Rong L P, Chen L Z, Fu Q, Chen W F, Sun L Z, Jiang X Y, Mo Y
Department of Pediatrics, the First Affiliated Hospital of Sun Yat-sen University, Guangzhou 510080, China.
Zhonghua Er Ke Za Zhi. 2016 Dec 2;54(12):936-940. doi: 10.3760/cma.j.issn.0578-1310.2016.12.013.
To study the clinical features and treatment of focal segmental glomerulosclerosis (FSGS) after renal transplantation in a child with ANCA-associated glomerulonephritis. The clinical and pathological data of the patient treated in the Department of Pediatrics as well as in the Department of Organ Transplantation in November 2015 in the First Affiliated Hospital of Sun Yat-sen University, who was diagnosed with de novo FSGS after renal transplantation with a primary disease ANCA-associated glomerulonephritis, was analyzed retrospectively. Reports on "ANCA-associated glomerulonephritis" "(renal OR kidney) transplantation" "focal segmental glomerular sclerosis" were searched and reviewed. A ten years old female was definitely diagnosed with ANCA-associated glomerulonephritis on the 81 day after the onset of primary ANCA associated glomerulonephritis. Because of progressive decline of renal function, a hemodialysis period for 7 months was administered following the pulsed methylprednisolone as well as cyclophosphamide treatment. The renal transplantation was then carried out 18 months later, the renal function recovered 7 days later while proteinuria reappeared 28 days after renal transplantation. Based on the anti-rejection treatment, 3 times pulsed methylprednisolone administration did not make difference on reducing the proteinuria and then a renal biopsy was conducted and the transplanted kidney proved to be a newly developed FSGS. Consequently, plasma exchange therapy was administrated. When the plasma exchange course finished, the proteinuria decreased significantly (from 3.270 g/24 h to 0.370 g/24 h). No reports were retrieved either in Chinese databases or at PubMed as well as Medline databases. FSGS appears in transplanted kidney in patient with a primary renal disease as ANCA associated glomerulonephritis with early proteinuria after transplantation as well as negative P-ANCA and MPO. Pathology of renal biopsy revealed FSGS while the pathology of other recipient was not FSGS. The patient had no response to pulsed methylprednisolone therapy. Instead, plasma exchange therapy was an alternative also effective treatment for de novo FSGS in transplanted kidney.
研究儿童抗中性粒细胞胞浆抗体(ANCA)相关性肾小球肾炎肾移植后局灶节段性肾小球硬化(FSGS)的临床特征及治疗方法。回顾性分析2015年11月在中山大学附属第一医院儿科及器官移植科诊治的1例原发性疾病为ANCA相关性肾小球肾炎、肾移植后诊断为新发FSGS患者的临床及病理资料。检索并复习关于“ANCA相关性肾小球肾炎”“(肾或肾脏)移植”“局灶节段性肾小球硬化”的报道。1例10岁女性在原发性ANCA相关性肾小球肾炎发病81天后确诊。因肾功能进行性下降,在给予甲泼尼龙冲击及环磷酰胺治疗后进行了7个月的血液透析。18个月后进行肾移植,术后7天肾功能恢复,但肾移植后28天再次出现蛋白尿。在抗排斥治疗基础上,3次甲泼尼龙冲击治疗对降低蛋白尿无明显效果,随后进行肾活检,移植肾证实为新发FSGS。因此,给予血浆置换治疗。血浆置换疗程结束后,蛋白尿明显下降(从3.270 g/24 h降至0.370 g/24 h)。在中国数据库以及PubMed和Medline数据库中均未检索到相关报道。FSGS出现在原发性肾脏疾病为ANCA相关性肾小球肾炎的肾移植患者中,移植后早期出现蛋白尿,且抗蛋白酶3(P-ANCA)和髓过氧化物酶(MPO)阴性。肾活检病理显示为FSGS,而其他受者的病理并非FSGS。该患者对甲泼尼龙冲击治疗无反应。相反,血浆置换治疗是移植肾新发FSGS的一种有效替代治疗方法。