郎格罕细胞组织细胞增生症肺部病变临床特点分析

为探讨郎格罕细胞组织细胞增生症(LCH)患儿肺部病变临床特点，对20例郎格罕细胞组织细胞增生症患儿进行分析，其中男12例，女8例；就诊年龄4个月～6岁6个月。结果显示20例患儿均具有间质性肺损害，但出现呼吸道症状者仅占57％。有阵发性咳嗽ll例，气促、呼吸衰竭1例，反复呼吸道感染1例，无咯血、气胸、紫绀者；肺部体征有呼吸音粗糙6例，肺部罗音1例；活动期患儿可有血气和肺功能异常；X线异常多出现在呼吸道症状之前，其中双肺纹理粗多ll例，肺门影增粗模糊2例，网点状阴影10例，片状影10例，肺囊泡5例，毛玻璃样1例，肺气肿1例，胸膜增厚1例，胸腺肿大2例。提示胸部高分辨率CT扫描有利于早期发现LCH的肺部改变；早期、全身、强化疗有利于肺部病变的恢复。     

新生儿朗格罕斯细胞组织细胞增生症1例报告

目的了解新生儿朗格罕斯细胞组织细胞增生症(LCH)的临床、实验室特点及诊断要点。方法回顾性分析1例新生儿LCH的临床资料。结果女性患儿,生后1 d因少吃、少哭,皮疹入院,头部、四肢、躯干、脚趾、口腔可见多发皮疹,突出皮面,大小不等,部分破溃结痂。皮肤活检提示病变组织表达S-100(+),CD1α(+),符合LCH诊断。结论新生儿生后有特殊皮疹,需警惕LCH,应进行皮肤活检,以免误诊。     

儿童肺隔离症伴肺结核一例

患儿,男,14岁,因"反复咳嗽2个月,加重20d"入院,患儿2个月前因受凉后出现咳嗽,初为单声咳嗽,以后转为阵发性咳嗽,夜间明显,同时患儿低热,体温波动在37.8℃～38.2℃,当地医院胸片提示左下肺片状及云絮状影,诊断为肺炎.经治疗12 d后无明显好转入住我院呼吸科,纤支镜检查为慢性气管支气管内膜炎,经呼吸科头孢西丁抗感染治疗后咳嗽、发热好转出院.出院后1个多月来门诊复查,胸部CT平扫示左侧胸腔见弧形液体密度影,左肺下叶见均匀分布的细颗粒影,并可见大片状团絮状影,提示左肺下叶病变伴胸腔积液.因CT示胸部病变遂再次入住呼吸科,经我科会诊建议行胸部增强CT及心血管三维重建检查发现胸10椎体水平降主动脉向左发出—支血管伸入左下肺病灶供血(图1),诊断肺隔离症伴炎症后转入我胸心外科.     

儿童肺受累的朗格罕细胞组织细胞增生症病例分析北大核心CSCD

目的 了解肺受累的朗格罕细胞组织细胞增生症（Langerhans cell histiocytosis,LCH）儿童的临床表现及影像学特点.方法 回顾分析首都医科大学附属北京儿童医院呼吸二科近4年来因肺部症状或肺部影像学异常而就诊,并通过活检确诊为肺受累的LCH的14例患儿临床表现和影像学特征.结果 14例中男10例,女4例,男女比2.5∶1;中位发病年龄为1.3岁.所有均伴有多系统受累,最常见的是伴皮肤受累（10例,71％）,其次为肝受累（8例,57％）及骨受累（7例,50％）.最常见的表现为咳嗽伴发热（7例,50％）,呼吸道表现无特异性,3例患儿无呼吸道症状,但肺CT提示广泛肺间质改变.最常见的肺CT表现为结节合并囊泡（6例,43％）,其他表现为仅囊泡无结节（5例,36％）、仅结节无囊泡（1例）、既无结节也无囊泡（2例,14％）.结节可多发可散发,多为小结节,可大小不等,可含有囊腔.囊泡大小不等,形态不均,壁厚不均,可融合,可多发也可散发,可呈蜂窝样.7％患儿合并气胸.结论 肺受累的LCH常伴有皮肤、肝脏等多系统受累表现,易被误诊,仔细查找皮疹对于LCH的诊断有重要作用.高分辨率肺CT的特征性表现[肺间质改变、结节和（或）合并囊泡]有助于诊断.     

郎格罕细胞组织细胞增生症评估与治疗指南介绍

2009年4月国际组织细胞协会发布了《郎格罕细胞组织细胞增生症评估与治疗指南》(以下简称"指南")[1],对于郎格罕细胞组织细胞增生症(LCH)的诊断、评估和治疗规范提出了新的建议,介绍如下.一、LCH病理诊断标准1987年国际组织细胞协会的《郎格罕细胞组织细胞增生症病理诊断标准》中,确诊指标中的金标准有"电镜检查发现病变细胞内含Birbeck颗粒",由于近年来发现郎格素(langerin,CD207)表达阳性可以代表Birbeck颗粒,所以郎格素具有与Birbeck颗粒相同的确诊意义.因此,在新版"指南"中规定,上述两者具备其中1项即可确诊."指南"认为,只有在颈椎的扁平椎或齿状突( odontoid peg)孤立性受累、无软组织受累的LCH患者,由于活检风险大于组织诊断的需要,可以将Birbeck颗粒作为必需项目.     

儿童Erdheim-Chester病2例报告并文献复习

目的探讨儿童Erdheim-Chester病(ECD)的临床特点。方法回顾分析2例ECD患儿的临床表现、诊断及α干扰素治疗效果,并结合文献进行总结。结果 2例男性患儿,分别为4、5岁,均表现为多饮、多尿、骨痛、肝脏增大等,与郎格罕斯细胞组织细胞增生症相似,但病理表现为黄色类脂质肉芽肿,免疫组化中CD1a(-)、S-100(-)。与成人ECD相比,儿童骨骼系统同样最易受累,神经系统比成人更易受累,心脏、皮肤等受累少于成人。2例患儿α干扰素治疗效果尚可。结论 ECD是一种临床上罕见的非郎格罕斯细胞性组织细胞增生症,早期识别、早期诊断可改善预后。     

原发于鼻咽部的郎格罕细胞组织细胞增生症(LCH)1例

患儿 ,女 ,6岁 ,因“鼻塞 3个月 ,间断鼻衄 2个月”入院。当地医院依“鼻炎”治疗无效 ,3月来又先后于多家医院就诊 ,头颅CT、MRI示“右侧鼻咽部、颞下窝、蝶窝占位病变 ,左侧上颌窦、筛窦、乳突异常信号” ,诊为“鼻咽癌” ,行右侧鼻腔、鼻嗯、翼腭窝肿物切除术送病理可见郎格罕细胞 ,免疫组化S 1 0 0蛋白、CD1a阳性 ,诊断为“郎格罕细胞组织细胞增生症”转入我院。入院后行上颌窦区CT平扫 :双侧副鼻窦炎 ,右侧颅底骨及蝶骨大翼可见大片骨缺损 ,右侧咽壁软组织影增厚 ,右乳突蜂房变密 ,鼓室变小。依据患儿有上颌窦、蝶窦、筛窦、乳突、…     

儿童肺部间质性病变影像学检查

为探讨常用的各种影像学检查在儿科肺部间质性病变诊断中的价值 ,特别是高分辨率CT(HRCT)在间质性病变诊断中的意义。将45例(间质性肺炎21例 ,郎格罕组织细胞增生症12例 ,致纤维化肺泡炎4例 ,特发性肺含铁血黄素沉着症8例)患儿的普通X线胸片、常规CT片及HRCT片进行回顾性比较、分析 ,并对普通X线胸片和HRCT ,以及常规CT和HRCT的结果进行一致性和差异性检验。结果 :①HRCT对肺内网状阴影、小结节状阴影、肺气肿和细支气管病变的显示率均超过50% ,网状阴影显示率高达86.7% ;常规CT扫描和普通胸片对以上征象的显示率则较低 ,特别是对于磨玻璃样阴影和细支气管病变的显示率更低。②HRCT与普通X线胸片及常规CT比较 ,在显示肺内网状阴影、小结节阴影(直径<1cm)、肺气肿、细支气管病变及磨玻璃样阴影时 ,结果具有显著或非常显著一致性(P<0.05,<0.005);对肺内网状阴影、小结节阴影、磨玻璃样阴影、肺气肿和细支气管病变的阳性显示 ,HRCT与普通X线胸片及常规CT比较 ,差异有非常显著意义(P<0.005)。提示HRCT在显示儿科肺部间质性病变的网状阴影、小结节阴影、磨玻璃样阴影、肺气肿和细支气管病变时优于常规CT和普通X线胸片 ,可在病情判断及疗效观察等方面为临床提供更大的帮助     

29例小儿间质性肺疾病临床、放射及病理分析

目的提高对小儿间质性肺疾病的认识。方法收集2001年3月至2009年12月住院肺活检的弥漫性肺疾病患儿29例,对其临床表现、肺CT或高分辨CT(HRCT)、肺组织病理特点进行回顾性分析。结果临床表现为咳嗽29例,呼吸困难15例,肺部湿啰音7例,杵状指趾9例。胸CT或HRCT显示:19例以磨玻璃影为主,5例为结节影,5例以多发实变影为主。根据肺病理的改变,结合临床和影像学特点,确诊特发性间质性肺炎18例,包括非特异性间质性肺炎(NSIP)8例、急性间质性肺炎(AIP)3例、隐源性机化性肺炎(COP)3例、淋巴细胞性间质性肺炎(LIP)2例、呼吸性细支气管炎伴间质性肺炎(RBILD)1例、未分型特发性间质性肺炎1例;其他间质性肺疾病11例,包括弥漫性泛细支气管炎2例、过敏性肺炎2例、类脂性肺炎2例、肺泡蛋白沉着症2例、肺泡微石症1例、特发性肺含铁血黄素沉着症1例、结节病1例。其中21例患儿应用糖皮质激素治疗,16例有效。结论小儿间质性肺疾病的主要临床表现为咳嗽和呼吸困难,肺CT表现主要为磨玻璃影、结节影,肺组织病理为小儿间质性肺疾病诊断的主要依据。     

儿童EB病毒相关平滑肌肿瘤1例

7岁女性患儿,因双下肢疼痛1年余入院.腰椎MRI提示椎旁团块状软组织肿块;头颅MRI提示双侧海绵窦区结节状肿块;胸部CT示右肺下叶外基底段及左肺上叶前段高密度结节灶.腰椎病变组织病理活检示EB病毒相关平滑肌肿瘤.基因检测示ITK基因存在c.725_730delAGAGTA(p.K242_S243del)新发突变.治疗上...     

Chronic eosinophilic pneumonia in a 13-year-old child

We report a case of a 13-year-old girl with an asymptomatic isoniazid-resistant tuberculosis contact. Six months after the contact had been made, chest radiography showed left upper lobe infiltrates without hilar lymphadenopathy, which led to the start of an antituberculous treatment. Tuberculin skin test remained negative and blood tests showed hypereosinophilia. One month after the onset of the treatment, she presented with asthenia, weight loss, and cough. She was admitted to our unit with a diagnosis of drug-resistant tuberculosis. Blood tests showed the persistence of hypereosinophilia. Chest radiograph and high-resolution lung computed tomography (CT) scan showed alveolar peripheral condensations on both upper lobes without significant hilar lymphadenopathy. Bronchoalveolar lavage (BAL) showed a normal total cell count with 44% of eosinophils. Microbiological analyses were all negative. Chronic eosinophilic pneumonia (CEP) was confirmed after the elimination of other different eosinophilic lung diseases. The patient was highly responsive to high doses of oral corticosteroids. Dyspnoea and cough disappeared within one week and chest CT scan showed regression of the lung infiltrates within one month. No relapse occurred during the following nine months.     

Pulmonary alveolar microlithiasis

Summary An isolated case of pulmonary alveolar microlithiasis in a boy aged 16 years is reported. His main complaint was shortness of breath. Lung function tests showed a restrictive type of insufficiency. X-ray of the chest and lung biopsy confirmed the diagnosis. The literature on the subject is reviewed. From the Government T. B. Hospital, Mangalagiri, Guntur District, Andhra.     

CT scan patterns of pulmonary alveolar proteinosis in children

Background. To study computed tomographic (CT) findings in children with pulmonary alveolar proteinosis (PAP) more extensively. Objective. To describe the CT features at the time of diagnosis and after therapeutic broncho-alveolar lavage (BAL). Materials and methods. We retrospectively reviewed the CT scans of five children (aged 3 months to 4 years) examined because of incidental bronchitis (n = 1), disease in a sibling (n = 1) and relapsing fever, cough and dyspnoea (n = 3). Each patient had an initial CT scan. Two asymptomatic cases were not treated but were followed up by plain chest films. The other three had BAL and follow-up CT. Results. Initial CT in all cases showed a diffuse reticulomicronodular pattern associated in three cases with posterior bilateral alveolar infiltrates. CT in the two asymptomatic patients remained unchanged or slightly improved without BAL. After BAL, a variable decrease of lung infiltrates was observed. Conclusions. Correlation between the extent of alveolar consolidation and severity of disease was found. Anatomical and pathological considerations allow us to consider that the classical reticulomicronodular pattern is not due to an interstitial infiltration but to alveoli filled with the abnormal material characteristic of PAP. Received: 11 February 1998 Accepted: 26 June 1998     

儿童肺放线菌病一例并文献复习

目的总结儿童肺放线菌病的临床特征、影像学表现、诊治方法及预后。方法回顾性分析浙江大学医学院附属儿童医院呼吸科2019年12月收治的1例肺放线菌病患儿的临床资料。以“儿童”或“小儿”和“肺放线菌病”为关键词在万方数据库、中国期刊全文数据库、中国维普数据库,以“pulmonary”或“thoracic”和“actinomycosis”和“pediatric”或“children”或“child”为关键词在PubMed数据库检索1975年1月至2020年1月相关文献进行文献复习。结果患儿男,12岁6月龄,因“咳嗽伴胸痛20 d余”收入院,病初3 d有发热,外院胸部CT示“右肺中叶炎性病变,不除外空洞形成”,2次纤维支气管镜检查示“右中叶支气管黏膜充血水肿”,且1次灌洗液示结核杆菌DNA阳性,但结核菌素试验及结核感染T细胞检测均阴性。入院后患儿有咳嗽伴胸痛,体温正常,再次行纤维支气管镜检查,灌洗液送检病原微生物宏基因测序,检测出放线菌属(检出序列数222),格雷文尼放线菌(检出序列数185)。入院后予头孢哌酮舒巴坦针静脉滴注抗感染2周,出院后口服阿莫西林克拉维酸钾片6周,随访至2020年4月患儿无临床症状,且胸部影像学提示病变已明显吸收好转。共检索到符合条件的外文文献8篇,未检索到中文文献,共报道62例肺放线菌病患儿,文献报道最小年龄为27月龄。该病临床特征无特异性,主要表现为胸壁肿物(8例)、咳嗽(23例)、疼痛(胸部、背部、肩部、腋窝)(24例)、发热(25例)、体重下降(26例)等。结论儿童肺放线菌病的临床特征及影像学表现均不典型,临床上极易误诊,支气管肺泡灌洗液病原微生物宏基因测序有助于确诊,经抗菌药物治疗预后良好。     

Pediatric interstitial lung disease

This study was done to ascertain the symptomatology, clinical features and investigations pertaining to interstitial lung diseases (ILD) in children. The medical records of 16 children admitted over a 4-year period from June 2000 to May 2004 with progressive cough, dyspnea, and chest X-ray/High Resolution Computerized Tomography (HRCT) abnormalities suggesting ILD were retrospectively evaluated. Clinical findings, investigations, chest skiagrams, HRCT, bronchoalveolar lavage (BAL) and lung biopsy reports were analyzed. An acute presentation of symptoms was seen in 4 cases (25%). Velcro crackles were the commonest clinical finding, present in 15 cases (93.8%). Serial X-rays revealed findings suggestive of ILD in 12 cases (75%) and HRCT was diagnostic in 13 cases (86.6%). Spirometry done in 5 cases showed a restrictive ventilatory defect, BAL analysis done in 8 cases demonstrated increased neutrophils and lung biopsy done in 5 cases was consistent with idiopathic pulmonary fibrosis. Mean survival duration of 2 years and 7 months after initial diagnosis was observed.     

Pulmonary langerhans cell histiocytosis masquerading as tuberculosis in an infant

A 4-month-old infant presented with continued fever, unresolving bronchopneumonia and household contact with sputum-smear-positive tuberculosis (TB) and showed marginal improvement on anti-TB chemotherapy. Recurrent pneumothorax prompted the clinical diagnosis of TB to be revised. High-resolution CT scan of the chest and open lung biopsy confirmed the diagnosis of pulmonary Langerhans cell histiocytosis. Treatment with prednisolone and vinblastin resulted in settling of fever and resolution of respiratory symptoms and signs. In communities where the prevalence of TB is high, unusual presentations should prompt consideration of alternative diagnoses.     

全肺灌洗治疗婴儿肺泡蛋白沉积症一例并文献复习

目的探讨婴儿全肺灌洗治疗肺泡蛋白沉积症的安全性、可行性及操作要点。方法报道2018年11月上海交通大学医学院附属上海儿童医学中心重症医学科收治的1例肺泡蛋白沉积导致呼吸衰竭的患儿的临床表现、基因检查及全肺灌洗治疗方法;并以"whole lung lavage""pediatric""pulmonary alveolar proteinosis""全肺灌洗""儿童""肺泡蛋白沉积"为检索词,检索PubMed、Springer Link、中国知网、维普、万方数据库建库至2019年4月相关文献并进行总结。结果患儿男,3月龄,因"咳嗽、气促伴口唇青紫1周"入院。查体示肝脾肿大。血常规示轻度贫血(血红蛋白96 g/L),白细胞、血小板及C反应蛋白正常。白蛋白偏低(31 g/L),天冬氨酸转氨酶轻度升高(115 U/L);乳酸脱氢酶显著升高(>6600 U/L),铁蛋白显著升高(>4500μg/L),血氨轻度升高(165μmol/L)。胸部CT显示两肺透光度减低,斑片状高密度影及磨玻璃样影。基因检查显示SLC7A7基因存在突变(c.625+1G>A)。支气管肺泡灌洗液过碘酸希夫反应(PAS染色)阳性。患儿诊断为重症肺炎、呼吸衰竭、赖氨酸尿性蛋白耐受不良、肺泡蛋白沉积症。行左右肺全肺灌洗治疗后患儿顺利撤机,脱离吸氧并出院。文献检索共11篇,中文0篇、英文11篇,纳入21例患者,进行全肺灌洗后呼吸功能好转者占76%(16/21)。结论全肺灌洗可有效改善婴儿肺泡蛋白沉积导致的呼吸衰竭,改善患儿呼吸功能;且操作具有可行性及安全性。     

血栓性肺栓塞八例临床特征分析

目的分析儿童血栓性肺栓塞(PTE)临床特征以提高对该病的认识和诊治能力。方法回顾性分析浙江大学医学院附属儿童医院2014年3月至2019年3月收治的8例PTE患儿(男4例、女4例)的临床表现、实验室检查、影像学特点和诊治情况。结果8例PTE患儿年龄9小时龄~14岁10月龄。发热4例、咳嗽加剧4例、气促3例、胸痛2例、腹痛和背痛1例、咯血2例、发绀1例、下肢水肿2例。肺部局部呼吸音降低2例、痰鸣音3例、胸膜摩擦音1例。超声发现胸腔积液5例。6例血浆D-二聚体水平升高(0.66~9.96 mg/L),5例超敏C反应蛋白升高(10.78~78.00 mg/L)。胸部增强CT提示肺动脉或静脉充盈缺损,其中肺动脉栓塞7例、肺静脉栓塞1例。原发病包括肺炎支原体肺炎4例、肾炎2例和先天性心脏病术后2例。7例患儿接受抗凝治疗后痊愈,1例因家长拒绝进一步治疗自动出院。结论对于有肺炎支原体肺炎、免疫紊乱、长期激素治疗、心血管有创操作等高危因素的患儿,出现发热、咳嗽加重、呼吸急促、胸痛和腹背痛伴胸腔积液等,要注意PTE可能,尽早行胸部增强CT检查以确诊。抗凝可以作为儿童PTE首选治疗方法。     

Severe isolated pulmonary Langerhans cell histiocytosis in a 6-year-old girl

Langerhans cell histiocytosis (LCH) usually affects different organs or bones. Isolated pulmonary disease is rare in childhood. We report about a 6-year-old girl with progressive pulmonary insufficiency, onset of clubbing at 4 years of age and honeycombing lung infiltrations on X-ray films. The radiological suspicion of primary pulmonary LCH was confirmed by the presence of CD1a positive cells in the bronchoalveolar lavage fluid. Other organs were not involved. The girl was treated according to the LCH-III International Study Protocol with a good response. Follow-up showed no reactivation of LCH but a reduced vital capacity and signs of interstitial pulmonary involvement on a CT scan. CONCLUSION: Langerhans cell histiocytosis should be considered in the aetiology of cystic lung diseases. Early responders to treatment have a high likelihood of becoming free of disease. However, pulmonary fibrosis is an important mechanism of lung remodelling in pulmonary Langerhans cell histiocytosis and the long-term prognosis is unclear.