ABSTRACT
Objective To compare the clinical features of diffuse capillary endothelial proliferative Henoch-Schonlein purpura nephritis ( DEP-HSPN ) with or without crescent formation. Methods The clinical data of 110 children with DEP-HSPN admitted to Dalian Central Hospital and the First Affiliated Hospital of Dalian Medical University from January 2008 to January 2018 were analyzed retrospectively. Among them,24 cases were divided into non crescentic group and 86 cases into crescentic group. The clinical characteristics and prognosis of the two groups were statistically analyzed. Results The clinical manifestations of children without crescent body formation group were all type III. There were 35 cases of type III and 51 cases of type V in the crescent body formation group. Compared with non-crescent formation group,the proportion of gross hematuria(83. 7%(72／86) vs. 29. 2%(7／24),χ2＝10. 396),urine red blood cell count ((112. 4±20. 3)／HP vs. (45. 2±10. 6)／HP,t＝9. 697),24 h urine protein ((2471. 6 ±242. 0) mg／d vs. (1358. 5±109. 3) mg／d,t＝6. 372) and serum creatinine (( 44. 9 ± 9. 6) μmol／L vs. (32. 3±5. 2) μmol／L,t＝5. 390) increased significantly,the serum albumin (( 22. 8±3. 8) g／L vs. ( 35. 1 ±5. 7) g／L,t＝4. 806)decreased significantly (all P＜0. 05). Both groups had non-simple IgA deposits in the mesangial region. The proportion of complete remission and asymptomatic hematuria was 70. 8%( 17／24) and 29. 2%( 7／24) in the non-crescent group, 58. 1%( 50／86) and 41. 9%( 36／86) in the crescent group,respectively,with no significant difference ( χ2＝1. 330,1. 196, all P＞0. 05) . Conclusion When DEP-HSPN is accompanied by crescent formation, gross hematuria, urinary erythrocyte count and the proportion of massive proteinuria increase significantly. Combined immunosuppressive therapy in acute stage and long-term sequential treatment in remission stage can achieve good prognosis.
ABSTRACT
Objective To investigate the clinical and pathological characteristics of Henoch-Sch?nlein purpura nephritis with diffuse capillary endothelial cell proliferation as pathological manifestation. Methods The clinical manifestations and pathology of capillary endothelial proliferative purpura nephritis (DEP-HSPN) diagnosed by renal biopsy were retrospectively analyzed in 50 children in recent 5 years. Results The pathological lesions in 50 cases included simple DEP-HSPN in 11 cases (7 males and 4 females) and capillary endothelial cell proliferation combined with crescents formation (non-simple DEP-HSPN) in 39 cases (27 males and 12 females). There was no significant difference in the course of disease and age between the two groups (P>0.05). The clinical type of 11 cases of simple DEP-HSPN was type Ⅲ. In 39 cases of non-simple DEP-HSPN, 16 cases were type Ⅲ and 23 cases were type Ⅴ. All of the children had hematuria and proteinuria. The incidence of gross hematuria, urine red blood cell count, 24 h urine protein, and serum creatinine levels in children with non-simple DEP-HSPN were significantly higher than those in simple DEP-HSPN group, but the plasma albumin level was significantly lower than that in simple DEP-HSPN group. It was easy to have crescent formation in DEP-HSPN, and the rate of crescent formation was 11.1％ (5.0％-27.6％). The incidence of segmental lesions and renal tubular interstitial damage was low. All children had non simple IgA deposits in the mesangial area. In the 50 children treated for 1 year, 22 had complete remission, 28 had asymptomatic hematuria, and none had active nephropathy and renal insufficiency. In 32 cases of non-simple DEP-HSPN, the 24 h urinary protein, plasma albumin level, and the incidences of gross hematuria and microscopic hematuria were statistically different before treatment and 1, 3, 6, 12 months after treatment (P<0.01). The 24 h urine protein and gross hematuria gradually decreased with the prolongation of treatment, while the level of plasma albumin was gradually increased. Conclusions DEP-HSPN is characterized by gross hematuria and proteinuria. The onset is acute and it is easy to have crescent formation. When combined with crescent formation, the clinical symptoms are more severe. The combination of strong immunosuppressive agents and long-term sequential follow-up treatment is effective in acute stage. The prognosis is good.
ABSTRACT
OBJECTIVE: To investigate the outcomes of childhood diffuse endocapillary proliferation Henoch-Schönlein purpura nephritis (DEP-HSPN) in response to early diagnosis and prompt treatment. METHODS: Eleven cases of DEP-HSPN in children were investigated in comparison to HSPN without diffuse endocapillary proliferation (non-DEP-HSPN). RESULTS: DEP-HSPN had a higher prevalence of nephrotic syndrome but a lower prevalence of hematuria compared to non-DEP-HSPN. IgA, IgG and IgM antibody deposition was found in DEP-HSPN by histopathological examination. Proteinuria cleared in all 11 cases through treatment with steroids and/or immunosuppressive drugs. However, half of the DEP-HSPN patients continuously had hematuria after treatment. CONCLUSION: The early diagnosis and prompt initiation of immunosuppressive treatment based on renal biopsy are important for achieving favorable outcomes.
Subject(s)
Humans , Male , Female , Child, Preschool , Child , Adolescent , Nephritis/drug therapy , Nephritis/pathology , IgA Vasculitis/drug therapy , IgA Vasculitis/pathology , Biopsy , Glucocorticoids/therapeutic use , Hematuria , Immunosuppressive Agents/therapeutic use , Kidney/pathology , Prednisone/therapeutic use , Proteinuria , Treatment OutcomeABSTRACT
Purpose To analyze clinicopathologic and prognostic features in 9 cases of children endocapillary proliferative glomerulone-phritis with hepatitis B virus antigen deposition ( HBV-ECPGN) . Methods Retrospective analysis of demographic information, clini-cal manifestations, laboratory parameters, pathological and prognostic features was carried out for 9 cases of HBV-ECPGN and 13 cases of acute poststreptococcal infection endocapillary proliferative glomerulonephritis ( APS-ECPGN) for comparison. Renal biopsy tissue were fixed in formalin and embedded in paraffin, stained with HE, PAS and PAM-Masson. Immunohistochemical study with EliVision method was performed. Three cases were submitted for electron microscopy. Results There were 7 males and 2 females ( M ∶ F=7 ∶ 2) of HBV-ECPGN. The median age was 10. 3 years. Serum C4 deposition ratio HBV-ECPGN was significantly greater than APS-ECPGN group (P<0. 05). There was an average of 11. 2 weeks of HBV-ECPGN kidney disease duration, which was significantly lon-ger than an average of 3. 8 weeks of APS-ECPGN group (P<0. 05). There was no disease relapse in all cases during 53. 55 months follow-up. C4d deposit was significantly stronger in all HBV-ECPGN cases compared with control group (APS-ECPGN cases). There were no significant differences in deposit of IgG, IgM, IgA, C3d and C1q between the two groups. HBsAg deposit in juxtaglomerular sites was identified in all cases. Conclusions Serum C4 decrease is more common in HBV-ECPGN than APS-ECPGN. Which may be associated with HBV infection, there is longer disease duration of HBV-ECPGN. C4d deposit is significantly stronger than control group, suggesting pathogenesis of HBV-ECPGN and APS-ECPGN is different. HBsAg deposit may be closely related to the pathogene-sis of HBV-ECPGN. HBsAg deposit in juxtaglomerular sites may be characteristic of HBV-ECPGN.