Clinical and Pathological Findings and Prognosis of Henoch-Schonlein Purpura Nephritis in Children
|Keywords||Pathological grade Crescentic Proteinuria The extent of damage Irritability Clinical manifestations Pathological type Clinical classification Zhejiang University Nephrotic syndrome|
Background: allergic purpura caused kidney damage known as allergic purpura nephritis (HSPN), is the children's secondary glomerular diseases. 1% to 5% may progress to end-stage renal failure. Accounted for 14% of the children with end-stage renal failure. The lack of a large sample of children allergic purpura nephritis, and also the lack of long-term follow-up results, clinical manifestations, the lack of a more comprehensive study on the pathological type and prognosis. Objective: In order to understand the intrinsic relationship between the children HSPN in clinical, pathological, and prognostic characteristics, and their mutual and improve understanding of the disease, and provide lessons for future diagnosis, treatment. In order to more effectively reduce the occurrence due the HSPN cause of end-stage renal failure, and to improve the prognosis of patients. Methods: The study is from January 1992 to February 2006 in the subsidiary of the Children's Hospital of Zhejiang University School of Medicine, Department of Nephrology, hospital, clinical diagnosis of HSPN, and renal biopsy HSPN children with pathologically confirmed, a total of 159 cases. HSPN diagnostic criteria for the in allergic purpura course (the majority of hematuria and (or) proteinuria and kidney tissue immunofluorescence examination membrane zone IgA deposition (IgA fluorescence brightness ≥) in six months), and the exclusion of systemic lupus erythematosus, chronic liver disease. Retrospective collection and analysis in children with clinical and pathological data and follow-up of their disease outcome. Clinical classification standards developed according to the Pediatric Branch of the Chinese Medical Association nephrology group (2000.11. Zhuhai). The pathological grading standard developed by the International Children Society of Nephrology (ISKDC). Tubular - interstitial pathological changes take Bohle A grade. Basis Counahan develop standards for prognosis. Case-control study, the analysis of the relationship between the clinical, pathological, prognostic features. Results: 1. Clinical features: 102 male and 57 females. Biopsy in age from 4 to 16 years old. Clinical classification for hematuria and proteinuria 74 cases, accounting for 46.6%; 70 cases of nephrotic syndrome, accounting for 44.0%; 10 cases of acute glomerulonephritis type, accounting for 6.3%; isolated hematuria or simple protein urinary type 4 cases; rapidly progressive glomerulonephritis type 1 cases. 89 cases (56.0%) urine abnormalities in the pathogenesis of a week, have urinary abnormalities between one week to January in 56 cases (35.2%). There are 109 cases (68.6%), rash repeated episodes of many. 2. Pathological features: mainly basic damage varying degrees of mesangial cell proliferation and matrix increase, can be divided into focal and diffuse glomerular lesions. Glomerular crescent formation or segmental lesions (sclerosis, adhesions, thrombosis, necrosis). ISKDC the standard Ⅱ, Ⅲ grade up, which Ⅱ level there are 71 cases, accounting for 44.7% (Ⅱ a28 cases, Ⅱ b43 patients), grade Ⅲ 76 cases, accounting for 47.8% (Ⅲ a23 cases, Ⅲ b53 cases), Ⅰ grade 6 cases, Ⅳ grade five cases, Ⅴ. Small tube - the qualitative pathological type display level accounted for 75.5%, followed by 20.1% for the class. Immune deposition type shows simply IgA type, and the proportion is closer to IgA IgM type IgA IgM IgG type, 45 cases (28.3%), 53 cases (33.3%), 40 cases (25.2%). IgA IgG, 21 cases (13.2%). 128 cases (80.5%) with C3 deposition, 68 cases (42.8%) with Fib deposition, and associated with the C4, C1q deposition rare, each one cases. Immune deposition type C3 deposition no correlation with the pathological grade (P> 0.05). Tubule - interstitial the typing ISKDC pathological grading positive correlation (P <0.01), a small tube - the more serious interstitial damage, then ISKDC grading heavier. 3. Urine abnormalities time from onset to clinical and pathological relations: gender, abdominal pain and other symptoms, and no correlation with the pathological grade (P> 0.05). Occurrences of skin purpura positive correlation (P <0.05) and pathological damage. Purpura many pathological damage compared to purpura occurs only once. Between clinical classification and pathological grade correlation (P <0.01), the more serious clinical manifestations, the pathological extent of the damage is more serious. No correlation between age and serum IgA levels, serum C3 levels correlated with pathological grading (P> 0.05). 4. Prognosis: regular follow-up and follow-up period of 6 months to 14 years. Follow-up results: A normal 99 cases, accounting for 62.3%; B mild urinary abnormalities 49 cases, accounting for 30.8%; C activity of six cases of kidney disease, accounting for 3.8%; D renal insufficiency in 5 patients (3.1%). The extent of the damage and the prognosis of kidney disease management was a positive correlation, P <0.01, the more serious pathological damage, poor prognosis the greater the risk. Conclusion: 1. Anaphylactoid purpura nephritis clinical type correlation with pathological grade, the more severe clinical manifestations, the degree of renal impairment is relatively heavier. The number of occurrences of skin purpura correlation with pathological grading, purpura occurred several pathological extent of the damage is relatively heavy. Tubule - between mass the pathological grade ISKDC grading a correlation. Tubule - interstitial damage severe is relatively heavy ISKDC classification. 2. Allergic purpura nephritis pathological grade prognosis correlation, the more serious pathological damage, poor prognosis greater the risk. Pathology for the above Ⅲ grade III (special cases Ⅳ - Ⅴ grade) should actively treated, long-term follow-up. 3. Timely kidney suitable allergic purpura nephritis cases should wear, and understanding of the clinical and pathological grade, to the decentralized treatment and improve the prognosis.