婴幼儿多发性大动脉炎14例临床特点分析

目的通过分析总结14例婴幼儿多发性大动脉炎(TA)的临床特点,并结合文献复习总结,以提高对该病的认识及诊疗水平。方法回顾性分析2016年7月至2019年5月在首都儿科研究所附属儿童医院住院的TA婴幼儿的临床资料及随访情况,并结合文献,分析总结该病的临床特点。结果14例患儿年龄为1个月23 d^28个月,男6例,女8例。临床表现中最常见的是发热[10例(71.4%)],高血压9例(64.3%),脉弱或无脉5例(35.7%)。按照病变血管部位的临床分型,广泛型11例(78.5%),头臂动脉型3例(21.4%),本组患儿无胸腹主动脉及单纯肺动脉型。14例TA患儿中,12例有颈总动脉、颈动脉、锁骨下动脉及冠状动脉及其分支(前降支、回旋支)受累(85.7%);11例肾动脉受累(78.6%);9例腋动脉受累(64.2%);8例腹主动脉受累(57.1%);6例降主动脉受累(42.9%);6例胸主动脉受累(42.9%);6例肠系膜上动脉受累(42.9%);5例股动脉受累(35.7%);5例肺动脉受累(35.7%);4例肱动脉受累(28.6%)。14例患儿中,误诊11例,诊断不清3例,误诊时间为18 d^2个月。误诊病例中,8例误诊为不典型川崎病。14例患儿中,7例治疗后大部分病变血管范围逐渐减少,受累较轻血管甚至可以完全恢复正常血管状态。4例患儿血管影像学检查较前无明显加重或好转。9例出现高血压患儿应用降压药血压能控制在正常范围,但不能停用降压药。5例查体发现脉弱或无脉患儿均未改善。14例患儿中7例生长发育同正常同龄儿,7例落后于正常同龄儿身高体质量第25百分位。14例患儿随访2~22个月,均规律治疗,未出现复发。结论3岁以内TA累及血管较多,病情严重,误诊率高,经过治疗病情很快能够控制,但容易遗留血管病变,部分患儿预后不佳。     

儿童肺动脉吊带与双主动脉弓伴随的肺气管畸形比较

目的评价血管环中双主动脉弓与肺动脉吊带伴随的气管狭窄与气道畸形的异同。方法回顾性分析2010年7月至2015年7月的双主动脉弓和肺动脉吊带患儿的临床和螺旋CT影像学资料。结果双主动脉弓患儿16例,男11例、女5例,发病中位年龄3.5个月;肺动脉吊带患儿47例,男28例、女19例,发病中位年龄4个月。16例双主动脉弓患儿中14例伴随气道狭窄,累及多个分段者2例,均为累及分段Ⅱ、Ⅲ;仅累及1个分段者12例。47例肺动脉吊带患儿均发生不同程度气道狭窄,其中狭窄累及多个分段者27例,最常见的是同时累及分段Ⅱ、Ⅲ者19例;2例同时累及4个气道段,仅累及1个气道分段者20例。两者累及气道分段数量差异有统计学意义(χ~2=13.588,P=0.001)。16例双主动脉弓患儿合并气管性支气管1例,合并肺发育不良1例;47例肺动脉吊带患儿中合并支气管桥8例,合并气管性支气管3例,合并肺发育不良3例,支气管缺如(或气管憩室)5例,合并肺前肠畸形2例。两者合并气道畸形的差异有统计学意义(χ~2=5.333,P=0.021)。结论肺动脉吊带合并的气道狭窄和气道肺畸形较双主动脉弓更为突出。     

Ӥ�׶��ν�˶������CT���������о�

??Objective To investigate and analyze the characteristics of MSCT manifestation of infants and young children with intra-thoracic tuberculosis. Methods A retrospective analysis of MSCT chest performances of 76 cases of infants and young children ??ages 0-3 years??with intrathoracic tuberculosis from January 2010 to June 2012 in our hospital was done to find the characteristics and classify pulmonary lesions of it. Results In this study?? the male to female ratio was 48:28?? 7 cases were miliary tuberculosis?? 6 cases were with cavitates?? 15 cases with tuberculous meningitis. 6 cases had no pulmonary lesions?? 18 cases only had single lobe involved??and 31 cases had four or more than four lobes involved??among all the cases?? the right upper lobe was involved in 48 cases?? right middle lobe 43 cases?? right lower lobe 53 cases?? left upper lobe 36 cases?? left lower lobe 40 cases. We classified cases of pulmonary lesions into five types based on the CT features: ??1?? nodular type: pulmonary lesions were mainly nodular?? a total of 16 cases?? ??2?? inflammatory type: pulmonary lesions were ground-glass opacities?? patches?? cords and ??or?? consolidation?? 24 cases?? ??3?? mass type: pulmonary lesions were mainly clumps?? 19 cases?? ??4?? military type: 7 cases?? ??5?? endobronchial tuberculosis type: 4 cases.Totally 71 cases of hilar and mediastinal adenopathy were found?? and mainly in 2R ??75.00%???? 4R ??76.32%???? district 5 ??57.89%???? district 7 ??76.32%???? 8 ??68.42%?? and 10R ??75.00%???? 11R ??64.47%??. Most of the swollen lymph nodes in plain CT scan were medium-density?? and calcification was found in 40 cases. Totally 39 cases had enlarged axillary lymph nodes. There were 8 cases with left pleurisy?? 9 cases with right pleurisy. Conclusion The main MSCT manifestation of infants and young children with intra-thoracic tuberculosis is pulmonary lesions with hilar and mediastinal adenopathy?? but the majority of pulmonary lesions of them on CT are different with primary complex?? hilar and mediastinal adenopathy??enlarged axillary lymph nodes and lymph nodes with calcification are common??while endobronchial tuberculosis??pleurisy and cavitas are rare.     

儿童Leigh综合征4例临床分析

目的探讨儿童Leigh综合征的临床表现、诊断及预后。方法回顾性分析4例通过基因检测确诊的Leigh综合征患儿临床资料,并复习相关文献。结果 4例患儿中,男3例、女1例,其中3例婴幼儿起病、1例学龄期起病,主要表现为智力运动发育落后、肌张力低下、惊厥、喂养困难、眼睑下垂、眼外肌麻痹及眼球震颤、易惊、活动不耐受等。头颅MRI,脑干、双侧苍白球、丘脑、小脑齿状核、中脑导水管周围对称性长T1长T2异常信号;其中累及中脑3例、丘脑1例、小脑齿状核1例;2例提示脑萎缩。肌电图检查均正常。血、脑脊液乳酸均增高。线粒体DNA(mt DNA)检测,1例为mt DNA 8993 TG突变,其余3例mt DNA9176 TC突变。学龄期起病患儿发病1月后死于呼吸衰竭,另3例仍在随访中,有智力运动发育落后,但无明显倒退。结论儿童Leigh综合征临床表现多样,诊断主要依靠典型临床表现及MRI、血和/或脑脊液乳酸水平升高为依据,基因检测为诊断金标准。     

流行性病毒感染致危重症儿童急性肾损伤临床分析

目的 分析流行性病毒感染危重症患儿急性肾损伤(AKI)的发生情况,并探讨血肌酐及尿量在AKI诊断中的意义.方法 回顾性分析急诊科住院的甲型H1N1流感病毒感染患儿(H1N1组)及肠道病毒EV71感染患儿(EV71组)临床资料.结果 共28例,H1N1组18例(男6例,女12例),平均年龄5.4岁,EV71组10例(男8例,女2例),平均年龄1.1岁.(1)H1N1组4例发生AKI,平均受累脏器5.3个,2例1期患儿治疗后痊愈;2例3期患儿死亡;14例未发生AKI,平均受累脏器3.0个,死亡4例.(2)EV71组:3例发生AKI(1期)者与3例血肌酐升高45.0％～ 47.6％者平均受累脏器5.7个,均死亡;4例无血肌酐升高者平均受累AKI脏器3.0个,病情好转.结论 流行性病毒感染所致危重症,发生AKI者受累脏器更多;H1N1感染AKI程度轻者早期积极干预预后相对好,程度重者预后差;E V71感染发生AKI则预后极差.诊断儿童AKI血肌酐较尿量的敏感性高.     

小儿先天性冠状动脉瘘的临床诊断与分析

目的探讨先天性冠状动脉瘘的临床诊断特点.方法回顾性分析有完整病史资料并经手术治疗的小儿先天性冠状动脉瘘21例.结果21例中右冠状动脉瘘15例占71.4%,左冠状动脉瘘6例占28.6%,19例引流到右心系统占90.5%,2例引流到左心系统占9.5%;合并其他畸形3例占14.3%.结论先天性冠状动脉瘘最重要的临床诊断特征是心前区异常位置的连续性杂音,杂音的性质与分流部位、压力相关.彩色多普勒血流显像仪和升主动脉或冠状动脉造影能够明确诊断,可显示迂曲扩张的冠状血管与其引流的心腔部位     

Recurrent Staphylococcus aureus abscess and fatal pneumococcal septicemia due to IRAK‐4 deficiency

We describe the case of an infant with recurrent episodes of staphylococcal skin abscess and subsequent lethal pneumococcal meningitis/septicemia due to interleukin‐1 receptor‐associated kinase 4 (IRAK‐4) deficiency. In this case, systemic signs of inflammatory response were poor and delayed. Among all other reported cases of IRAK‐4 deficiency, none involved severe viral or fungal disease, and the range of infecting bacteria was narrow.     

Tuberculous osteomyelitis of the long bones and joints

Skeletal tuberculosis(TB) is usually a rare osteoarticular disease in which bones or joints are involved. We studied 4 such cases admitted to Iranian National Research Institute of Tuberculosis and Lung Diseases from 2000 to 2003. In the initial stages of the disease, diagnosis is very difficult and conventional radiographies often reveal nonspecific findings. Our experience in these cases showed that a high index of suspicion is the most important step in early diagnosis of skeletal tuberculosis.     

腹型过敏性紫癜患儿的临床、内镜及病理学特点

目的研究腹型过敏性紫癜患儿的临床表现、内镜及病理学特点,以提高对腹型过敏性紫癜的诊断水平。方法以浙江大学医学院附属儿童医院2002年1月-2007年5月住院的57例行上消化道内镜检查时未出现皮肤紫癜患儿为研究对象,回顾性分析腹型过敏性紫癜患儿的临床、内镜及病理学资料。结果消化道症状主要为腹痛46例（80．7％）、呕吐32例（56．1％）、便血11例（19．3％）、腹泻4例（7．0％）、腹胀1例（1．8％）;同时伴关节肿痛3例（5．3％）。实验室检查主要为外周血白细胞增高（33例,57．9％）,1／5左右的病例出现C反应蛋白升高、白蛋白降低或腹腔积液情况。内镜下主要表现37例为略高出黏膜的点状出血和淤斑或成片状（64．9％）,14例出现糜烂或多发溃疡伴出血（24．6％）,4例颗粒状或结节状增生（7．0％）,弥漫性出血2例（3．5％）。病变在十二指肠降段出现率最高且最严重（55例,96．5％）,其次为十二指肠球部（32例,56．1％）、胃（18例,31．6％）,食道较少（1例,1．8％）。组织病理学呈毛细血管炎表现,3例（5．3％）的患儿同时伴幽门螺杆菌感染。49例（86．0％）患儿在内镜检查后1～7d内出现皮肤紫癜,8例至出院未出现皮肤紫癜,其中2例失访,6例（10．5％）随访至今（1～5年）未见皮肤紫癜。结论十二指肠降部病变在腹型过敏性紫癜极其常见,上消化道内镜检查对腹型过敏性紫癜有极高的诊断价值。     

Early intervention in case of child abuse: Co-operation between the hot-line and local facilities

The Center for Child Abuse Prevention (CCAP), a citizens' organization, was started in May 1991 with such members as pediatricians, psychiatrists, lawyers and people working at child welfare offices. A hotline dealing with child abuse was set up immediately after the establishment of CCAP on 20 May 1991, and in the 2 years it has been in operation this hot-line has received 3189 calls out of which we picked 30 cases that CCAP was consulted about by helping persons or therapists affiliated with such facilities as hospitals, public health centers, etc. in order to evaluate how CCAP co-operates with these organizations. We reviewed the CCAP records of these 30 cases, comparing them with the materials concerning other hot-line calls, while at the same time interviewing the helping persons and therapists who had called in those cases, ascertaining their motives and the later course of the cases. This follow-up study was begun in June 1992 so that the longest follow-up period from the first call was 2 years and 7 months and the shortest 7 months. A total of 73.3% of these 30 cases required emergency intervention, and 53.4% were extremely severe, life-threatening cases of child abuse. The age distribution of these 30 victimized children peaked between 4 and 6, and 13 and 15 years. The perpetrator was the biological mother in 55.9% of the cases, the biological father in 29.4% and the stepfather in 8.9%. The abuse was physical in 58.3% of the cases, sexual in 8.9%, emotional in 5.6%, and involved neglect in 22.2%. These percentages differ from the general trend seen in the total hot-line cases, but were very similar to those reported by the Child Guidance Center. In the majority (56.7%) of the cases CCAP was consulted about, CCAP went beyond its hot-line service and co-operated to intervene, organizing network sessions in which the helping persons and therapists involved could discuss how best to deal with the problem at hand in five of these cases. In 22 of these 30 cases, the persons involved are still co-operating with CCAP after successful intervention through which the victimized children were rescued from critical situations. It is vitally important to set up networks between the professionals of the various support agencies involved if we are to intervene successfully in cases of child abuse. Indeed, one should only proceed with the intervention once the various facilities involved have agreed on who should take care of therapy and/or care, and on how to proceed and at what pace.     

Many faces of Hirschsprung's disease

Hirschsprung's disease (HSCR) is the main genetic cause of functional intestinal obstruction with an incidence of 1/5000 live births. The etiology of HSCR is complex and is presumed to be a sex-influenced multifactorial disorder, with contributions from several genes. All the genes involved in HSCR are also involved with the early development of the enteric nervous system. HSCR is known to be associated with a chromosomal abnormality in 12 % of cases, and with other congenital anomalies in additional 18 % of cases. It is recommended that patients, including newborns, with HSCR undergo a careful assessment by a clinician trained in dysmorphology. Echocardiography, ultrasonography for urogenital malformations and skeletal x-rays should be routinely performed in cases with HSCR to rule out associated anomalies. HSCR associated with dysmorphic features or any additional systemic anomaly should prompt chromosomal studies. Genetic counseling should be provided to families of HSCR patients as the recurrence risk varies from 4 % to up to 50 % depending on whether it is non-syndromic or part of a specific syndrome.     

小儿肠系膜乳糜囊肿

目的探讨小儿肠系膜乳糜囊肿的临床特点及诊治方法。方法回顾性分析我院1987～2003年经手术治疗及病理检查证实的6例小儿肠系膜乳糜囊肿的临床表现及治疗经过。结果本组男4例，女2例，年龄7个月～10岁（平均3岁10个月）。病程30h～2年，病程在7d以内4例。5例表现为急腹症，1例表现为慢性复发性腹痛。术中证实空肠系膜囊肿4例，回肠系膜囊肿2例，囊肿压迫肠管并发肠梗阻2例，并发肠扭转1例，并发乳糜腹2例。3例行囊肿及部分肠管切除吻合术，3例行单纯囊肿切除术，囊内液均呈乳糜状，苏丹Ⅲ染色及乳糜试验阳性。结论小儿肠系膜乳糜囊肿主要表现为腹痛伴腹腔囊性包块，常以急腹症为首发表现，手术切除囊肿及受累肠管预后良好。     

舌下含服粉尘螨滴剂治疗支气管哮喘伴变应性鼻炎的疗效

目的 探讨舌下含服粉尘螨滴剂特异性免疫治疗支气管哮喘(哮喘)伴变应性鼻炎的疗效及安全性.方法 选取516例年龄4～13岁哮喘伴变应性鼻炎患儿.其中291例完成1 a舌下免疫治疗(免疫治疗组),非免疫治疗对照组225例.患儿均完成10种常见变应原皮肤点刺试验,点刺试验结果呈阳性反应.免疫治疗组根据皮肤点刺试验结果分尘螨过敏组80例、尘螨及蟑螂过敏组71例、尘螨及花粉过敏组74例、尘螨及狗毛过敏组66例.应用粉尘螨滴剂进行临床免疫治疗,记录治疗前后哮喘控制问卷(ACQ)评分、鼻炎症状评分、用药情况和不良反应.结果 1.治疗12个月后,免疫治疗组和非免疫治疗对照组ACQ评分分别为(0.28±0.33)分和(1.07±0.68)分,与治疗前[(1.76±0.75)分和(1.55±0.62)分]比较,分别下降了(74.03±37.66)%和(29.32±44.53)%,2组ACQ评分比较差异有统计学意义(Z=-154.109,P<0.000 1).2.治疗12个月,免疫治疗组和非免疫治疗对照组鼻炎症状评分分别为(0.337±0.479)分和(0.560±0.634)分,较治疗前[(0.899±0.667)分和(0.892±0.688)分]分别有70.8%和39.1%的患儿评分级别降低,2组比较差异有统计学意义(χ2=51.949,P<0.000 1).3.治疗12个月,免疫治疗组和非免疫治疗对照组治疗哮喘月均用药评分分别为(20.91±18.03)分 和(85.22±47.84)分,与治疗初始月均用药评分[(113.41±35.02)分和(108.86±35.24)分]比较,分别下降了(75.10±28.80)%和(20.60±39.52)%.4.治疗12个月,免疫治疗组肺功能呼气峰流速(预计值百分比)[(91.38±8.82)%]较治疗前上升(8.84±9.64)%.5.免疫治疗结束,免疫治疗组粉尘螨试验阳性级别降低,与非免疫治疗对照组比较差异有统计学意义(χ2=70.850,P<0.000 1).6.各免疫治疗亚组ACQ评分、月均用药评分和粉尘螨皮试阳性级别降低的差异均无统计学意义.7.与用药相关的皮疹、鼻咽痒和哮喘发作不良反应发生率为24.7%,未出现过敏性休克等严重不良反应.结论 特应性舌下免疫治疗方法安全有效,是治疗儿童哮喘伴变应性鼻炎的重要措施之一.     

儿童系统性红斑狼疮合并脑后部可逆性脑病综合征4例并文献复习

目的 分析儿童系统性红斑狼疮（SLE）合并脑后部可逆性脑病综合征（PRES）的临床特征,提高对本病的认识。方法 报告北京协和医院诊断的4例儿童SLE合并PRES的临床资料,在PubMed数据库检索相关病例行文献复习,分析儿童SLE合并PRES的临床表现、影像学检查、治疗及预后情况。结果 ①4例SLE患儿均伴有狼疮性肾炎,从确诊SLE至出现PRES的时间1~63个月,出现PRES时1例正在接受甲泼尼龙及环磷酰胺(CTX)冲击治疗。PRES均以惊厥、头痛起病,并伴血压升高。②PubMed数据库检索到11例SLE合并PRES的儿童病例,结合本文报道的4例,15例进入分析。女性14例。年龄最小8岁。从确诊SLE至出现PRES的间隔中位时间为6个月（1个月至8年）。15例出现PRES时均有惊厥发作,10例伴头痛,7例呕吐,9例意识丧失,7例视力障碍。15例均有血压升高。12例有狼疮性肾炎。治疗SLE予甲泼尼龙或CTX冲击治疗分别为4和3例,予羟氯喹2例,予环孢素和利妥昔单抗各1例,PRES分别发生在免疫抑制剂治疗后的2 d至4年。15例行头颅MRI检查示大脑后循环皮质下白质受累为主。12例予降血压治疗,10例予抗惊厥药物短期治疗。9例SLE处于活动期,继予糖皮质激素和免疫抑制剂治疗;6例非活动期的SLE患儿减停糖皮质激素及免疫抑制剂。15例神经系统症状均恢复,随访均未遗留神经系统后遗症。9例复查头颅MRI示颅内病变完全或基本消失。结论 儿童SLE合并PRES主要表现为惊厥、头痛、意识障碍和视觉障碍。对于有狼疮性肾炎的SLE患儿血压升高时,尤其同时予大剂量糖皮质激素或CTX等免疫抑制剂治疗时,应警惕PRES的发生。早期诊断和治疗PRES预后较好。     

机器人辅助腹腔镜下Lich-Greqoir手术治疗儿童原发性膀胱输尿管反流

目的探讨机器人辅助腹腔镜下Lich-Greqoir手术治疗儿童原发性膀胱输尿管反流的可行性、安全性和手术技巧。方法回顾性分析2014年5月至2019年2月上海市儿童医院收治的11例原发性膀胱输尿管反流患儿临床资料,其中男童10例,女童1例,均应用达芬奇系统辅助腹腔镜行Lich-Greqoir输尿管再植手术。患儿平均年龄9.4岁(3.2~18岁)。其中双侧4例,单侧7例。反流程度Ⅱ~Ⅳ级(Ⅱ级2侧,Ⅲ级3侧,Ⅳ级10侧)。结果患儿均采用Lich-Gregoir术式,术中患侧输尿管平均直径9 mm(5~15 mm),平均黏膜下隧道长度4.4 cm(3~6 cm)。术中无中转开放手术,单侧输尿管手术平均时间152 min(132~175 min),双侧输尿管手术平均时间257 min(249~264 min),术后平均住院时间4.5 d(3~6 d),1例双侧VUR患儿术后出现短期尿潴留,延长留置导尿管2周后痊愈。患儿平均随访时间2.5年(6个月至5.3年),所有病例均无发热性尿路感染,B超均未见患侧输尿管肾盂积水加重。术后完成VCUG 9例(其中双侧4例,单侧5例),其中1例双侧病例术后发现左侧反流Ⅰ级。结论机器人辅助腹腔镜下Lich-Greqoir手术治疗儿童原发性膀胱输尿管反流安全、有效,可以应用于3岁以上儿童的治疗。     

儿童伴IRF4基因重排大B细胞淋巴瘤6例病例系列报告并文献复习

背景 儿童伴干扰素调节因子4(IRF4)基因重排大B细胞淋巴瘤(LBCL)在WTO淋巴瘤分类(2017)中被定义为一种独特亚型,临床罕见,有其特殊的临床特征,有必要将其与其他LBCL进行区分.目的 探讨儿童伴IRF4基因重排LBCL的临床表现、病理特点、治疗方案和预后等方面的特征.设计病例系列报告.方法 回顾性收集20...     

新生儿低血糖性脑损伤磁共振成像动态变化研究

目的探讨弥散加权成像（DWI）、常规磁共振成像（MRI）在新生儿低血糖性脑损伤不同阶段的动态变化。方法回顾分析了2005年9月至2008年9月,中国医科大学附属盛京医院新生儿科收治的经MRI确诊的20例低血糖性脑损伤患儿（病例组）的临床资料,并随机选取同期住院MRI正常的20例单纯性低血糖患儿为对照组。 结果病例组平均最低血糖值低于对照组（P < 0.01）,低血糖持续时间长于对照组（P < 0.01）。病例组于低血糖发生后3.8（1～11）d完成首次MRI检查,受累部位主要为枕叶11例、枕顶叶8例,顶叶1例,受累部位在DWI均表现为高信号,常规MRI相应部位12例表现为T1加权成像（T1WI）、T2加权成像（T2WI）正常信号,仅6例表现为T1WI低信号、T2WI高信号;11例于首次检查后11.4（8～15）d完成第2次MRI检查,首次检查受累部位DWI 7例转为正常信号,4例低信号,常规MRI均表现为T1WI低信号、T2WI高信号。3例于6个月随访,提示枕叶DWI正常信号,T1WI低信号、T2WI高信号。结论新生儿低血糖性脑损伤早期DWI表现为异常高信号的部位,与晚期常规MRI表现为T1WI低信号、T2WI高信号的部位一致,这种一致性提示DWI异常高信号对于低血糖性脑损伤早期病情判断具有一定价值。     

儿童创伤性骺板及骨骺损伤的多排螺旋CT诊断分析

目的探讨儿童骺板及骨骺损伤的多排螺旋CT影像学表现。方法收集首都医科大学附属北京儿童医院2009年1月至2011年3月收治的X线平片或CT检查可疑骨骺损伤的33例患儿临床资料,年龄1d至14岁,中位年龄7岁,男14例,女19例,病史为1h至3个月。分别行x线平片及CT检查,分析其多排螺旋CT表现。结果33例患儿共29处骺板受累,其中2例为双骨干骺端骨折,6例经多排螺旋CT证实排除骺板骨折,9例X线平片未发现骨骺损伤而多排螺旋CT发现骺板骨折。骨折位于肱骨远端8例,肱骨近端1例,桡骨近端2例,桡骨远端3例,股骨远端2例,胫骨近端1例,胫骨远端8例,腓骨远端1例,指骨2例,趾骨1例。按Salter—Harris分型,Ⅰ型2例（6．9％）,Ⅱ型19例（65．5％）,Ⅲ型4例（13．8％）,Ⅳ型4例（13．8％）。结论多排螺旋CT可以了解骨骺受累情况,直观观察骨骺移位以及关节内情况,及时提示临床进行相应处理,对病变的预后起着重要的作用。     

Giant lobar emphysema — Neonatal diagnosis

Giant emphysema of a lung lobe has distinctive features. Classically there is overdistension of the affected lung lobe, with one lobe only being involved, and, 50% of cases occur in the newborn infant [1, 4, 8].The authors describe a particularly severe example with marked mediastinal shift and initially the hemithorax on the side of the lesion was opaque. Angiography was carried out and followed by resection when the infant was 4 months old.