Yu Xiao-Juan, Yu Feng, Song Di, Wang Su-Xia, Song Yan, Liu Gang, Zhao Ming-Hui
Renal Division, Department of Medicine, Peking University First Hospital, Institute of Nephrology, Peking University, Key Laboratory of Renal Disease, Ministry of Health of China, Key Laboratory of CKD Prevention and Treatment, Ministry of Education of China, Beijing 100034, China.
Department of Nephrology, The First Affiliated Hospital of Chinese PLA General Hospital, 51 Fucheng Road, Beijing 100048, China.
ScientificWorldJournal. 2014;2014:680502. doi: 10.1155/2014/680502. Epub 2014 Aug 11.
The current study aimed to investigate the spectrum of etiologies and associated disorders of renal biopsy-proven thrombotic microangiopathy (TMA) patients.
The clinical, laboratory, and renal histopathological data of patients with renal TMA from 2000 to 2012 in our institute were collected and reviewed.
One hundred and nine TMA patients were enrolled in this study. The mean age was 34.0 ± 11.1 years. Seventy patients (64.2%) were male and thirty-nine patients (35.8%) were female. There were eight patients (7.3%) with hemolytic uremic syndrome (HUS). Sixty-one patients (56.0%) were secondary to malignant hypertension. Fourteen patients (12.8%) were pregnancy-associated TMA. Other associated disorders included 17 patients with connective tissue disorders, 2 patients with hematopoietic stem cell transplantation, 4 patients with Castleman's disease, 1 patient with cryoglobulinemia, and 2 patients with glomerulopathy. During followup, 8 patients died due to severe infection, 17 patients had doubling of serum creatinine, and 44 had end-stage renal disease. In multivariate analysis, male, elevated serum creatinine, and decreased hemoglobin were independently associated with poor renal outcomes.
Renal TMA changes consisted of different disorders with various etiologies. aHUS, pregnancy-associated TMA, and malignant hypertension accounted for the majority of patients in our cohort.
本研究旨在调查经肾活检证实的血栓性微血管病（TMA）患者的病因谱及相关疾病。
收集并回顾了2000年至2012年我院肾TMA患者的临床、实验室及肾组织病理学数据。
本研究纳入了109例TMA患者。平均年龄为34.0±11.1岁。70例（64.2%）为男性，39例（35.8%）为女性。8例（7.3%）患有溶血性尿毒症综合征（HUS）。61例（56.0%）继发于恶性高血压。14例（12.8%）为妊娠相关TMA。其他相关疾病包括17例结缔组织病患者、2例造血干细胞移植患者、4例Castleman病患者、1例冷球蛋白血症患者和2例肾小球病患者。随访期间，8例患者死于严重感染，17例患者血清肌酐翻倍，44例患者进入终末期肾病。多因素分析显示，男性、血清肌酐升高和血红蛋白降低与不良肾脏结局独立相关。
肾TMA变化由不同病因的多种疾病组成。非典型溶血尿毒综合征（aHUS）、妊娠相关TMA和恶性高血压占我们队列中患者的大多数。
