Pediatric Nephrology Unit, Department of Pediatrics, University of Florence, Florence, Italy.
Kidney Blood Press Res. 2010;33(5):399-404. doi: 10.1159/000320385. Epub 2010 Oct 14.
We retrospectively analyzed etiological, pathological and clinical features of the patients with hemolytic uremic syndrome (HUS) observed in the Pediatric Nephrology Unit at AOU Meyer of Florence. From January 1997 to December 2008, 22 cases were identified, with an annual incidence of 0.05 cases per 100,000 inhabitants, and 0.34 cases per 100,000 children <15 years old. 60% of the patients were D+ and 40% were D-, with an age distribution from 12 days to 13 years. Twenty patients (90%) had oligoanuria, lasting 6.4 ± 4 days for D+ patients versus 11.8 ± 4 days for D- patients. The development of chronic kidney disease positively correlates with the initial blood pressure value, the length of oligoanuria, and hospitalization. Microbiological investigations showed an association of D+HUS with different strains of Shiga toxin-producing Escherichia coli in 54% of the cases. D-HUS was associated with complement factor H deficiency in one patient. In the other cases, the triggering factors were pertussis, urinary tract infections and upper airway infections. While clinical and prognostic features correspond with literature data, in Tuscany the annual incidence is lower, and the percentage of D-HUS patients is higher than that observed in other studies.
我们回顾性分析了佛罗伦萨 AOU Meyer 儿科肾脏病科观察到的溶血性尿毒症综合征(HUS)患者的病因、病理和临床特征。1997 年 1 月至 2008 年 12 月,共确诊 22 例,发病率为每 10 万人 0.05 例,每 10 万 15 岁以下儿童 0.34 例。60%的患者为 D+,40%为 D-,年龄分布从 12 天至 13 岁。20 例(90%)患者少尿,D+患者持续 6.4±4 天,D-患者持续 11.8±4 天。慢性肾脏病的发展与初始血压值、少尿持续时间和住院时间呈正相关。微生物学调查显示,54%的 D+HUS 与不同株产志贺毒素大肠杆菌有关。D-HUS 与 1 例补体因子 H 缺陷有关。在其他病例中,触发因素为百日咳、尿路感染和上呼吸道感染。虽然临床和预后特征与文献数据相符,但在托斯卡纳,年发病率较低,D-HUS 患者的比例高于其他研究。