Division of Nephrology, Department of Medicine, St. Elizabeth's Medical Center, Boston, MA, USA; Department of Medicine, Tufts University School of Medicine, Boston, MA, USA.
Department of Neurology, Boston Medical Center, Boston, MA, USA; Department of Neurology, St. Elizabeth's Medical Center, Boston, MA, USA.
Am J Med Sci. 2023 Jun;365(6):538-544. doi: 10.1016/j.amjms.2023.03.011. Epub 2023 Mar 17.
Minimal change disease (MCD) is a well-known cause of fulminant acute nephrotic syndrome (NS) and has been associated with thrombotic complications. We report the case of a 51-year-old woman with previous biopsy-proven MCD in remission who presented with worsening headache and acute confusion shortly after a relapse of the NS and was diagnosed with cerebral venous thrombosis (CVT) complicated by intracranial hemorrhage and midline shift. One month prior, she had been initiated on an oral contraceptive agent during remission of the NS. After initiation of systemic anticoagulation, her condition rapidly deteriorated, and she passed away before being able to undergo catheter-based venous thrombectomy. We conducted a systematic literature review and identified 33 case reports of adults with NS-associated CVT. The most common symptoms were headache (83%), nausea or vomiting (47%), and altered mental status (30%). 64% of patients presented at time of initial diagnosis of the NS and 32% during a relapse. Mean urinary protein excretion was 9.32 g/day and mean serum albumin was 1.8 g/dL. 91% of patients received systemic anticoagulation, and 19% died. The outcome in the remaining cases was favorable with only one report (5%) of residual neurological deficit. Of the available kidney biopsy results, MCD was the most common diagnosis (70%), raising the hypothesis that the fulminant acute onset of the NS might be a predisposing factor for this serious thrombotic complication. Clinicians should have a high index of suspicion for CVT in patients with the NS who present with new-onset neurological symptoms, including headache and nausea.
微小病变病(MCD)是暴发性急性肾病综合征(NS)的已知病因,并与血栓并发症有关。我们报告了一例 51 岁女性,此前曾因 MCD 而进行活检并处于缓解期,在 NS 复发后不久出现头痛加重和急性意识混乱,并被诊断为伴有颅内出血和中线移位的脑静脉血栓形成(CVT)。一个月前,她在 NS 缓解期开始服用口服避孕药。在开始全身抗凝治疗后,她的病情迅速恶化,在能够进行基于导管的静脉血栓切除术之前去世。我们进行了系统的文献回顾,共确定了 33 例与 NS 相关的 CVT 成年患者的病例报告。最常见的症状是头痛(83%)、恶心或呕吐(47%)和精神状态改变(30%)。64%的患者在初次诊断 NS 时出现症状,32%在复发时出现症状。平均尿蛋白排泄量为 9.32g/天,平均血清白蛋白为 1.8g/dL。91%的患者接受了全身抗凝治疗,19%的患者死亡。其余病例的预后良好,只有一例(5%)报告有残留的神经功能缺损。在可获得的肾活检结果中,MCD 是最常见的诊断(70%),这提出了一个假设,即 NS 的暴发性急性发作可能是这种严重血栓并发症的一个诱发因素。对于出现新发神经症状(包括头痛和恶心)的 NS 患者,临床医生应高度怀疑 CVT。