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06 July 2013, Volume 28 Issue 7 Previous Issue    Next Issue

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Discussion of relationship between renal pathology and glomerular podocyte injury in childhood Henoch-Schönlein purpura nephritis. REN Xian-guo*， ZHANG Pei*， DU Li-fang， GAO Yuan-fu *， FAN Zhong-min*,GAO Chun-lin *， MAO Song*， HE Xu*， XIA Zheng-kun*， REN Xian-qing， LIU Guang-ling. 2013, 28(7): 512-515.  Abstract ( )   Abstract: Objective    To analyze relationship between renal pathology and glomerular podocyte food processes fused in childhood Henoch-Schönlein purpura nephritis. Methods    Renal pathological types of 72 children suffering from HSPN were reviewed during the period from January 1st 2008 to January 1st 2011 from Nanjing Command of Jingling Hospital. Pathological grading and immunofluorescence typing were analyzed. The information of podocyte food processes fused was showed by electron microscopy. Results    Pathological findings were classified as ISKDC（the International Society of Kidney Disease in Children） grade Ⅱin 34 （47.2%） and gradeⅢ in 38 （52.8%）. Glomerular podocyte injury was classified by electron microscope as food processes of podocyte fused extensively in 21 cases，food processes of podocyte fused segmentally in 35 cases， food processes of podocyte without fusion in 11cases.There was no renal glamorous in nephridial tissue by electron microscope in five cases. Conclusion    The childhood HSPN is expressed with foot process of podocytes fused more extensively on electron microscope besides glomerular pathologic change. The more serious  the degree of podocyte injury is，the more obvious the pathologic changeis.

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Management of central airway stenosis by means of the flexible bronchoscopic balloon dilatation tracheoplasty in children. 张 磊a，殷 勇a，张 静a，王 薇a，朱 铭b 2013, 28(7): 516-521.  Abstract ( )   Abstract: Objective    To assess the efficiency and safety of the flexible bronchoscopic balloon dilatation tracheoplasty in children， and discuss the optimal indication and combined therapy of central airway stenosis in children. Methods         From July 2009 to April 2012， 34 cases were diagnosed as central airway stenosis by chest CT scan and bronchoscpy in Shanghai Children's Medical Center，and received the treatment of the flexible bronchoscopic balloon dilatation tracheoplasty. The data about the efficiency， complication and prognosis of the treatment were collected. Results    In the 34 cases， 16 cases were improved after the treatment and the overall response rate was 47.1%. According to the pathology of the stenosis， these cases were divided into three types: 9 cases were membranous stenosis， 5 cases were muscular stenosis and 20 cases were osseous stenosis. The response rate was 47.1%， 100%， and 20% respectively. According to the anatomy morphology of the stenosis， 20 cases were short distance stenosis and the response rate was 75%； 12 cases were long distance stenosis and the response rate was 8.3%； 2 cases were funnel stenosis and the response rate was 0. Among these cases， 3 cases accepted the operation of pulmonary artery sling and tracheal sliding angioplasty， the total response rate being raised to 55.9%. The most common complication in the flexible bronchoscopic balloon dilatation tracheoplasty was transient oxygen desaturation. Conclusion    The severity， length， pathology， indication and time of the treatment should be considered completely before the flexible bronchoscopic balloon dilatation tracheoplasty. The balloon dilatation therapy is suitable for membranous with short distance stenosis. The combined interventional treatment is good for muscular stenosis，and the surgery and palliative treatment is an option for osseous with long distance stenosis.

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Clinical analysis of methylmalonic acidemia in 95 children. LI Qi-liang， SONG Wen-qi. 2013, 28(7): 522-524.  Abstract ( )   Abstract： Objective    To accumulate knowledge of methylmalonic acidemia （MMA） screening by studying the clinical characteristics and laboratory characteristics of the disease. Methods    The clinical manifestations and the results of laboratory tests of 95 cases of MMA were analyzed. Results    The age of onset was from 1 day to 12 years. Six cases had the episodes of vomiting， lethargy in 14 cases and recurrent difficulty with feeding in 4 cases， seizures in 24 cases， mental retardation and movement disorders in 18 cases， dysarthria in 3 cases， debilitation and BLE edemas in 10 cases， jaundice in 7 cases， complaints of precordium in 6 cases， anaemia in 3 cases. The laboratory tests showed that the counts of white blood cells（WBC）were increased in 26 cases and the counts of red blood cells （RBC）were decreased in 26 cases. The results of urine routine tests were abnormal in 37 cases. The results of renal function tests were abnormal in 18 cases. The results of hepatic function tests were abnormal in 17 cases. The results of myocardial enzyme were abnormal in 40 cases. The results of blood gas test were abnormal in 11 cases. The levels of glucose were decreased in 6 cases. The levels of lactic acid were increased in 62 cases. The levels of blood ammonia were increased in 50 cases. Remarkable elevation of urinary methymalonic concentration was confirmed in 97% patients by gas chromatography -mass spectrometry （GC-MS）. Remarkable elevation of C3， C3/C0 and C3/C2 concentrations was confirmed in 89% patients by liquid chromatography-tandem mass spectrometry （LC-MS/MS）. Conclusion    The main clinical features of MMA include lethargy， seizure， vomiting，developmental retardation or regradation， hyperarmnonemia and hyperlactacidemia. Urine organic acids analysis by GS-MS and dry blood spots （amino acids and acylcarnitine） analysis by LC-MS/MS are very important to the diagnosis of MMA.

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Correlative study between plasma N-terminal pro-brain natriuretic peptide and right ventricular Tei index in children with pulmonary arterial hypertension.  GAO Yi-hua*， I-Seok Kang，Geena Kim. 2013, 28(7): 525-528.  Abstract ( )   Abstract：Objective    To investigate the correlation among plasma N-terminal pro-brain natriuretic peptide （NT-proBNP） and right ventricular Tei （RVTei） and right ventricular fractional area change （RVFAC） in children with pulmonary hypertension. Methods    From January 2007 to November 2012，echocardiography and serological examination data of 38 patients from Samsung Seoul Hospital in Korea were retrospectively analyzed. Patients were divided into two groups ：16 cases with 30＜PASP＜70mmHg （mild to moderate） and 22 cases with PASP≥70mmHg （severe）. Results    （1）RVFAC， PASP and plasma concentration of NT-proBNP had a statistically significant difference（P＜0.05） between mild to moderate PAH group and severe PAH group . RVTei index had no difference between the two groups（P＞0.05）. （2）Correlation analysis：the plasma concentration of NT-proBNP had a positive correlation （r = 0.544，P＜0.05）with PASP，a negative correlation（r = -0.767，P＜0.05）with RVFAC；RVFAC had a negative correlation（r = -0.711，-0.767，P＜0.05） with PASP and the plasma concentrations of NT-proBNP； the RVTei had no correlation with the plasma concentration of NT-proBNP， PASP or RVFAC（r =  -0.041，-0.048，0.016，P＞0.05）. Conclusion            Whether there are correlations between the plasma concentration of NT-proBNP and RVFAC depends on the degree of PAH. This suggests that the plasma concentration of NT-proBNP and RVFAC can be used as evaluation of right ventricular function in children with pulmonary hypertension.

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Investigation of the relationship between mutation of thyroglobulin gene and congenital hypothyroidism with goiter.       ZHANG Yuan-feng*，LI-Tang,SU Nai-lun,ZHANG Li-qin，MA Shao-chun. 2013, 28(7): 529-532.  Abstract ( )   Abstract：Objective    To identify thyroglobulin （ TG） gene mutation in patients with congenital hypothyroidism with goiter，in order to provide full evidence for gene diagnosis of congenital hypothyroidism. Methods    Totally 11 patients with congenital hypothyroidism with goiter in Qingdao Women and Children hospital from Jan. 2012 to Aug. 2012 were enrolled in this study. The 7，14，22，33 and 38 exons of TG gene were amplified through PCR and the products were sequenced directly. The type and characteristic of TG gene mutation in patients with congenital hypothyroidism with goiter in this region were analyzed. Results    Two single nucleotide polymorphism of TG gene were identified，but no gene mutation was observed.  TG c.3218-81T＞G （rs 853324，homozygous）in 1 patient and c.3218-81T＞G（rs 853324，heterozygosis）in 6 patients were found. Conclusion    The incidence of TG gene mutation is very low in patients with congenital hypothyroidism with goiter from Qingdao City. It suggests that TG gene mutation may not serve as the mutation hotspot gene of congenital hypothyroidism with goiter in Qingdao City．

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Relationship of fractional exhaled nitric oxide with childhood asthma control test and bronchial provocation test.    LUO Xue-qin， LIU En-mei，FU Zhou， GONG Cai-hui，LUO Jian. 2013, 28(7): 533-536.  Abstract ( )   Abstract： Objective    To find out the fractional exhaled nitric oxide（ FENO） in different control level and the relationship of FENO with childhood asthma control test （C-ACT） and bronchial provocation test（BPT）. Methods    Totally 48 asthmatic children who underwent routine checkups since March 2011 through September 2011 were enrolled in the study. The patients completed the detection of FENO， they also underwent pulmonary  function  test（PFT），  bronchial  provocation test （BPT）and C-ACT. Those indexes and relationships were analyzed in different control groups. Results          The geometrical mean FENO of asthmatic children was （22.5 ± 2.0） × 10-9 mol/L. Uncontrolled group had higher FENO than controlled group and partly controlled group， and FENO was  slightly  related to FEV1% Pred，so did PC20，but no relevance had been found between FENO， C-ACT or PC20. Conclusion    FENO can be an useful tool in managing asthma when used together with C-ACT，PFT and BPT.

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The trends of diseases and cause-specific death in hospitalized children in the hospitals of urban Beijing from 2003 to 2009.   LI Xia*，DU Zhong-dong*，ZHOU Zhong-shu，KONG Jian*，PAN Yue-song*. 2013, 28(7): 537-539.  Abstract ( )   Abstract：Objective    To analyze the evolution of disease composition and causes of death in hospitalized children in hospitals of urban Beijing. Methods    Medical data of 151 336 hospitalized children in Beijing Children's Hospital and the Sino-Japanese Friendship Hospital aged 0～18 years from 2003 to 2009 were analyzed to summarize changes of disease composition and mortality of each age group and year. According to international nosology ICD-10 classification criteria， first diagnosis of each child was classified into five catogeries： communicable diseases， non-communicable diseases， perinatal diseases， injuries and others. Results    The first three diseases of hospitalized children were respiratory infection， congenital malformation and neonatal disease； the top five single diseases for hospitalization were pneumonia， inguinal hernia， cancer， upper respiratory tract infection and allergic purpura.The top three cause-specific death of hospitalized children were congenital heart disease， cancer and pneumonia. Congenital heart disease was the first cause of death in infants under the age of 1 year， while cancer as the first cause of death in more than 1 year old children.The top five diseases of hospitalized children were different in each age group. Conclusion    The number of congenital malformations and cancer has significantly increased in hospitalized children in the hospitals of urban Beijing， but pneumonia still accouns for the most common disease in these children. Congenital heart disease is the first cause of death in infants， while cancer in more than 1 year old children， which is basically the same in developed countries.