Pediatric Nephrology Unit, Bordeaux University Hospital, Bordeaux, France.
Department of Pediatrics, Bayonne Medical Centre, Bayonne, France.
Pediatr Nephrol. 2019 Mar;34(3):533-537. doi: 10.1007/s00467-018-4174-1. Epub 2018 Dec 17.
Hemolytic uremic syndrome (HUS) has been associated with a number of infectious agents. We report here the case of an infant with severe Bordetella pertussis infection who developed HUS.
CASE DIAGNOSIS/TREATMENT: A 2-month-old preterm male was admitted for severe Bordetella pertussis infection. Symptoms leading to a diagnosis of hemolytic uremic syndrome (HUS) rapidly appeared: hemolytic anemia, thrombocytopenia, and acute kidney injury. He was treated with 25 days of peritoneal dialysis and received complement-targeting therapy with eculizumab (five injections over 2 months), in addition to blood transfusions, antibiotics, and respiratory support. The outcome was favorable. The genetic workup found a complement factor H gene variant which has been associated with atypical HUS. This variant was located in the C3b-binding site and functional tests revealed that it perturbed the regulatory activity of factor H.
This case suggests that pertussis is a strong trigger of HUS and that complement investigations are necessary to guide treatment and understand the pathophysiology.
溶血性尿毒症综合征（HUS）与许多感染因子有关。我们在此报告一例严重百日咳博德特氏菌感染婴儿发生 HUS 的病例。
病例诊断/治疗：一名 2 个月大的早产男婴因严重百日咳博德特氏菌感染入院。导致溶血性尿毒症综合征（HUS）诊断的症状迅速出现：溶血性贫血、血小板减少和急性肾损伤。他接受了 25 天的腹膜透析治疗，并接受了 eculizumab（2 个月内注射 5 次）的补体靶向治疗，此外还进行了输血、抗生素和呼吸支持治疗。结果良好。基因检测发现了一种补体因子 H 基因突变，该突变与非典型 HUS 有关。该变体位于 C3b 结合位点，功能测试显示它扰乱了因子 H 的调节活性。
该病例提示百日咳是 HUS 的强烈诱因，需要进行补体检查以指导治疗并了解病理生理学。
