CAJ | 학술논문

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 as-sociated 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 pro-liferative 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EliVision法IgG、IgA、IgM、C3d、C4d、C1q、HBsAg、HBcAg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),IgG、IgM、IgA、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HBsAg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C4 d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HBsAg沉积有关,HBsAg沉积则可能与HBV-ECPGN发病机制相关；HBsAg在肾小球旁器部位的沉积是HBV-ECPGN的特征性标志。
목적：탐토인동모세혈관내증생성신소구신염반을형간염병독항원침적( endocapillary proliferative glomerulonephritis as-sociated with hepatitis B virus antigen deposition, HBV-ECPGN)적림상병리특정급예후。방법회고성분석9례HBV-ECPGN (실험조)여13례인동경전급성련구균감염후모세혈관내증생성신소구신염( acute poststreptococcal infection endocapillary pro-liferative glomerulonephritis, APS-ECPGN)(대조조)적림상표현、실험실삼수、신병병정화림상예후등。신천자표본균경광경、면역조화검사,3례동시송전경검사。광경검사포괄석사절편HE、PAS화PAM-Masson염색,면역조화EliVision법IgG、IgA、IgM、C3d、C4d、C1q、HBsAg、HBcAg염색。결과실험조9례중남녀비위7∶2,평균년령10.3세；혈청학C4하강비례명현대우대조조(P<0．05)；실험조신병병정평균11.2주,명현장우대조조(평균3．8주)(P<0．05)；실험조평균수방53.55개월,균미견신염복발。면역조화C4d침적명현고우대조조(P<0．05),IgG、IgM、IgA、C3d화C1q적침적량조상비차이무현저성(P>0．05)；소유실험조병례균유HBsAg침적,침적부위이신소구방기부위최위명현。결론여APS-ECPGN불동,HBV-ECPGN경다견혈청학C4하강화경강적신조직내C4 d침적,림상상다이급성신염발병,신병병정교장。제시이자재발병궤제상존재차이。혈청학C4하강가능여HBsAg침적유관,HBsAg침적칙가능여HBV-ECPGN발병궤제상관；HBsAg재신소구방기부위적침적시HBV-ECPGN적특정성표지。
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.