Onal Hasan, Aktuglu-Zeybek Cigdem, Altun Gürkan, Ozyilmaz Isa, Alhaj Safa, Aydin Ahmet
Division of Metabolic Diseases, Department of Pediatrics, Ministry of Health Bakirkoy Maternity and Children Teaching Hospital.
Ann Trop Paediatr. 2007 Mar;27(1):81-6. doi: 10.1179/146532807X170556.
Systemic capillary leak syndrome (SCLS) is a rare disorder of unknown pathophysiology, characterised by episodic life-threatening hypotension, haemoconcentration and hypo-albuminaemia. A 5-month-old child presented with episodes of relapsing diarrhoea, vomiting and hyponatraemia and developed generalised oedema during treatment. Clinical and laboratory findings were consistent with acute SCLS. Treatment with careful fluid replacement, fresh frozen plasma and aminophylline in the acute phase and teophylline and gingko biloba in the chronic phase led to sustained remission during follow-up for over 1 year. To our knowledge, this is the youngest case of SCLS in the literature and is unusual in that it presented with diarrhoea.
系统性毛细血管渗漏综合征（SCLS）是一种病因不明的罕见疾病，其特征为发作性危及生命的低血压、血液浓缩和低白蛋白血症。一名5个月大的儿童出现反复腹泻、呕吐和低钠血症发作，并在治疗期间出现全身性水肿。临床和实验室检查结果与急性SCLS相符。急性期通过谨慎补液、输注新鲜冰冻血浆和使用氨茶碱进行治疗，慢性期使用茶碱和银杏叶进行治疗，在随访1年多的时间里实现了持续缓解。据我们所知，这是文献中报道的最年轻的SCLS病例，且以腹泻为表现较为罕见。
