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06 December 2017, Volume 32 Issue 12 Previous Issue    Next Issue

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Expert consensus on integrated traditional Chinese and western medicine in the diagnosis and treatment of Mycoplasma pneumoniae in children （2017） Childhood Pneumonia Union of China Association of Chinese Medicine 2017, 32(12): 881-885.  DOI: 10.19538/j.ek2017120601 Abstract ( )

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Paying attention to the bronchiolitis in children LU Quan 2017, 32(12): 886-889.  DOI: 10.19538/j.ek2017120602 Abstract ( )   Bronchiolitis is a common respiratory disorder in childhood and bronchiolitis generally refers to a group of disorders in which a variety of pathogenic factors cause bronchiolar damage，resulting in reactive inflammatory cell infiltration and fibrous tissue hyperplasia. Pathologically bronchiolar diseases are divied into primary and secondary categories. Bronchioles are the end point of airway conduction area and its unique anatomical and physiological characteristics make it a good place for many diseases. Acute infectious bronchiolitis is one of the most common types，while bronchiolitis obliterans（BO） is attracting more and more attention in pediatric clinical practice and diffuse pan-bronchiolitis（DPB） has also been reported in children. Pediatricians need to improve their knowledge of bronchiolitis. Bronchiolar diseases and diffuse interstitial lung fibrosis are quite overlapped. It is necessary to take a holistic view of the intrinsic relationship between lung parenchyma and pulmonary interstitial. It should be regarded as the only correct choice to make standard diagnosis and treatment of bronchiolitis.

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Anatomy and physiological basis and classification of bronchiolitis LIU Han-min 2017, 32(12): 890-893.  DOI: 10.19538/j.ek2017120603 Abstract ( )   Bronchiolitis has been one of the hot spots in the researches on diseases of respiratory system nowadays. However， the concepts and classifications of this disease remain unclear. In this paper， the denotative and connotative concepts of bronchioles and bronchiolitis were analyzed from the views of anatomy and development biology， and the classification changes of adult bronchiolitis were introduced. These content can be used as references for the systematic studies of pediatric bronchiolitis.

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Acute infectious bronchiolitis ZHAO Cheng-song，ZHAO Shun-ying，WEN Xiao-hui 2017, 32(12): 893-895.  DOI: 10.19538/j.ek2017120604 Abstract ( )   Acute infectious bronchiolitis could be caused by virus，bacteria，fungus and Mycoplasma pneumoniae with non-specific histology in children. Fever，cough and wheeze were the clinical manifestations；while centrilobular nodules，tree-in-bud and bronchiolar wall thickening were the main HRCT findings. The short-term prognosis was often good，although long-term prognosis was not optimistic with asthma and recurrent wheezing left. Since acute Mycoplasma pneumoniae bronchiolitis cases seemed to increase recently，it should be paid attention to by pediatrician.

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Research advance of association between viral bronchiolitis and asthma in children ZHANG Hai-lin，Lü Fang-fang 2017, 32(12): 895-900.  DOI: 10.19538/j.ek2017120605 Abstract ( )   Viral bronchiolitis is one of the most common health problems in children worldwide. The most common pathogen is respiratory syncytial virus（RSV），and other pathogens include rhinovirus，human metapneumovirus，bocavirus and so on. Infant hospitalized for viral bronchiolitis will have recurrent wheezing or asthma later，disease severity，virus type，atopy and genetic polymorphisms  will increase the risk of asthma. The definition of viral bronchiolitis is not uniform，and the difficulty of defining wheezing and its frequency is a great obstacle to study the relationship between them. Future studies should give full consideration to the heterogeneity of bronchiolitis，and observe and compare the risk of asthma in different phenotypes of bronchiolitis.

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Etiology and pathogenesis of bronchiolitis obliterans ZHAO De-yu，PAN Jing 2017, 32(12): 900-903.  DOI: 10.19538/j.ek2017120606 Abstract ( )   Bronchiolitis obliterans is associated with many factors；clinical common causes include infection，allogeneic hematopoietic stem cell transplantation，allogeneic organ transplantation，inhalation injury and autoimmune diseases，such as adverse drug reactions. The main pathogenesis is that the airway epithelial tissue is excessively activated by the tissue injury resulting from inflammation or immune response which triggered a series of related immune response，accompanied by respiratory function recovery disorder，eventually leading to the occurrence of BO.

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Progress in the treatment of bronchiolitis obliterans XU Dan，CHEN Zhi-min 2017, 32(12): 903-907.  DOI: 10.19538/j.ek2017120607 Abstract ( )   Bronchiolitis obliterans（bronchiolitis obliterans BO） is a chronic airflow limitation caused by bronchial inflammatory injury. The clinical manifestations are persistent cough, wheezing and dyspnea after airway injury. The conventional bronchodilator therapy is invalid for BO. At present, the treatment for BO is still in lack of uniform standard. The early stage is the key period of clinical treatment. Treatment for children BO includes general supportive treatment, use of corticosteroids, bronchodilators, macrolides，intravenous immunoglobulin and other antibiotics，leukotriene receptor antagonists and immunosuppressants，as well as pulmonary physical therapy and lung transplantation.

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Diffuse panbronchiolitis NONG Guang-min，CHEN Xun 2017, 32(12): 907-912.  DOI: 10.19538/j.ek2017120608 Abstract ( )   Diffuse panbronchiolitis（DPB），an idiopathic inflammatory disease，is characterized by chronic respiratory bronchioles of the bilateral lung. The etiology and pathogenesis of DPB is unknown. The main etiology is now believed to be related to gene and infection. Pathological examination is the gold standard for DPB diagnosis. Low-dose and long-term treatment with macrolides has been shown to significantly improve the prognosis of DPB patients.

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Imaging features of  bronchiolitis YUAN Xin-yu 2017, 32(12): 912-916.  DOI: 10.19538/j.ek2017120609 Abstract ( )   Bronchiolitis is the most common lower respiratory inflammation in infants younger than 5 years. Pathogens are variable, and the majority of bronchiolitis were caused by infection. The radiological presentations of bronchiolitis include direct signs （bronchiol-ectasis，tree-bud，central lobular nodules） and indirect signs（air trapping，Mosic sign）. Bronchiolitis caused by different entities will present different presentations. The combination of clinical information and radiological findings will provide evidence for correct diagnosis.

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Chronic suppurative lung disease in children FENG Yong，CAI Xu-xu，SHANG Yun-xiao 2017, 32(12): 917-922.  DOI: 10.19538/j.ek2017120610 Abstract ( )   The term pediatric chronic suppurative lung disease has a history of one hundred years, but with the progress of diagnosis and treatment technology and changes of disease spectrum, the concept of chronic suppurative lung disease has changed greatly in recent years. The concept has been redefined and a series of researches have been conducted. Chronic suppurative lung disease is a clinical syndrome and mainly manifest as repeated attacks of chronic wet cough. There are varied causes, and the diagnosis and  etiology are particularly important. The updates on this concept complete a disease spectrum, and can help to early find out children who may develop into bronchiectasis. Early treatments and interventions can improve outcomes and reduce mortality.

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Long-term home invasive ventilation management in children ZHU Bi-zhen，WU Jin-zhun 2017, 32(12): 923-928.  DOI: 10.19538/j.ek2017120611 Abstract ( )   The benefits of home invasive ventilation are obvious，compared with hospital invasive ventilation. More and more evidence-based medical researches have verified the feasibility and safety of long-term home invasive ventilation. Children with chronic invasive ventilator dependence at home are a diverse group of children who may have one or more organ dysfunction，involving respiratory，nervous，digestive，cardiovascular，skeletal and endocrine system. This article will introduce 4 critical clinical questions related to long-term home invasive ventilation presented in the 2016 American Thoracic Society Clinical Practice Guideline，i.e. medical management mode，evaluation before discharge，training of the long-term home care-givers，and the instruments and equipment necessary for the treatment，and discuss each topic through combination with the relevant literature and actual situation in China.

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Height velocity targets for the first year in response to recombinant human growth hormone（rhGH） treatment in pre-pubertal Growth Hormone Deficiency（GHD） children SU Zhe，DU Min-lian，MA Hua-mei，et al 2017, 32(12): 929-932.  DOI: 10.19538/j.ek2017120612 Abstract ( )   Objective　To describe height velocity in pre-pubertal Growth Hormone Deficiency（GHD） children without recombinant human growth hormone（rhGH） treatment and explore the height velocity targets for the first year in response to rhGH treatment. Methods　Analyze retrospectively the height velocity data without（HV0） and one year after （HV1） rhGH treatment in physiologic dose［0.7 U/（kg·w）］ in pre-pubertal GHD children above 3 years old who were diagnosed from Jan，2000 to Dec，2009 in our hospital. The GHD patients who were included for calculation of HV0 had peak GH value in GH provocative test less than 7 ng/ml. HV0 was calculated according to age（HV0-CA，342 patients） and bone age（HV0-BA，257 patients） respectively.  According to the peak GH value in GH provocative test，the patients who were included for calculation of HV1 were divided into GHD-1 group（＜0.33 nmol/L，140 patients） and GHD-2 group（7.0～9.9 μg/L，33 patients）. Results　Within every bone age group，GHD-1 group had significantly higher HV1 than GHD-2 group（P＜0.05），11.0（10.5-11.5） cm/a（n＝34） vs. 9.9（9.1-10.8） cm/a（n＝6） when bone age was less than 3 years，10.4（9.8-10.9） cm/a（n＝48） vs. 8.8（8.3～9.2） cm/a（n＝8） when bone age was between 3 to 5 years，and 9.5（9.1-9.9） cm/a（n＝58） vs. 8.5（8.0-9.1） cm/a（n＝19） when bone age was between 6 to 10 years. The mean HV1 of GHD-2 was very close to the 25th percentile（P25） of GHD-1 group. They both were significantly higher than HV0-BA. Conclusion　The recommended height velocity target for the first year after rhGH treatment in pre- pubertal GHD children is the P25 of HV1 of GHD-1 group. It should be at least 9.9 cm/a，8.7 cm/a and 8.3 cm/a when the bone age is less than 3 years，3 to 5 years and 6 to 10 years， respectively.

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Long-term effect of gonadotropin-releasing hormone analogue treatment on glucose and lipid metabolism and function of hypothalamic pituitary gonadal axis in Chinese girls with ICPP ZHANG Qian-ru，CHEN Rui-min，CAI Chun-yan，et al 2017, 32(12): 933-936.  DOI: 10.19538/j.ek2017120613 Abstract ( )   Objective　To observe the influence of gonadotropin-releasing hormone analogue（GnRHa） treatment on body mass index（BMI），glucose and lipid metabolism，hypothalamic pituitary gonadal axis（HPGA） function in Chinese girls with idiopathic central precocious puberty（ICPP） after withdrawal. Methods　The girls，who were treated with GnRHa for two years and then withdrawn，were enrolled in this study. The data of height，weight，BMI，fasting blood glucose（FBG），blood lipid，insulin，serum hormones associated with the HPGA，and the volumes of uterine and ovarian were obtained and compared at withdrawal，the first and second year after withdrawal respectively. Results　A total of 65 girls with ICPP were studied. Compared with withdrawal，BMI，sex hormone and other indexes at the first and second year after withdrawal were increased significantly（P＜0.05），but the level of BMI-Z score，FBG and lipid metabolism index did not change significantly（P＞0.05）. Dehydroepiandrosterone sulfate and testosterone at second year after withdrawal were significantly higher than those at withdrawal（P＜0.05）. After withdrawol for（14.16±6.70） months，32 girls emerged menarche，whose average age was（11.86±0.75） years old. Conclusion　HPGA function of ICPP girls who had treated with GnRHa gradually recovered after withdrawal. The treatment of GnRHa has been found no influence on weight status，but might increase the level of insulin and androgen.

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Determination of a steroid profile in dried blood spots specimen by liquid chromatography-tandem mass spectrometry ZHAN Xia，HAN Lian-shu，YE Jun，et al 2017, 32(12): 937-941.  DOI: 10.19538/j.ek2017120614 Abstract ( )   Objective　To establish a method to determine steroids in dried blood spots specimens by liquid chromatography-tandem mass spectrometry（LC-MS/MS） assay. Methods　Two dry blood spots（DBS） of diameter of 3 mm with different concentration of androstenedione，testosterone，17α-hydroxyprogesterone，progesterone，11-deoxycortisol，21-deoxycortisol and cortisol，were punched. Then the two DBS were extracted with 1∶1 methanol/acetonitrile solution，which was labeled with stable isotope internal standards. The extraction of DBS was dried under nitrogen. After reconstituted，the steroids were analyzed by LC-MS/MS. The linearity，precision and accuracy were evaluated. Seven DBS samples of 21-hydroxylase deficiency patients and 23 DBS samples of normal infants were collected，and then the steroids for these samples were analyzed by LC-MS/MS. Results　The intra-day and inter-day precisions were 3.3%～10.9%，and the intra-day and inter-day accuracies were 100.4%～116.5%. The lowest limits of detection（LLOD） of these seven steroids were 0.03～0.25 μg/L. The lowest limits of quantitation（LLOQ） of these seven steroids were 0.125～1.00 μg/L. The linearities of different steroids were 0.997～0.999 respectively. The results of non-parametric test about steroids concentration in infants and 21-OHD patients showed that，the concentration of androstenedione，17-hydroxyprogesterone，progesterone，21-deoxycortisol and cortisol in the 21-OHD patients were significantly higher than those steroids in normal infants，P＜0.05. Conclusion　A method for measurement of the steroids in DBS by LC-MS/MS has been established，which shows good precision，accuracy and linearity. This method can be applied to clinical testing and follow-up therapy of CAH patients，and it is expected to be used as a second-tier test for CAH newborn screening.

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Genetic studies of 77 cases of short stature with unknown etiology YANG Lu-lu*，WANG Wei，SHI Jin-xiu，et al 2017, 32(12): 942-946.  DOI: 10.19538/j.ek2017120615 Abstract ( )   Objective　To detect pathogenic genes of short stature with unknown etiology by a targeted next generation sequencing panel to analyze the correlation between genotypes and clinical phenotypes. Methods　A total of 77 children diagnosed with unexplained short stature were enrolled for the study. These children were treated in Ruijin Hospital of Shanghai Jiao Tong University from 2007 to 2015. To search for genetic variation in 187 candidate genes which were associated with growth， we constructed a targeted next generation sequencing panel encompassing the coding regions of 187 genes. According to ACMG Guidelines，the sites of variation were determined. Sanger sequencing was used to verify the suspected pathogenic genes variation. The relationship between genotype and clinical phenotype was analyzed. Results　Including 5 pathogenic variants， one likely pathogenic variant and one variant of uncertain significance， we identified 7 heterozygous variants of 7 cases in 77 cases of short stature with unknown etiology. A pathogenic variant p.D2407fs of ACAN gene was found in a case with advanced bone age. There were 3 reported pathogenic variants， including p.A72G， p.I282V and p.P491S of PTPN11 gene， which were diagnosed as Noonan syndrome. A case carrying known pathogenic variant COL2A1（p.R904C） was diagnosed as Stickler syndrome. We still got one likely pathogenic variant COMP（p.D401N）， which could cause multiple epiphyseal dysplasia. There was a familial short stature of delayed bone age carrying a variant（p.S289Y） of uncertain significance，in which the genotype was in accordance with the clinical phenotype. Conclusion　The ACAN gene defection is associated with the idiopathic short stature with advanced bone age. The likely pathogenic variant COMP（p.D401N） may cause multiple epiphyseal dysplasia. The newly-found heterozygous varians（p.S289Y） of GHSR gene may result in short stature，which needs further function verification.

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Expression differences of microRNA-126 in mast cell and its influence on release of IL-4 XU Chang-di，TANG Heng，ZHAO De-yu，et al 2017, 32(12): 947-950.  DOI: 10.19538/j.ek2017120616 Abstract ( )   Objective　To observe the expression features of peripheral blood microRNA（miR）-126 in children with asthma and to investigate the influence of miR-126 downregulation on release of interleukin（IL）-4 in mast cells and its possible mechanism. Methods　The peripheral blood of healthy subjects（control group） and astham children（asthma group） were enrolled. The asthma group included 6 children who were treated in Nanjing Children’s Hospital from September to December of 2015. The levels of serum miR-126 were measured in both groups by quantitative real-time polymerase chain reaction（RT-PCR）. After stimulation with lipopolysaccharide（LPS） on P815，miR-126 expression was detected by RT-PCR. P815 were transfected with miR-126 negative control（negative control group） and miR-126 inhibitor（miR-126 inhibitor group） respectively. The levels of IL-4 in supernatants were detect by ELISA，and the levels of phosphorylated P38 were detected by Western Blot in two groups. Results    The levels of peripheral blood miR-126 in asthma group were significantly higher than those in control group（P＜0.05）. In LPS stimulated P815，miR-126 expression increased（P＜0.05）. The levels of IL-4 and phosphorylated P38 in the miR-126 inhibitor group were lower than those in the negative control group，with statistical significance（P＜0.05）. Conclusion    The expression of peripheral blood miR-126 increases in asthma children. Down-regulation of miR-126  may suppress IL-4 release from mast cells by reducing the expression of phosphorylated P38.

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Clinical characteristics and diagnosis analysis of intrathoracic tuberculosis in children YIN Hong-yun*， SHI Xiang， GE Yan-ping， et al 2017, 32(12): 951-955.  DOI: 10.19538/j.ek2017120617 Abstract ( )   Objective　To explore the value of clinical manifestations and routine examinations for the diagnosis of intrathoracic tuberculosis in children. Methods　The medical records of children under 16 years of age due to intrathoracic diseases in Shanghai Pulmonary Hospital affiliated to Tongji University School of Medicine from January 2009 to December 2011 were collected. According to the final diagnosis，the patients were divided into 2 groups：intrathoracic tuberculosis and non tuberculosis. The clinical manifestations and routine auxiliary examination results of the 2 groups were retrospectively analyzed. The children were divided according to age＜10 years and over 10 years old in 2 groups，and the clinical data were also compared. Results　（1）Pathological diagnoses were confirmed at 72.30% of proportion at whole patients，70.73% in TB group. The positive rate of tuberculosis bacteriology was 21.85% in TB group. （2）The middle course of history accounted for 56.30% in TB children. At the onset of illess，the proportion of fever and predisposition to tuberculosis，positivity of tuberculin skin test（TST） and serumal tuberculosis antibody，and average value of ESR，CRP，PLT and D-Dier in blood were significantly higher in TB group than those in non-TB group. （3）About 59% of patients in the non-TB group were cured or were markedly improved by antibiotic treatment，which was effective in 10% of the TB group. （4）Diagnostic accordance rate by radiology was 53.36% and the rate of misdiagnosis as tuberculosis accounted for 11.71% in non-TB group. Conclusion　Clinical features combined with medical history and laboratory results are helpful for the identification of tuberculosis in children under 16 years old.

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Clinical observation on the auxiliary treatment with andrographolide sulphonate for Mycoplasma pneumoniae pneumonia in 80 cases WANG Zheng-jun，RUAN Lian-ying，JI Ya-nan, et al 2017, 32(12): 956-958.  DOI: 10.19538/j.ek2017120618 Abstract ( )

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Incontinentia pigmenti-a familial report YANG Xiao-meng，ZHOU Ai-yan，LIU Yong, et al 2017, 32(12): 959-960.  DOI: 10.19538/j.ek2017120619 Abstract ( )