Simonin Mathieu, Corbeau Pierre, Durand Philippe, Rosenzwajg Michelle, Filleron Anne, Tran Tu-Anh
Department of Pediatrics, Bicêtre University Hospital, Assistance Publique des Hôpitaux de Paris, Le Kremlin Bicêtre, France.
Department of Immunology, Nîmes University Hospital, Montpellier-Nîmes University, Nîmes, France.
Eur J Paediatr Neurol. 2016 Nov;20(6):953-956. doi: 10.1016/j.ejpn.2016.08.003. Epub 2016 Aug 15.
Systemic capillary leak syndrome (SCLS) is a rare disease characterized by recurrent episodes and a triad of "leak attacks": hypovolemic shock, generalized edema, hemoconcentration and paradoxical hypoalbuminemia.
Here we report a case of pediatric idiopathic SCLS with an episode of cough and fever followed two days later by myalgia, livedo, acrocyanosis, and five days later by edema, tachycardia, hypotension, and generalized tonic-clonic seizure. Moreover, we provide evidence for an LPS-induced overproduction of interferon-gamma and interleukin-17 by the patient's peripheral blood mononuclear cells one year after the attack.
This observation suggests the involvement of IL-17 in the pathogenesis of this disease.
系统性毛细血管渗漏综合征（SCLS）是一种罕见疾病，其特征为反复发作以及“渗漏发作”三联征：低血容量性休克、全身性水肿、血液浓缩和矛盾性低白蛋白血症。
在此，我们报告一例小儿特发性SCLS病例，该病例先出现咳嗽和发热，两天后出现肌痛、网状青斑、手足发绀，五天后出现水肿、心动过速、低血压和全身性强直阵挛发作。此外，我们提供证据表明，在发作一年后，患者外周血单个核细胞中脂多糖诱导γ干扰素和白细胞介素-17产生过量。
该观察结果提示白细胞介素-17参与了本病的发病机制。
