Babar Masud Iqbal, Ahmad Irfan, Rao Muhammad Suleman, Iqbal Raghib, Asghar Shakeel, Saleem Mazhar
Department of Paediatric, Sheikh Zayed Medical College, Rahim Yar Khan.
J Coll Physicians Surg Pak. 2011 Aug;21(8):487-90.
To determine the frequency of clinical features of Celiac disease (CD) and Celiac crisis in children.
Case series.
Paediatrics Unit, Sheikh Zayed Medical College and Hospital, Rahim Yar Khan, from September 2009 to September 2010.
Forty children aged between 4 to 13 years of either gender, presenting with complaints of recurrent diarrhea, abdominal distention, severe emaciation and dehydration were included. The information about breast feeding, weaning diets, age of introduction of wheat diets, onset of diarrhea, characteristics and frequency of stools, growth, vaccination status, symptoms in 1st degree relatives, restriction of Gluten diet in the past and anthropometric measures were recorded. Serological tests against anti-Tissue Transglultaminase (anti-tTG) antibodies were obtained in all cases. Upper gastrointestinal endoscopies were performed and multiple biopsies were taken from distal parts of duodenum.
Among the forty children, twenty four (60%) were females and 16 were males (40%). The mean age was 6.35 ± 2.83 years. Thirty five (87.5%) parents were cousins. Breast feeding was not exclusively given and the Gluten containing weaning diets were given as early as 3.5 months of age. Thirty (75%) children presented with typical sign and symptoms of CD. Celiac crisis presented with profuse diarrhea, severe dehydration; abdominal distention; pedal edema, carpopedal spasm due to tetany; wasted muscles; head drop and inability to stand. The serum TtG antibodies in thirty-eight cases (95%) were above the cut off level of 7 u/ml ranging from 35-99 u/ml. The histopathology of specimens from distal duodenum revealed lesions of M3 type in thirteen (32.5%) and M2 type in eighteen cases (45%). All cases recovered and improved on follow-up after strict adherence to gluten-free diet (GFD).
Majority of children with Celiac disease presented with typical symptom, while Celiac crisis was characterized by severe dehydration, weakness and calcium deficiency signs. Most were the product of consanguineous marriages and were given Gluten-containing weaning foods as early as the 4th month of life.
确定儿童乳糜泻（CD）和乳糜泻危象的临床特征发生率。
病例系列研究。
2009年9月至2010年9月，拉希姆亚尔汗市谢赫扎耶德医学院及医院儿科病房。
纳入40名年龄在4至13岁的儿童，男女不限，均有反复腹泻、腹胀、严重消瘦和脱水症状。记录母乳喂养、断奶饮食、引入小麦饮食的年龄、腹泻发作情况、大便特征及频率、生长发育、疫苗接种状况、一级亲属的症状、过去有无麸质饮食限制以及人体测量指标等信息。所有病例均进行了抗组织转谷氨酰胺酶（抗tTG）抗体的血清学检测。进行了上消化道内镜检查，并从十二指肠远端取了多处活检组织。
40名儿童中，24名（60%）为女性，16名（40%）为男性。平均年龄为6.35±2.83岁。35名（87.5%）儿童的父母为近亲。并非完全采用母乳喂养，含麸质的断奶饮食最早在3.5个月时就开始给予。30名（75%）儿童出现了典型的乳糜泻症状和体征。乳糜泻危象表现为大量腹泻、严重脱水、腹胀、足部水肿、因手足搐搦导致的手足痉挛、肌肉消瘦、头部下垂和无法站立。38例（95%）患儿的血清TtG抗体高于7 u/ml的临界值，范围为35 - 99 u/ml。十二指肠远端标本的组织病理学检查显示，13例（32.5%）为M3型病变，18例（45%）为M2型病变。所有病例在严格遵循无麸质饮食（GFD）后随访时均康复并有所改善。
大多数乳糜泻患儿表现出典型症状，而乳糜泻危象的特征为严重脱水、虚弱和缺钙体征。大多数患儿是近亲结婚的产物，早在出生后第4个月就开始食用含麸质的断奶食品。
