Semm Galactose-deficient IgA1 Related Gene Research In Children With Henoch-schonlein Purpura

Background and Objective:Henoch-Sch?nlein purpura(HSP) is the most common childhood systemic vasculitis, may be the disease from infancy to adulthood, while 75% of the patients were children under 10 years of age. HSP can affect various body organs, mainly affects the skin, joints, digestive tract and kidneys of the four organs, the degree of involvement of the kidneys to determine their ultimate prognosis. Some children with recurrent disease and a poor prognosis. There are reported that underglycosylated Ig A1 plays an important role in the pathogenesis of Henoch Schonlein purpura, Ig A1 O-galactose iscomposed of beta 1, 3 galactosyl transferase(beta 1, 3 ga Iactosyltransferase, beta 1, 3 GT) in its specific molecular chaperone Cosmc(core 1 beta 3- Gal- T specific molecular chaperone) assistance. this paper discusses the relevance of the level of Gd-glycosylated Ig A1 in different groupings and clinical symptoms. At the same time, to further explore the expression levels caused by Henoch Schonlein purpura underglycosylated Ig A1 related genes.Research subjects and Methods :In our hospital in 2013 October-2014 year in March admitted 44 cases of children Henoch Schonlein purpura as the research object, the choice of gender, age matched healthy children as a control group of 12 cases. In children with Henoch Schonlein purpura according to clinical symptoms and urinary protein degree is divided into mild, moderate,severe.including 22 mild cases, 12 moderate cases, 10 cases of severe. Serum Ig A1 levels were measured by immunoturbidimetry,serum gd-glycosylation Ig A1 was detected by binging of Vicia villosa lection using ELISA technique.Henoch Schonleinpurpura patients according to whether the renal involvement, divided into 19 cases of Henoch Schonlein purpura nephritis group and 25 cases of non nephritis group, By using centrifugal ficoll density gradient separation of peripheral blood mononuclear cells, using CD19 magnetic bead separation of peripheral blood B lymphocytes, using real-time fluorescent quantitative(RT-PCR) analysis of peripheral blood lymphocyte B 1, 3 galactosyl base transferase(C1GALT1) and their partners(Comsc) nucleic acid protein expression levels.The data of multiple groups were compared using analysis of variance(F test), between the two two groups were compared with LSD.t test, the relationship between two variables by linear correlation analysis, P<0.05 had significant difference.Results:1. Serum Ig A1 and Galactose-deficient Ig A1.The Ig A1 level of the control group,mild group,moderate group and severe group were(1.86±0.05) 、( 3.16±0.13)、(3.42±0.53)、(4.61±1.07),and gd-glycosylation Ig A1 were(0.14±0.00)、(0.18±0.01) 、(0.20±0.02) 、(0.24±0.02) in all groups, comparison of levels of Serum Ig A1 and Galactose-deficient Ig A1 among the four groups,the differences were statistically significant(P<0.05). Henoch-Sch?nlein purpura from each group, the group with mild levels of serum Ig A1 and Galactose-deficient Ig A1 was lower than the severe group, the difference was statistically significant(P<0.05); there was no significant difference between mild and moderate group(P>0.05); the level of serum Ig A1 and underglycosylated Ig A1 were higher than the healthy control group(P<0.05).2. Galactose-deficient Ig A1 related gene expressionThe Ig A1 level of HSPN,HSP and control group were( 3.73±0.59) 、(3.21±0.18) 、(1.86±0.05),and the level of Galactose-deficient Ig A1(AU/mg) were(0.23±0.06)、(0.17±0.01)、(0.14±0.00) in all groups. Henoch-Sch?nlein purpura nephritis groupwas higher than Henoch-Sch?nlein purpura group and healthy control group, the differences were statistically significant(p<0.05).The C1GALT1 gene expression level of Henoch-Sch?nlein purpura nephritis, Henoch-Sch?nlein purpura, healthy controls were(0.74±0.59)、(0.75±0.27)、(1±0.28),There were no significant differences between the three groups(P>0.05).The expression level of Comsc gene were(0.33±0.13)、(0.40±0.18),、(1±0.16).,the level of Comsc gene expression were differences in all groups(P<0.05),the expression of Comsc gene in children with Henoch Schonlein purpura compared with the healthy control group was decreased,The Comsc gene expression level had no significant differences in of HSP and HSPN(P>0.05). Pearson correlation analysis showed that C1GALT1, Comsc gene expression and Galactose-deficient Ig A1 had no correlation, the correlation coefficients were respectively 0.032, 0.081, P values were more than 0.05.Conclusions:Children with the presence of serum Galactose-deficient Ig A1, and abnormal Ig A1 Increased,particularly in children with severe Henoch Schonlein purpura and Henoch Schonlein purpura nephritis, the abnormal serum Ig A1 level may, with the severity of Henoch Schonlein purpura in children with the disease severity associated with proteinuria. Henoch Schonlein Purpura nephritis chaperone gene expression was significantly decreased, but its expression level decreased Galactose-deficient Ig A1 had no positive correlation, suggesting that multiple factors involved in the formation of Galactose-deficient Ig A1.