【摘要】 目的　探讨C1q肾病的诊断和鉴别诊断。方法　分析 8例C1q肾病患儿肾活检组织中C1q沉积的发生率、强度和分布特点 ,并与同期 3 5 2例原发性和 42例继发性肾小球疾病作比较。结果　 8例C1q肾病患儿系膜区均有显著的C1q沉积 ,阳性率为 10 0 % ,平均沉积强度达 2 1+ ;2 0例狼疮性肾炎患儿中 ,13例系膜区有显著的C1q沉积 ,阳性率为 65 % ,平均沉积强度达 1 7+ ;2 2例乙肝病毒相关性肾炎中 ,5例系膜区有显著的C1q沉积 ,阳性率为 2 3 % ,平均沉积强度达 0 5 +。在原发性肾小球疾病中 ,5例膜增殖性肾小球肾炎患儿中的 3例系膜区有显著的C1q沉积 ,阳性率为 60 % ,平均沉积强度达 1 6+ ;其他病理类型的患儿均缺乏。结论　儿童C1q肾病的诊断除依赖于免疫荧光发现系膜区有显著的C1q沉积外 ,尚需与狼疮性肾炎、乙肝病毒相关性肾炎和膜增殖性肾小球肾炎相鉴别更多还原

【Abstract】 Objective The evidence of predominant C1q deposits in mesangial regions is essential to diagnose C1q nephropathy, but a few reports have indicated that the changes were also found in lupus nephritis (LN) and membranoproliferative glomerulonephritis (MPGN). In this study, C1q immunostainings were preformed in various kinds of immune-complex-mediated glomeruonephritis in order to clarify the deposition of C1q in the other kind of glomerular diseases which will be helpful to improve the diagnosis and differential diagnosis of C1q nephropathy. Methods Five hundreds renal biopsy specimens were collected in children with kidney diseases from 1995-2000, including 8 cases of C1q nephropathy, 42 cases of secondary kidney diseases (SKD) and 450 cases of idiopathic kidney diseases (IKD). In 42 children with SKD, 20 cases were diagnosed as LN, others as hepatitis B virus associated glomerulonephritis (HBV-GN). In 450 children with IKD, the pathologic types included 137 cases of minor change (MC), 45 cases of focal segmental glomerulosclerosis (FSGS), 5 cases of membranoproliferative glomerulonephritis (MPGN), 165 cases of mesangial proliferative glomerulonephritis (MsPGN, including IgM and IgA nephropathy) and 98 cases of other pathologic types. All renal specimens were detected under light microscope, electron microscope and immunofluorescence microscope. The incidence, distribution and intensity of C1q location were analyzed and compared among different diseases. Results Intense C1q immunostainings were found in mesangial regions in all 8 cases with C1q nephropathy, the incidence was 100%, the average fluorescence intensity was 2.1+. In 20 cases with LN, 13 cases had the bright C1q immunostainings in mesangial regions, the incidence reached 65%, the average fluorescence intensity was 1.7+. In 22 cases with HBV-GN, only 5 cases had similar intense C1q deposits in mesangial regions, the incidence reduced to 23%, the average fluorescence intensity decreased to 0.5+. In 450 cases of IKD, no C1q immunostainings were observed in other pathologic types except for 3 of 5 cases of MPGN, the incidence in MPGN was 60%, the average fluorescence intensity was 1.6+. Conclusion The diagnosis of C1q nephropathy depends on the predominant C1q deposits in mesangial regions by immunofluoresence findings with the exclusion of LN, HBV-GN and MPGN in advance.更多还原