The medical field in China has witnessed encouraging progress in specialized theoretical research and clinical practice concerning childhood diffuse parenchymal lung diseases/childhood interstitial lung diseases (chDPLD/chILD) after many years of hard work. However, we have also encountered many tasks and challenges. We must approach the problem with a holistic perspective, and collect, accumulate and analyze, in a uniform way, the data from all over the country. We should try our best to obtain more pathological materials for further analysis of the diagnosis and treatment as well as clinical research. The diagnosis protocol and treatment recommendations should be revised regularly. Moreover, we emphasize the adoption of the clinico-radio-genetic-pathological (C-R-G-P) management model and the multi-disciplinary team (MDT) approach to the diagnosis and treatment of chDPLD/chILD. In this way, we will be able to improve our cognitive understanding and enrich our experience in the prevention and management of chDPLD/chILD further more.

目的 探讨儿童肝肺综合征( HPS)的临床特点及诊治措施.方法 分析1例仅有呼吸系统表现、隐源性肝硬化相关的HPS患儿临床资料,并复习相关文献.结果 患儿,男,10岁10个月,呼吸困难伴发绀0. 5年,肝硬化及门脉高压,肺内血管扩张.诊断:HPS(极重度),隐源性肝硬化、门脉高压、胃底食管静脉曲张.文献复习:儿童HPS发病率低,多发生于肝脏功能严重障碍的肝硬化和门脉高压患儿,仅表现为呼吸系统症状时易误诊,对比增强超声心动图、99m锝标记清蛋白核素等多种方法联合检测肺内血管扩张、分流可提高诊断率.HPS预后差,原位肝移植是唯一根治方法.结论 难治性低氧血症及有严重肝脏疾病的患儿要考虑并发HPS的可能.

"儿童弥漫性肺实质疾病/肺间质疾病治疗建议"的制定，是一项既困难又颇具挑战性的工作，但治疗的规范化又是临床医生迫切需要的，尽管本次只是提供了专家观点相对一致的治疗原则，我们坚信今后一定能逐渐完善，再上一个台阶。.

Importance:A cluster of influenza-associated deaths occurred among children during pandemic 2009 influenza A (H1N1) in China, but the risk factors and causes for death have not been clarified. Objective:We describe the clinical findings regarding 2009 influenza A (H1N1)-associated pediatric deaths in China, including the risk factors for death. Methods:The definition of 2009 influenza A (H1N1)-associated pediatric death is death in a child who is younger than 14 years and has laboratory-confirmed influenza. We collected data of total 810 hospitalized patients with 2009 influenza A (H1N1) infection from September 2009 to February 2010 in 17 hospitals across China. The clinical characteristics, laboratory abnormalities, and treatment course were retrospectively studied. Results:Of the 810 patients hospitalized with 2009 influenza A (H1N1) infection, 19 (2.3%) died. Ten patients died from severe pneumonia and acute respiratory distress syndrome; eight died from encephalopathy/encephalitis; one died from secondary fungal meningitis. Patients who died were more likely than patients who survived to have neutrophilia, lymphopenia, elevated C-reactive protein, and elevations of lactate dehydrogenase, creatine kinase, creatine kinase-MB, aspartate aminotransferase and alanine aminotransferase. There were no significant differences in the median age, median time from onset of illness to admission, underlying chronic disease, and initiation of antiviral therapy within 48 hours of illness onset, between patients who died and those who survived. Interpretation:The risk factors for pediatric death associated with 2009 influenza A (H1N1) infection are different from those of seasonal influenza. The most common causes of death are viral pneumonia, acute respiratory distress syndrome, and encephalopathy/encephalitis.

OBJECTIVE:To evaluate the value of clinical signs in the identification of Mycoplasma pneumonia in children's community acquired pneumonia.METHOD:We searched the Cochrane library, PubMed, CNKI, Wan Fang and VIP databases. According to the inclusion and exclusion criterias, we selected and extracted the related information in the literature. According to the QUADAS evaluation system, we established the quality evaluation standard to evaluate the quality of the included studies and analyzed the difference of the clinical manifestations between Mycoplasmae pneumoniae and non-Mycoplasma pneumoniae in children's community acquired pneumonia. We used the RevMan 5.3 software to do the meta-analysis and collected the data according to the requirements. We calculated the pooled sensitivities, specificities and 95%CIs. Then we calculated the negative and positive likelihood ratio, the ratio of the diagnosis and the pre-/post-test probabilities with 95% CIs.RESULT:A total of 11 articles were included in the literature. In summary, the cases of the clinical signs of true positive (TP) and false positive (FP) were as follows : chest pain: TP: 287, FP: 1090; rales: TP: 1906, FP: 6886; headache: TP: 590, FP: 2051; pleural effusion: TP: 10, FP: 16; consolidation: TP: 75, FP: 83; emphysema: TP: 443, FP: 116. The pooled sensitivity, the pooled specificity, the diagnostic ratio (DOR) and 95% CI were: chest pain: pooled sensitivity: 0.12, 95% CI: 0.10-0.13, pooled specificity: 0.89, 95% CI: 0.88-0.90, DOR: 1.05, 95% CI: 0.92-1.21; rales: pooled sensitivity: 0.66, 95% CI: 0.64, 0.67, pooled specificity: 0.36, 95% CI: 0.35, 0.37, DOR: 1.12, 95% CI: 1.02, 1.22; headache: pooled sensitivity: 0.23, 95% CI: 0.21-0.25, pooled specificity: 0.80, 95%CI: 0.79-0.80, DOR: 1.16, 95%CI: 1.05-1.29; pleural effusion: pooled sensitivity: 0.04, 95% CI: 0.02, 0.08, pooled specificity: 0.98, 95% CI: 0.96, 0.99, DOR: 1.28, 95% CI: 0.56, 2.89; consolidation: pooled sensitivity: 0.32, 95% CI: 0.26, 0.39, pooled specificity: 0.87, 95% CI: 0.84, 0.90, DOR: 1.88, 95% CI: 1.23, 2.90; emphysema: pooled sensitivity: 0.22, 95% CI: 0.17, 0.29, pooled specificity: 0.73, 95% CI: 0.69, 0.77, DOR: 1.05, 95% CI: 0.68, 1.61.CONCLUSION:The value of clinical symptoms and signs in the identification of mycoplasma pneumonia in children's community acquired pneumonia was not significant. Although the clinical symptoms/signs of chest pain, headache, rales and chest X-ray manifestations of pleural effusion, consolidation, emphysema could suggest Mycoplasma pneumoniae infection, the presence or absence of any clinical signs were not positive or negative indicators for the identification of Mycoplasma pneumoniae infections.

Objective To understand the causes and the ratio of cause constituents in children with chronic cough in Beijing. Methods Patients with chronic cough treated at respiratory clinic of the Children' s Hospital Affiliated to Capital Institute of Pediatrics from May 2009 to April 2011 were included in this study. Etiologic diagnostic procedure suggested for children by the Subspecialty Group of Respiratory Diseases,the Society of Pediatrics,Chinese Medical Association in 2008 was implemented to manage the patients. After three - month follow - up,the etiological data was analyzed. Results Totally 213 patients with chronic cough aged 1. 1 to 14. 0 years old were enrolled,inclu-ding 40 cases(18. 8% )aged≤3 years old,134 cases(62. 9% )aged ﹥ 3 to 6 years old,and 39 cases(18. 3% ) aged ﹥ 6 years old. The majority of patients with positive allergen tests were sensitized to inhaled allergens. One child had positive result in 24 - hour esophageal pH monitoring,but his cough didn't respond well to the specific treatment for gastroesophageal reflux,so he wasn't diagnosed as gastroesophageal reflux cough. The 4 leading causes of the 213 pa-tients with chronic cough were cough variant asthma(CVA)in 92 cases(43. 2% ),CVA associated with upper airway cough syndrome(UACS)in 57 cases(26. 8% ),UACS in 28 cases(13. 2% ),respiratory infection and post - infec-tion cough(PIC)in 26 cases(12. 2% ),while other causes were found in 8 cases(3. 7% ),and unknown etiology in 2 cases(0. 9% ). The incidence of CVA ranked top 1 in all the 3 age groups,followed by PIC in ≤3 years old group, while CVA associated with UACS in the other 2 age groups. Conclusions CVA,CVA associated with UACS,UACS and PIC were the 4 leading causes for children with chronic cough in Beijing. Children in different age groups had dif-ferent constituents ratio of causes of chronic cough.

婴幼儿时期是一个特殊的生长发育阶段.近年来的研究表明,有些特殊类型的弥漫性肺实质疾病/肺间质疾病(diffuse parenchymal lung disease/interstitial lung disease,DPLD/ILD)仅存在于婴幼儿时期,而且其临床、影像、病理具有一定特征[1-6],需要早期识别、早期诊断和及时治疗,现就婴幼儿时期特有的ILD叙述如下,旨在提高我国儿科医师对这些疾病的认识.

近年来国际儿科界对儿童弥漫性肺实质疾病/肺间质疾病(children's diffuse parenchymal lung disease/children's interstitial lung disease,chDPLD/chILD 或pediatric diffuse parenchymal lung disease/pediatric interstitial lung disease,pDPLD/pILD,也称儿童弥漫性肺疾病,DLD)的研究进展很快,美、英、法等国都有了chDPLD/chILD研究协作组或攻关小组,各国研究团队展开了网络登记、病因分析、诊治策略、遗传因素探讨等多方位研究.中华医学会儿科学分会呼吸学组儿童弥漫性肺实质疾病/肺间质疾病协作组(以下简称协作组)自2009年成立以来以国际化诊断模式(临床-影像-病理诊断,clinical-radiologic-pathologic diagnosis,C-R-P diagnosis)为样板,在提高协作组的chDPLD/chILD诊断水平方面取得了阶段性的进展,已步入国内chDPLD/chlLD领域的先进行列[1-3].

弥漫性肺实质疾病/肺间质疾病(DPLD/ILD)是一组病因不同,但临床、影像学和组织病理学类似的异质性疾病,目前认为包括200余种疾病.儿童,特别是婴幼儿DPLD/ILD(chDPLD/chILD)与成年人既有相似性,又有特殊性,例如chILD的病因及预后与成年人存在明显的差别,通常成年人普遍诊断的原发性肺间质纤维化(IPF)在儿童几乎不存在,并且chILD的预后通常较成年人好.

OBJECTIVETo investigate the high-resolution computed tomographic (HRCT) features of infants with diffuse lung disease (DLD) for improving the diagnostic accuracy clinically.METHODTotally 75 infants under 2 years of age with DLD (2010-2013) were involved in this study. Among them, 56 were males and 19 females, aged from 2 days to 24 months (mean age was 10.9 months). According to the clinical or pathological data, the cases were enrolled into three groups, including systemic diseases-associated infantile DLD (30 cases), alveolar structure disorders-associated infantile DLD (23 cases), and infantile DLD specific to infancy (22 cases). Retrospectively, HRCT images, from the three groups respectively, were analyzed and compared. HRCT presentations including airway disorders, interstitial disorders and air space disorders were reviewed. Inter-reviewers consistency check was performed, the consistency between reviewers was good (K = 0.64;P = 0.03, < 0.05), as well as χ(2) test.RESULTAmong the three groups, some of the HRCT sings (bronchiectasis, thickened bronchiolar wall, mosaic sign, reticular, intralobular nodules and consolidations) had significant differences (χ(2) = 24.52, 6.08, 18.00, 12.56, 9.11 and 11.50, P < 0.05) .CONCLUSIONThe HRCT features of infantile pulmonary DLD/interstitial LD with different causes were as follows, compared to the other two groups, intralobular nodules was the main feature of the systemic diseases-associated infantile DLD, thickened bronchiolar wall, mosaic sign and consolidations were rare as well. Meanwhile, bronchiectasis was more common in alveolar structural disorders-associated infantile DLD, and reticular opacity was rarely seen. Associated clinical data, the HRCT presentations would help clinicians to make accurate diagnosis.

OBJECTIVE:To report a case of I73T mutation in the pulmonary surfactant protein (SP)-C gene associated with pediatric interstitial lung disease, and study the clinical diagnosis and review related literature, to investigate the role of gene detection in the diagnosis of interstitial lung disease in infants and children.METHOD:The clinical, radiological, and genetic testing information of the case was analyzed and related literature was reviewed.RESULT:(1) An 8-month-old girl was hospitalized because of cough, tachypnea, continuous oxygen therapy and failure to thrive. Physical examination on admission revealed tachypnea, slight cyanosis and the three concave sign was positive, respiratory rate of 50 times/minute, scattered fine crackles could be heard over both lungs, clubbing fingers were found. No other abnormalities were noted. Laboratory test results: pathogenic examination was negative, multiple blood gas analysis suggested hypoxemia. Chest CT showed ground-glass like opacity, diffused tubercle infiltration. The I73T mutation in SP-C gene was identified by SP-related gene sequencing. (2) The review of related literature: Data of 3 infants with I73T mutation in SP-C gene showed that all the 3 cases had tachypnea and dyspnea, chest CT revealed diffuse infiltration or diffuse ground glass pattern in lungs, the major pathology of lungs was nonspecific interstitial pneumonia (NSIP).CONCLUSION:A case of interstitial lung disease with I73T mutation in SP-C gene was preliminarily diagnosed in an infant. Gene test provides an important tool in the diagnosis of such pediatric interstitial lung disease.

OBJECTIVE To report a case of pulmonary surfactant protein (SP) gene mutation associated with pediatric interstitial lung disease, and study the clinical diagnosis process and review of related literature, to understand the relationship between interstitial lung disease and SP gene mutation in infants and children. METHOD The clinical, radiological, histological, and genetic testing information of a case of SP gene mutation related pediatric interstitial lung disease were analyzed and related literature was reviewed. RESULT A 2-year-old girl without a history of serious illness was hospitalized because of the shortness of breath, cough, excessive sputum, and the progressive dyspnea. Physical examination on admission revealed tachypnea, slight cyanosis, and the retraction signs were positive, respiratory rate of 60 times/minute, fine crackles could be heard through the lower lobe of both lungs; heart rate was 132 beats/minute. No other abnormalities were noted, no clubbing was found. Laboratory test results: pathologic examination was negative, multiple blood gas analysis suggested hypoxemia. Chest CT showed ground-glass like opacity, diffused patchy infiltration. Bronchoalveolar lavage fluid had a large number of neutrophils, and a few tissue cells. Eosinophil staining: negative. Fluconazole and methylprednisolone were given after admission, pulmonary symptoms and signs did not improve, reexamination showed no change in chest CT. Then lung biopsy was carried out through thoracoscopy. Histopathology suggested chronic interstitial pneumonia with fibrosis. The heterozygous mutation of R219W in the SFPTA1 and the S186N in SFTPC were identified by SP-related gene sequencing. The review of related literature showed that polymorphisms at the 219th amino acid in SP-A1 allele were found in adults with idiopathic pulmonary fibrosis (IPF), but there is no related literature in pediatric cases. The patient in this report had a mutation at the SP-A1 allele consistent with related literature. Data of 17 young children with mutation in SP-C gene showed that all the 17 cases had dyspnea and tachypnea, chest CT revealed diffuse opacities in lungs, the pathology of lungs was NSIP and CPI. There were 17 kinds of mutation and the common mutation was I73T. The mutation of S186N in SFTPC in our case has never been shown in previously published literature. CONCLUSION A case of interstitial lung disease with S186N gene mutation in SFTPC was preliminarily diagnosed in an infant. The SP-C gene mutations and polymorphisms are associated with pediatric interstitial lung disease.

<正>将反复呼吸道感染作为儿科的一个独立疾病来认识已有几十年的历史。1987年第一届全国小儿呼吸道疾病学术会议经讨论制定了"反复呼吸道感染的诊断参考标准"(以下简称"参考标准")。"参考标准"规定0～2岁、>2～5岁及>5～14岁小儿1年内患上呼吸道感染依次在7次、6次和5次以上;患下呼吸道感染依次在3次、2次和2次以上(若上呼吸道感染次数不够,可累加下呼吸道感染次数,反之则不能)即可诊断为反复呼吸道感染。

OBJECTIVE:To summarize the chest CT features and outcome of necrotizing pneumonia (NP) caused by Mycoplasma pneumoniae in children and to review the changes of common inflammatory parameters in NP patients to help clinicians understand the proper timing of CT scan.METHOD:The imaging data from 30 cases of Mycoplasma pneumoniae pneumonia in NP group and 24 cases with non-necrotizing Mycoplasma penumoniae pneumonia (control group) were analyzed retrospectively. The changes of common inflammatory parameters in NP group and control group were compared.RESULT:(1) The chest CT findings of NP (30 cases): 28 cases showed unilateral pneumonia, and 20 cases showed single lobar consolidation, 10 cases had multiple lobes involvement; pulmonary cavities were seen in 27 cases. There were decreased enhancement areas in the consolidation (22 cases). (2) The dynamic changes of CT signs during follow-up: The CT scan performed during the 1 - 2 months after onset of disease (23 cases) showed that pulmonary consolidation in 2 cases (9%) were absorbed, 18 cases (78%)had cavities in lung, 16 cases (70%) had pleural thickening, 2 cases (9%) atelectasis and 1 case (4%) bronchopleural fistula;the CT scan performed during the 2 - 3 months after onset of disease (11 cases) showed pulmonary consolidation in 7 cases (64%) were absorbed, 10 cases (91%) pleural thickness, 7 cases (64%) with cavities in lung, 5 cases (45%) atelectasis, 2 cases (18%) pulmonary lobe cysts and 1 case bronchopleural fistula. The CT scan performed at 3.5 years of disease course (10 cases) showed that there were no pulmonary consolidation in any of the cases, 4 cases had atelectasis, 4 cases had pulmonary cysts, and 1 case had band-like scars. (3) There were significant differences between NP group and control group in the maximum peripheral blood WBC, proportion of neutrophil and C-reactive protein(CRP, mg/L) (P < 0.01, 0.01, 0.001, respectively), and there was significant difference between the 2 groups in the duration of fever, abnormal WBC(d) and CRP(d) (P < 0.001).CONCLUSION:The chest CT features of NP caused by Mycoplasma pneumoniae in children were single lobular consolidation in most cases, NP had decreased parenchymal enhancement and cavity in the consolidation, and recovery was slow, the outcome included recovery, atelectasis or lobar cystic degeneration. The clinicians should pay more attention to the common inflammatory parameters when they suspect the Mycoplasma pneumoniae pneumonia is progressing into necrosis and make correct decision for chest CT examination.

OBJECTIVE:Interstitial lung disease in children represents a heterogeneous group of disorders of both known and unknown causes. This study aimed to understand better the causes of the disease in children and to provide information on the current approach to diagnosis and management of the disease.METHOD:Through the Pediatric Diffuse Parenchymal Lung Disease/Pediatric Interstitial Lung Disease Cooperative Group of China, data of 93 cases of interstitial lung disease of children from 11 hospitals were collected with the same questionnaire in 2009. Respiratory tract secretions were obtained for bacterial culture. Respiratory virus antigen examination, mycoplasma antibody, EB virus, cytomegalovirus, and herpes simplex viruses antibody detection were performed. Cells in the sputum, gastric juice and bronchoalveolar lavage fluid (BALF) were tested for hemosiderin. The CT or high resolution CT (HRCT) of the lung and blood-gas analysis were also performed. Fourteen cases underwent lung biopsy and 25 cases underwent bronchomicrocopy. Data were then pooled and discussed through a series of meetings.RESULT:Fifty-three cases were male, 40 were female and their age ranged from 8 months to 14 years. Thirty-nine cases were diagnosed as bronchiolitis obliterans (BO); 39 as idiopathic pulmonary hemosiderosis (IPH); 7 as idiopathic interstitial pneumonia (IIP) of unknown causes, of whom 4 cases had non specific interstitial pneumonia, 1 case as acute interstitial pneumonia and 1 case as lymphocytic interstitial pneumonia, 1 case as idiopathic pulmonary fibrosis; 2 cases as secondary interstitial lung disease, one was secondary to SLE, one to human immunodeficiency virus (HIV) infection; 2 cases had hypersensitive pneumonitis; 2 cases had pulmonary alveolar proteinosis; 1 case had bronchiolitis obliterans organizing pneumonia; 1 case had lipoid pneumonia;1 case of diffuse panbronchiolitis; 1 case of microlithiasis alveolaris pulmonum. Forty two cases had cough, 24 of them also had tachypnea, 8 cases had clubbing. HRCT showed that 56 cases had groundglass-like opacification; 30 cases had mosaic appearance; 1 case had diffuse micronodular opacities, 1 case had diffuse reticulonodular opacities and cysts.CONCLUSION:Interstitial lung disease in children is a heterogeneous group of disorders. In this study diagnoses of 11 diseases were made, the top third diagnoses were BO, IPH and IIP, respectively.