乳糜泻危象:一种罕见而隐匿的乳糜泻并发症

Maria Barca, M. Boccia, Marta Improta, V. Discepolo
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摘要

乳糜泻危象是乳糜泻(Celiac Disease,CeD)的一种紧急且可能致命的并发症,多发于幼儿期。危机可能发生在发病初期,也可能发生在对无麸质饮食(GFD)依从性较差的糜烂性胃炎患者身上。压力、手术和感染都可能成为诱发因素。诊断依据是急性发作的快速进展性腹泻,至少伴有两种情况,包括严重脱水、代谢性酸中毒、双选择性溶血、肾功能障碍、神经系统改变、低蛋白血症和体重减轻。血液学表现,如血小板减少、INR升高、再生障碍性贫血和白细胞减少症则很少见。治疗的基础是静脉补液、纠正低白蛋白血症、酸中毒和硫酸血症,同时严格执行 GFD。难治性病例也可使用类固醇。本文介绍了最近的一例患者,该患者出现了严重的腹腔危象,危及生命,其特征是下肢无力、严重腹胀、腹泻和外周水肿导致无法行走。
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Crisi celiaca: una complicanza rara e insidiosa della celiachia
Coeliac Crisis is an urgent and potentially fatal complication of Coeliac Disease (CeD), which is most frequent in early childhood. The crisis may occur at onset or develop in CeD patients with poor compliance to the gluten-free diet (GFD). Stress, surgery and infections can act as triggers. The diagnosis is based on the acute onset of rapidly progressive diarrhoea associated with at least two conditions among severe dehydration, metabolic acidosis, dyselectrolytemia, renal dysfunction, neurological alterations, hypoproteinemia and weight loss. Haematological manifestations, such as thrombocytopenia, increased INR, aplastic anaemia and leukopenia, are rare. The cornerstones of therapy are intravenous rehydration, correction of hypoalbuminemia, acidosis and dyshionemia along with a strict GFD. Steroids may be also used in refractory cases. The paper describes a recent case of a patient presenting with a severe life-threatening coeliac crisis characterized by inability to walk due to lower limb weakness, severe abdominal distension, diarrhoea and peripheral oedema.
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