Long-term prognosis of primary focal segmental glomerulosclerosis in children.
	    		
		   		
		   			
		   		
	    	
    	 
    	10.3760/cma.j.cn112140-20220228-00161
   		
        
        	
        		- VernacularTitle:儿童原发性局灶节段性肾小球硬化症的长期预后分析
 
        	
        	
        	
        		- Author:
	        		
		        		
		        		
			        		Ying Chao PENG
			        		
			        		
			        		
			        			1
			        			
			        		
			        		
			        		
			        		
			        		;
		        		
		        		
		        		
			        		Chun Lin GAO
			        		
			        		
			        		
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			        		;
		        		
		        		
		        		
			        		Tao SUN
			        		
			        		
			        		
			        			1
			        			
			        		
			        		
			        		
			        		
			        		;
		        		
		        		
		        		
			        		Pei ZHANG
			        		
			        		
			        		
			        			1
			        			
			        		
			        		
			        		
			        		
			        		;
		        		
		        		
		        		
			        		Zheng Kun XIA
			        		
			        		
			        		
			        			1
			        			
			        		
			        		
			        		
			        		
			        		
		        		
		        		
		        		
		        		
		        			
			        		
			        		Author Information
			        		
		        		
		        		
			        		
			        		
			        			1. Department of Pediatrics, Jinling Hospital, the First School of Clinical Medicine, Southern Medical University, Nanjing 210002, China.
			        		
		        		
	        		
        		 
        	
        	
        	
        		- Publication Type:Journal Article
 
        	
        	
            
            	- MeSH:
            	
	        			
	        				
	        				
				        		
					        		Adolescent;
				        		
			        		
				        		
					        		Child;
				        		
			        		
				        		
					        		Female;
				        		
			        		
				        		
					        		Glomerulosclerosis, Focal Segmental/complications*;
				        		
			        		
				        		
					        		Humans;
				        		
			        		
				        		
					        		Hypertension;
				        		
			        		
				        		
					        		Kidney Failure, Chronic/etiology*;
				        		
			        		
				        		
					        		Male;
				        		
			        		
				        		
					        		Prognosis;
				        		
			        		
				        		
					        		Retrospective Studies;
				        		
			        		
				        		
					        		Sclerosis
				        		
			        		
	        			
	        			
            	
            	
 
            
            
            	- From:
	            		
	            			Chinese Journal of Pediatrics
	            		
	            		 2022;60(9):894-900
	            	
            	
 
            
            
            	- CountryChina
 
            
            
            	- Language:Chinese
 
            
            
            	- 
		        	Abstract:
			       	
			       		
				        
				        	Objective: To investigate risk factors for the long-term prognosis of primary focal segmental glomerulosclerosis (FSGS) and associated with renal prognosis in children. Methods: A retrospective study was conducted by collecting clinical data including general information, clinical features and renal pathological findings of 124 children with primary FSGS in Department of Pediatrics of Jinling Hospital from January 2003 to December 2019. The cumulative renal survival rate was calculated by Kaplan-Meier survival analysis. The risk factors related to renal prognosis were identified by Cox regression risk model analysis and receiver operating characteristic (ROC) curve. Results: Among 124 children, 94 were males (75.8%) and 30 were females (24.2%). The children were 16 (14, 17) years of age at the time of kidney biopsies. There were 102 cases (82.3%) aged from 13 to 18 years. The period of follow-up was 64.8 (32.1, 86.0) months. There were 49 cases (39.5%) with nonspecific variant, 33 cases (26.6%) with tip variant, 22 cases (17.7%) with collapsing variant, 14 cases (11.3%) with cellular variant and 6 cases (4.8%) with periportal variant. The data of Kaplan-Meier survival analysis showed that cumulative renal survival rates of end-stage kidney disease (ESKD) or ≥50% decline in estimated glomerular filtration rate (eGFR) from baseline at the year of 5, 10 and 15 after renal biopsies were 66.9%, 51.4% and 21.0% respectively. Multivariate Cox regression analysis showed that hypertension, glomerular segmental sclerosis ratio, moderate to severe chronic tubulointerstitial lesions were independent risk factors for progressing to ESKD or ≥50% reduction in eGFR from baseline in pediatric FSGS (HR=5.28, 1.03, 7.81, 95%CI 2.77-10.05, 1.01-1.04, 4.08-14.98, all P<0.01). ROC curve analysis showed glomerular segmental sclerosis ratio (AUC=0.734, P<0.05, optimal cut-off value=25.4%, sensitivity=50.0%, specificity=88.6%), moderate and severe chronic renal tubulointerstitial lesions (AUC=0.724, P<0.05, sensitivity=46.3%, specificity=98.6%) had good efficacy in evaluating renal outcomes of FSGS. Conclusions: The long-term prognosis of FSGS in children is poor. The risk factors of poor prognosis in children with FSGS are hypertension, moderate to severe chronic renal tubulointerstitial lesions and glomerular segmental sclerosis (≥25.4%).