Rai Ayesha Aslam, Butt Muhammad Osama, Laeeq Syed Mudassir, Abbas Zaigham, Luck Nasir Hasasan, Hassan Syed Mujahid, Tasneem Abbas Ali, Mubarak Muhammad
Department of Hepatogastroenterology, Sindh Institute of Urology and Transplantation (SIUT), Karachi.
Department of Histopathology, Sindh Institute of Urology and Transplantation (SIUT), Karachi.
J Coll Physicians Surg Pak. 2015 Oct;25 Suppl 2:S134-6. doi: 10.2015/JCPSP.S134S136.
Celiac crisis is a life-threatening condition in which patients have profuse diarrhea and severe metabolic disturbances. Refractory Celiac Disease (RCD) is a rare condition defined as persistence of symptoms despite being on Gluten Free Diet (GFD) for 6 months. Neurological involvement in Celiac Disease (CD) is seen in around 8 - 10% of adult patients; however, it is rare in children. Herein we present a case of an adolescent presenting with neurological symptoms and celiac crisis and diagnosed as RCD, type 1. He was treated with high dose steroids. This case underscores the need to consider RCD in patients presenting in celiac crisis and showing no improvement on GFD.
乳糜泻危象是一种危及生命的状况，患者会出现大量腹泻和严重的代谢紊乱。难治性乳糜泻（RCD）是一种罕见病症，定义为尽管已遵循无麸质饮食（GFD）6个月但症状仍持续存在。乳糜泻（CD）的神经系统受累在约8 - 10%的成年患者中可见；然而，在儿童中较为罕见。在此，我们报告一例青少年患者，其出现神经系统症状和乳糜泻危象，并被诊断为1型RCD。他接受了高剂量类固醇治疗。该病例强调了对于出现乳糜泻危象且在GFD治疗下无改善的患者，需要考虑RCD的可能性。
