McGraw Kathryn E, Porter Amanda P, Moffitt Alyssa M, Golden Marina E M, Stewart Heather
Mercer University School of Medicine, Savannah, GA.
Memorial Health University Medical Center, Savannah, GA.
HCA Healthc J Med. 2024 Aug 1;5(4):459-464. doi: 10.36518/2689-0216.1669. eCollection 2024.
Atypical hemolytic uremic syndrome (aHUS) is a thrombotic microangiopathy that presents with a triad of hemolytic anemia, thrombocytopenia, and acute kidney impairment. It can be attributed to mutations in an array of different complement proteins leading to the overactivation of the complement system, the most impacted being the alternative pathway. Though rare, influenza B has been documented as a potential trigger to the development of aHUS.
We discuss a 10-year-old girl with a history of aHUS who was found to have a repeat episode of aHUS following an influenza B infection. There have only been a few reports of aHUS triggered by influenza B, making this a unique case. Given the recurrence and atypical features present in this case, a genetic workup was obtained, which showed a heterozygous mutation of complement protein CD46. The presence of mutations in CD46 is a known predisposing factor to aHUS, but influenza B infection is rarely implicated as a trigger to aHUS. The prognosis of aHUS varies and is dependent on the complement mutation specific to the individual.
Patients with CD46 mutations have been shown to have high rates of relapse but less long-term kidney damage, as seen in this case. Clinicians should be aware of the association between influenza B and aHUS to improve patient outcomes.
非典型溶血性尿毒症综合征(aHUS)是一种血栓性微血管病,表现为溶血性贫血、血小板减少和急性肾损伤三联征。它可归因于一系列不同补体蛋白的突变,导致补体系统过度激活,受影响最大的是替代途径。虽然罕见,但乙型流感已被记录为aHUS发生的潜在触发因素。
我们讨论一名有aHUS病史的10岁女孩,她在感染乙型流感后再次出现aHUS发作。仅有少数关于乙型流感引发aHUS的报道,因此这是一个独特的病例。鉴于该病例中存在复发和非典型特征,进行了基因检查,结果显示补体蛋白CD46存在杂合突变。CD46突变的存在是aHUS的已知易感因素,但乙型流感感染很少被认为是aHUS的触发因素。aHUS的预后各不相同,取决于个体特异性的补体突变。
如本病例所示,已证明携带CD46突变的患者复发率高,但长期肾损害较少。临床医生应了解乙型流感与aHUS之间的关联,以改善患者预后。