儿童毛细血管内增生性肾小球肾炎伴乙型肝炎病毒抗原沉积9例临床病理分析

Endocapillary proliferative glomerulonephritis with hepatitis B virus antigen deposition in children: a clinicopathologic analysis of 9 cases

目的探讨儿童毛细血管内增生性肾小球肾炎伴乙型肝炎病毒抗原沉积(endocapillary proliferative glomerulonephritis associated with hepatitis B virus antigen deposition,HBV-ECPGN)的临床病理特征及预后。方法回顾性分析9例HBV-ECPGN(实验组)与13例儿童经典急性链球菌感染后毛细血管内增生性肾小球肾炎(acute poststreptococcal infection endocapillary proliferative glomerulonephritis,APS-ECPGN)(对照组)的临床表现、实验室参数、肾病病程和临床预后等。肾穿刺标本均经光镜、免疫组化检查,3例同时送电镜检查。光镜检查包括石蜡切片HE、PAS和PAM-Masson染色,免疫组化Eli Vision法Ig G、Ig A、Ig M、C3d、C4d、C1q、HBs Ag、HBc Ag染色。结果实验组9例中男女比为7∶2,平均年龄10.3岁;血清学C4下降比例明显大于对照组(P<0.05);实验组肾病病程平均11.2周,明显长于对照组(平均3.8周)(P<0.05);实验组平均随访53.55个月,均未见肾炎复发。免疫组化C4d沉积明显高于对照组(P<0.05),Ig G、Ig M、Ig A、C3d和C1q的沉积两组相比差异无显著性(P>0.05);所有实验组病例均有HBs Ag沉积,沉积部位以肾小球旁器部位最为明显。结论与APS-ECPGN不同,HBV-ECPGN更多见血清学C4下降和更强的肾组织内C4d沉积,临床上多以急性肾炎发病,肾病病程较长。提示二者在发病机制上存在差异。血清学C4下降可能与HBs Ag沉积有关,HBs Ag沉积则可能与HBV-ECPGN发病机制相关;HBs Ag在肾小球旁器部位的沉积是HBV-ECPGN的特征性标志。

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.