Ferjani Maryem, Zaimi Yosra, Trad Nouha, Hammi Yousra, Ayari Myriam, Ayadi Shema, Sayari Taha, Gargah Tahar
Department of Pediatrics, Charles Nicolle Hospital, Tunis, Tunisia.
Department of Gastroenterology, Charles Nicolle Hospital, Tunis, Tunisia.
Int Med Case Rep J. 2023 Sep 29;16:633-639. doi: 10.2147/IMCRJ.S401983. eCollection 2023.
Nephrotic syndrome (NS) is associated with a hypercoagulable state and may be complicated by thrombotic events. Venous thrombosis is well-acknowledged, while arterial thrombosis is rather unusual.
We present the case of a 20-year-old woman with a 12-year history of idiopathic NS revealed by extensive cerebral venous thrombosis with pulmonary embolism treated with anticoagulation therapy and oral corticosteroid therapy followed by mycophenolate mofetil (MMF). The thrombophilia assessment did not show any abnormalities. The evolution was marked by the occurrence of several NS relapses controlled by oral corticosteroid therapy until 2017. Subsequently, the patient had not presented a relapse of her disease. The anticoagulant treatment and the MMF were therefore stopped. One year later, the patient presented with severe diffuse acute abdominal pain associated with postprandial vomiting and bilateral lower limb edema. Laboratory results confirmed a NS relapse. An abdominal CT scan revealed acute thrombosis of the superior mesenteric artery with acute mesenteric ischemia. Intraoperative exploration showed mesenteric ischemia with extensive necrosis of the small intestine making their resections incompatible with life. The patient died after 48 hours.
Mesenteric arterial thrombosis, which is a rare but life-threatening NS complication, should always be considered, especially in the case of acute non-specific digestive symptoms.
肾病综合征(NS)与高凝状态相关,可能并发血栓形成事件。静脉血栓形成已得到充分认识,而动脉血栓形成则较为罕见。
我们报告一例20岁女性,有12年特发性NS病史,因广泛脑静脉血栓形成合并肺栓塞就诊,接受抗凝治疗、口服糖皮质激素治疗,随后使用霉酚酸酯(MMF)。血栓形成倾向评估未显示任何异常。病情演变过程中,多次肾病综合征复发,均通过口服糖皮质激素治疗得到控制,直至2017年。此后,患者未再出现疾病复发。因此停用了抗凝治疗和MMF。一年后,患者出现严重的弥漫性急性腹痛,伴有餐后呕吐和双侧下肢水肿。实验室检查结果证实肾病综合征复发。腹部CT扫描显示肠系膜上动脉急性血栓形成伴急性肠系膜缺血。术中探查显示肠系膜缺血,小肠广泛坏死,无法进行切除手术。患者在48小时后死亡。
肠系膜动脉血栓形成是一种罕见但危及生命的肾病综合征并发症,应始终予以考虑,尤其是在出现急性非特异性消化症状的情况下。