Clinicopathological features and prognosis of IgA nephropathy with crescentic formation in children

Abstract

Objective To observe the clinical, pathological features and prognosis of IgA nephropathy in children with crescent formation. Methods A total of 38 cases of children with IgA nephropathy diagnosed by renal biopsy at Qilu Hospital of Shandong University were retrospectively analyzed. According to whether there were crescent formation, they were divided into crescents formation group (crescent formation≥10%, n=18) and control group consisting of children with complete clinical data and matching age, gender and course of disease (no crescent formation, n=20). The clinical, pathological features and prognosis of the two groups were compared. Results Fourteen cases (77.78%) of crescents formation group were associated with nausea hematuria, and 12 cases (66.67%) had hypertension, 8 patients in control group (40%) had gross hematuria, and 6 cases (30%) had high blood pressure. The differences were statistically significant (P<0.05). In crescents formation group, Alb and eGFR were significantly lower than those of control group. 24 h urine protein and Scr were significantly higher than those of control group and there were significant statistically differences between two groups (P<0.05). Nephropathological changes in crescent formation group were mainly in grade Ⅱ and grade Ⅲ and were usually combined with tubular interstitial damages. In addition to IgA immunoglobulin deposition, they often accompanied by IgG and IgM immunoglobulin deposition. In control group, nephropathological changes were mainly in grade Ⅰ and grade Ⅱand tubular interstitial damages were rare. IgA was the main form of deposition in control group. After 12 weeks steroid treatment and (or) immunosuppressive therapy, urinary protein turned negative in 7 cases in crescent formation group, 24 h urinary protein level declined by half than before in 7 cases and no obvious change in 4 cases. In control group, urinary protein turned negative in 12 cases, 6 cases showed a more than 50% decline in urinary protein level and 2 cases had no obvious change. During the 1-3 years of follow-up, renal function of 14 cases and 19 cases was normal in the crescent formation group and control group respectively, serum creatinine of 3 cases and 1 case increased mildly in the crescent formation group and control group respectively, 1 case needed dialysis treatment in the crescent formation group and the one in the control group who had slightly increasement of Scr did not reach the standard of dialysis treatment. Conclusions The clinical and pathological manifestations of children with IgA nephropathy with crescent formation are more severe and more likely to manifest renal insufficiency, suggesting that crescent formation is one of the indicators of poor prognosis. Key words: Glomerulonephritis, IgA; Children; Clinical pathology; Crescent; Prognosis