙 川芎嗪诱导大鼠脑组织热休克蛋白70合成

目的：探讨黄芩甙、川芎嗪是否通过诱导热休克蛋白(HSP70)合成增加来保护脑组织。方法：SD大鼠随机分为①正常对照组,②热休克处理组,③感染性脑水肿组,④黄芩甙组,⑤川芎嗪组。其中黄芩甙和川芎嗪又分为小剂量、治疗量及大剂量各3组。采用Western印迹杂交技术检测各组的HSP70的表达。结果：正常对照组、黄芩甙小剂量组、川芎嗪小剂量组及感染性脑水肿组均有一定量的HSP70合成,而黄芩甙、川芎嗪的治疗量及大剂量组以及热休克处理组均见明显的HSP70条带。结论：黄芩甙、川芎嗪能诱导脑组织HSP70合成明显增加,两药对感染性脑水肿的治疗机制可能与HSP70合成增加有关。     

Bronchial challenge with purified protein derivative of Mycobacterium tuberculosis in asthma.

Response to bronchial challenge (BC) with purified protein derivative of Mycobacterium tuberculosis (PPD), was studied in children with bronchial asthma and correlated with Mantoux test and serum immunoglobulin E (IgE) against PPD (PPD-Sp IgE). Nearly 59% patients with bronchial asthma and 12.2% patients with pulmonary tuberculosis gave positive BC. Children with extra-pulmonary tuberculosis and normal children did not show positive BC. In asthma, 25% gave early (EAR), 50% gave late (LAR) and 25% gave both early and late (DAR) asthmatic response. Forced expiratory flow volumes in 1 sec (FEV.1) of 13 age and sex matched asthmatic and normal children showed similar volumes before BC, however, the values were significantly lower in asthma at 20 min (p less than 0.005) and 24 h (p less than 0.005) after BC. There was no relationship between response to BC and the severity or chronicity of asthma. PPD-Sp IgE was estimated by the radioimmunoassay method (Pharmacia Diagnostics). It was detected in 75% with positive BC and none of the controls. The titre was of Phadebas RAST Class III in 66.7%, Class II in 22.2% and Class I in 11.1%. The presence of early Mantoux reactions, positive BC with PPD and serum PPD-Sp IgE suggest the existence of Type I or Arthus type of reactions to PPD, which could cause hyperreactive airways in some cases of asthma.     

Imaging of biliary complications following paediatric liver transplantation

Biliary complications (BC) are well recognised following paediatric liver transplantation. We reviewed retrospectively 169 consecutive liver transplants performed in 139 children. BC occurred in 36/169 grafts (21 %) in 35/139 patients (25 %). Biliary obstruction was present in 18/169 grafts (11 %), biliary leakage in 14/169 grafts (8 %) and a combination of obstruction and leakage was present in 4/169 (2 %) grafts. BC were as likely to present radiologically as they were with either clinical and/or biochemical abnormalities. Most BC (26/36, i. e. 72 %) occurred in the first 2 weeks following transplantation. Ultrasound and cholangiography were the principle imaging modalities used for detection of these complications. False negative ultrasound examinations occurred in three patients with biliary obstruction and in three patients with biliary leakage. False negative cholangiograms occurred in two patients with biliary leakage. Ultrasound is important in the post-operative surveillance of paediatric liver transplants, with cholangiography having a complementary role in those with BC.     

Biliary complications after transplantation in children: Role of imaging modalites

Among a series of 140 liver transplantations in children, 21 biliary complications (BC) (15%) are reported. BC were identified from 2 days to 3 months after LT. Positive US findings were present in 20 cases. Cholangiography was obtained by opacification of a surgical drain in 3 cases, per-operatively in 3 and by PTC in 15. Drainage was placed in 9 and ballon dilatation performed in 2. Causes of BC include hepatic artery (HA) thrombosis in 7, HA stenosis in 1, anastomosis stricture in 7, anastomosis kink in 3, mucocele of cystic duct remnant in 2 and sludge in 1. Treatment was surgical in all, but 2 cases were treated percutaneously. There is a great difference in severity of prognosis between complications secondary to HA thrombosis and isolated BC. Role of US in diagnosis and of PTC and interventional radiology in treatment are emphasized.Presented at the ESPR meeting in Munich 1990. Selected for publication by an International Group of the ESPR