Objective. The objective is to explore the aberrant sirtuin-6 (SIRT6) and Vanin-1 (VNN1) protein expression in peripheral blood monocytes (PBM) of children with primary nephrotic syndrome (PNS) and its diagnostic and prognostic values. Methods. 83 child patients with nephrotic syndrome (NS) and 65 healthy volunteers were enrolled in the study. The test of SIRT6 and VNN1 was performed by the Western blot. The receiver operator characteristic (ROC) curve was used to analyze the diagnostic and prognostic value of SIRT6 and VNN1 for child patients with NS. The logistic regression was used to analyze the association of SIRT6 and VNN1 with the prognosis of NS child patients. Results. SIRT6 in monocytes in the study group was inferior versus the control, while VNN1 outweighed it. The AUC of the combined detection of SIRT6 and VNN1 for the diagnosis of NS was 0.854, with a sensitivity of 80.0% and a specificity of 80.7%. The AUC of combined detection of SIRT6 and VNN1 for the prognosis of NS was 0.860, with a sensitivity of 84.6% and a specificity of 79.2%. The logistic regression analysis showed that less than 21.09 in SIRT6 was the number of risk factors for the prognosis of NS child patients ( P < 0.05 ). Conclusion. SIRT6 and VNN1 are provided with diagnostic and prognostic values for NS.
Background: Although the use of belimumab in children with lupus nephritis has increased over the past few years, there are limited data on the safety of belimumab in such patients with hypogammaglobulinemia. There are few reports of an association between hypogammaglobulinemia and infection in LN patients receiving belimumab treatment. Methods: We reviewed 27 patients with lupus nephritis and nephrotic-range proteinuria admitted to Hebei Children's Hospital from January 2019 to June 2022. In all 27 patients, 12 received intravenous (IV) belimumab (at a dose of 10 mg per kilogram of body weight) plus standard systemic lupus erythematosus (SLE) therapy (SoC) (belimumab group), and the other 15 received SoC (glucocorticoids plus cyclophosphamide or mycophenolate mofetil) (control group). Estimated SLEDAI-score, total amount of urine protein in 24 hours, the serum level of IgG, IgM, IgA and C3, total B lymphocyte count (BLC) , total white lymphocyte count (WBC), erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) level were measured 5 times (at week 0, 4, 12, 24 and 52, respectively) in two groups. Results: Hypogammaglobulinemia was observed in 22/27 (81.5%) participants prior to initiatial treatment of LN patients with nephrotic range proteinuria. Participants developed hypogammaglobulinemia by week 4, 5 patients in the belimumab group and 1 patient in control group received 1-2 IVIG treatments at weeks 16-26 due to severe or recurrent infections. The incidence of infection was significantly higher in patients in the belimumab group than in the control group, and the IVIG serum level was significantly lower than the control group. However, infection rates and serum IgG levels were not significantly different between the two groups at weeks 24 and 52. We also found that CRP level of patients in the belimumab group was significantly lower than in the control group at week 4 and week 24 respectively (P<0.05), and ESR level of patients in the belimumab group was also significantly lower than in the control group at week 12 (P<0.05). At week 52, WBC of patients in the belimumab group was significantly higher than in the control group(P<0.05). Conclusions: Hypogammaglobulinemia is a complication of refractory LN,obtaining IgG level before initiating belimumab in pediatric patients with refractory lupus nephritis,and close monitoring of hypogammaglobulinemia after belimumab use in pediatric patients. Immunoglobulin replacement therapy should be initiated as soon as possible if patients develop recurrent infections.
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