Table of Content

15 December 2014 Volume 32 Issue 12

  

Analysis of the mutations of SMPD1 gene in Niemann-Pick disease

LI Zhenbiao, LUO Qiang, SHI Dandan, ZHANG Jiyao, DONG Wei, WANG Huaili

. 2014, 32(12):  1101.  doi:10.3969 j.issn.1000-3606.2014.12.001

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Objectives　To study the molecular genetics of Niemann-Pick's disease (NPD), and its implication in the diagnosis of NPD. Methods The clinical data and blood samples of three unrelated families were collected. The genomic DNA was extracted from peripheral blood. The six coding exons and their flanking intronic sequences of SMPD1 gene in all members of three pedigrees were amplified by polymerase chain reaction (PCR). The SMPD1 gene sequencing results were compared with the normal sequence from Genbank to identify possible causative mutations. The amplification products of exons where mutations were located were cloned into TA vector for further confirmation. Results Family 1 proband had homozygous T107C mutation and the parents had heterozygous T107C mutation. The homozygous delete mutation (c.108-113delGCTGGC) was detected and confirmed by TA cloning in all members of family 2 and 3. The 20 normal control members did not have this delete mutation. Conclusions The genetic basis of NPD in the proband of family 1 is the homozygous T107C mutation in SMPD1 gene, while parents in family 1 are carriers of recessive T107C mutation. The homozygous mutation c.108-113delGCTGGC exists in SMPD1 gene in all members of the family 2 and 3. This delete mutation is considered to be genetic polymorphism.

Clinical and genetic features of three patients with non-classical isovaleric aciduria

　LI Xiyuan, HUA Ying, DING Yuan, WU Tongfei, SONG Jinqing, LIU Yupeng, WANG Qiao, ZHANG Yao, LI Mengqiu, QIN Yaping, YANG Yanling

. 2014, 32(12):  1107.  doi:10.3969 j.issn.1000-3606.2014.12.002

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Objective　To explore the clinical, therapeutic and genetic features of IVD gene in late-onset non-classical isovaleric aciduria. Methods One boy and two girls presented with intractable vomiting were admitted. Urine organic acids and blood acylcarnitines profiles were analyzed. Isovaleric aciduria was diagnosed and confirmed by IVD gene analysis. The patients were treated with leucine-restricted diet and the supplements of L-carnitine and glycine. Results Three patients had recurrent vomiting, drowsiness, odor of sweaty feet and metabolic acidosis from the age of 1 to 2 years. All of them had normal intelligence and leukopenia. One had oligocythemia. The blood isovalerylcarnitines (4.6 to 8.2 μmol/L) and urine isovalerylglycines (36.1 to 1783.56 mmol/mmol creatinine) were elevated. Six mutations were found in their IVD gene. Four mutations (c.157C>T, c.214G>A, c.1183C>G and c.1208A>G) were reported. Two (c.1039G>A and c.1076A>G) were novel. The patients completely recovered after treatment with protein-restricted diet and the supplements of L-carnitine and glycine. Currently, they were aged 19 months to 14 years with normal physical and psychomotor development. Conclusions The clinical features of late-onset non-classical isovaleric aciduria are complex. It is onset in infants and young children and characteristic of recurrent vomiting and metabolic acidosis, which can be diagnosed by the blood acylcarnitine spectrum, urine organic acid analysis, and confirmed by genetic analysis. L-carnitine supplement and diet intervention has significant effects.

A case of neonatal death due to argininosuccinic aciduria

DING Yuan, MA Yanyan, Wu Tongfei, LI Xiyuan, LIU Yupeng, WANG Qiao, YANG Yanling

. 2014, 32(12):  1112.  doi:10.3969 j.issn.1000-3606.2014.12.003

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Objectives　To report the first Chinese case of early onset argininosuccinic aciduria. Methods A girl aged three days was admitted because of vomiting and lethargy from the second day of life. General laboratory examination, blood amino acids analysis, urine organic acids tests and gene studies were performed for the diagnosis. Results Severe hyperammonemia, liver dysfunction, metabolic acidosis, hypokalemia and hypocalcemia were found. Bood citrulline was extremely elevated (1098.12 μmol/L vs normal range 5 to 25 μmol/L), while blood arginine was decreased. Urine orotic acid, uracil and argininosuccinic acid were significantly elevated. Two known heterozygosis mutations on ASL gene, c.544C>T (p.R182X) and c.706C>T (p.R236W), confirmed the diagnosis of argininosuccinic aciduria. Unfortunately, protein-restricted diet with L-arginine supplement showed no effect. The patient died at the 23th day of life. Conclusions Argininosuccinic aciduria is a severe inherited metabolic disorder. Clinical diagnosis is difficult. It is characterized biochemically by severe citrullinemia. Urine organic acids analysis and ASL gene analysis are important for the differential diagnosis. In this study, a case of neonate death due to early-onset argininosuccinic aciduria was diagnosed by post-mortem investigation. ASL gene study is helpful for the genetic counseling and prenatal diagnosis of the disease.

The prognosis of neonatal hyperthyrotropinemia

　WU Wanliang, PAN Jianping, LI Fengxia

. 2014, 32(12):  1116.  doi:10.3969 j.issn.1000-3606.2014.12.004

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Objective　To study the prognosis of neonatal hyperthyrotropinemia (HT). Methods Children with HT were followed up regularly to evaluate the growth index, thyroid function and effectiveness of treatment. Results One hundred and ninety-one neonates with serum TSH level of 5.6~10 mU/L at the screening were followed up until 24 months old. Serum TSH gradually returned to normal range in 182 cases. Serum TSH increased to be >10 mU/L in 5 cases who were treated with levothyroxine sodium. Serum TSH fluctuated between 5.6 and 10 mU/L in 4 cases who were continually followed up. Among 44 cases of serum TSH at 10~20mU/L, serum TSH gradually returned to normal range after levothyroxine sodium treatment in 38 cases; serum TSH increased to >20 mU/L in 7 cases for whom continuous treatment with levothyroxine sodium was required. In 242 cases of HT neonates, 18 cases had received continuous levothyroxine sodium replacement treatment for 2 years and were followed-up continuously. Conclusions Most of the newborns with HT recover within 2 years, while a few develop thyroid dysfunction and require follow-ups.

Gene array analysis in 56 cases of children with growth retardation

FU Chunyun, CHEN Shaoke, CHEN Rongyu, FAN Xin, LUO Jingsi, LI Chuan

. 2014, 32(12):  1119.  doi:10.3969 j.issn.1000-3606.2014.12.005

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　Objective　To investigate the genetic basis of the children with growth retardation. Methods From January to October 2013, the 56 patients with growth retardation were enrolled in this study. Genomic DNA was extracted from peripheral blood and was analyzed with gene array chips. Results Abnormalities were found in 12 patients (6 cases of sex chromosome abnormalities and 6 cases of autosomal aberration) and the detection rate was 21.4%. Four patients had the copy-number variations of smaller than 2.5Mb in size which could not be found by karyotyping analysis. Conclusions SNP-array gene chip could be used in the genetic diagnosis of growth retardation.

One case of childhood Graves'disease complicated with systemic lupus erythematosus: clinical manifestation and diagnosis　

GUO Qingmin, ZHUO Zhihong, WANG Huaili, CHEN Xiaoxin, MA Wei, XIE Lei, LI Fengyan

. 2014, 32(12):  1122.  doi:10.3969 j.issn.1000-3606.2014.12.006

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　Objective　To investigate the clinical characteristics, diagnosis and the differential diagnosis of Graves’ disease complicated with systemic lupus erythematosus (SLE) in children. Methods A child diagnosed of Graves’ disease complicated with SLE were reviewed. Results A 13-year-old girl, with fever and convulsions as the onset symptom, had the following examines performed: T3, T4, TSH, brain MRI, TPOAb, TGAb, anti-dsDNA, anti-smDNA, anti-ENA antibodies, antinuclear antibodies, renal and thyroid biopsies. SLE with lupus nephritis, Graves’ disease, cerebral infarction and cerebral white matter demyelination were diagnosed. Conclusions In addition to common diseases, autoimmune disease should also be considered for children with long-time fever and convulsions. Autoantibodies should be checked for children diagnosed of autoimmune thyroid disease.

The relationship between LTC4S, ALOX5 genetic polymorphism and clinical efficacy of leukotriene receptor modulators in children with asthma

YUAN Shuhua, YIN Yong, DONG Wenfang, ZHANG Hao, WANG Wei, ZHANG Lei, ZHANG Jing

. 2014, 32(12):  1126.  doi:10.3969 j.issn.1000-3606.2014.12.007

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Objective　To study the effect of genetic polymorphism of leukotriene C4 synthase (LTC4S) and 5-lipoxygenase (ALOX5) on efficacy of leukotriene receptor antagonist (LTRA) in children with moderate persistent asthma. Methods Seventy-two children with moderate persistent asthma who visited the out-patient clinic of Shanghai Children’s Medical. Center from June 2011 to June 2013 were divided into two groups, each of which first had ICS or LTRA+ICS for twelve weeks and then had the other for another twelve weeks. Polymerase chain reaction - restriction fragment length polymorphism (PCR-RFLP) was used to assess the genetic polymorphism of LTC4S RS730012 and ALOX5 RS2115819. Pulmonary function, clinical symptoms and C-ACT score were evaluated before and after treatment. Results After the treatment with LTRA, 75% forced expiratory flow (FEF75) was improved more significantly in patients with A/C or C/C genotype at LTC4S (RS730012) locus than in patients with A/A genotype. After the treatment with LTRA+ICS, there was no difference of pulmonary function among patients with different genotypes at ALOX5 (RS2115819). Conclusions The SNP of LTC4S (RS730012) is associated with the efficacy of montelukast in asthmatic patients because of the improvement of small airway function.

Analysis of etiology and diagnostic clues of childhood pleural effusion in 2419 cases　

LI Xiaoyong, LI Yonggang, PAN Zhengxia, WU Chun

. 2014, 32(12):  1132.  doi:10.3969 j.issn.1000-3606.2014.12.008

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Objective　To investigate the etiology and diagnostic clues of childhood pleural effusion. Methods Two thousand four hundred and nineteen hospitalized cases of pleural effusion from Jan. 1994 to Jan. 2014 were included and divided into infection group and non-infection group. Then the clinical data were collected. Results There were 1523 males (63.0%) and 896 females with an average age of 6.5±4.0 years old. There were 2353 cases in the infection group and 66 cases in the non-infection group. The incidence of fever in infection group was much higher than that in non-infection group, whereas the incidence of dyspnea and moderate-to-large pleural effusion was lower (all P<0.01). There was a significant difference of age distribution among children with different infectious pleural effusion. In infection group, the paragonimus szechuanensis pleurisy occurred preferentially in children 3-7 years old, the tuberculous pleurisy occurred preferentially in children >7 years old and the purulent pleurisy occurred preferentially in children ≤ 3 years old. The prognosis of infection group was much better than that of non-infection group (P<0.01). Conclusions Infection is the predominant cause of childhood pleural effusion. The differential diagnosis from non-infectious pleural effusion should consider the incidence, clinical manifestations and effusion quantity.

Clinical analysis of Mycoplasma pneumoniae pneumonia in children under 2 years old

CHEN Meng, YANG Jun, ZHAO Deyu

. 2014, 32(12):  1135.  doi:10.3969 j.issn.1000-3606.2014.12.009

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Objective　To analyze the clinical characteristics of Mycoplasma pneumoniae pneumonia in children under 2 years old. Methods The clinical features and treatment outcomes were analyzed in 57 children under 2 years old and compared with 153 children more than 2 years old. Results Compared with children more than 2 years old, in children under 2 years old, the number of cases with fever was fewer, the fever peak was lower and the lasting time was shorter. However, in children under 2 years old, the severe pulmonary signs were more common, the incidence of wheeze was higher, meanwhile, the count of white blood cells (WBCs) was significantly increased, more small pieces of fluff shadow was showed on chest X-ray, and the pulmonary complications were fewer, less corticosteroid was needed for treatment. After one-week therapy, the chest X-ray shadows were disappeared in most of children. Complications out of lungs, such as rash, liver function damage and cardiac damage can be found in both groups and no significant difference (P>0.05). Conclusions mycoplasma pneumoniae pneumonia in children under 2 years old had the characteristics of mild symptoms, severe pulmonary signs, few pulmonary complications, light systemic inflammations and mild radiological changes.

Clinical risk factor analysis of childhood refractory Mycoplasma pneumoniae pneumonia

MEI Yuxia, JIANG Jinjin,CAI Bin, ZHUANG Cheng, CHEN Ruohua

. 2014, 32(12):  1138.  doi:10.3969 j.issn.1000-3606.2014.12.010

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Objective　To identify the clinical risk factors of refractory Mycoplasma pneumoniae pneumonia (RMPP) and their values in early diagnosis. Methods The retrospective analysis of clinical data was conducted in 142 children with Mycoplasma pneumoniae pneumonia (MPP) admitted to Changhai Hospital of Second Military Medical University from Jan. 2012 to Jan. 2014. All children were divided into two groups, RMPP group (n=112) and MPP group (n=30). The comparison was made between two groups in clinical data. The factors were analyzed by the multifactor logistic regression. Results As compared to MPP, RMPP had longer fever duration, the higher ratios of large consolidation shadows, extrapulmonary complications and increased CRP level (P<0.05). The results of logistic regression indicated that the clinical risk factors included large consolidation shadows (OR=6.57, 95%CI: 2.10-20.56), extrapulmonary complications (OR=11.66, 95%CI: 2.42-56.08) and CRP (OR=14.87, 95%CI: 2.67-82.79) (P<0.01). Conclusions Large consolidation shadows, extrapulmonary complications and CRP are clinical risk factors of RMPP. CRP elevation and lung imaging changes are valuable in early diagnosis of RMPP.

The analysis of risk factors of nonalcoholic fatty liver diseases in primary school students

XU Renying, FENG Haixia, WAN Yanping, ZHOU Yiquan, LU Liping, ZHANG Xiaomin, CHEN Zhiqi, JIANG Ying, TAN Tao, WU Yingjie

. 2014, 32(12):  1141.  doi:10.3969 j.issn.1000-3606.2014.12.011

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Objective To evaluate the relationship between birth weight and nonalcoholic fatty liver diseases (NAFLD) in Chinese primary school students. Methods A cross-section study was conducted in five elementary schools in Gao Hang Town, Shanghai and 2163 students were enrolled in the study (1120 boys/1043 girls). Height, body weight, waist circumference and percent of body fat (bioelectrical impedance analysis) were measured by professional nutritionist after training. Birth weight, feeding pattern, height and body weight of parents were obtained by a self-completed questionnaire. NAFLD was diagnosed by ultrasound. The risk factors of NAFLD were analyzed. Results The prevalence of NAFLD in the study population was 8.9%. The prevalence of NAFLD was significantly higher in boys than that in girls (12.5% vs 5.0%, P<0.01). Logistic regression showed that sex (OR=1.97, 95%CI: 1.21-3.21) and percent of body fat (OR=1.12, 95%CI: 1.07-1.17) were the risk factors of NAFLD, and normal BMI was the protective factor of NAFLD (OR=0.09, 95%CI: 0.04-0.19) in the study population. Conclusions The prevalence of NAFLD is higher in boys than that in girls. Also overweight, and high percent of body fat are risk factors of NAFLD in children.

The expressions of miR-196b and its prognostic significances in pediatric acute myeloid leukemia

XU Lihua,CEN Jiannong, HE Hailong, SHEN Hongjie, YANG Naichao, YAN Qing, HU Shaoyan

. 2014, 32(12):  1145.  doi:10.3969 j.issn.1000-3606.2014.12.012

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Objective　To evaluate the expression of miR-196b in newly diagnosed pediatric acute myeloid leukemia (AML) and its clinical significance. Methods Fifty-two AML children were enrolled in this study and 30 non-leukemia compared children were selected as controls. The expressions of miR-196b were detected in bone marrow samples by real-time quantitative PCR (q-RT-PCR) and the results were expressed in 2-ΔΔCt. Results miR-196b expressions were significantly higher in M4-5 and lower in non- M4-5 of AML children than those in control (P<0.01), with a lowest level in t (15;17) and a highest level in MLL subtypes (P<0.01). The miR-196b expressions were significantly different among different prognosis groups (P<0.01) and the level in the favorable prognostic group was lower than in poor prognosis group. It was also found that miR-196b expression was lower in remission group than that in no-remission group after the first induction remission therapy (P<0.05). Meanwhile, the expression of miR-196b in the children with WBC≥100×109/L were statistically higher than that in the children with WBC<100×109/L (P<0.01), and miR-196b level was positively correlated with the platelet counts (r=0.302, P=0.030). Conclusions miR-196b expression is increased in poor prognosis group of AML children, and high expression of miR-196b is related with low response rate and poor prognosis. miR-1966 is expected to become a new target for the treatment of AML.

Changes of matrix metalloproteinase 9 expression in Kawasaki disease after intravenous immunoglobulin therapy

ZHOU Cuizhen, XIE Lijian, HUANG Min, WANG Renjian, SHEN Jie, XIAO Tingting

. 2014, 32(12):  1150.  doi:10.3969 j.issn.1000-3606.2014.12.013

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　Objective　To explore the mechanism of intravenous immunoglobulin (IVIG) treatment on Kawasaki disease (KD). Methods Thirty-six KD patients and 13 patients with pneumonia or upper respiratory infection (control group) were selected. The gene expression profiles of peripheral white blood cells from 4 KD patients (three male, one female) pre-and post- IVIG therapy and one pneumonia patient (male) were analyzed by Agilent gene chip. The gene expression was detected in 32 KD patients and 12 control patients by real-time PCR. Results The expressions of IL-1β, S100A9, S100A12 and MMP9 were significantly down-regulated in response to IVIG. The expressions of IL-1β, S100A9, S100A12 and MMP9 mRNA were significantly different among pre-treatment, post-treatment and control groups (P<0.01). The expressions of IL-1β and MMP9 mRNA were significantly down-regulated in response to IVIG (P<0.01). The expression of IL-1β mRNA was significantly higher in KD patients than that in control group. The expression of MMP9 mRNA was significantly higher in KD patients pre and post treatment than that in control group (P<0.01). The expression of MMP9 mRNA was significantly higher in KD patients complicated with coronary artery lesion (CAL) than that in KD patients without CAL complication (P=0.001). Conclusions The effects of IVIG on KD may be mediated by IL1B, S100A9, S100A12 and MMP9. MMP9 gene expression may be related to complication of CAL in KD.

The characteristics of T wave alternans in aortic stenosis in school-aged children

ZHANG Haiyan, LI Yun

. 2014, 32(12):  1155.  doi:10.3969 j.issn.1000-3606.2014.12.014

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Objective　To explore the characteristics of T wave alternans (TWA) in aortic stenosis (AS) in school-aged children. Methods TWA was analyzed in 15 AS patients and 60 age-matched normal children using Microvolt T-Wave Alternans and the incidence of sustained TWA was compared between two groups. Myocardial ischemia during treadmill test was studied in sustained TWA and non-sustained TWA in AS patients. Results Compared with normal children, the incidence of sustained TWA was significantly increased in AS group (P<0.01). In AS school-aged patients, the incidence of myocardial ischemia is higher in sustained TWA than in non-sustained TWA during treadmill test (P<0.05). Conclusions AS school-aged children have electrophysiologic basis for sunstained TWA. TWA is closely related to myocardial ischemia during treadmill test. Sustained TWA could be used as an important electrophysiologic index to assess the cardiac function in children with AS.

The reliability and validity of simplified COMFORT scale in assessment of sedation levels in children on mechanical ventilation　

FENG Yuguang, SUN Liping, WANG Zhuheng, LI Yuwei, GUO Qinghua, ZHOU Guanhua

. 2014, 32(12):  1158.  doi:10.3969 j.issn.1000-3606.2014.12.015

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Objective　To compare the scores from the simplified comfort scale with those from the bispectral index (BIS) and analyze the variation among different evaluators in children in an intensive care unit, and to investigate the reliability; validity of simplified comfort scale. Methods Twenty children between 3 and 16 years of ages on mechanical ventilation and sedation were simultaneously classified based on the BIS and the simplified comfort scale evaluated by 4 independent evaluators. The Kappa test was performed and the correlation between the two methods (Pearson correlation) was tested. Results In total, 70 observations were performed on 20 patients. Based on the Kappa coefficient, the agreement among evaluators ranged from 0.62 to 0.77 (p<0.001). There was a correlation between the BIS and the simplified Comfort scale (r=0.53-0.61，p<0.01). Conclusions Due to the strong consistency between the independent evaluators and the correlation between the two methods, the reliability and validity of simplified comfort scale are preferable and simplified comfort scale is useful in classifying the level of sedation in children on mechanical ventilation.

The expressions of insulin receptor substrate in adipocytes during catch-up growth in neonatal rats with intrauterine growth retardation

　WANG Chengbin, ZHENG Ruidan, GAO Jinzhi, LIAO Lihong, YE Juan, YING Yanqin, NING Qin, LUO Xiaoping

. 2014, 32(12):  1162.  doi:10.3969 j.issn.1000-3606.2014.12.017

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Objective　To investigate the expressions of insulin receptor substrate-1 (IRS-1) and insulin receptor substrate-2 (IRS-2) in adipocytes during catch-up growth in neonatal rats with intrauterine growth restriction (IUGR) and their correlations with the insulin resistance. Methods Sprague-Dawley rats (clean grade) were randomly divided into control group and food-restricted group after fertilization. Food-restricted group were received about 30% of food amount consumed in control group every day through the whole pregnant period to establish IUGR animal model, and were fed increased amount of breastmilk from postnatal day 1 to 21 to establish the period of catch-up growth in IUGR animal model (IUGR-CG). Fasting serum glucose, insulin and triglyceride were measured in blood from heart ventricles of 4-week old SD rats and insulin resistance index was calculated. Pre-adipocytes and mature adipocytes were obtained from SD rats at different age (1-week, 3-week, 5-week and 7-week old) and the former were induced to differentiate toward mature adipocytes. The levels of IRS-1, IRS-2 in the two kinds of mature adipocytes were detected by Real-Time PCR and Western blot. Results The expression levels of IRS-1, IRS-2 mRNA in mature adipocytes of IUGR-CG rats were significantly lower than those of IUGR rats at 5-weeks and 7-weeks old (P<0.05) while the expression levels of IRS-1, IRS-2 mRNA in differentiated adipocytes of IUGR-CG rats were significantly lower than those of IUGR rats at 5-weeks old (P<0.05). The expression levels of IRS-1, IRS-2 protein in two kinds of adipocytes (mature and differentiated adipocytes) of IUGR-CG rats were significantly lower than those of IUGR rats from postpartum week 1 through 7 (P<0.05). Conclusions IRS-1 and IRS-2 expression levels are downregulated in adipocytes during catch-up growth of IUGR rats, which may be closely related with insulin resistance.

The curative effect of ursodeoxycholi acid on retinopathy of prematurity in newborn rats　

　ZHANG Guoqing, LI Jing, BU Jun, SUN Jianhua, HUANG Ping

. 2014, 32(12):  1168.  doi:10.3969 j.issn.1000-3606.2014.12.018

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Objective　To explore the curative effect of ursodeoxycholi acid on retinopathy of prematurity (ROP) caused by high concentration of oxygen in newborn rats. Methods The model of ROP was established. Neonatal rats were divided into normal control group, ROP model group, low dose ursodeoxycholi acid treatment group (10mg/kg) and high dose ursodeoxycholi acid treatment group (40mg/kg). Rats were sacrificed at days 17. The new retinal vessels were observed and counted under fluorescence microscope. Results The new retinal vessels in ROP rats were hyperplastic, twisted and unevenly distributed. There was significant difference in the number of new retinal vessels among different groups (P=0.000). The number of new retinal vessels of rats in ROP group, low-dose group and high-dose group was significantly more than that in control group (P=0.000). The number of new retinal vessels in low-dose group and high-dose group was significantly less than that in ROP group (P<0.05). The number of new retinal vessels in high-dose group was less than that in low-dose group (P>0.05). Conclusions Ursodeoxycholi acid could inhibit the angiogenesis of retina and could have curative effect on ROP.

The role of endoplasmic reticulum stress in the hyperoxia-induced lung injury in premature rats　

　WANG Qin,DONG Wenbin, CHE Zhongli, HE Na, YU Li, LI Qingping, ZHAI Xuesong, LEI Xiaoping

. 2014, 32(12):  1171.  doi:10.3969 j.issn.1000-3606.2014.12.019

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　Objective　To explore the effects of endoplasmic reticulum (ER) stress in the hyperoxia-induced lung injury in premature rats. Methods Forty-eight premature Wistar rats were randomized into two groups 12 hours after birth: hyperoxia group (n=24) inhaled 95% oxygen and control group (n=24) inhaled air. Eight rats were sacrificed in each group on day 1, 3, 7 after the treatment and the left lungs were embedded. The pathological changes in the HE stained sections of lung tissues were observed. The expressions of ER related protein ERp57 and c/EBP homologous protein CHOP were detected by immuno histochemistry and the apoptosis of lung cells was detected by TUNEL analysis. Results The typical pathological characteristics of acute lung injury were observed in hyperoxia group. The expressions of ERp57 and CHOP were increased with the exposure time in hyperoxia group, and were significantly higher than in control group (P < 0.05). The apoptosis rate of lung cells in hyperoxia group was significantly higher than in control group (P < 0.01). There was significant positive correlation between cell apoptosis index and expressions of Erp57 and c/EBP homogeneous protein. Conclusions ER stress initiated apoptosis participates and plays an important role in the process of hyperoxia-induced lung injury in premature rats.

Protective effects of mouse nerve growth factor on brain in epileptic young rats

ZANG Huanhuan, CHEN Lang, LIU Rui, CHEN Qiaobin, FANG Qiong, LIN Lin

. 2014, 32(12):  1176.  doi:10.3969 j.issn.1000-3606.2014.12.020

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　Objective　To explore the effects of mouse nerve growth factor (mNGF) on expression of metallothionein I/II (MT I/II) and cytochrome C (Cyt C) in hippocampus of pentylenetetrazol (PTZ)-induced epileptic (EP) young rats. Methods Fifty SD rats aged 19 days were randomly divided into control group, EP group, mNGF low, medium, and high dose groups. Each group had 10 rats. Control group was injected with normal saline every day, and EP group was intraperitoneally injected with PTZ 40 mg/(kg·d) for 21 days in succession. The mNGF low, medium, and high dose groups were respectively intramuscularly injected with mNGF 500, 1 000, 2 000 AU/(kg·d) for 7 days in succession after PTZ injection. Changes of body weight, behavioral performance were recorded. The positive cells of MT I/II, Cyt C were examined by immunohistochemisty. The levels of MT I, Cyt C mRNA in hippocampus were measured by real-time PCR. Results The number of MT I/II, Cyt C positive cells and the levels of MT I, Cyt C mRNA in hippocampus had significant differences among groups (F=15.98-105.76, P =0.000). The number of MT I/II, Cyt C positive cells and the levels of MT I, Cyt C mRNA of EP group were higher than those in control group, mNGF low, medium, and high dose groups (P<0.05). The number of MT I/II, Cyt C positive cells of mNGF low group were higher than those in mNGF high dose group (P<0.05). The levels of MT I, Cyt C mRNA of mNGF low group were higher than those in mNGF medium and high dose groups (P<0.05). The number of MT I/II, Cyt C positive cells and the levels of MT I, Cyt C mRNA had no differences between mNGF medium and high dose groups (P>0.05). Conclusions As a stress protein, metallothionein is involved in the process of chronic epilepsy along with Cyt C. mNGF has neuroprotective effects on the hippocampus of epileptic rats in dose dependent manner.

New advances in the treatment of multisystem Langerhans cell histiocytosis

Zhang Zhen

. 2014, 32(12):  1191.  doi:10.3969 j.issn.1000-3606.2014.12.022

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　Langerhans cell histiocytosis (LCH) is a rare proliferative disorder of dendritic cells and the reticular cells. It is divided into single-system langerhans cell histiocytosis (SS-LCH) and multi-system langerhans cell system (MS-LCH). MS-LCH has a very poor prognosis. The key of survival is timely and effective treatment. The treatment protocols include chemotherapy, immunotherapy and stem cells transplantation. In this review, the new advances in the treatment of MS-LCH were systemically reviewed.

Advances in research of the relationship between adhesion molecules and pathogenesis of Kawasaki disease

CHAO Lumen, CHEN Baiyu

. 2014, 32(12):  1194.  doi:10.3969 j.issn.1000-3606.2014.12.023

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Adhesion molecules (AM) are a class of molecules that can mediate cell-to-cell and cell-to-matrix interaction. They participate in the cellular recognition, signal transduction, cell proliferation and differentiation, cell stretching and movement through ligand-to-receptor interaction. AM are the molecular basis of immune response, inflammation, blood coagulation, tumor metastasis, wound healing and a series of physiological and pathological processes. Kawasaki disease (KD) is an acute systemic small vasculitis syndrome, mainly affecting coronary artery. KD is the main cause for acquired heart disease in children. To study the relationship between AM and pathogenesis of KD is important in the understanding of KD pathogenesis, prevention and treatment of coronary artery lesions complicated with KD. This review focused on the relationship between AM and pathogenesis of KD.