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Objective To explore the alternations of CD4 + T cell subsets and their cytokines and transcription factors in children with Henoch - Schonlein purpura(HSP). Methods Forty - one children were enrolled in this stud-y,including 41 in acute stage of HSP,35 cases of HSP in clinical remission stage and 30 healthy children as healthy control group. The percentages of helper T lymphocytes(Th)1,Th2,regulatory T cells(Treg)and Th17 subsets were determined by flow cytometry(FCM). The concentrations of plasma interferon - γ( INF - γ),interleukin( IL) - 4, transforming growth factor - β1(TGF - β1 )and IL - 17 were examined by ways of enzyme - linked immunosorbent assay(ELISA). The expressions of T - bet,GATA3,Foxp3 and ROR - γt mRNA in peripheral blood mononuclear cells were examined by means of reverse transcription - polymerase chain reaction(RT - PCR). Results (1)During the a-cute and recovery stage of HSP,compared with the control group,the percentages of Th2 and Th17 cell subsets,the le-vels of plasma IL - 4 and IL - 17,and the expressions of GATA3 and ROR - γt mRNA were significantly higher(all P ﹤ 0. 05). The percentages of Th1 and Treg cell subsets,the levels of plasma INF - γ and TGF - β1 and the expres-sions of T - bet and Foxp3 mRNA were lower(all P ﹤ 0. 05). The imbalances of Th1 / Th2 and Treg/ Th17 appeared during the acute stage of HSP.(2)During the recovery stage of HSP,the percentages of Th1,Th2,Th17 and Treg,and the ratio of Treg / Th17 were of no difference compared with those in acute stage( all P ﹥ 0. 05). The ratio of Th1 / Th2,and the expressions of T - bet and Foxp3 mRNA were increased(all P ﹤ 0. 05),while the levels of plasma IL - 4 and IL - 17,the expressions of GATA3 and ROR - γt mRNA were decreased(all P ﹤ 0. 05)compared with those in acute stage. In the recovery stage of HSP,the imbalances of Th1 / Th2 and Treg/ Th17 were still obvious .(3)There was a positive correlation in every 2 cytokines of Th1,INF - γ and T - bet. And the same correlation existed in Th2, IL - 4 and GATA3,in Treg,TGF - β1 and Foxp3,and in Th17,IL - 17 and ROR - γt(P ﹤ 0. 05). Conclusions The imbalance of Th1 / Th2 and Treg/ Th17 is critical in pathological mechanism of HSP. The disturbance of immune tole-rance induced by Treg cells is important in HSP.
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Objective To investigate the correlations between the clinical manifestations based on pathologic grades and renal pathological features of Henoch - Schonlein purpura nephritis(HSPN)in children. Methods The clinical data of 77 patients with HSPN in the Department of Nephrology,Anhui Provincial Children's Hospital from Ja-nuary 2004 to March 2014 were retrospectively analyzed. The relationship between clinical manifestation and pathologi-cal features was analyzed. Results Among the 77 patients,21 cases(27. 3% )had both abdominal symptoms,and ar-thritis was reported in 15 cases(19. 5% ),28 cases(36. 4% )had abdominal symptoms and arthritis,and 13 cases (16. 9% )had no such symptoms. Hematuria and proteinuria were the most common clinical types[48. 1%(37 / 77 ca-ses)],followed by simple hematuria or proteinuria[27. 3%(21 / 77 cases)],nephrotic syndrome[23. 4%(18 / 77 ca-ses)],and chronic nephritis[1. 3%(1 / 77 cases)]. The major of pathological changes in HSPN were grade Ⅱ[46. 8%(36 / 77 cases)]and grade Ⅲ[45. 5%(35 / 77 cases)],the minority of them were grade Ⅰ[6. 5%(5 / 77 cases)]and grade Ⅳ[1. 3%(1 / 77 cases)]. The severity of urine protein was positively associated with pathologic classification (r s = 0. 472,P = 0. 000). According to the glomerular deposition of immune complex,there were 6 types. The percen-tage of deposition of IgA + IgM was 62. 3%(48 / 77 cases),IgA + IgG + IgM was 19. 5%(15 / 77 cases),IgA 14. 3%(11 / 77 cases),that of IgA + IgG 1. 3%(1 / 77 cases),and the IgM 1. 3%(1 / 77 cases),no Ig 1. 3%(1 / 77 cases). In these cases,76. 6%(59 / 77 cases)had complements C3 deposition;pathologic stage characterized by Ⅲ level and a-bove were common[54. 2%(32 / 59 cases)],Ⅱ level 42. 2%(25 / 29 cases),Ⅰ level 3. 4%(2 / 59 cases). Among the different types of immune complex depositions,there was no statistically significant difference in pathological types of distribution,while the clinical type and complements C3 deposition were significantly associated with pathologic classifi-cation(rs = 0. 361,P = 0. 001). Sixty - two cases were rated as level 1(80. 5% ),and 15 cases was level 2(19. 5% );in different clinical group,rating in glomeruli was statistically different(χ2 = 17. 2,P = 0. 004). Renal tubular interstitial rating of all the patients were level 1(100% ). Conclusions The severity of urine protein,complements C3 deposition is associated with pathologic classification. Pathologic classification can basically reflect the renal damage in HSPN.
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Objective To investigate the association between 2 single nucleotide polymorphisms( SNPs) [rs2243250 in interleukin(IL)- 4 gene and rs1800925 in IL - 13 gene]in Henoch - Schonlein purpura(HSP)and Henoch - Schonlein purpura nephritis(HSPN)in Han Chinese children. The level of IL - 4 and IL - 13 in serum were compared between HSP and HSPN. Methods A case - control study was adopted in this research,and 383 children with HSP or HSPN were recruited in this study,409 normal children served as controls. The genotypes of 2 SNPs were detected by using polymerase chain reaction - restriction fragment length polymorphism(PCR - RFLP),and the serum levels of IL - 4 and IL - 13 in HSP and HSPN patients were detected by using enzyme linked immunosorbent assay (ELISA). All the data were analyzed by SPSS 16. 0 software. Results (1)There was no association(polymorphism of SNP rs2243250 in IL - 4 gene)in HSP group and HSPN group compared with the healthy control group(P ﹥ 0. 05);and the polymorphism of another SNP(rs1800925 in IL - 13 gene)was associated with HSP(P = 0. 037),and the fre-quency of T allele of the study group was higher than that of the healthy control group(P = 0. 027). But there was no difference between the study group(HSP group and HSPN group)and the healthy control group in frequency of TT genotype(P = 0. 132),and there was no association between HSPN group and healthy control group(P = 0. 487);also there was no association between polymorphism of this SNP in HSP group and HSPN group(P = 0. 129).(2)There was an association between polymorphism of IL - 13 gene and IL - 13 level in serum,patients with TT genotype had a higher serum IL - 13 level than any other genotypes(P ﹤ 0. 05),but there was no statistically significant difference in IL - 4 level between TT genotype and other genotypes(P ﹥ 0. 05).(3)There was an association between polymorphism of IL - 13 gene and IL - 13 level in serum patients with HSPN who had a higher level of serum IL - 13 than the patients with HSP( P ﹤ 0. 05),but there was no difference in serum IL - 4 level between HSP group and HSPN group. Conclusions The polymorphism of SNP(rs1800925 in IL - 13 gene)was associated with HSP group,and the poly-morphism of this SNP might affect the level of IL - 13 in serum. The patients with HSPN have a higher level of serum IL -13 than the patients with HSP,and high level of IL -13 may be a risk factor in the progression from HSP to HSPN.
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Objective To summarize the clinical characteristics and laboratory test results of children with Henoch - Schonlein purpura(HSP),and further to analyze the risk factors for HSP combined with cardiac damage. Methods The clinical and laboratory tests findings from 707 children diagnosed as HSP at Nanjing Children's Hospi-tal were retrospectively analyzed,who were recruited from November 2011 to December 2012. The possible risk factors for HSP with cardiac damage in children were recorded,including gender,age,predisposing causes,gastrointestinal symptoms,joint pain,kidney disorders,serum electrolytes,anti - streptolysin 〝O〝 test,erythrocyte sedimentation rate, and complement level were summarized. Chi - square test and Logistic regression were performed to analyze the risk fac-tors of cardiac damage in children with HSP. Results Among 707 cases,192(27. 2% )patients were combined with car-diac damage,115 male and 77 female,and the proportion of men to women was 1. 00: 0. 67;age ranged from 11 months to 15 years and 4 months(6 years and 5 months for median age),6 patients ﹤ 3 years old occupying 3. 1% ,103 patients≥3 - 7 years old occupying 53. 7% ,82 patients≥7 - 14 years old occupying 42. 7% ,1 patient≥14 years old occupying 0. 5% ,and the age of onset in preschool and school age. Electrocardiogram(ECG)abnormalities were found in 190 patients,the main manifestations including long Q - T interval,ST - T segment falling down and sinus bradycar-dia,and one or more items of abnormal myocardial enzymes existed in 24 cases;echocardiography was performed in 35 cases of children,but no abnormality was detected,no obvious symptoms such as flustered or chest tightness or precor-dial distress. Statistical analysis showed that gender,predisposing causes,mixed HSP,complement level were related to the incidence of cardiac damage in children with HSP(P ﹤ 0. 05). Furthermore binary Logistic regression identified that in male patients,the ratio of X1 vs OR ratio was 0. 654(95% CI 0. 462 - 0. 926,P ﹤ 0. 05),for predisposing causes,the ratio of X2 vs OR ratio was 2. 63(95% CI 1. 838 - 3. 765,P ﹤ 0. 001),for mixed HSP,the ratio of X3 vs OR ratio was 2. 452(95% CI 1. 301 - 4. 621,P ﹤ 0. 01),which were independent factors for cardiac damage in chil-dren with HSP. Conclusions ECG and/ or myocardial enzyme spectrum abnormalities are the main clinical ma-nifestations of cardiac damage in children with HSP. Male patients,predisposing causes of the respiratory tract infec-tion,mixed HSP and hypocomplementemia were high risk factors in the development of cardiac damage,which require special consideration clinically,and earlier ECG and myocardial enzymes examination,early diagnosis and treatment are necessary to avoid the occurrence of severe cases.
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Paciente de 35 años de edad, quien debuta con síndrome de pulmón riñón secundario a vasculitispulmonar y depósitos renales granulosos de IgA, en quien se diagnostica vasculitis sistémica porIgA, posteriormente, presenta compromiso pancreático y cutáneo (Púrpura de Henoch Schõnlein),falleciendo ocho meses más tarde por linfoma difuso de células B de alto grado. Este es elreporte de caso número catorce en el que se establece asociación entre neoplasia hematológicay Púrpura de Henoch Schõnlein
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Humans , Lymphoma , IgA Vasculitis , RheumatologyABSTRACT
Objective To explore clinical significence of soluble vascular cell adhesion molecular(SVCAM - 1),immunoglobulin A (IgA) anti- endothelial cells antibodies(AECA) in Henoch-Schonlein purpura(HSP) and their relationships. Methods SVCAM-1 were detected by ELISA between 55 cases with HSP (40 cases in acute stage and 15 cases in recovery) and 20 controls. Results The levels of serum SVCAM - 1 of the HSPN cases were significantly increased compared with the other HSP. In addition, the serum level of SVCAM - 1 in acute stage HSP children was significantly higher than that in recovery stage and normal controls. IgA AECA positive rate in HSPN was higher than that of without nephritis group. The level of serum SVCAM - 1 in IgA AECA positive children was higher than that of IgA AECA negative children. Conclusion SVCAM - 1 and IgA AECA may participate in the pathogegesis of HSP, and the level of molecule is correlated with the progress of HSP.
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Objective To explore the relationship of immunoglobulin A(IgA) nephropathy (IgAN) and Henoch - Schonlein purpura nephritis(HSPN) in children by making comparison in the aspects of clinical symptoms, laboratory tests and renal pathology. Methods Process the data of 69 cases of HSPN and 33 cases of IgAN respectively in clinical symptoms' frequency, indices of urinalysis and blood cell count, protein quantity in urine of 24 hours, serum level of immunoglobulins, C3 and C4, as well as renal pathologic results. Results In IgAN group, the incidence of male was higher than that of female, respiratory tract infections usually occur as prodrome. Macroscopic hematuria was the most prominent clinical feature, while rash, joint pain, abdominal symptoms seldom happened. Level of hemoglobin in IgAN group was decreased significantly. There were no significant differences in edema, hypertension, proteinuria degree, level of plasm albumin, level of serum IgA, IgG, IgM, IgE, C3 and C4, as well as the features of renal pathologic changes between IgAN and HSPN.Conclusions IgAN and HSPN have similar features in clinical symptoms,level of immunoglobulins and complements and renal pathology. But it is still under discussion whether they are different stages of the same disease.
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0.05) ,but the differences reached statistical significance compared the positive ratios of two index together to urine NAG and ?2 - MG (X2 = 4.41,7.28 P
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Objective To explore the pathogenic effect of serum platelet derived growth factor(PDGF) in Henoch - Schonlein pur-pura nephritis(HSPN) and the influence of heparin. Methods The levels of serum PDGF were detected before and after heparin therapy by double antibody ELISA in the peripheral blood of patients. All patients were injected with heparin calcium[100 U/(kg?d)] for two to three weeks. Results The levels of serum PDGF(596.23 ? 199.43) ng/L in 30 HSPN cases before heparin therapy were significantly higher than those of the control group(259.76?69.58) ng/L (P