Table of Content

15 February 2017 Volume 35 Issue 2

  

Role and influence of coinfection factor to refractory Mycoplasma pneumoniae pneumonia in children

LU Yanhong，ZHANG Xinxing，YAN Yongdong，GU Wenjing，CHEN Zhengrong，ZHU Canhong，WANG Yuqing， HUANG Li， SHAO Xuejun，JI Wei

. 2017, 35(2):  81.  doi:10.3969/j.issn.1000-3606.2017.02.001

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　Objective　To study the role and influence of coinfection factor to refractory Mycoplasma pneumoniae （MP） pneumonia (RMPP) in children. Methods　Clinical data and sputum specimen of 286 hospitalized children of RMPP were collected from January, 2013 to December, 2015. According to the results of pathogen detection, children were divided into RMPP coinfection group and simple MP infection group. Clinical features and laboratory tests were compared between the two groups. Results　The durations of fever and hospitalization of the RMPP coinfection group were 12.5（11.0-14.0）days and 12.0 （9.0-14.0）days, respectively, which were significantly longer than those of simple MP infection group, 11.0（9.0-13.0）days and 10.0（8.0-12.0）days (Z=3.947, P<0.001, Z=3.519, P<0.001). In the RMPP coinfection group, white blood cell count, the percentage of neutral granulocytes, pleteltes count, lactate dehydrogenase and the incidence of C-reaction protein (CRP) greater than 40 mg/L, and CRP greater than 100 mg/L, elevated CK-MB of were higher than those of simple MP infection group (all P<0.05), but the incidence of CRP greater than 8 mg/L and elevated ALT in the two groups showed no significant difference (both P>0.05). The incidence of pneumothorax and respiratory failure in children of RMPP coinfection group were higher than those in the simple MP infection group (χ2=5.873, P=0.015, χ2=11.995, P=0.001), but the incidence of lobar pneumonia, pleural effusion, atelectasis in the two groups had no significant difference (all P>0.05). The incidence of blood system damage, myocardial damage, skin and joint damage and electrolyte disorders in RMPP coinfection group were higher than those of simple MP infection group (all P<0.05). Conclusions　Children with RMPP coinfection tend to be with prolonged duration of fever, increased systemic inflammation, higher incidence of pulmonary and extrapulmonary complications, and longer duration of hospitalization.

Mycoplasma pneumoniae pneumonia with secondary thrombocytosis in infants and young children

ZHU Ruochen, JIANG Qin, WU Liangxia, ZHANG Jianhua

. 2017, 35(2):  86.  doi:10.3969/j.issn.1000-3606.2017.02.002

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Objectives　To analyze the clinical characteristics and significance of Mycoplasma pneumoniae pneumonia (MPP) combined with secondary thrombocytosis in infants and young children. Methods　Clinical features, laboratory and imaging data of the infants and young children with MPP were collected, and compared between the two groups of children with and without secondary thrombocytosis. Results　Sixty-seven (67) infants and young children with secondary thrombocytosis (28 males and 39 females, with onset at 2 years and 2 months after birth in average) and 269 infants and young children with normal platelet counts (138 males and 131 females, at 2 years and 3 months after birth in average) were included. Significant longer durations of hospitalization（P =0.018） and fever（P =0.000）, higher temperature peak（P =0.000）, as well as higher morbidity of refractory MPP（P =0.001） and more complications（P =0.000）were observed in the group of MPP with secondary thrombocytosis. Moreover, the laboratory data of white blood cell count（P=0.000）, C-reactive protein（P=0.000），procalcitonin （P=0.000）, erythrocyte sedimentation rate（P=0.000）and higher morbidity of multiple pathogen infection（P =0.033） were observed between the two groups. Conclusions　More severe clinical manifestations and higher complication morbidity could be observed in the group of  MPP combined with secondary thrombocytosis, implicating the value of platelet count  in clinical assessment of MPP in infants and young children at early stage.

Expression and verification of miRNAs in peripheral blood of Mycoplasma pneumoniae pneumonia

　DING Ying, LEI Xiaoli, SUN Dan, LI Yuqin, CHU Chu, CHEN Zhengrong, JI Wei, ZHOU Weifang

. 2017, 35(2):  90.  doi:10.3969/j.issn.1000-3606.2017.02.003

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Objective　To identify the differentially expressed microRNAs（miRNA）in plasma of Mycoplasma pneumoniae pneumonia (MPP) by miRNA microarray. Methods　36 cases of hospitalized children with MPP were enrolled (MPP group). 27 cases of healthy children were enrolled as control group (NC group) in the same period. Microarray chip was used to find out differentially expressed miRNAs of plasma from 3 cases with MPP group and 3 cases with NC group.  Real-time quantitative PCR was chosen to detect the expression of miR-222-3p and miR-492 in leukocytes from MPP group (n=36) and NC group (n=27).  Results　26 miRNAs differentially expressed between the two groups were screened out by miRNA microarray assays, of which the expression of 19 miRNAs was elevated, while 7 miRNAs declined. Compared with NC group, the expression of miR-222-3p and miR-492 increased in the MPP group (P＜0.01).  Conclusions　Some miRNAs are differently expressed in peripheral blood of MPP patients, which may be involved in the immunopathogenesis of MPP. Key

Treatment and risk factors of diffuse alveolar hemorrhage in 62 children

ZHANG Hui, TIAN Xiaoyin, MENG Qingqing, CHEN Ming, TIAN Qingin, LUO Zhengxiu

. 2017, 35(2):  94.  doi:10.3969/j.issn.1000-3606.2017.02.004

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 Objective　To investigate the possible risk factors for prognosis of diffuse alveolar hemorrhage (DAH) in children and to improve the recognition of the disease. Methods　The study included 62 DAH pediatric patients hospitalized from January, 2006 to January, 2016. Clinical data were retrospectively analyzed. According to the basic diseases, children were divided into immune associated DAH and non-immune associated DAH to explore the effect of early glucocorticoid treatment on the two groups of DAH. Based on the prognosis, the patients were divided into the death group and the survival group to analyze its related risk factors. Results　Of the 62 patients, 20 were of immune associated DAH, 42 of non-immune associated DAH. There was no significant difference of early treatment with glucocorticoid between the two groups (P>0.05). In our cohort, 30 patients died, the total mortality was 48.4％ (30/62). Pediatric critical illness score may be the independent risk factor for DAH mortality. Conclusions　DAH is an acute, life-threatening event, the lower the pediatric critical illness score, the higher risk of death.

Effect of pediatric obstructive sleep spnea hypopnea syndrome with attention deficit hyperactivity disorder on sleep and quality of life　

WU Jiali， GU Meizhen, CHEN Shumei, CHEN Wei, NI Kun, XU Hongmin, LI Xiaoyan

. 2017, 35(2):  98.  doi:10.3969/j.issn.1000-3606.2017.02.005

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Objective　Related factors of pediatric obstructive sleep apnea hypopnea syndrome (OSAHS) combined with attention deficit hyperactivity disorder (ADHD) were retrospectively analyzed in OSAHS children. Methods　The research included 437 cases of children hospitalized for OSAHS from January, 2014 to December, 2014. All the cases were divided into OSAHS group and OSAHS combined with ADHD group, according to the presence or absence of ADHD. Patient's general conditions (gender, age, height, weight, allergic rhinitis history, tonsil grade and adenoid grade), OSA-18 quality of life score, intention-hyperactivity score, polysomnography parameter (apnea hypopnea index, lowest oxygen) were collected as variables. SPSS20.0 was used to perform statistical analyses. Results　There were 437 cases in this research, 298 of them were males, and 139 of them were females. Of 437 patients, 147 had OSAHS combined with ADHD (33.64%).  Higher apnea hypopnea index (P < 0.01) and severer oxygen deficiency (P < 0.01) were found in OSAHS combined with ADHD group than those in OSAHA group. Five projects of OSA - 18 score of in OSAHS combined with ADHD group were all higher than those in OSAHS group (P < 0.01). Conclusions　The incidence of pediatric OSAHS combined with ADHD in this research was more than 30%. More OSAHS combined with ADHD were found in male children. The quality of life was lower in pediatric OSAHS combined with ADHD. Oxygen deficiency was suggested to be the most important risk factor of ADHD in pediatric OSAHS.

Joint attention characteristics of children with autism spectrum disorders

CHEN Yumei,CHEN Zhuoming, LIN Zhenping, LIANG Junjie, LI Jinping

. 2017, 35(2):  102.  doi:10.3969/j.issn.1000-3606.2017.02.006

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 Objective　To analyze the joint attention characteristics of children with autism spectrum disorders (ASD). Methods　2 - 6 years old children with ASD (n=43) are selected to assess their joint attention, and were compared with Gesell adaptive developmental age matched with healthy controls (n=15). Results　Adaptability age and sex ratio of the two groups showed no significant difference (P > 0.05), but physiological age showed significant difference (P < 0.05). Compared with the healthy controls with same developmental age, children with ASD have obvious defects in both initiative and responsive joint attention abilities (P <0.01). For children with ASD, their joint attention behavior and adaptability age are positively correlated (P <0.01), but initiative joint attention behavior is not correlated with adaptability age (P > 0.05). Conclusions　Children with ASD have obvious defects in both initiative and responsive joint attention abilities. With the improvement of adaptability age, the responsive joint attention ability may be increased correspondingly, while it is still difficult to improve initiative joint attention ability.

Heat shock protein 70 in liver fibrosis in infants with biliary atresia and its impact on prognosis　

　ZHAO Jinlei, FAN Xiaoge, JIN Sanding, LI Ping, ZHANG Hecheng

. 2017, 35(2):  106.  doi:10.3969/j.issn.1000-3606.2017.02.007

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Objective　To investigate the role of heat shock protein 70 (HSP70) in liver fibrosis in infants with biliary atresia (BA) and its impact on prognosis. Methods　Fourty-six (46) cases of infants with BA undergoing elective Kasai surgery were selected. In the same period, 30 cases of children with choledochal cyst and 17 cases of children with portal vein cavernous transformation were selected. The expressions of HSP70 in liver tissues were detected using immunohistochemical staining. The liver fibrosis in children with BA was detected using Sirius red-saturated picric acid staining. The expressions of HSP70 proteins in different fibrotic liver tissues were detected by using double staining. All postoperative BA infants were followed up and ended at June 30, 2016. Results　The proportion of high expression of HSP 70 proteins in BA infants were significantly higher than that in children with choledochal cyst and vein cavernous transformation (P<0.05). Rank correlation analysis showed that the expressions of HSP70 in liver tissues were positively correlated with the degree of liver fibrosis (r=0.861, P<0.05). 15 patients died among 46 cases of BA infants. The survival rate in BA children with mild liver fibrosis was 82.4%, which was significantly higher than 58.6% in the severe group (P<0.05). The survival rate in HSP70 protein low expression group was 85.0%, while in HSP70 protein high expression group was 53.8%. Kaplan-meier survival analysis showed that the median survival time in the HSP70 protein low expression group was (34.0±2.6) months, while in the HSP70 protein high expression group was (18.3±2.2) months, the difference was statistically significant (χ2=4.765, P=0.029). Conclusions　The expressions of HSP70 proteins in liver tissues in infants with BA were high and were positively correlated with the degree of hepatic fibrosis. It suggested the possible involvement of HSP70 in the process of liver fibrosis. The upregulated expressions of HSP70 often indicated poor prognosis in children. It could be used as determining biomarker for prognosis.

Pathological patterns of renal biopsy with immunofluorescence relate to the therapeutic effects of corticosteroid in childhood nephrotic syndrome

　SHAN Wenjie, GONG Yinliang, ZHU Yaju, JIN Jing, WU Weilan, CHEN Huimin, DONG Yu, LI Yufeng

. 2017, 35(2):  111.  doi:10.3969/j.issn.1000-3606.2017.02.008

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　Objective　To explore the relationship of pathological patterns of renal biopsy with immunofluorescence the therapeutic effects of corticosteroid in childhood nephrotic syndrome. Method　Renal biopsy materials and clinical data of the children diagnosed with nephrotic syndrome in the Department of Pediatric Nephrology of Shanghai Xinhua Hospital from January, 1990 to December, 2015 were retrospectively reviewed and analyzed. Results　The renal pathological patterns by immunofluorescence of 252 patients showed that IgA was presented in 13 cases (5.16%), IgM in 78 cases (30.95%), complement in 19 cases (7.54%), IgG+A+M in 17 cases(6.75%), IgG+A+M+complement in 22 cases (8.73%), IgA+complement in 2 cases (0.79%), IgM+complement in 55 cases (21.83%), and there was no immune complex present in 46 cases (18.25%). The renal pathological patterns were found that 157 (62.3%) minimal change nephropathy syndrome (MCNS), 35 (13.89%) mesangioproliferative glomerulonephritis (MsPGN), 60 (23.81%) focal and segmental glomerulosclerosis (FSGS). In the 4 weeks of oral administration of prednisone, complete remission was achieved in 167 cases (77.31%), partial remission was achieved in 31 cases (14.35%), and no remission was shown in 18 cases (8.33). There was statistically difference in the distribution of renal pathological patterns among the 8 patterns by immunofluorescence (χ2=31.308， P=0.001). The therapeutic effects of corticosteroid  on the 8 patterns by immunofluorescence have no significant difference (P>0.05). Conclusions　The distribution of renal pathological patterns among the 8 patterns of immunofluorescence is significantly different.

Red blood cell casts induced acute renal failure in IgA nephropathy: a childhood case report with literature review

ZHANG Hongwen, CUI Jieyuan, SU Baige， YAO Yong

. 2017, 35(2):  115.  doi:10.3969/j.issn.1000-3606.2017.02.009

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 Objective　To explore the rare cause of renal failure in childhood IgA nephropathy. Methods　A six year-old boy presented with recurrent gross hematuria for 3 months and increased serum creatinine for 5 days, blood and urine routine test, renal function, urinary protein concentration and renal biopsy were performed for diagnosis. Results　The boy had three episodes of recurrent gross hematuria with a predisposed respiratory tract infection, he recovered within a week after antibiotic therapy from previous two episodes, but oliguria and renal failure were occurred in the third episode. Renal biopsy showed IgA nephropathy with presence of red blood cell casts in as much as 50% of the tubular lumen and acute tubular lesion. His renal function recovered gradually to normal within 4 weeks after treatment with anti-infection, diuresis and alkalization of urine. Conclusions　This article reported the renal failure case induced by tubular damage and obstruction by red blood cell casts in childhood IgA nephropathy.

Childhood hepatolenticular degeneration combined with thin basement membrane nephropathy: a case report with literature review

Cui Jieyuan, Zhang Hongwen

. 2017, 35(2):  118.  doi:10.3969/j.issn.1000-3606.2017.02.010

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 Objective　To analyze the diagnostic approach on hepatolenticular degeneration combined with thin basement membrane nephropathy. Methods　A girl presented with microscopic hematuria, liver dysfunction and hypocomplementemia was diagnosed with hepatolenticular degeneration combined with thin basement membrane nephropathy, her clinical data were summarized and analyzed retrospectively. Results　A ten years old girl presented with microscopic hematuria and liver dysfunction for a year, dysarthria for a month, and combined with hypocomplementemia but without proteinuria. Renal biopsy showed thin basement membrane nephropathy. Ceruloplasmin was 23.10 mg/L and urinary copper concentration was 120μg, respectively,  ocular slit lamp examination showed Kayser-Fleischer ring, cranial MRI showed preternatural signal in both basal and putamen nucleus, mutation analysis showed homozygous mutations in ATP7B and heterozygous mutation in COL4A3 gene, respectively. Conclussion　Hepatolenticular degeneration should be suspected in those cases with persistence microscopic hematuria, liver dysfunction and hypocomplementemia.

Prognostic analysis of hepatoblastoma in children

YAO Dongya, LUO Yuan, SHENG Guangyao

. 2017, 35(2):  121.  doi:10.3969/j.issn.1000-3606.2017.02.011

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  Objective　To study the prognosis of  hepatoblastoma (HB) in children.　Methods　Retrospective analysis on the clinical data and follow-up of children with HB admitted from September 2010 to February 2015 and sum up the clinical outcome and prognosis. Results   Thirty-six children with HB (23 males and 13 females) were enrolled. The median age on diagnosis was 20 months. All the 36 patients received follow-up, the median follow-up time was 37 months (7～71 months).(1) Of the 36 patients, 8 died. The prognosis of patients with stage Ⅰ or Ⅱ was significantly better than those with stage Ⅲor Ⅳ (P<0.05). The prognosis of patients with fetal type was significantly better than those with other subtypes (P<0.05). (2) Serum AFP level of 35 newly diagnosed cases increased, AFP level of survivors returned to normal after treatment. Of the 8 died patients, 3 patients had AFP elevated when relapsed, 5 cases had a persistently high AFP level after adjuvant chemotherapy. The levels of LDH, CRP, and AST at diagnosis had significant influence on the prognosis (P<0.05). Conclusions　Serum AFP can be a monitoring indicator for recurrence and prognosis of hepatoblastoma.  POG / CCG staging, pathology classification are key points when assessing the prognosis of hepatoblastoma. The prognosis of patients with high levels of LDH, CRP, and AST might be worse.

Atrial flutter in infants ：treatment analysis and follow-up

WANG Yefeng, CHEN Zhi, XIAO Yunbin, ZENG Min, YANG Zhou, WANG Xiang

. 2017, 35(2):  125.  doi:10.3969/j.issn.1000-3606.2017.02.012

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Objectives　To assess treatment outcomes and prognosis in infants with atrial flutter (AFL). Methods　Thirty-four (34) cases of infants with AFL in Hunan Children’s Hospital had been analyzed for clinical features, treatment outcomes and followup between March, 2009 and September, 2015. Based on ECG characteristics, the patients had been divided into simple and complex AFL groups.  Based on age, they had been divided into neonates and non-neonates group. The aim of this study is  to compare the clinical effects of drug treatment in different types of AFL. Results　With digitalis alone, the cardioversion rate was 37.5%，no significant difference was observed between simple and complex AFL groups (45.8% vs 12.5%, P=0.206). Combining with other drugs, the cardioversion rate was 54.5%, which showed significant difference between simple and complex AFL groups (76.9% vs 22.2%，P=0.036). The overall cardioversion rate was 70.6%, which showed significant difference between simple and complex AFL groups（87.5% vs 30%, P=0.003). There was no significant difference in pharmaceutical cardioversion rate between neonates and non-neonates group (85.7% vs 60.0%, P=0.216). Two cases with symptoms of heart failure used synchronized cardioversion. One patient restored to sinus rhythm, and another case was still recurrent of AFL after repeated electrical cardioversion, and eventually died of cardiogenic shock. After treatment, 9 patients were still with paroxysmal AFL and atrial tachycardia episodes, including 3 cases of simple type and 6 cases of complex type who were discharged with oral digoxin and propafenone treatment at home. 24 patients were followed up (3 months to 3 years and 4 months). 16 cases restored to sinus rhythm during hospitalization had no recurrence of AFL. Conclusions　The overall treatment effects of AFL in infants were good. In simple type of AFL, most of patients did not need long-term antiarrhythmic drug therapy and the prognosis was good. The prognosis of treatment with conventional drug was poor in complex AFL group, with a higher rate of recurrence of AFL.

ATP1A3 mutations in childhood alternating hemiplegia: a report of two cases with literature review

ZHANG Ting, MA Jiannan, XIAO Nong

. 2017, 35(2):  129.  doi:10.3969/j.issn.1000-3606.2017.02.013

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 Objective　To explore clinical manifestations, genetic diagnosis and treatment of childhood alternating hemiplegia. Methods　Two patients were clinically diagnosed as alternating hemiplegia. ATP1A3 gene sequencing was performed on these two children and their parents. Literatures on childhood alternating hemiplegia were reviewed. Results　Both patients were female. The first symptom of the first girl was alternating hemiplegia occurring at 4-month-old. For the second girl, the first symptom was seizure occurred at 6-month-old, and the typical symptoms including alternating hemiplegia occurred at the second year of course. Heterozygous missense mutations of c.2401G>A (p.D801N) and c.2731G>C (p.A911P) were found in ATP1A3 gene of these two girls, the latter hasn’t been reported in the Human Gene Mutation Database (HGMD) Professional. Conclusions　ATP1A3 gene sequencing should be conducted for children clinically diagnosed as alternating hemiplegia, which has important significance for diagnosis and genetic counseling.

Role of SCN1A gene variations in the development of familial febrile seizures

GUO Jiacheng，ZHAO Wu

. 2017, 35(2):  133.  doi:10.3969/j.issn.1000-3606.2017.02.014

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Objective　To determine the role of SCN1A gene variation in the development of familial febrile seizures （FS） . Methods　Clinical data were collected from 8 familial FS pedigrees, and peripheral venous blood samples were collected from the probands and other available family members. All 26 coding exons and exon-intron boundaries at least 50 bases of the human SCN1A gene were amplified by polymerase chain reaction, the products were subsequently sequenced. To novo variation, other family members were screened for the corresponding exons. Two hundred age-matched healthy children were served as normal controls. Results　A total of 33 variations in the SCN1A gene were identified in these families. Of these variations, one was a missense mutation; the remaining 32 variations were previously submitted as single nucleotide polymorphisms (SNPs). A c.2650G>A heterozygous missense mutation in exon 15 of the SCN1A gene found in the proband of family 4 was inherited from his father who had seizures with fever in early childhood. The c.2650G>A mutation was absent in the 400 alleles of normal controls. To the best of our knowledge, the SCN1A c.2650G>A mutation has neither been reported in the NCBI SNP database nor in the literature to date. The c.2650G>A mutation changes a glycine at amino acid 884 in the SCN1A protein to a serine (p.Gly884Ser). Protein sequence analysis showed that the p.Gly884Ser is located at a highly conserved region between the 4th and 5th transmembrane segment of the homologous domain II of voltage-gated sodium channel 1 subunit (DIIS4-S5). Conclusions　The pathogenesis of familial febrile seizures was related to the SCN1A variation, the mutation outside the region of the voltage sensor (S4) and ion channel pore (S5-S6) of the voltage gated sodium channel α-subunit may be an important factor to cause mild phenotype epilepsy syndrome.

Hydrogen sulfide protects intestinal mucosa in a neonatal rat model of necrotizing enterocolitis by upregulating the expression of HO-1

ZENG Zhaojun, ZHONG Sen, WANG Jianing, TANG Junming, ZHANG Lei, WANG Jintang, ZHAO Yang

. 2017, 35(2):  138.  doi:10.3969/j.issn.1000-3606.2017.02.015

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Objective　To explore the protective effects of GYY4137, a new hydrogen sulfide donor, on intestinal mucosa in a neonatal rat model of necrotizing enterocolitis (NEC), and its potential mechanism. Methods　Sixty SD rats were randomly assigned into 4 groups: group A (control group), group B (NEC group), group C (NEC with GYY4137 treatment, H2S donor group), and group D (NEC with GYY4137 and Znpptreatment, HO-1 inhibitor group). The SD rat models of NEC were established using simulated milk feeding-hypoxia-cold stress-Lipopolysaccharides. The injury degree of intestinal mucosa was evaluated using HE-staining, and its mechanisms were investigated using biochemical indicators and Western blotting. Results　Compared with control group, the pathology score and the total superoxide dismutase (T-SOD) in the NEC group was significantly higher, the concentrations of methane dicarboxylic aldehyde (MDA) and necrosis factor α (TNF-α) were lower（P<0.05）. Compared with those in NEC group, the pathology score and the concentration of MDA and TNF-α in the H2S donor group were significantly lower, the T-SOD, and the HO-1 expression was higher. The pathology score and the level of MDA and TNF-α were significantly increased after treated with HO-1 inhibitor Znpp, and T-SOD was significantly decreased.. Conclusions　The GYY4137, as a new H2S donor, could attenuate the injury of intestinal mucosa in a neonatal rat model of NEC by upregulating the expression of HO-1.

Progress on roles of type 2 innate lymphoid cells in the pathogenesis of asthma

JIANG Qin

. 2017, 35(2):  148.  doi:10.3969/j.issn.1000-3606.2017.02.017

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Type 2 innate lymphoid cell (ILC2) is a new member of the innate lymphoid cell family discovered recently. After being exposed to antigens, ILC2 was activated by epithelial cell-derived cytokines, lipid mediators and TNF family member TL1A to promote structural and immune cell responses in the airways. These cells are critical components of the innate immune response involved in the pathogenesis of asthma. This paper summarizes the progress on the roles of type 2 innate lymphoid cells in the pathogenesis of asthma.

The latest development in diagnosis and treatment of intestinal neuronal dysplasia

XIE Jingjing

. 2017, 35(2):  153.  doi:10.3969/j.issn.1000-3606.2017.02.018

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Intestinal neuronal dysplasia is a common disease of chronic constipation in children, which is one of the Hirschsprung disease allied disorders. In recent years, the diagnostic methods of intestinal neuronal dysplasia have been improved, but diagnostic criteria are not standardized yet. The treatments include conservative treatment and surgical treatment. Special attention should be paid to save the integrity of the anal canal during operation. Moreover, using intestinal transplantation of neural stem cells and small intestinal transplantation to treat intestinal neuronal dysplasia has gained increasing attention.