| Objective:To explore the clinical manifestations,pathological features and risk factors related to the prognosis of IgA nephropathy?IgAN?in children.Methods:The clinical and pathological data of children with IgAN diagnosed in Children's Hospital of Chongqing Medical University from January 2003 to December 2018 were analyzed retrospectively.The prognosis of the children who were followed up for more than 1 year after biopsy was collected,including the prognosis of 1 year,more than 5 years and the last follow-up.The clinical outcome was divided into four grades:Grade A:clinical recovery?no hypertension,normal urine test and renal function?;Grade B:mild abnormal urine test,that is microscopic hematuria or mild proteinuria(<25mg·kg-1·d-1);Grade C:severe abnormal urine test,that is,gross hematuria or moderate and severe albuminuria(?25mg·kg-1·d-1),normal renal function,may be accompanied by hypertension;Grade D:renal insufficiency,dialysis or death.The relationship between clinical manifestations,pathological features and prognosis was analyzed.Results:1.524 children with IgAN accounted for 17.24%of the renal pathological biopsies in the same period,and the ratio of male to female was1.69:1.The average age was 9.67±2.75 years old,and the peak age of onset was 6-12 years old.2.54.01%of the children with IgAN had predisposing factors before onset,of which 88.34%were respiratory infections.The most common initial manifestation was macroscopic hematuria?285 cases/524 cases,54.39%?.The most common clinical classification was nephrotic syndrome type?251 cases/524 cases,47.90%?.3.Grade?was the most common type of Lee classification?263cases/495 cases,53.13%?.There were significant differences in the distribution of Lee classification among children with different clinical classification?P<0.001?.The main type of Lee classification in children with isolated hematuria type was grade?.In children with hematuria and proteinuria type,acute nephritis type,or nephrotic syndrome type,the main type of Lee classification was grade?.2 cases of acute rapidly progressive glomerulonephritis type were grade?.Lee classifications of chronic glomerulonephritis type were grade?and grade?.4.In Oxford classification,M1?320 cases/359cases,89.14%?and E1?183 cases/359cases,50.97%?were common,S1?64 cases/359 cases,17.83%?and T1/T2?7 cases/359 cases,1.95%?were rare.5.Among the 524 children with IgAN,319 cases were followed up for more than 1 year,with the follow-up time of 1.00-10.42 years,and the median follow-up time was 2.67 years.Among the 1-year follow-up nodes,149 cases?48.69%?had grade A,131 cases?42.81%?had grade B,22 cases?7.19%?had grade C,and 4 cases?1.31%?had grade D.Among the nodes followed up for more than 5 years,51 cases?68.92%?had grade A,17cases?22.97%?had grade B,6 cases?8.11%?had grade C,and no one followed-up to grade D was performed.Up to the last follow-up node,there were 194 cases?60.82%?with grade A,94 cases?29.46%?with grade B,25cases?7.84%?with grade C,6 cases?1.88%?with grade D.6.In univariate analysis,the IgA/C3 ratio?P=0.036?was correlated with the prognosis of 1-year follow-up.When renal tubular atrophy/interstitial fibrosis?T??P=0.008?lesions existed,the prognosis of1-year follow-up was worse.7.In univariate analysis,when segmental glomerulosclerosis?S?lesion existed,the prognosis of follow-up for more than 5 years was worse.Clinical classification?P=0.032?and renal C3 deposition intensity?P=0.019?were correlated with the prognosis of follow-up for more than 5 years.8.In univariate analysis,the higher the mean arterial pressure?MAP?at the initial diagnosis?P=0.007?,the presence of lesions including endocapillary hypercellularity?E??P=0.013?,segmental glomerulosclerosis?S??P=0.003?,and balloon adhesion?P=0.007?,the worse the prognosis at the last follow-up was.Lee classification?P=0.037?,the ratio of crescent formation?P=0.009?,and renal IgA deposition intensity?P=0.002?were correlated with the prognosis of the last follow-up.9.Logistic regression analysis showed that there were no independent risk factors for the prognosis of follow-up for 1 year and more than 5years?P>0.05?.Logistic regression analysis results showed that MAP at the initial diagnosis?P=0.011?,segmental glomerulosclerosis?S??P=0.33?,the ratio of crescent formation more than 50%?P=0.028?,and balloon adhesion formation?P=0.047?were independent risk factors for poor prognosis at the last follow-up of IgAN.Conclusion:Macroscopic hematuria was the most common first manifestation of children with IgAN in our center.Nephrotic syndrome type was the most common clinical classification,and grade?was the most common Lee classification.The distribution of Lee classification was different in children with different clinical classification.Oxford classifications of M1 and E1 were common,S1 and T1/T2 were rare.The short-term prognosis of children with IgAN was good,and there were still a few children with renal insufficiency.The ratio of IgA/C3 and renal tubular atrophy/interstitial fibrosis?T?were correlated with short-term prognosis of IgAN.Clinical classification,segmental glomerulosclerosis?S?and renal C3 deposition intensity were correlated with long-term prognosis of IgAN.MAP at the initial diagnosis,segmental glomerulosclerosis?S?,balloon adhesion,the ratio of crescent formation more than 50%were independent risk factors for poor prognosis in children with IgAN. |
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