Prognostic Factors in Children with Henoch-Schonlein Purpura Nephritis

PURPOSE: The long term disease course and prognostic factors were evaluated in childhood Henoch-Schonlein purpura nephritis(HSPN). METHODS: A total of 75 children(44 boys and 31 girls) with HSPN were included in this study. The onset age was 8.0+/-3.1 years(2.3-15.3 years), and the follow-up period was 4.3+/-3.6 years(1.0-17.1 years). Kidney biopsy was done in 24 children(32%). Initial clinical and laboratory findings were evaluated. In addition, polymorphisms of the renin angiotensin system(RAS) genes(insertion/deletion in intron 16 of ACE gene, M235T in AGT gene, and A1166C in AGTR gene) were analysed. The initial and last clinical states were classified into 4 groups as follows:A, normal; B, minor urinary abnormalities; C, active renal disease (nephrotic-range proteinuria and/or hypertension with serum creatinine < or =1.5 mg/dL); D, renal insufficiency. RESULTS: At the onset, the clinical states of the patients were B in 26(35%), C in 46(61%), and D, in 3(4%). The distribution of the RAS gene polymorphism of HSPN were not different from that of 100 healthy control subjects. At the last follow-up, the clinical states of the patients were A in 23(31%), B in 38(50%), C in 9(12%), and D in 5(7%). A multiple logistic regression identified age at the onset and initial urine protein excretion as significant prognostic factors. Analysis of genotypes of the 3 RAS genes as prognostic values revealed no statistical significance. CONCLUSION: Older age at onset and severe proteinuria were identified as poor prognostic factors of childhood HSPN. Implication of the RAS gene polymorphism in HSPN could not be validated in this small-scale retrospective study.

The Analysis of Renal Risk Factors in Henoch-Schonlein Purpura / 대한신장학회잡지

PURPOSE: Henoch-Schonlein purpura (HSP) is a multisystem disorder affecting predominantly skin, gastrointestinal tract, joint and kidneys, as well as the central nervous, cardiopulmonary and musculoskeletal system. Most patients with renal involvement have a good prognosis. However, some patients develop end-stage renal disease. Therefore, severity of renal involvement is considered to contribute to the outcome. The aim of this study was to evaluate the clinical renal risk and prognostic factors of HSP. METHODS: We had collected the clinical and laboratory data of 125 patients with acute HSP who visited Chungbuk National University Hospital from March 1992 to April 2002. Data were expressed as the mean+/-SD and statistical analysis was performed using Wilcoxon rank sum test, Mantel-Haenszel test, Fisher's Exact test, Student t-test. p<0.05 was considered as significant. RESULTS: The patient population consisted of 87 boys and 38 girls ranging from 1 to 14 years in age. Recurrance number of purpura in the HSP patients with renal involvement were significantly higher than those without renal involvement (p<0.01). 24-hour urine protein/creatinine ratio in the HSP patients with renal involvement were significantly higher than those without renal involvement (p< 0.01). But serum C3, C4, CH50, anti-streptolysin titers and so forth had no correlation with renal involvement. In the HSN patients, 24-hour creatinine and creatinine clearance have no correlation with renal involvement. Fifteen of 87 boys (17.2%) in this study developed scrotal involvement, which showed no significantly difference in patients with or without renal involvement. CONCLUSION: It is important that 24-hour urine protein/creatinine ratio at acute stage shows a significant relation with renal involvement. Results suggest that recurrence number of purpura are important to renal involvement in HSP. Based on these findings, futher prospective and/or controlled studies among more patients are thus necessary in order to prevent renal involvement in HSP.

Henoch-Schonlein Nephritis in Korean Children: Renal Outcome and Prognostic Factors / 대한신장학회잡지

Purpose: This retrospective study has been undertaken to find out the clinical outcome of children with Henoch-Schonlein nephritis and its relationship with initial clinical presentations and/or renal pathologic findings. METHODS: Study population consisted of 54 children with HS nephritis who have been admitted to the Pediatric department of Kyungpook University Hospital from 1993 to 2003, and biopsy was done with indications of heavy proteinuria (1 g/m2/day) lasting over 1 month, nephrotic syndrome, and persistent hematuria and/or proteinuria over 1 years. Patients were clinically divided into 3 groups; isolated hematuria, hematuria with proteinuria and heavy proteinuria (including nephrotic syndrome). Biopsy findings were graded from I-VI according to International Study of Kidney Disease in Children. RESULTS: Mean age of presentation was 7.9+/-2.7 years and slight female predominance was noted (24 boys and 30 girls). Histopathologic grading showed grade I in 5, grade II in 17, grade III in 29, and grade VI in 3 cases. Clinical outcome at the follow- up period less than 4 years (45 cases) and more than 5 years (18 cases) showed normal urinalysis in 17 (38%) and 10 (56%), persistent isolated hematuria in 14 (31%) and 1 (5%), hematuria with mild proteinuria in 14 (31%) and 7 (39%), respectively. No patients persist heavy proteinuria more than 3 years. Clinical outcome according to histopathologic grading showed that normalization of urinalysis was significantly lower in grade III when compared to grade II (p<0.05). Disappearance of proteinuria takes significantly longer in children with crescent formation (p<0.05). CONCLUSION: The majority of children with mild HS nephritis had good prognosis. The severity of histopathologic grade was well correlated with improvement of urinalysis. Our study suggests that renal biopsy provides a valuable prediction of prognosis even in mild HS nephritis without renal functional impairment.

A Case of Henoch-Schonlein Nephritis Mimicking Acute Poststreptococcal Glomerulonephritis in Histology

A girl aged 21 months with Henoch-Schonlein purpura(HSP) developed heavy proteinuria with hematuria 8 days after the appearance of purpuric rash, swelling and tenderness of both ankle joints. Her clinical and laboratory features demonstrated nephrotic and nephritic syndrome. The percutaneous renal biopsy revealed diffuse mesangial proliferative glomerulonephritis. Unlike usual HSP nephritis, immunoglobulin A deposition was not detected in the mesangium or the capillary of the glomeruli. Instead, numerous subepithelial electron-dense deposits("humps") mimicking acute poststreptococcal glomerulonephritis were found.

Clinical Study of the Correlation of Tumor Necrosis Factor alpha and the Proteinuria of Henoch-Schonlein Nephritis and Idiopathic Nephrotic Syndrome

PURPOSE: It is not clear that the development of glomerular injury and aggravation by tumor necrosis factor alpha (TNF-alpha) is related to intrarenal or serum concentration of TNF-alpha. So, we studied the relationship between the concentration of TNF-alpha and aggravation of glomerular damage in the Henoch-Schonlein nephritis(HSN) and idiopathic nephrotic syndrome(INS). METHODS: We collected the sera and urines of 21 patients with Henoch-Schonlein purpura(HSP) and 22 patients with INS visited Chungbuk National University hospital from March 1998 to March 2001. The concentration of TNF-alpha in the sera and urines were measured by sandwich ELISA. RESULTS: Serum TNF-alpha levels in the HSP patients with renal involvement were significantly higher than those without renal involvement(P=0.009). But urine TNF-alpha levels have no correlation with renal involvement(P=0.088). In the HSN patients, proteinuria have a significant correlation with serum TNF-alpha levels(P=0.004) but less correlation with urine TNF-alpha levels(P=0.053). Otherwise, proteinuria have no correlation with serum TNF-alpha levels(P=0.763) but have a significant correlation with urine TNF-alpha levels(P=0.007) in INS. CONCLUSION: These result suggest that the serum concentration of TNF-alpha would be important to glomerular involvement in HSP. And, it is interesting that proteinuria shows a significant relation with serum TNF-alpha levels in the HSN, but with urine TNF-alpha levels in the INS. This means the major production of TNF-alpha may be originated by extrarenal inflammation in the HSN and by intrarenal tubulo-interstitial damage due to proteinuria in the INS.

The analysis of clinical correlated factors of renal damage in henoch-schonlein purpura / 实用儿科临床杂志

Objective To study the clinical correlated factors of renal damage in henoch-schonlein purpura (HSP).Methods One hundred cases with HSP were retrospectively studied. Accoding to the urinary route examination, the patients were devided into 2 groups, HSP with (49 cases) and without renal damaged (51 cases). In addition, the group of HSP with renal damaged was further diveded into 2 groups, HSP with urinary abnormality passingly (27 cases) and persistently (22 cases). Clinical indicators were statistical analysed.Results To be compared with HSP without renal damaged, age,apread of the purpura, abdominal pain,hemorrhage of digestive tract were higher in HSP with renal damaged (P