CELIAC CRISIS: A RARE OR RARELY RECOGNIZED DISEASE
AbstractBackground: 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. Methods: 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. Results: 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). Conclusion: Celiac crisis is a common but under recognized problem in developing countries. Commonest presenting feature is neuromuscular paralysis and biochemical abnormality is hypokalaemia.Keywords: Celiac Disease; Celiac crisis; electrolyte abnormality; neuromuscular weakness
Sondheimer JM, SundaramS. Celiac disease (gluten enteropathy). In: Hay WW, Levin MJ, Sondheimer JM, Deterding RR, editors. Current diagnosis and treatment: pediatrics. New York: McGraw Hill;2004. p. 602–3.
Hussain S, Sabir MU, Afzal M, Asghar I. Celiac disease-clinical presentation and diagnosis by anti-tissue transglutaminase antibody titer in children. J Pak Med Assoc 2014;64(4):437–41.
Khuhro AA, Parkash A, Cheema HA. Frequency of common clinical presentation of celiac disease in children. Eur Acad Res 2015;10:13223–31.
Cheema HA, Arshad R, Zaidi Z. Celiac disease-an under reported entity in northern Pakistan. Pak Pediatr J 2013;37(2):86–90.
Silano M, Agostoni C, Guandalini S. Effect of the timing of gluten introduction on the development of celiac disease. World J Gastroenterol 2010;16(16):1939–42.
Mones RL, Atienza KV, Youssef NN, Verg B, Mercer GO, Rosh JR. Celiac crisis in the modern era. J Pediatr Gastroenterol Nutr 2007;45(4):480–3.
Walia A, Thapa BR, Celia crisis. Indian Pediatr 2005;42(11):1169.
Babur MI, Ahmad I, Rao SM, Iqbal R, AsgharS, Saleem M. Celiac disease and celiac crisis in children. J Coll Physicians Surg Pak 2011;21(8):487–90.
Patwari AK, Anand VK, Kapoor G. Clinical and nutritional profile of children with celiac disease. Indian Pediatr 2003;40(4):337–42.
Fasano A. Clinical presentation of celiac disease in the pediatric population. Gastroenterology 2005;128(4 Suppl 1):68–73.
Andersen DH, Di Sant`Agnese PA. Idiopathic celiac disease. I. Mode of onset and diagnosis. Pediatrics 1953;11(3):207–23.
Jamma S, Rubio-Tapia A, Kelly CP, Murray J, Sheth S, Schuppan D, et al. Celiac crisis is a rare but serious complication of disease in adults. Clin Gastroenterol Hepatol 2010;8(7):587–90.
Baranwal AK, Singhi SC, Thapa BR, Kakkar N. Celiac crisis. Indian J Pediatr 2003;70(5):433–5.
Rubio-Tapia A, Kyle RA, Kaplan EL, Johnson DR, Page W, Erdtmann F, et al. Increased prevalence and mortality in undiagnosed celiac disease. Gastroenterology 2009;137(1):88–93.
Atikou A, RabhiM, Hidani H, El alaoui Faris M, Toloune F. Celiac crisis with quadriplegia due to potassium depletion as presenting feature of celiac disease. Rev Med Interne 2009;30(6):516–8.
Gupta T, Mandot A, Desai D, Abraham P, Joshi A. Celiac crisis with hypokalemic paralysis in a young lady. Indian J Gastroenterol 2006;25(5):259–60.
Mehta S, Jain K, Mulye S. Unresponsive and refractory hypokalemia in celiac crisis-a predictor of high mortality. J Pediatr Sci 2015;7:222.
Gutierrez S, Toro M, Cassar A, Ongay R, Isaguirre J, Lopez C, et al. Celiac crisis: presentation as bleeding diathesis. Acta Gastroenterol Latinoam 2009;39(1):53–4.
Wolf I, Mouallem M, Farfel Z. Adult celiac disease presented with celiac crisis. Severe diarrhea, hypokalemia and acidosis. J Clin Gastroenterol 2000;30(3):324–6.
Ozaslan E, Köseoğlu T, Kayhan B. Celiac crisis in adults: report of two cases. Eur J Emerg Med 2004;11(6):363–5.
Altamimi E. Celiac crisis with severe hypokalemia and paraplegia as a first presentation of celiac disease in a child. Jordan Med J 2012;46(1):61–3.
Lloydstill JD, Grand RJ, Khaw KT, Shwachman H. The use of corticosteroids in celiac crisis. J Pediatr 1972;81(6):1074–81.