Table of Content

15 October 2018 Volume 36 Issue 10

  

Effects of obesity on lung function in mild-to-moderate allergic and nonallergic asthmatic children

LIANG Fanmei, HUANG Ying, ZHANG Xueli, XU Xiaowen, WANG Jian, PENG Jiayu

. 2018, 36(10):  729.  doi:10.3969/j.issn.1000-3606.2018.10.001

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　Objective　To explore the effects of obesity on basic lung function and airway hyperresponsiveness (AHR) in children with mild-to-moderate allergic and non-allergic asthma. Methods　According to the skin prick test (SPT), 230 children with mild-to-moderate asthma were divided into allergic group and non-allergic group. Furthermore, based on different body mass index (BMI) Z score, they were also divided into allergic normal body mass group, allergic overweight group, allergic obese group, nonallergic normal body mass group, nonallergic overweight group and nonallergic obese group. The lung function and bronchial provocation test of each group were detected and compared. Results　There were significant differences in peak expiratory flow (PEF), maximal expiratory flows at the 75% of vital capacity (MEF75), MEF50, MEF25, and maximal midexpiratory flow (MMEF) among different groups of mild-to-moderate asthmatic children, and the levels of all above markers were the lowest in nonallergic obese group, followed by nonallergic overweight group (P<0.05). There was no correlation between the BMI-Z score and the indexes of allergic asthma (P>0.05). The pulmonary function indexes (PEF, MEF75, MEF50, MEF25 and MMEF) of nonallergic asthma children were negatively correlated with BMI-Z score (r=-0.314~-0.273, P all <0.01). There was no significant difference in the incidence rate of different grades of AHR among different groups (P>0.05). After bronchial provocation test, the rates of decrease in PEF, MEF75, MEF25 and MMEF were significantly different among the groups (P<0.05), and the lowest rate was found in the nonallergic obese group. Conclusion　Obesity has a greater impact on lung function in nonallergic asthma children, and mainly affects ventilation function.

Risk factors for severe complications caused by digestive tract foreign bodies in children

　GU Zhujun, LIU Haifeng, CHENG Weiwei, LIN Kai, WANG Xing, WANG Ling, HU Zhihong

. 2018, 36(10):  734.  doi:10.3969/j.issn.1000-3606.2018.10.002

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Objective　To explore the risk factors of serious complications caused by foreign bodies in digestive tract in children. Method　The clinical data of 285 children under 12 years old with foreign bodies in digestive tract admitted from November 2012 to November 2017 were retrospectively analyzed. According to the severity of complications caused by foreign bodies in digestive tract, the selected children were divided into risk group and general group for analysis and comparison. Results Among 285 children (182 males,103 females) the types of foreign bodies included coins, batteries, magnetic foreign bodies, hair, pins, screws, and so on. There were 42 cases in risk group and 243 cases in general group. There were significant differences in the percentage of esophageal foreign body retention time >24 hours, the shape of foreign bodies, the types of foreign bodies and the retention sites of foreign bodies in digestive tract between the two groups (P<0.01). These factors mentioned above were risk factors for serious complications. Conclusion　The serious complications of foreign bodies in children’s digestive tract can be reduced by correctly judging risk factors, choosing proper treatment methods and proper treatment timing.

Clinical analysis of colorectal polyps in 670 children

MAO Kangwei, LIAN Min, LIU Zhifeng, PAN Jian, ZHENG Bixia, YAO Yuqian, LI Dalei

. 2018, 36(10):  738.  doi:10.3969/j.issn.1000-3606.2018.10.003

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Objective　To explore the clinical features of colorectal polyps in children and the therapeutic value of endoscopic mucosal resection (EMR). Method　The clinical data of 670 children with colorectal polyps who underwent EMR immediately after the diagnosis from 2012 to 2017 were retrospectively analyzed. Results　Among 670 children (423 males, 232 females), the average diagnosis age was 4.50±2.69 years old, with high incidence at the age of 3 to 6 years (43.4%). The clinical presentation was dominated by hemafecia (90.3%). Most of the polyps were solitary (92.5%), mostly in the rectum (49.0%) and sigmoid colon (35.2%) with diameter <2 cm (78.9%). For polyps with diameter ≥2 cm, titanium clips should be used to close the wounds, while for those with diameter <2 cm, titanium clips have no effect on postoperative bleeding. Postoperative bleeding mainly occurred on the first day after operation. Postoperative pathological findings suggested that juvenile polyp was predominant. Conclusions　Colorectal polyps are most common in preschool boys and most of them are juvenile polyps. EMR supplemented with metal titanium clips can reduce postoperative bleeding.

Clinical characteristics and drug sensitivity analysis of invasive Salmonella infection in children

　WANG Hongmei, LEI Min, HUANG Baoxing, ZHOU Gaofeng, LEI Yanling, TAN Kun, DENG Jikui

. 2018, 36(10):  741.  doi:10.3969/j.issn.1000-3606.2018.10.004

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Objective　To explore the pathogen serotype distribution and drug resistance characteristics of invasive Salmonella infection in children. Method　The clinical characteristics, serotype distribution and drug sensitivity of 66 children with invasive Salmonella infection confirmed by blood or pus culture from January 2012 to December 2015 were retrospectively analyzed. Results　Among 66 children (48 males and 18 females) included, there were 60 hospitalized and 6 outpatient children, and 43 were under 2 years old. Strains were mainly found from May to October (80.30%, 53/66). The main clinical manifestations of hospitalized children were fever (55 cases, 91.67%), abdominal pain and / or diarrhea (32 cases, 53.33%), anemia (36 cases, 60.00%), cough (28 cases, 46.67%), hepatomegaly (24 cases, 40.00%) and splenomegaly (12 cases, 20.00%). Among all the children, 45 (68.18%) had increased white blood cell counts, 56 (84.85%) had decreased proportion of eosinophils (eosinophils disappeared in 15 cases), and 51 (77.27%) had increased level of hypersensitive C-reactive protein. Salmonella serotypes were mainly group D and group B, accounting for 39.39% (26 cases) and 33.33% (22 cases) respectively. The invasive Salmonella strains resistant to imipenem and ertapenem were not detected, and the sensitivities of the detected strains to cefepime, ceftazidime, ceftriaxone and aztreonam were all >70.00%. Conclusion　Invasive Salmonella infection is most common in children under 2 years of age, with prevalence from May to October. Salmonella serotypes were mainly group D and group B, and the clinical manifestations are diverse. Antibiotic treatment should be carefully selected in combination with serotype and drug susceptibility results.

Holocarboxylase synthetase deficiency: a case report

YIN Zhiping, WANG Yizhong, ZHANG Ting, XIAO Yongmei

. 2018, 36(10):  745.  doi:10.3969/j.issn.1000-3606.2018.10.005

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Objective　To explore the clinical manifestations, diagnosis, treatment and prognosis of holocarboxylase synthetase deficiency (HLCSD) in children. Method　The clinical manifestations and laboratory tests results in a child with HLCSD were retrospectively analyzed. Genetic mutations in peripheral blood of the child and her family members were analyzed by second-generation gene sequencing. Results　The girl presented with recurrent refractory rash and the age at onset was 4 months. Humoral immunity showed a decrease in the level of total immunoglobulins. Blood tandem mass spectrometry showed a marked increase in the level of 3-hydroxy-isovalyl carnitine. Urine gas chromatography mass spectrometry showed a significant increase in lactic acid, 3-hydroxy-isovaleric acid and pyruvic acid. The second-generation gene sequencing revealed that there were heterozygous mutations of c.1544G>A (S515N) and c.1993C>T (R665X) in exon 9 and 11 of the HLCS gene in the child, and R665X was a new mutation. His parents are genetic mutation carriers. Conclusions　The clinical manifestations of HLCSD are atypical. For children having refractory rash with neurological abnormalities, growth and development disorders, persistent acidosis, hyperlactacidemia and other manifestations, HLCSD should be suspected, and the diagnosis can be confirmed early by blood tandem mass spectrometry, urine gas chromatography mass spectrometry and gene screening.

Evaluation of myocardial damage by ventricular global longitudinal strain, ESR, and CRP  in the acute stage of Kawasaki disease in children

　PANG Gaofeng, XU Min, GONG Mingxia, LIU Fei, MENG Jun, GE Zhixiang, YANG Ling

. 2018, 36(10):  748.  doi:10.3969/j.issn.1000-3606.2018.10.006

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Objective　To explore the value of left ventricular myocardial global longitudinal strain (GLS) obtained by two-dimensional speckle tracking echocardiography (STE) in the assessment of acute myocardial injury in children with Kawasaki disease (KD) and its relationship with c-reactive protein (CRP) and erythrocyte sedimentation rate (ESR). Method The clinical data of 68 hospitalized children with acute KD from January 2010 to January 2017 were retrospectively analyzed. According to the presence or absence of coronary artery injury, KD children were divided into non-coronary artery injury group (53 cases) and coronary artery injury group (15 cases). The Simpon method was used to measure the left ventricular ejection fraction (LVEF), and QLA10.0 software was used to track the collected myocardial images and obtain GLS to analyze the myocardial movement. Results　of CRP and ESR in acute phase of KD children were collected and compared with those of 56 healthy children. Correlation between inflammatory indexes and GLS in children with KD was analyzed. Results The levels of GLS, CRP and ESR in children with acute KD were higher than those in healthy controls, and there were significant differences (P all<0.01). There was no difference in LVEF between the two groups (P>0.05). The levels of CRP and ESR in non-coronary artery injury group were significantly higher than those in coronary artery injury group, and there were significant differences (P all<0.01). There was no difference in LVEF and GLS between the two groups (P>0.05). In acute phase of KD, GLS was positively correlated with CRP and ESR (r=0.62, 0.57; P all<0.05). Conclusion　The GLS obtained by STE was significantly elevated in the acute phase of KD and was associated with the increased CRP, but was not related to coronary artery injury.

Analysis of risk factors of severe hand, foot, and mouth disease complicated with cardiopulmonary failure

LI Xiaonian

. 2018, 36(10):  752.  doi:10.3969/j.issn.1000-3606.2018.10.007

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Objective　To explore the risk factors of severe hand, foot, and mouth disease (HFMD) complicated with cardiopulmonary failure. Methods　The clinical data of 179 children with severe HFMD admitted from December 2011 to December 2016 were retrospectively analyzed. According to whether the children were complicated with cardiopulmonary failure, they were divided into cardiopulmonary failure group (33 cases) and non-cardiopulmonary failure group (146 cases). Univariate analysis and multivariate logistic regression analysis were used to analyze the risk factors for severe HFMD with cardiopulmonary failure. Results　Univariate analysis revealed that there were significant differences between two groups in sex composition, tremor, disturbance of consciousness, irregular respiratory rhythm, limb weakness, slow heart rate, high blood pressure, peripheral circulation disorders, peripheral blood leukocyte count, pharyngeal reflex, frequent vomiting, abnormal cerebrospinal fluid leukocyte count, increased brain natriuretic peptide (BNP) levels, EV-71 infection, increased levels of S100 beta protein, neuronspecific enolase and troponin. Multivariate logistic regression analysis showed that frequent vomiting, peripheral circulation disorder, enterovirus EV-71 infection, irregular respiratory rhythm, and elevated BNP levels were five independent risk factors of severe HFMD with cardiopulmonary failure. Conclusions　Frequent vomiting, circulatory disturbances, EV-71 infection, respiratory rhythm dysfunction, and elevated BNP levels are indicative of severe HFMD complicated with cardiopulmonary failure.

Mutation analysis of CYP21A2 gene in children with 21-hydroxylase deficiency in Henan

　YANG Haihua, WANG Huizhen, CHEN Yongxing, WANG Xiaohong, YANG Wei, CHEN Qiong, SHEN Linghua, LIU Xiaojing, WEI Haiyan

. 2018, 36(10):  756.  doi:10.3969/j.issn.1000-3606.2018.10.008

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Objective　To explore the genetic characteristics of 21 hydroxylase deficiency (21-OHD) in Henan area. Methods　The genetic test results of 21-OHD children and their families of Han nationality originally from Henan province were retrospectively analyzed from 2009 to 2016. Sanger sequencing and multiplex ligation-dependent probe amplification (MLPA) methods were performed to detect mutations in CYP21A2 gene. For children with negative mutation of CYP21A2 gene, next generation sequencing (NGS) was performed to confirm the diagnosis. Results　A total of 55 patients from 54 families with a disease course of one month to 8 years were studied. There were 49 cases (37 boys and 12 girls) of clinically diagnosed salt wasting (SW) type and 6 cases (3 boys and 3 girls) of simple virilizing (SV) type. The CYP21A2 gene mutation was detected in 46 children, and the most frequent mutation identified was c.293-13A/C>G (I2G), followed by partial or complete deletion of the gene, c.955G>T (p.Q319X), c.515T>A (p.I172N) and c.1069G>T (p.R357W). Among 9 children with negative CYP21A2, there were 7 children whose diagnosis was corrected to congenital adrenal dysplasia due to positive NROB1 (DAX1) mutations detected by NGS analysis. Conclusions　The most common mutation type of CYP21A2 in children diagnosed with 21-OHD clinically in Henan is c. 293-13a/c >G (I2G), followed by the large fragment deletion of the gene.

Analysis of TTN gene mutation in familial dilated cardiomyopathy in children

　CUI Qingyang, LU Jun

. 2018, 36(10):  761.  doi:10.3969/j.issn.1000-3606.2018.10.009

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　Objective　To explore the characteristics of TTN gene mutation in familial dilated cardiomyopathy. Method The blood samples were collected from a 6-year-old boy with  confirmed familial dilated cardiomyopathy and his mother who had familial dilated cardiomyopathy with familial dilated cardiomyopathy, as well as his father, grandparents and his maternal grandmothers without who didn’t have familial dilated cardiomyopathy history, and genomic DNA was extracted. Nextgeneration DNA sequencing (NGS) was used to detect 213 cardiovascular disease-related genes in the child, and the suspicious sites were identified by bioinformatics analysis and then verified by Sanger sequencing. Results　Three missense mutations of TTN gene on chromosome 2, c.53498A>T (p.Tyr17833Phe), c.66590G>A (p.Arg22197Gln) and c.8434G>C (p.Val2812Leu), were carried by the child. Each of the above mutation could affect the functional expression of the protein. Conclusion　This is the first time to find three variants of c.53498A>T, c.66590G>A and c.8434G>C in TTN gene.

Predictive analysis of intravenous immunoglobulin unresponsive Kawasaki disease

　XIAO Lan, ZHANG Jing, YI Lunyu, QIU Ling, YANG Ying, YE Xiaochun

. 2018, 36(10):  765.  doi:10.3969/j.issn.1000-3606.2018.10.010

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　Objective　To evaluate the value of different scoring systems in the prediction of intravenous immunoglobulin (IVIG) unresponsive Kawasaki disease in Chinese children, and to explore the risk factors of IVIG unresponsive Kawasaki disease, so as to establish a predictive scoring system for IVIG unresponsive Kawasaki disease in Chinese children. Method　The clinical data of 1 655 children with Kawasaki disease in China were analyzed retrospectively. The Kobayashi score, Egami score, Sano score, Fu score, Formosa score and Tang score were used to predict IVIG unresponsiveness of patients and their predictive capability were evaluated. Univariate analysis, logistic regression analysis and receiver operating characteristic curve (ROC) were used to analyze the independent risk factors of Kawasaki disease unresponsive to initial IVIG treatment in Chinese children, and a new predictive scoring system was attempted to establish. Results　The incidence of IVIG unresponsive Kawasaki disease is higher in patients with lower platelets and serum sodium, higher percentage of neutrophils and total bilirubin (P<0.05). The above four risk factors were used to establish a new predictive scoring system, and the score ≥2 points suggested IVIG unresponsive Kawasaki disease. The new predictive scoring system have a sensitivity of 70.4%, a specificity of 72.7%, and an area under the ROC curve of 0.767. Conclusion　Platelets, percentage of neutrophils, total bilirubin, and serum sodium were associated with IVIG unresponsive Kawasaki disease. The new scoring system established in this study has high sensitivity and specificity, but its predictive efficacy in other regions and populations needs further confirmation.

The correlation of D-Dimer, N-terminal pro-brain natriuretic peptide and inflammatory markers with the prognosis of severe pneumonia in children

HUANG Caizhi, YANG Longgui, ZHANG Cong, ZHOU Zhou, MO Liya

. 2018, 36(10):  772.  doi:10.3969/j.issn.1000-3606.2018.10.011

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Objective　To explore the correlation of D-Dimer (DD), N-terminal pro-brain natriuretic peptide (NT-ProBNP), inflammatory markers such as procalcitonin (PCT), interleukin (IL-6), white blood cell count (WBC) and C-reactive protein (CRP) with the prognosis of severe pneumonia in children. Methods　A total of 107 children with severe pneumonia were divided into non-mechanical ventilation group and mechanical ventilation group according to whether they needed mechanical ventilation. Mechanical ventilation group was further divided into non-invasive ventilation group and invasive ventilation group. According to the intensive care time, the patients were divided into intensive care time ≤7 days group and intensive care time >7 days group. According to the outcome at discharge, the patients were divided into survival group and death group. The levels of DD, NTProBNP, PCT, IL-6, WBC and CRP were compared and analyzed among different groups, and the indexes were compared and evaluated by binary logistic regression analysis and receiver operating characteristic (ROC) curve. Results　The levels of DD, NTProBNP, PCT and IL-6 in mechanical ventilation group, invasive ventilation group, intensive care time >7 days group and death group were significantly higher than those in non-mechanical ventilation group, non-invasive ventilation group and intensive care time ≤7 days group and survival group (P all < 0.05), but there was no difference in WBC and CRP among those groups (P all > 0.05). Multivariate logistic regression analysis showed that DD had statistical significance in risk assessment of death in children with severe pneumonia (OR=2.72, 95%CI: 1.32~5.60, P<0.01). ROC analysis showed that the maximum area under the curve used to predict the survival of severe pneumonia in children was DD (0.89), followed by IL-6 (0.85), PCT (0.84) and NT-ProBNP (0.80). When combining DD, NT-ProBNP, PCT and IL-6, the area under the curve was 0.91, with the diagnostic sensitivity of 0.91 and the specificity of 0.85. Conclusions　DD, NT-ProBNP, PCT and IL-6 have certain clinical values in assessing the severity of severe pneumonia and its prognosis in children. The combination of four indicators can improve the predictive ability of survival. DD may be an independent biological indicator to predict the risk of death in children with severe pneumonia.

Anti-neutrophil cytoplasmic antibody-related vasculitis in children : a report of 3 cases and  literature review

　SU Hui, SONG Xiaoxiang, CHENG Jiang, MIN Yue, FENG Qihua

. 2018, 36(10):  777.  doi:10.3969/j.issn.1000-3606.2018.10.012

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Objective　To explore the clinical features, diagnosis and treatment of Anti-neutrophil cytoplasmic antibody associated vasculitis (ANCA) in children, and raise awareness of the disease. Methods　The clinical data of 3 children with ANCA associated vasculitis were retrospectively analyzed from 2013 to 2017 in the hospital. Results　Of the 3 cases , 2 were girls and 1 boys . One case was positive for MPO, one case was positive for P-ANCA, one case was negative for P-ANCA after one year of treatment, and the other two cases were negative for ANCA related antibodies because of pulmonary infection. The initial performance of 3 cases was different. 2 cases had different degrees of renal injury, 2 cases had different degrees of lung injury, 1 case had skin involvement, and 1 case had liver involvement. In case 2 , after the therapy of glucocorticoid combined with cyclophosphamide, the patient had improved significantly. However, the patient was automatically discharged from hospital due to recurrence of infection and economic problems during the course of the disease. Cases 1 and 3 were treated with hormones, support, etc., and their condition improved and discharged. Conclusions　The first clinical manifestation of ANCArelated vasculitis in children vary widely, and when multiple organs such as kidney and lungs are damaged , we should be alert to ANCA-related vasculitis , and early diagnosis and early treatment can improve their prognosis .

Clinical and genetic characteristics of congenital disorders of glycosylation type Id: a case report

WANG Dan, HE Yan, GUO Hu

. 2018, 36(10):  781.  doi:10.3969/j.issn.1000-3606.2018.10.013

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Objective　To explore the clinical and genetic characteristics of congenital disorders of glycosylation type Id (CDG-Id). Method　The clinical data and genetic test results of a case of CDG-Id were retrospectively analyzed and related literature were reviewed. Results　A male infant began to have convulsions 2 months after birth, with motor development retardation and low muscle strength and low muscle tension. Brain magnetic resonance imaging showed widened bilateral extracranial space in frontotemporal region and the full bilateral lateral ventricles. Genetic sequencing revealed that there were two heterozygous mutations in the ALG3 gene, C. 494A > G (p. His165Arg) and C. 33del (p. Gly12fs), so the infant was diagnosed of CDG-Id. Conclusions　CDG-Id, a rare type of CDG, is an autosomal recessive inherited disease. The neurological symptoms are the most prominent manifestation. The ALG3 gene detection is helpful for diagnosis.

Chediak-Higashi syndrome: a case report

TANG Fajuan, LI Xihong, PAN Lingli

. 2018, 36(10):  784.  doi:10.3969/j.issn.1000-3606.2018.10.014

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Objective　To explore the clinical features and prognosis of Chediak-Higashi syndrome. Method　The clinical data of Chediak-Higashi syndrome in a child were retrospectively analyzed and the related literature were reviewed. Results　A one-yearold boy presented with fever, localized albinism, hepatosplenomegaly and panhematopenia. Blood smear showed the inclusion body in some neutrophils and lymphocytes, and multiple inclusion bodies were observed in a neutrophil. Bone marrow examination revealed large abnormal granules in the cytoplasm of some neutrophils. Genetic testing revealed that the LYST gene had a homozygous mutation, c.2311C>T (the nucleotide number 2311 in the coding region changed from cytosine to thymine), resulting in the change in amino acid (p.Q771X, nonsense mutation). The inheritance pattern was autosomal recessive inheritance, and the disease phenotype was congenital leukocyte granular abnormal syndrome. Conclusion　Chediak-Higashi syndrome is a rare autosomal recessive hereditary disease with a poor prognosis. The diagnosis relies mainly on bone marrow examination and genetic testing.

Clinical and genetic analysis of COPA syndrome in one case

HE Tingyan, QI Zhongxiang, LUO Shuli, XIA Yu,LI Chengrong, HUANG Ying, YANG Jun

. 2018, 36(10):  787.  doi:10.3969/j.issn.1000-3606.2018.10.015

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　Objective　To investigate clinical features, immunologic and genetic characteristics of COPA syndrome. Method　Characteristics of clinical materials, immunological data and gene mutation of the first case in China with COPα gene mutation were retrospectively analyzed. Result　A 10-year-3-month old girl was admitted due to mental deterioration for half a month and edema for six days. She looked a little pale. The blood pressure was 170/98 mmHg. There were scattered bluish purple ecchymosis in her extremities and obvious edema in her face and lower limbs. Abdominal distension and positive mobile dullness were found. Urinary analysis revealed of positive urine protein and red blood cells. Rheumatoid factor, antinuclear antibody and anti-double-stranded DNA antibody were positive. Anti-Sm antibody was negative. CT images showed lesions of left lower lung lobe and a small amount of pleural and pericardial effusion. MRI revealed of effusion and partial synovial thickening of left ankle and foot joints. Renal pathology showed moderate-to-severe diffuse hyperplasia of glomerular mesangial cells with endothelial cell hyperplasia. IgG deposited at glomus, capillary loops, and mesangial area. The gene sequencing results showed COPα gene c.433C>T,p.P145S heterozygous mutation. Flow cytometry detection deployed a significantly increased Th17 cell ratio (6.3%). Conclusions　The main clinical features of COPA syndrome include lung lesions, arthritis, renal damage and positive autoantibodies . Th17 cell ratio is significantly increased. Gene sequencing analysis shows pathogenic variation of COPα gene.

The efficacy of tacrolimus and cyclosporine A in the treatment of refractory nephrotic syndrome: a meta-analysis

WEN Min, DANG Xiqiang

. 2018, 36(10):  791.  doi:10.3969/j.issn.1000-3606.2018.10.016

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　Objective　To explore the efficacy of tacrolimus (TAC) and cyclosporine A (CsA) in the treatment of refractory nephrotic syndrome (RNS). Methods　The databases of PubMed, Cochrane Library, Wanfang, VIP, CNKI were searched to retrieve randomized controlled clinical trials published from January 2000 to June 2017. Meta-analysis was performed using RevMan 5.3 software using complete remission rate, overall remission rate and recurrence rate as indicators. Results　A total of 426 articles were retrieved. According to inclusion and exclusion criteria, 6 articles were finally included with 302 RNS patients in total. Meta-analysis showed that there were significant differences in complete remission rate (RR=2.01, 95%CI: 1.15~3.51, Z=2.44, P=0.01) and total remission rate (RR=2.53, 95%CI: 1.10~5.83, Z=2.19, P=0.03) while there was no difference in recurrence rate (RR=0.62, 95%CI: 0.30~1.27, Z=1.31, P=0.19) between TAC group and CsA group. Conclusion　Compared with CsA, TAC has certain advantage in improving the complete remission rate and total remission rate of RNS.

Research progress in Kawasaki disease complicated with macrophage activation syndrome

ZHOU Libing

. 2018, 36(10):  796.  doi:10.3969/j.issn.1000-3606.2018.10.017

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Macrophage activation syndrome (MAS) is a rare complication of Kawasaki disease and more attention is needed for it in clinic because of its insidious onset, rapid progression, multiple organ system involvement and high mortality. There is no international unified diagnostic criteria and treatment regimen for Kawasaki disease complicated with macrophage activation syndrome at present, so it is often missed or misdiagnosed. This article reviews the epidemiology, immunological mechanism, diagnosis and treatment of Kawasaki disease complicated with macrophage activation syndrome.

Research progress in very early onset inflammatory bowel disease

HU Menglong

. 2018, 36(10):  801.  doi:10.3969/j.issn.1000-3606.2018.10.018

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Very early onset inflammatory bowel disease (veo-IBD) is an IBD that is developed and diagnosed before the age of 6 years. IBD is an inflammatory reaction of uncontrollable intestinal epithelial cells caused by autoimmune disorder which is resulted from pathogenic factors such as intestinal commensal bacteria and pathogens stimulated by environmental factors. In recent years, the incidence and the prevalence of veo-IBD have continued to rise, and the diagnosis of ulcerative colitis is more common and it is more serious condition and has longer course of the disease compared to older-onset IBD. For veo-IBD, in addition to early onset, recurrent abdominal pain, diarrhea, mucous bloody stool, growth retardation and severe perianal diseases, different pathogenic genes can cause different clinical manifestations. The article reviews the trend of veo-IBD in recent years, the main pathogenesis, clinical manifestations, diagnosis and treatment in veo-IBD.