1.Progress on molecular genetics in atypical hemolytic uremic syndrome
International Journal of Pediatrics 2011;38(2):147-150
Atypical hemolytic uremic syndrome(aHUS) has recently been shown to be a rare disease of genetic predisposition, including genes of complement factor H(CFH), membrane cofactor protein(MCP, CD46)and complement factor Ⅰ(CFI), which are complement regulatory genes. Genes mutation is about 50%,involving the three genes mutation, including nonsense mutation, missen mutation, silent mutation, splice mutation and insertion mutation. Autosomal dominant inheritance and autosomal recessive inheritance have been reported, however, for autosomal dominant inheritance, the three genes mutations are incomplete penetrance.
2.The biological characteristics of p300/CBP-associated factor and relationship with disease
International Journal of Pediatrics 2011;38(2):139-141
p300/CBP-associated factor (PCAF)is an important histone acetyltransferase in eukaryotic cells. PCAF can acetylizes histone and non-histone. PCAF participates in various biological processes of cells and in the interaction between virus and cells. The imbalance of PCAF would lead to abnormal development of various organs, and related to the development of some tumors.
3.The function of T helper type 17 cell in intestinal mucosal immunity
International Journal of Pediatrics 2011;38(2):155-158
The intestinal mucosa is the biggest lymph organ in human body, which consists of the first line of defensive system with the respiratory mucosa and urinary mucosa. The intestinal mucosa is responsible to defend bacteria, viruses, dietary pathogens, non-steroid anti-inflammatory drug and so on. If the intestinal mucosal immune system is destroyed, the human will suffer many diseases such as infectious diseases,autoimmune diseases. Therefore, it plays an important part in physical health. T helper type 17 (Th17) cells different from Th1 cells and Th2 cells are a distinct lineage of T cells. The discovery of Th17 cells has ruined the constitutional pattern of Th1/Th2. Th17 cells, which are important parts of intestinal mucosal immune system,play critical roles in intestinal autoimmune diseases, tissue inflammation and the infection of various pathogenic bacteria. This paper will review the differentiation of Th17 cell and its function in intestinal mucosal barrier,intestinal autoimmune diseases and intestinal infectious diseases.
4.Analysis of frequent relapses related factors of primary nephrotic syndrome in children
Shuying FAN ; Bili ZHANG ; Wenhong WANG ; Xuan ZHANG
International Journal of Pediatrics 2011;38(2):198-200
Objective To explore the influencing factors of frequent relapse primary nephritic syndrome (PNS) in children according to the statistical analysis of their clinical data and laboratory examinations. Method We collected in-hospital and out-patient medical records of children who were diagnosed PNS from Jan. 2007 to Feb. 2010 and whole clinical data were analyzed. Results In 245 cases of children with steroid sensitine nephrotic syndrome, non-frequent replapse (NFR) accounted for 82.4% and frequent relapse (FR) accounted for 17.6%. In univariate analysis, period of first relapse between initial treatment remission within 3 months , level of serum total protein and cholesterol and IgE at onset had statistical significance in FR children and NFR children, which were significant predictors of FR. The logistic regression analysis revealed that the first relapse after initial treatment remission within 3 months and increasing of serum IgE level (more than 358 IU/ml)were risk factors of FR. Among frequent relapses factors, infection, especially upper respiratory tract infection was the first (65. 1%). The relapse with motivation mainly in January to March, accounted for 30. 4%. Conclusion FR had a proportion of 17. 6% in children with PNS under regular glucocorticoid treatment. It showed seasonality feature that relapse. peak in winter and spring. Level of the serum total protein less than 40g/L and cholesterol more than 10mmol/L at onset can be predictive index of FR. Infections are the main motivation of relapse, but the relapse by irregular treatment and specific constitutions should be pay more attentions too.
5.The expression and clinical significance of the serum Eotaxin, IL-13 and total IgE in children with asthma
Chunyan MA ; Yajing ZHANG ; Lihua NING ; Hua CHEN ; Fang ZHOU ; Xin WANG
International Journal of Pediatrics 2011;38(2):195-197
Objective To study the nosogenesis and the relationship of different levels of serum Eotaxin,IL-13 and total IgE(TIgE)in asthmatic children. Methods Thirty children with asthma aging from 5 to 12 years were selected as study group (attack period and remission period), and 22 healthy children were selected as control group. Serum Eotaxin and IL-13 were measured with enzyme linked immunosorbent assay, and serum TIgE was determined with enzyme-linked fluoroimmune assay in the study group and control group, and pulmonary function was measured at the same time in the study group. Results (1) There were significant differences in pulmonary function between the attack period and the remission period (P < 0. 001). (2) There were significant differences in the level of serum Eotaxin, IL-13 and IgE between the study group and the control group,especially for the attack period and the control group(P <0. 001). (3) There were significant positive correlations between the level of serum Eotaxin and IL-13, Eotaxin and TIgE, IL-13 and TIgE respectively in the study group (P <0. 05). (4) There were negative correlcations between the level of serum Eotaxin and FEV1 ,PEF,and serum IL-13 and FEV1, PEF in asthmatic children (P < 0. 05), there were no correlations between the level of serum TIgE and FEV1, PEF in the study group (P > 0. 05). Conclusion Serum Eotaxin, IL-13 and TIgE were participated in the nosogenesis of asthma, and the level of serum Eotaxin and IL-13 can be a assessment of asthmatic patients' condition.
6.Progress of genetic etiology and detection method in congenital heart disease
International Journal of Pediatrics 2011;38(2):188-191
The etiology of congenital heart disease (CHD) incluses genetic factors, environmental factors and maternal factors. The genetic etiology of CHD includes chromosomal abnormalities, single gene defects and multiple genetic defects. The genetic testing method of CHD has made great progress in karyotype analysis,fluorescence in situ hybridization, quantitative PCR, multiplex ligation dependent probe amplification, et al.
7.Clinical progress of rotavirus vaccine in children
International Journal of Pediatrics 2011;38(2):185-187
Rotavirus is an important pathogen, which can cause severe diarrhea in infants and children.Because of lacking of specific therapy, an effective vaccine is particularly urgently needed. Vaccine safety,efficacy of the vaccine, possible outcomes of continuous expression of the vaccine' s exogenous antigens and vaccine' s induction on the immune response of bodies are the key factors to rotavirus vaccine, Present review discussed and analyzed the related characteristics of rotavirus vaccine abroad, in order to put forward further guidance on the research, and promote the development of rotavirus vaccine in China.
8.Progress of immunology in epilepsy
International Journal of Pediatrics 2011;38(2):121-124
Epilepsy is the most common neurological disease in children. Nowadays, the functional disturbances of the immune system, both humeral and cell-mediated immunity, have been detected more often in patients with epilepsy than in normal individuals, which include changes of cytokine, autoantibody and immunoglobulin in humoral immunity, and changes of lymphocyte subset, lymphocyte hyperpalsy zone and nature killer cell in cellular immunity. Not only the peripheral blood of the patients is abnormal, but also the cerebrospinal fluid can be found unusual. Some antiepileptic drugs (AEDs) can exert an effect on the function of immune system as well. Elucidation of connections between epileptogenesis, immunology and effectiveness of antiepileptic drugs therapy is to be explored.
9.Applications of the zebrafish in the studies of developmental pediatrics
International Journal of Pediatrics 2011;38(2):178-180
Zebrafish as a new animal model is gradually being recognized. Easy fed, growing rapidly,transparent embryo, strong reproduction, especially similar with human in gene structure, body regulation patterns, and the development of organs and disease, all those characteristics make zebrafish significant in development of vertebrate animal and human disease. This review focuses on discussing the applications of the major organs of zebrafish in the studies of developmental pediatrics.
10.Bocavirus infections and wheezing in infant
International Journal of Pediatrics 2011;38(2):166-168
Wheezing in infants and young children are common respiratory diseases, the incidence rate of it is increasing year by year. Viral infection of the respiratory tract is one of the most important provocative causes of infant wheezing disorders. Recent studies have discovered the relationship between Bocavirus and infant wheezing. To study the relationship between Bocavirus and infant wheezing will be important to prevent infant wheezing disorders.