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Objective: To investigate the relationship between serum galactose-deficient immunoglobulin A1 (Gd IgA1), soluble fms like tyrosine kinase receptor l (sFlt-1), immunoglobulin A (IgA) /complement C3 (C3) ratio, renal injury, and therapeutic effect in children with purpura nephritis (HSPN) . Methods: A total of 151 children diagnosed with HSPN in the The Affiliated Hospital of Chengdu University were selected from December 2019 to December 2021 as the case group. Another 100 healthy volunteer children who underwent physical examinations during the same period were selected as the control group. The serum Gd-IgA1, sFlt-1, IgA/C3 ratio, renal function index [urea nitrogen (BUN), blood creatinine (Scr), 24 h urinary protein (UP) quantification (24hUPE) and UP/Scr] between the two groups were compared. Pearson correlation analysis was used to investigate the correlation between serum Gd-IgA1, sFlt-1, IgA/C3ratio and renal function indicators. The serum Gd-IgA1, sFlt-1, and IgA/C3 ratios of children with different renal pathological grades and different therapeutic effects were compared. Results: The Gd-IgA1, sFlt-1, IgA/C3 ratio, BUN, Scr, 24hUPE and UP/Scr of the case group were all higher than those of the control group (P＜0.05) . Pearson correlation analysis showed: Gd-IgA1, sFlt-1, IgA/C3 ratios were positively correlated with BUN, Scr, 24hUPE and UP/Scr (P＜0.05) . With the elevated renal pathological grade, the serum Gd-IgA1,sFlt-1, and IgA/C3 ratios in HSPN patients continue to increase (P＜0.05) . The Gd-IgA1, sFlt-1, and IgA/C3 ratios of the invalid group were higher than those of the valid group (P＜0.05) . Conclusion: Gd-IgA1, sFlt-1, and IgA/C3 ratios are involved in the disease progression in children with HSPN, which can lead to renal injury, aggravate renal pathological changes, clinical guidance for treatment can be based on changes in the above indicators. [ABSTRACT FROM AUTHOR]

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Objective: To analyze the relationship between monocyte chemoattractor protein-1 (MCP-1), soluble vascular cell adhesion molecule-1 (sVCAM-1) and children with purpura nephritis and renal injury. Methods: 108 children with purpura nephritis admitted to our hospital from January 2018 to December 2020 were selected as the observation group, and 108 healthy children were selected as the control group. The expression levels of serum MCP-1 and sVCAM-1 in the two groups were detected, and the relationship between the expression levels of serum MCP-1 and sVCAM-1 and renal function indexes in children with purpura nephritis was analyzed. The area under the receiver operating characteristic curve (AUC) was used to evaluate the efficacy of serum MCP-1 combined with sVCAM-1 in diagnosing renal injury. Results: The expressions of MCP-1 and SVCAM-1 in the observation group were higher than those in the control group (P<0.05). The expression levels of 24 h urinary protein (24 h Upro), cystatin C (Cys-C) and serum creatinine (SCr) in the observation group were higher than those in the control group (P<0.05). Pearson correlation analysis showed that the expression levels of MCP-1 and sVCAM-1 in serum of children with purpura nephritis were positively correlated with the expression levels of Upro, Cys-C and SCr at 24 hours (P<0.05). Among 108 children with purpura nephritis, 34 had renal injury. The expression levels of MCP-1 and sVCAM-1 in the renal injury group were higher than those in the non-renal injury group (P<0.05). ROC curve analysis showed that the AUC of serum MCP-1 combined with sVCAM-1 to determine renal injury in children with purpura nephritis was 0.862, which was higher than the single index of MCP-1(0.660) and sVCAM-1(0.663) (P<0.05). Conclusions: The expression levels of MCP-1 and SVCAM-1 in serum of children with purpura nephritis are related to the degree of renal involvement, the combination of MCP-1 and SVCAM-1 in the diagnosis of renal injury is effective, which provides a new reference for monitoring the evolution of the disease and is worthy of clinical attention. [ABSTRACT FROM AUTHOR]

Objective: To research the correlation of serum levels of IL-21. TGF-β1. TNF-α and IgAl in children with allergic purpura and the purpura nephritis. Methods: 57 cases with allergic purpura who were treated in our hosβ1tal from November 2015 to May 2016 were selected and 29 cases were diagnosed with general allergic purpura, and another 28 cases were diagnosed with purpuric nephritis. 30 healthy children were selected as the control group. Then the serum levels of EL-21. TGF-pl. TNF-α. IgAl. immunoglobulin A (IgA). C3 and C4 between the three groups were observed and compared. Results: The serum levels of IL-21. TGF-β1, TNF-α. IgAl and IgA in the purpuric nephritis group were higher than those of the control group and the general allergic purpura group, and the differences were statistically significant (P<0.05): The serum levels of IL-21. TGF-β1. TNF-α. IgAl and IgA in the general allergic purpura group were higher than those of the control group, and the differences were statistically significant (P<0.05): There was no statistically significant difference about the C3 and C4 in the three groups (P>0.05). Conclusion: The serum levels of IL-21. TGF-β1, TNF-α and IgAl may be involved in the development of henoch-schonlein purpura and purpura nephritis. [ABSTRACT FROM AUTHOR]

Objectives: To investigate the clinical characteristics, pathology, and prognosis of children with diffuse endocapillary proliferative Henoch-Schönlein purpura nephritis (DEP-HSPN)., Methods: A retrospective analysis was performed on the clinical, pathological, and prognosis data of 44 children with DEP-HSPN and 765 children without DEP-HSPN. The children with DEP-HSPN were diagnosed by renal biopsy in Jiangxi Provincial Children's Hospital from January 2006 to December 2021., Results: Among the 809 children with purpura nephritis, 44 (5.4%) had DEP-HSPN, with a mean age of (8±3) years, and there were 29 boys (65.9%) and 15 girls (34.1%). Compared with the non-DEP-HSPN group, the DEP-HSPN group had a significantly shorter time from onset to renal biopsy and a significantly higher proportion of children with respiratory infection or gross hematuria, and most children had nephrotic syndrome. The DEP-HSPN group had significantly higher levels of 24-hour urinary protein, urinary protein grading, microscopic hematuria grading, serum creatinine, and blood urea nitrogen and significantly lower levels of serum albumin and complement C3 ( P <0.05). The DEP-HSPN group had a higher pathological grading, with predominant deposition of IgA in the mesangial area and capillary loops, and higher activity scores in the modified semi-quantitative scoring system ( P <0.05). The Kaplan-Meier survival analysis showed that there was no significant difference in the renal complete remission rate between the two groups ( P >0.05)., Conclusions: Children with DEP-HSPN have a rapid onset, severe clinical manifestations and pathological grading, and high activity scores in the modified semi-quantitative scoring system. However, most of the children with DEP-HSPN have a good prognosis, with a comparable renal complete remission rate to the children without DEP-HSPN.

lthough the etiology of Henoch-Schönlein purpura (HSP) remains unclear, influenza vaccinations have been implicated as possible triggers for HSP. We describe four patients with HSP following influenza vaccinations which developed during the pandemic of influenza A (H1N1) 2009 and review the literature concerning HSP associated with this vaccine. HSP in patients developed in October and November, 2009. Four patients exhibited purpura, three patients complained of arthralgias, and one patient had both abdominal pains and renal involvement. Reviewing the literature, 11 patients with HSP following influenza vaccinations have been reported. Eight patients were children and five patients had past histories of immunologically mediated diseases including HSP, drug eruptions, and food allergy. While a favorable outcome was noted in most patients, one patient developed end-stage renal failure and another patient exhibited chronic glomerulonephritis. Although the precise reason for clustering of our patients with HSP following influenza vaccination was unclear, increasing use of the recent influenza vaccine associated with the pandemic of influenza A (H1N1) 2009 might explain this phenomenon. Because the incidence of HSP caused by influenza vaccination was very low, influenza vaccination should not be limited for this reason. However, caution may be required with its use in children with immunologically mediated diseases such as HSP. [ABSTRACT FROM AUTHOR]

Abstract: Generally, prognosis of hypertensive encephalopathy in childhood is favorable. We reported a 5-year-old girl who presented with a headache attack and EEG abnormalities after hypertensive encephalopathy due to purpura nephritis. The patient had suffered from hypertensive encephalopathy due to purpura nephritis, which soon ameliorated. Five months later, she developed attacks of headache, vomiting and disturbed consciousness with left side-predominant EEG abnormalities. Although carbamazepine and sodium valproate failed to improve her condition, zonisamide eradicated both the symptoms and EEG abnormalities, and an attack has not reoccurred for 5 years since completion of her treatment. It is noteworthy that delayed-onset complications can occur in child hypertensive encephalopathy, cases of which should be followed up prudently. Zonisamide should be considered for treatment of attacks of headaches with an epileptic character. [Copyright &y& Elsevier]

Renal involvement in Henoch-Schönlein purpura: A multivariate analysis of prognostic factors. This study is the first report in which the relationship between the progression of renal involvement in Henoch-Schönlein purpura (HSP) and various factors was evaluated using a multivariate analysis. Sixty-five (33.5%) of 194 patients with HSP developed renal involvement from three days to 17 months after the onset of the disease. The plasma coagulation factor XIII (F XIII) activity of 97 patients was examined, and 51 (54.3%) of them showed a decreased activity. A univariate analysis showed that an age at the onset of more than seven years, persistent purpura and a decreased F XIII activity all increased the risk of developing renal involvement. A Cox regression model analysis indicated severe abdominal symptoms, persistent purpura and decreased F XIII activity to be significant risk factors and their hazard ratios were 3.26, 11.53 and 2.27, respectively. Corticosteroid treatment had a hazard ratio of 0.36 and was considered to decrease the risk of developing renal involvement. Based on these findings, patients who have the risk factors for renal involvement should be treated with corticosteroids at the onset of the disease to prevent developing renal involvement. [ABSTRACT FROM AUTHOR]

Background: Given the importance of neutrophil recruitment in the pathogenesis of glomerulonephritis (GN), the representative neutrophil chemoattractant C-X-C motif chemokine 1 (CXCL1)/GROα and the adhesion molecule E-selectin in glomerular endothelial cells (GECs) play a pivotal role in the development of GN. Endothelial Toll-like receptor 3 (TLR3) is thought to be involved in the inflammatory response via innate immunity. However, the role of endothelial TLR3 signaling in the expression of neutrophil chemoattractants and adhesion molecules remains to be elucidated. Thus, we aimed to examine this issue., Methods: We treated normal human GECs with polyinosinic-polycytidylic acid (poly IC), an authentic double-stranded RNA, and analyzed the expressions of CXCL1 and E-selectin using quantitative real-time reverse transcription-polymerase chain reaction, western blotting, and enzyme-linked immunosorbent assay. To further elucidate the poly IC-induced signaling pathway, we subjected the cells to RNA interference against TLR3, interferon (IFN)-β, nuclear factor (NF)-κB p65, and IFN regulatory factor (IRF) 3. We also used immunofluorescence to examine the endothelial expression of CXCL1 in biopsy specimens from patients with crescentic and non-crescentic purpura nephritis (PN)., Results: We found that the activation of TLR3 induced the endothelial expression of CXCL1 and E-selectin, and that this involved TLR3, -NF-κB, IRF3, and IFN-β. Intense endothelial CXCL1 expression was observed in biopsy specimens from patients with crescentic PN., Conclusion: These findings support a role for glomerular antiviral innate immunity in the pathogenesis of GN. Intervention of glomerular TLR3 signaling may therefore be a suitable therapeutic strategy for treating GN in the future., (© 2018 S. Karger AG, Basel.)

Purpura nephritis and autosomal dominant polycystic kidney disease are relatively rare kidney disorders. We present a case complicated by these two diseases. A 68 year-old man with polycystic kidney disease was referred to our hospital with a high fever lasting 3 days and pyuria. Pyelonephritis was suspected based on computed tomography findings of bilateral swelling of the kidney. Inflammation subsided gradually after the initiation of antimicrobial therapy. However, approximately 3 weeks later, the patient developed a fever and skin purpura on the extremities, stomach colic pain, gross hematuria, and increased proteinuria was evident. Therefore, we diagnosed Henoch-Schönlein purpura complicated with nephritis based on biopsies of the skin and the kidney. Immunosuppressant therapy was administered; every symptom was relieved and proteinuria decreased for approximately 20 months.