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Clinical And Pathological Findings And Prognosis Of Henoch-Schonlein Purpura Nephritis In Children

Posted on:2007-06-01Degree:MasterType:Thesis
Country:ChinaCandidate:H D FuFull Text:PDF
GTID:2144360212989988Subject:Academy of Pediatrics
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Background: Henoch-Schonlein purpura (HSP) is a leukocytoclastic vasculitis involving small vessels with the deposition of immune complexes containing IgA. Henoch-Schonlein purpura nephritis (HSPN) is an important complication of HSP, one of the most common secondary glomerulonephritis in children. HSPN is also the predictive factor of HSP's outcome. 1~5% HSPN maybe develop end-stage renal failure. 14% of end-stage renal failure in children caused by HSPN. Now the studies about HSPN are lack of large sample studies and long term follow up, also very few studies about the clinicopathologic findings and prognosis.Objective: To investigate the relationship between clinicopathologic findings and the prognosis in the HSPN children, therefore decrease the occurrence of end-stage renal failure and improve the life quality of the patients.Methods: This is a retrospective study. From January 1992 to February 2006. 159 cases who admitted to our nephrology department were diagnosed with HSPN. All the diagnosis of these 159 cases were confirmed by renal biopsy. The criteria of the diagnosis include: 1 .Hematuria and/or proteinuria in HSP children (mostly onset in 6 months after the diagnosis of HSP) 2.The immunofluorescence and electron microscopy analysis show IgA deposition (≥++). The pathologic classification wasbased on the criteria established by the International Study of Kidney Diseases in Children (ISKDC). The tubulointerstitial classification system proposed by Bohle and co-workers. The clinical data were collected. All the patients were followed in the clinic after discharge.Results: 1.clinical characteristics: 102 cases were male, 57 cases were female, male/female ratio: 1.79/1. Biopsy was done at age of 4 to 16 (9.38±2.58). All the patients were divided into five clinical groups: 74 cases (46.6%) manifested proteinuria and hematuria, 70 cases (44.0%) were nephrotic syndrome, 10 cases were actue nephritis, 4 cases were just simplified hematuria or proteinuria, one cases was rapidly progressive glomerulonephritis. 89 patients (56.0%) showed abnormal urinalysis in 1 week after the onset of HSP.56 cases (35.2%) had abnormal urinalysis in 1 week~1 month after the onset of HSP.109 cases had recurrent skin rashes. 2. Pathologic findings: AH the patients classified with ISKDC standard, most of them were grade II and III. 71 cases (44.7%) were grade II(IIa 28 cases, IIb 43 cases), 76 cases (47.8%) were grade III (IIIa 23 cases, IIIb 53 cases), 6 cases were grade I, 5 cases were grade IV, only one cases was grade V. The tubulointerstitial classification showed + in 75.5% patients, ++ in 20.1% patients. The study showed IgA deposit in 45 cases (28.3%), IgA+IgM deposit in 53 cases (33.3%), IgA+IgM+IgG deposit in 40 cases (25.2%), IgA+IgG deposit in 21 cases (13.2%). There were 128 patients (80.5%) showed C3 deposit as well. 68 cases (42.8%) were accompanied with Fib deposit. Immuro complex deposition and C3 deposition was not associated with pathologic classification. But tubulointerstitial changes were consistented with the degree of glomerular damage. 3. The relationship between clinic manifestation and pathologic findings: The gender, abdominal pain, the duration from onset of HSP to abnormal urinalysis was not associated with pathologic changes. The recurrence of skin rashes was related to the pathologic changes. Clinical type was associated with pathologic classification, severe clinical signs predicted severe pathologic impairment. Age, serum IgA level and C3 level was not related to pathologic classification. 4. Prognosis: All the patients were followed for 6 months to 14 years. 99 cases (62.3%) had normal renal function and urinalysis. Slightlyabnormal urinalysis in 49 cases (30.8%), 6 cases had progressive renal disease. Renal dysfunction in 5 cases (3.1%). The degree of pathologic impairment was associated with prognosis. Severe pathologic impairment predicted poor prognosis. Conclusion:1. The clinic classification of HSPN is associated with pathologic patterns, severe clinical signs predicted severe pathologic impairment. The recurrence of skin rashes was related to the pathologic changes. Tubulointerstitial changes are consistent with the degree of glomerular damage.2. Pathologic classification is a predictive factor of prognosis. Severe pathologic impairement indicates poor prognosis. Patients with grade III or more pathologic pattern need vigorous treatment and long term follow up.3. It is necessary and important for patient with HSPN who is suitable to renal biopsy in time. Since that we can know the classification of ISKDC, treat actively with different degree and improve the prognosis of patients.
Keywords/Search Tags:Henoch-Schonlein
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