Waheed Nadia, Cheema Huma Arshad, Suleman Hassan, Fayyaz Zafar, Mushtaq Iqra, Hashmi Almas
Department of Hepatology & Nutrition, Children Hospital & Institute of child health, Lahore, Pakistan.
J Ayub Med Coll Abbottabad. 2016 Oct-Dec;28(4):672-675.
Celiac crisis is a serious life threatening complication of celiac disease characterized by profuse diarrhoea, severe dehydration and metabolic disturbances leading to neuromuscular weakness, cardiac arrhythmias and sudden death. It has been described as rare condition and not well documented in the literature. To improve awareness and facilitate diagnosis of this condition, we studied risk factors, pattern of presentation and management plans of celiac crisis.
It was a descriptive cross sectional study. Patients presenting in emergency room(ER) with profuse diarrhoea leading to severe dehydration, neuromuscular weakness, and metabolic acidosis and electrolyte abnormalities enrolled in the studies after positive serology and small bowel biopsy suggestive of celiac disease.
Total 126 patients out of 350 fulfilled the criteria including 54 (42.8%) male and 71 (56.3%) female. The mean age at presentation was 5.25±1.18 years. Risk factors were poor social status (97.60%), consanguinity (96.77%), early weaning with gluten contained diet (93.54%), and Presenting complaints were loose motion (100%), loss of neck holding (96.77%), dehydration (96.77%), polyuria (95.96%), inability to walk (67.74%), abdominal distension (85.86%). Electrolytes imbalances were hypokalaemia (2.4±0.55), hypocalcaemia (7.29±0.66), hypomagnesaemia (1.89±0.50), hypophosphatemia (2.8±0.68), hypoalbuminemia (3.05±0.48) and metabolic acidosis (96%). One hundred & twenty patients were stabilized with GFD and correction of dehydration, acidosis and electrolyte imbalance. Six patients needed parenteral steroids ant total parenteral nutrition (TPN). Recovery time from crisis was mean 5.4±2.73 days (range 3-20 days).
Celiac crisis is a common but under recognized problem in developing countries. Commonest presenting feature is neuromuscular paralysis and biochemical abnormality is hypokalaemia.
乳糜泻危象是乳糜泻一种严重的、危及生命的并发症，其特征为大量腹泻、严重脱水及代谢紊乱，可导致神经肌肉无力、心律失常及猝死。它被描述为一种罕见病症，文献记载较少。为提高对该病症的认识并促进其诊断，我们研究了乳糜泻危象的危险因素、临床表现模式及治疗方案。
这是一项描述性横断面研究。因大量腹泻导致严重脱水、神经肌肉无力、代谢性酸中毒及电解质异常而就诊于急诊室的患者，在血清学检查及小肠活检结果提示乳糜泻阳性后纳入研究。
350例患者中共有126例符合标准，其中男性54例（42.8%），女性71例（56.3%）。就诊时的平均年龄为5.25±1.18岁。危险因素包括社会地位低下（97.60%）、近亲结婚（96.77%）、过早添加含麸质饮食（93.54%）。主要临床表现为腹泻（100%）、不能抬头（96.77%）、脱水（96.77%）、多尿（95.96%）、不能行走（67.74%）、腹胀（85.86%）。电解质紊乱包括低钾血症（2.4±0.55）、低钙血症（7.29±0.66）、低镁血症（1.89±0.50）、低磷血症（2.8±0.68）、低白蛋白血症（3.05±0.48）及代谢性酸中毒（96%）。120例患者通过采用无麸质饮食（GFD）及纠正脱水、酸中毒和电解质失衡病情得以稳定。6例患者需要胃肠外类固醇及全胃肠外营养（TPN）。从危象中恢复的平均时间为（5.4±2.73）天（范围3 - 20天）。
在发展中国家，乳糜泻危象是一个常见但未得到充分认识的问题。最常见的临床表现是神经肌肉麻痹，生化异常是低钾血症。
