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06 December 2014, Volume 29 Issue 12 Previous Issue    Next Issue

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Diffuse parenchymal lung disease/interstitial lung disease in infancy. NONG Guang-min. 2014, 29(12): 881-883.  DOI: 10.7504/ek2014120601 Abstract ( )   PDF (1034KB) ( )   Abstract：The literature is becoming progressively populated by reports of diffuse parenchymal lung disease/interstitial lung disease（DPLD/ ILD） that appear distinct to infants and young children. The classification， clinical features， management and outcome for these disorders are briefly mentioned.

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Congenital surfactant dysfunction disorders. ZHENG Yue-jie. 2014, 29(12): 883-885.  DOI: 10.7504/ek2014120602 Abstract ( )   PDF (1018KB) ( )   Abstract： Congenital surfactant dysfunction disorders（SDD） or inborn errors of surfactant metabolism（IESM） include the mutations in the genes encoding the surfactant proteins B（SFTPB）， surfactant protein C （SFTPC）， the ATP-binding cassette transporter A3 （ABCA3）， the thyroid transcription factor 1（NKX2.1）， and the receptor for granulocyte-macrophage colony stimulating factor （GMCSFR）. These disorders have now been recognized as the important etiology for diffuse interstitial lung disease （ILD） in children. The age of onset and severity of these disorders are highly varied. The presentation ranges from fatal RDS in full-term infants to ILD in older infants， children， and even adults.

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nfection  and  interstitial  lung  diseases  in  infants ZHAO De-yu. 2014, 29(12): 886-889.  DOI: 10.7504/ek2014120603 Abstract ( )   PDF (1052KB) ( )   Abstract： The interstitial lung disease （ILD） in infants has been better diagnosed with the application of high-resolution CT in recent years. Mutiple factors play important roles in the pathogenesis and development of ILD in infants， especially the infection. Moreover， the infants with ILD are also prone to infection which could further aggravate their ILD. So a correct understanding of the relationship between infection and ILD in infants is significantly important.

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Alveolar growth abnormalities interstitial lung disease ZOU Ying-xue. 2014, 29(12): 889-892.  DOI: 10.7504/ek2014120604 Abstract ( )   PDF (1053KB) ( )   Abstract：Though interstitial lung disease （ILD） can occur at any age in children， disorders more common in infancy and young children have received increased attention as an important group that is disproportionally affected， linked to lung development and lung injury， and represents disorders not seen in adult ILD. The specific disorders causesd by alveolar growth abnormalities（AGA） is the most common in infancy. The presentation， evaluation， treatment， and clinical course in infants are discussed in infants in this paper.

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Interstitial lung disease in infants of undefined etiology SHANG Yun-xiao， FENG Yong. 2014, 29(12): 892-897.  DOI: 10.7504/ek2014120605 Abstract ( )   PDF (1065KB) ( )   Abstract： Children’s interstitial lung disease is a group of relatively rare and complicated pulmonary disorders，which is more prevalent in infancy periods. Early diagnosis and treatment may alter the poor prognosis. In the last ten years， with the application of clinical-radiologic-pathologic diagnosis mode， more and more interstitial lung disease can be diagnosed with a known etiology in infants， but some of them are still lack of a defined etiology. This article describes the interstitial lung disease in infants of undefined etiology，which in turn can lead to better understanding and optimal patient management.

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Connective tissue disease-related interstitial lung disease in infant. CHEN De-hui. 2014, 29(12): 897-901.  DOI: 10.7504/ek2014120620 Abstract ( )   PDF (1055KB) ( )   Abstract：Interstitial lung disease （ILD） is a group of widely heterogeneous etiology disease.  Infant and early childhood special type of ILD belongs to a more complex etiology category.  Connective tissue disease （CTD） is a group of autoimmune disease involves multi-systems and organs in the whole body， lung is the organ most commonly involved.  Connective tissue disease-associated interstitial lung disease （CTD-ILD） and ILD have similar clinical symptoms， with their own characteristics， but different treatment options and prognosis.  Deeply understanding the infant CTD-ILD and ILD， learning their clinical analysis ideas， will become very important in carrying out extensive clinical research for prognosis and therapy in the future.

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Investigation into the incidence and causes of ketoacidosis in children with established T1DM. WEI Li-ya*， LI Pin， LIU Qian-qi， WEI Hai-yan， YANG Yu， CUI Wei， LU Ping， YAO Hui， CHEN Lin-qi， YANG Qiao-zhi， CHEN Rui-min， CHENG Xin-ran， REN Xiao-ya， ZHU Zhi-ying， SHI Xing， CHEN Yong-xing， WU Xian， WANG Ning， DUAN Jing， CHEN Xiao-hong，WANG Feng-yun， CHENG Yu-xian， WEI Yan， YANG Xiao-hong， GONG Chun-xiu. 2014, 29(12): 902-908.  DOI: 10.7504/ek2014120606 Abstract ( )   Abstract： Objective To investigate the occurrance of DKA in established T1DM children. Methods According to the registration system in the following-hospitals（Beijing Children’s Hospital of Capital Medical University， Children’s Hospital of Shanghai， Nanjing Children’s Hospital，Children’s Hospital of Zhengzhou， Children’s Hospital of Jiangxi， the First Affiliated Hospital of Xi’an Jiaotong University，First Affiliated Hospital of Kunming Medical University， Children’s Hospital of Wuhan， SooChow University Affiliated Children’s Hospital， Children’s Hospital of Liaocheng， Children’s Hospital of Fuzhou， Chengdu Women & Children’s Central Hospital）， we investigated the frequency and cause of DKA in children with established T1DM from December 1995 to June 2014. After the diagnosis of T1DM， the first time DKA was for group 1A， the second DKA for group 1B. We conducted a cross-sectional survey of blood glucose control status for patients with T1DM from December 2011 to May 2012 in Beijing Children’s Hospital. Patients who did not have DKA episode in the course of T1DM were selected as control group （group 2）. Results Totally 1676 children were newly diagnosed with T1DM by 12 hospitals， and 89 patients occurred 100 DKA after T1DM diagnosed. The incidence and frequency of DKA was 5.3% （89/1676） and 5.9% （100/1676）. The frequency was different in 12 hospitals， fluctuating between 1.1% and 24.1%. Compared with group 2， group 1A had high level of HbA1c ［（11.31±3.03）% vs. （8.26±1.53）%， P＜0.01］ and insulin dosage ［（0.85±0.42） IU vs. （0.71±0.31） IU， P＜0.01］. There were more patients with insulin bump in group 1A than group2 （25.0% vs. 11.2%， P＝0.01）， and few patients reached the standard of blood glucose monitoring （12.1% vs.40.1%， P＜0.01） and follow-up （21.2% vs. 46.6%， P＜0.01）. The main reasons of DKA in group 1A were infection （33.7%）， interrupting insulin therapy （21.3%） and eating disorder （20.2%）； one patient had DKA after islet stem cell transplantation. Infection was also the major cause of DKA in group 1B （4/10）， and 1 patient had DKA because of insulin bump failure. For DKA which occurred within different course， the distribution of causes was different （P＜0.01）. Within 1 year of T1DM duration， the major reason was interrupting insulin injection （39.3%）. For patients more than 1 year， it only accounted for 13.1%（8/61）； the major causes were infection （22/61） and eating disorder （16/61）. The major cause in mutiple hospitals with high DKA frequency was infection （50.0%）， while in other hospitals 28.1% of patients had DKA because of infection （P＜0.01）. Conclusion The frequency of DKA is 5.3%， which is different in 12 hospitals， with the highest up to 24.1%. Patients with DKA have poor glycemic control， and they can not regularly monitor blood glucose and follow-up. We should emphasize the education of diabetes. Patients with insulin pump and islet stem cell transplantation must also become a new focus of education. Hospitals with high DKA frequency should give patients information how to deal with other diseases.

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Model of multidisciplinary team treatment for intractable epilepsy in children. GU Shuo，WANG Zhi-ping， BAO Nan，YU Hong-zhen， YE Xiao-lai， GU Jie， LI Fei， ZHOU Ying， HONG Li， YIN Min-zhi， SONG Yun-hai， FAN Jia-jun， XU Min. 2014, 29(12): 909-912.  DOI: 10.7504/ek2014120607 Abstract ( )   Abstracts： Objective To study the diagnosis and treatment by the model of multidisciplinary team treatment for intractable epilepsy in children. Methods We retrospectively reviewed the clinical data of 43 pediatric cases of intractable epilepsy in our institution from 2012 to 2014，aged from 8 months to 12 years， with an average age of 5 years. Results Totally 29 cases could be found the related factors of epilepsy in the past history， including prenatal and perinatal factors in 9 cases， 8 cases of traumatic intracranial hemorrhage， 3 cases of spontaneous intracranial hemorrhage， 2 cases of disorders of cortical development， 2 cases of vascular malformation， 2 cases of tuberous sclerosis，1 cases of Sturge-Weber syndrome， 1 cases of infection， and intraventricular tumor after operation in 1 case. Five cases underwent surgery and the follow-up was from 3 to 18 months （mean 10 months）， 4 of 5 （80%） achieved seizure freedom （Engel Ⅰ）， and 1 （20%） demonstrated worthwhile improvement （Engel Ⅲ）. Conclusions Multidisciplinary team treatment is an effective model in diagnosis and treatment of childhood intractable epilepsy．Surgical intervention in children with intractable seizures can yield favorable seizure outcome.

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Epidemiological survey of asthma prevalence in children aged 0 to 14 in Changle rural area in Fujian province. ZHENG Jian-yun， CHENG Chao， QIU Hua-bo，CHEN Hong-chao， WANG Zi-jian， CHEN Yu-feng， LIN Ting，LI Dian， CHEN Dai-run， CHEN Zhen-can， CHEN Bin-le，LI Juan， TANG Su-ping,LIU Yan-lin，HUA Yun-han. 2014, 29(12): 913-917.  DOI: 10.7504/ek2014120608 Abstract ( )   Abstracts： Objective To explore the prevalence and risk factors of asthma in children aged 0～14 years old in Changle rural area， providing data for prevention and treatment of childhood asthma in rural area. Methods Multi-stage， stratified and random cluster sampling was used to recruit children. The same screening questionnaires for the national epidemiological survey of children’s asthma were distributed among parents of children aged 0～14 years old at schools，kindergartens and communities. Asthmatic children were picked among the screening-positive children based on on-the-spot inquiries，physical examinations，medical records and supporting test results．Further survey of asthmatics was carried out to investigate the diagnosis and treatment status of childhood asthma and other associated allergic diseases. Results Among a total of 6000 questionnaires， 5860 were completed with a response rate of 96.67%; and 135 （2.3%）children were recognized as asthma［typical asthma（n=119，2.03%）， cough variant asthma（n=13，0.22%） and suspected asthma（n=3，0.05%）］. The prevalence of asthma was higher in boys（n=81，2.51%） than in girls（n=38， 1.44%） （χ2=8.27，P＜0.05）. Conclusions The prevalence of asthma is 2.03% in children under 14 years old in Changle rural area and it varies in children with different genders and ages．Taking the age of onset into account， the asthma prevalence was lowest in the children aged 0～1 years old［0.37%（2/533）］ and highest［5.32%（20/376）］in those aged 3～4 years old．The main cause of asthma in children is upper respiratory tract infection， and the important risks include individual allergic history， family history and genders.

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Comparative analysis of epidemiological survey of childhood asthma in Xiamen. YANG Yun-gang*， WU Jin-zhun， ZHANG Jian-min， ZHU Bi-zhen， ZHONG Sheng-hui， FANG Mei， SHEN Yu-hong， YANG Yi-min， SHEN Tong， WU Xing-dong， CHEN Qi. 2014, 29(12): 918-921.  DOI: 10.7504/ek2014120609 Abstract ( )   Abstracts： Objective To study the epidemiology of asthmatic children from 0 to 14 years old in the city of Xiamen in a decade， in order to find a scientific basis for future children’s asthma prevention and treatment. Methods Secondary children’s asthma epidemiology study roster， preliminary screening and questionnaires were provided by Centre of Asthma Prevention and Education of Chinese Capital Institute of Pediatrics. All the asthma specialists who participated in this study were trained with the same content; we used cluster sampling methods， and nearly 10，000 children were involved in this secondary investigation; 95% of children in the study roster were involved in our investigations. Quality controls were set during the present investigations. All the data were duplex entered into the software of EPI-Info3.5.1 （America CDC）. Rate， mean and Chi square statistics were used as statistic methods. Results The actual surveys of children included 11845 and 11508 respectively， and the rate of lost of follow-up was 0.53% and 0.9% respectively in the year of 2000 and 2010. Average of the morbidity of asthma was 3.96% and 3.03% in the year of 2000 and 2010 respectively（P＜0.05）. The rate of misdiagnosis was 35.56% and 35.5% in the year of 2000 and 2010 respectively， （P＞0.05）.Two thirds of the children had their first episode within 3 years old. About 75.6% and 71.7% suffered from asthma within one year in the year of 2000 and 2010 respectively. Hospitalization rate of asthma was 45.78% and 34.5% in the year of 2000 and 2010 respectively. About 51.28% and 59.5% children with asthma suffered from allergic rhinitis， and the percentage of using antibiotics was 100% and 83.4% in the year of 2000 and 2010 respectively， （P＜0.01）. Conclusions The morbidity of asthma in children aged 0~14 years old decreased in a decade in Xiamen city， but the misdiagnosis rate is still near one thirds; more than half of the asthmatic children have allergic rhinitis at the same time. The morbidity， hospitalization rate and the rate of using antibiotics have decreased in this decade. We also need to pay more attention to the training of the pediatricians who don’t specialize in asthma in the battle of asthma prevention.

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Balloon dilation combined with cryotherapy in the treatment for subglottic stenosis in infants and young children. ZHANG Lei*， YIN Yong， ZHANG Jing， ZHANG Hao，WANG Wei，ZHU Ming. 2014, 29(12): 922-926.  DOI: 10.7504/ek2014120610 Abstract ( )   Abstract： Objective To study balloon dilatation with cryotherapy under bronchoscope for subglottic stenosis in infants and young children，and to explore the efficacy of this new technology，the timing of treatment and safety. Method From July 2009 to May 2014 in 28 SGS cases aged from 1 month to 3 years， bronchoscopic  balloon dilatation  treatment with CO2 cryotherapy was performed， and different  types of SGS treated by minimal invasi were assessed regarding efficacy, complications and outcome. Results    The infant SGS were divided into Ⅰ°~Ⅲ° by severity， and soft membrane SGS and scarring SGS by the causes of stenosis. Ⅰ° SGS included 16 cases, in which 8 cases soft membrane SGS were treated by balloon dilatation effectively， 9 cases after combined treatment; in scarring  SGS balloon dilatation was effective in 1 case， 4 cases after combined therapy; Ⅱ° SGS  included 8 cases， soft membrane SGS were treated effectively in 2 cases， 4 cases after combined treatment; in scarring SGS balloon dilatation was effective in 1 case， 2 cases after combined therapy; Ⅲ °SGS  included 4 cases， 1 case of soft membrane SGS and 1 case of scarring SGS responded well to integrated treatment .Conclusion    MSCT and bronchoscopy assessment is used in grading the extent and causes of stratification in infants with subglottic stenosis， and then we can select the appropriate treatment programs to improve the prognosis of the disease and improve life quality of SGS children.

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Effects of L-carnitine on plasma free carnitine levels and cardiac functions in children with cardiomyopathy TAN Wei-qun，LIU Lin，XU Ming-guo，WEN Peng-qiang，CUI Dong，MENG Xiang-chun，CHEN Shu-li，LI Cheng-rong. 2014, 29(12): 927-931.  DOI: 10.7504/ek2014120611 Abstract ( )   Abstract： Objective To investigate the effect of exogenous L-carnitine on plasma free carnitine levels and cardiac function in children with cardiomyopathy. Methods A total of 88 patients with myocardiopathy were divided into treatment group and control group randomly. The control group received conventional therapy. The treatment group received additional intravenous L-carnitine therapy of 100mg/kg.d for 14 days on the basis of conventional therapy, then changed to oral L-carnitine consistently. The oral dosage was 10ml once a day in patients under 6 years of age，10ml twice a day in patients over 7 years.The plasma free carnitine levels and the improvement of cardiac function including left ventricular ejection fraction（LVEF），cardiac output（CO）， stroke volume （SV）， cardiac index （CI）， EF slope （MVEF） and left ventricular wall motion fast filling phase fraction （LFFPW），combined with electrocardiography， the plasma free carnitine levels， C-reactive protein （CRP）， myocardial enzymes were analyzed before and after therapy. Results Compared with control group， or those before treatment，the LVEF，CO，SV，CIMVEF and LFFPW of the treatment group improved significantly.The plasma free carnitine levels increased markly and had significant difference compared with those before treatment or control group. Conclusion L-carnitine supplement significantly increases the plasma free carnitine levels and improves cardiac function in children with cardiomyopathy.L-carnitine had a good therapeutic effect in children with cardiomyopathy.

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The clinical and pathological characteristics of nonalcoholic steatohepatitis in children. WANG Li-min， ZHANG Hong-fei， DONG Yi， XU Zhi-qiang， CHEN Da-wei， GAN Yu， WANG Fu-chuan， ZHU Shi-shu. 2014, 29(12): 932-935.  DOI: 10.7504/ek2014120612 Abstract ( )   Abstract：Objective To explore relation skip between clinical and liver pathological characteristics of nonalcoholic steatohepatitis in children. Methods The data of 105 cases of nonalcoholic steatohepatitis children，who had been hospitalized from January 2008 to December 2012， were summarized and analyzed. Results （1）About 92.38% of all the group were male，11～13 years old were the most，46.67% were adiposity，0.95% had hypertension，and 33.33% had family history. （2）The patients ALT averaged 224.90±115.55 μ/L，AST 118.57±64.38 μ/L. About 50.48% of all TG was above normal，2.86% had diabetes， and 22.86% of insulin was above normal. About 83.81% had fatty liver by ultrasonogrophy. （3）Liver pathology: 56.19% of all had F≥3，29.53% of all G≥2，20.95% of all S≥3. NAS score 5～6 cent accounted for 41.90% of all，7～8 cent for 58.10% of all. The group of patients with F≥3 were higher than the group of F＜3 in average BMI，AST and the percentage of hyperglyceride and S≥3.（4）Liver fibrosis logistic analysis revealed that liver F grade and BMI affected liver fibrosis in NASH children. Conclusion NASH is more common in boys，and 11～13 years old is more. The group of patients F≥3 had higher than the group of F＜3 in average BMI，AST and the percentage of hyperglyceride，S≥3，liver F grade and BMI affect liver fibrosis.

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Clinical characteristics and follow-up of bronchiectasis in children. WANG Hao，XU Bao-ping，LIU Xiu-yun，HU Ying-hui，REN Yi-xin，SHEN Kun-ling. 2014, 29(12): 936-939.  DOI: 10.7504/ek2014120613 Abstract ( )   Abstract：Objective To investigate the general characteristics， undrelying causative factors and prognosis of bronchiectasis in chilren. Methods A total of 172 chilren diagnosed with bronchiectasis were included in the study. Demographics， clinical data， HRCT， lung function， and underlying etiology were collected and retrospectively analyzed. Part of the patients were followed up. Results Cough and expectoration were the two most common symptoms. Chest X-ray was significantly less sensitive （4.42%） than HRCT （100%） scan in diagnosis of bronchiectasis. Immunodeficiency， bronchiolitis obliterans and primary ciliary dyskinesia were the three main etiology causing widespread distribution in HRCT （P＜0.05）. Lung functions in 76.92% of the patients were abnormal. The underlying etiologies were identified in 113 patients （65.70%）， and pulmonary infecion（31.40%） was the most common. It was found that FEV1 decreased by 1.28% per year with the extension of the course in the follow-up patients. There was improvement of HRCT in one patient whose symptom and lung fuction were also improved. Conclusion Bronchiectasis usually presents nonspecific symptoms. Most patients can be found with the underlying causes， and pulmonary infection is the most common cause. HRCT is needed as early as possible in children with chronic respiratory symptoms. FEV1% declines slowly with the course extended. Part of the bronchiectasis children can be improved.

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Respiratory syncytial virus infection in children with primary immunodeficiency. LIU Ping， ZHAO Xiao-dong. 2014, 29(12): 940-943.  DOI: 10.7504/ek2014120615 Abstract ( )   Abstract： Objective In this study， we describe the clinical characteristics associated with respiratory syncytial virus（RSV） infection in children with primary immunodeficiency（PID）. Method Thirty specimens were collected from children with PID from Apr. 2009 to Sep. 2010 and analyzed for the presence of RSV using real-time chain reaction assay.To further verify the presence of the virus，viral isolation was used to ensure reproducibility. Results The presence of RSV was detected respectively in 11 （36.7%） of the 30 children studied, including symptomless patient 2 cases（18.2%），bronchitis 3 cases （27.3%）and pneumonia 6 cases（54.5%）.Two symptomless patient with RSV infection were treated with intravenous immune globulin （IVIG）. Conclusion PID children are at increased risk of RSV infection； IVIG can reduce the incidence of RSV pneumonia.

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Clinical and pathological features and follow-up studies of idiopathic membranous nephropathy in 20 children FU Hui，WU Yu-bin，DU Yue，ZHAO Cheng-guang. 2014, 29(12): 944-947.  DOI: 10.7504/ek2014120616 Abstract ( )   Abstract： Objective To investigate the children with idiopathic membranous nephropathy（IMN） about the clinical and pathological characteristics， efficacy and prognosis， providing the reference for the clinical diagnosis and treatment.Methods  Summarize and analyze the clinical manifestation， pathological features， treatment and prognosis of 20 cases of IMN patients in Paediatric Nephrology of Shengjing Hospital of China Medical University between 2006.2 and 2014.2. Results  （1） In 20 cases of children with IMN，there were 13 male cases and 7 female cases, male to female ratio 1.86∶1. The age ranged from 4 to 14 years， the mean age being 10.65 ± 3.18 years；from onset to renal biopsy the was 7 to 190 days，with an average of 59.1 ± 55.9 days.There were 5 cases of below nephrotic proteinuria in clinical manifestations， （including simple proteinuria in 1 case， hematuria and proteinuria in 4 cases），and 15 cases of nephrotic proteinuria， of which 1 case with acute renal insufficiency.（2）Renal pathology:light microscopy showed that there were 2 cases of stage I membranous nephropathy，17 cases of stage II membranous nephropathy and 1 cases of stage III membranous nephropathy，among which 16 cases were with mild mesangial cells proliferation and mesangial matrix increase，4 cases with partial glomerular sclerosis（percentage of glomerular sclerosis was 2.4%，3.4%，3.7% and 5.5%，respectively），and 7 cases with focal tubular atrophy and interstitial fibrosis.Immunofluorescence showed there was IgG and C3 deposition， partly with IgM，Fg，C1q，and IgA deposition.（3）According to the level of proteinuria the patients were treated with glucocorticoid and renin angiotensin converting enzyme inhibitor（ACEI） or combined with immunosuppressive therapy with a result of 12 cases completely relieved and 8 parthy relieved.（4）In the 19 cases followed up for 2-7 years，12 cases obtained complete remission， of which 2 cases replased in 1.5 year and 2 years after drug withdrawal. Eight cases had partial remission，of which 7 cases were still receiving sequential treatment，while 1 case lost follow-up because of refusing the use of immunosuppressant. Conclusion The onset of IMN is mainly in elder children and male is more than female.Nephrotic syndrome is the major clinical manifestations of IMN. Glucocorticoids combined with immunosuppressive drugs can achieve satisfactory therapeutic effect.