Risk factors for renal outcomes in children with antineutrophil cytoplasmic antibody-associated vasculitis: a nationwide retrospective study in China.

BACKGROUND: Pediatric antineutrophil cytoplasmic antibody-associated vasculitis (AAV) is a life-threatening systemic vasculitis featured by liability to renal involvement. However, there are few studies on the risk factors and predictive models for renal outcomes of AAV in children. METHODS: Data from 179 AAV children in multiple centers between January 2012 and March 2020 were collected retrospectively. The risk factors and predictive model of end-stage renal disease (ESRD) in AAV were explored. RESULTS: Renal involvement was the most typical manifestation (95.5%), and the crescent was the predominant pathological lesion (84.9%). The estimated glomerular filtration rate (eGFR) was evaluated in 114 patients, of whom 59.6% developed ESRD, and the median time to ESRD was 3.20 months. The eGFR [P = 0.006, odds ratio (OR) = 0.955, 95% confidence interval (CI) = 0.924-0.987] and the percentages of global glomerulosclerosis (pGGS; P = 0.018, OR = 1.060, 95% CI = 1.010-1.112) were independent risk factors for ESRD of renal biopsy. Based on the pGGS and eGFR at renal biopsy, we developed three risk grades of ESRD and one predictive model. The Kaplan‒Meier curve indicated that renal outcomes were significantly different in different risk grades (P < 0.001). Compared with serum creatinine at baseline, the predictive model had higher accuracy (0.86 versus 0.58, P < 0.001) and a lower coefficient of variation (0.07 versus 0.92) in external validation. CONCLUSIONS: Renal involvement is the most common manifestation of pediatric AAV in China, of which more than half deteriorates into ESRD. The predictive model based on eGFR at renal biopsy and the pGGS may be stable and accurate in speculating the risk of ESRD in AAV children. Supplementary file 2 (MP4 18937 KB).

Axial length, vitreoretinal pathology, and anterior chamber depth can predict postoperative refractive outcomes in phacovitrectomy/silicone oil removal.

AIM: To evaluate the postoperative refractive prediction error (PE) and determine the factors that affect the refractive outcomes of combined pars plana vitrectomy (PPV) or silicone oil removal (SOR) with cataract surgery. METHODS: The study is a retrospective, case-series study. Totally 301 eyes of 301 patients undergoing combined PPV/SOR with cataract surgery were enrolled. Eligible individuals were separated into four groups according to their preoperative diagnoses: silicone oil-filled eyes after PPV (group 1), epiretinal membrane (group 2), macular hole (group 3), and primary retinal detachment (RD; group 4). The variables affecting postoperative refractive outcomes were analyzed, including age, gender, preoperative best-corrected visual acuity (BCVA), axial length (AL), keratometry average, anterior chamber depth (ACD), intraocular tamponade, and vitreoretinal pathology. The outcome measurements include the mean refractive PE and the proportions of eyes with a PE within ±0.50 diopter (D) and ±1.00 D. RESULTS: For all patients, the mean PE was -0.04±1.17 D, and 50.17% of patients (eyes) had a PE within ±0.50 D. There was a significant difference in refractive outcomes among the four groups (P=0.028), with RD (group 4) showing the least favorable refractive outcome. In multivariate regression analysis, only AL, vitreoretinal pathology, and ACD were strongly associated with PE (all P<0.01). Univariate analysis revealed that longer eyes (AL>26 mm) and a deeper ACD were correlated with hyperopic PE, and shorter eyes (AL<26 mm) and a shallower ACD were correlated with myopic PE. CONCLUSION: RD patients have the least favorable refractive outcome. AL, vitreoretinal pathology, and ACD are strongly associated with PE in the combined surgery. These three factors affect refractive outcomes and thus can be used to predict a better postoperative refractive outcome in clinical practice.

Association between HLA alleles and sub-phenotype of childhood steroid-sensitive nephrotic syndrome.

BACKGROUND: Few studies have addressed the effects of human leukocyte antigen (HLA) alleles on different clinical sub-phenotypes in childhood steroid-sensitive nephrotic syndrome (SSNS), including SSNS without recurrence (SSNSWR) and steroid-dependent nephrotic syndrome/frequently relapse nephrotic syndrome (SDNS/FRNS). In this study, we investigated the relationship between HLA system and children with SSNSWR and SDNS/FRNS and clarified the value of HLA allele detection for precise typing of childhood SSNS. METHODS: A total of 241 Chinese Han individuals with SSNS were genotyped using GenCap-WES Capture Kit, and four-digit resolution HLA alleles were imputed from available Genome Wide Association data. The distribution and carrying frequency of HLA alleles in SSNSWR and SDNS/FRNS were investigated. Additionally, logistic regression and mediating effects were used to examine the relationship between risk factors for disease process and HLA system. RESULTS: Compared with SSNSWR, significantly decreased serum levels of complement 3 (C3) and complement 4 (C4) at onset were detected in SDNS/FRNS (C3, P < 0.001; C4, P = 0.018). The average time to remission after sufficient initial steroid treatment in SDNS/FRNS was significantly longer than that in SSNSWR (P = 0.0001). Low level of C4 was further identified as an independent risk factor for SDNS/FRNS (P = 0.008, odds ratio = 0.174, 95% confidence interval 0.048-0.630). The HLA-A*11:01 allele was independently associated with SSNSWR and SDNS/FRNS (P = 0.0012 and P = 0.0006, respectively). No significant HLA alleles were detected between SSNSWR and SDNS/FRNS. In addition, a mediating effect among HLA-I alleles (HLA-B*15:11, HLA-B*44:03 and HLA-C*07:06), C4 level and SDNS/FRNS was identified. CONCLUSIONS: HLA-I alleles provide novel genetic markers for SSNSWR and SDNS/FRNS. HLA-I antigens may be involved in steroid dependent or frequent relapse in children with SSNS as mediators of immunoregulation.