Sabeen Abid Khan, Muhammad Imran, Qamar Ali, Munir Iqbal Malik
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Later, after 12 days he again presented with jaundice, fever, anorexia, and dark colored urine and irritability. He was admitted for fulminant hepatic failure, his workup revealed direct hyperbilirubenemia, ANA +ve, and hyper IgG. Liver biopsy confirmed autoimmune hepatitis. Further workup for anemia showed reticulocyte count 7.1, LDH 423, direct and indirect coombs test was positive confirming autoimmune hemolytic anemia. Child responded well to Azathioprine and prednisolone with clinical improvement. We report a rare presentation of celiac disease with polyautoimmunity in a young child. Case reports of autoimmune hepatitis with CD patients have been reported in adult patients. Association of celiac disease with autoimmune hemolytic anemia and autoimmune hepatitis is a distinct and rare condition.</p>","PeriodicalId":45027,"journal":{"name":"Clinical Medicine Insights-Pediatrics","volume":" ","pages":"11795565221120565"},"PeriodicalIF":1.7000,"publicationDate":"2022-08-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/1c/e2/10.1177_11795565221120565.PMC9425893.pdf","citationCount":"1","resultStr":"{\"title\":\"Celiac Disease With Autoimmune Hemolytic Anemia and Autoimmune Hepatitis in a Young Child: Case Report and Literature Review.\",\"authors\":\"Sabeen Abid Khan, Muhammad Imran, Qamar Ali, Munir Iqbal Malik\",\"doi\":\"10.1177/11795565221120565\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<p><p>Celiac disease (CD) is a chronic autoimmune condition with intestinal and extra-intestinal features. Extra intestinal features including hematological, neurological, and endocrine symptoms are seen more frequently in elder children. A 4 years 7 months old male child presented in clinic with history of abdominal pain and diarrhea on and off for 1 year. On examination, he was hemodynamically stable, pale, and malnourished with distended abdomen. He was investigated for CD, Anti TTG IgA <0.1 (positive >10), Anti TTG IgG 13 (positive >10). To confirm celiac disease, Esophagogastroduodenoscopy (EGD) was done which was consistent with diagnosis of Celiac disease (MARSH Type 3a). Gluten free diet was advised. Later, after 12 days he again presented with jaundice, fever, anorexia, and dark colored urine and irritability. He was admitted for fulminant hepatic failure, his workup revealed direct hyperbilirubenemia, ANA +ve, and hyper IgG. Liver biopsy confirmed autoimmune hepatitis. Further workup for anemia showed reticulocyte count 7.1, LDH 423, direct and indirect coombs test was positive confirming autoimmune hemolytic anemia. Child responded well to Azathioprine and prednisolone with clinical improvement. We report a rare presentation of celiac disease with polyautoimmunity in a young child. Case reports of autoimmune hepatitis with CD patients have been reported in adult patients. 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引用次数: 1
摘要
乳糜泻(CD)是一种具有肠道和肠道外特征的慢性自身免疫性疾病。肠外特征包括血液学、神经学和内分泌症状更常见于年龄较大的儿童。一名4岁7个月大的男婴因腹痛和腹泻断断续续1年就诊。经检查,他血流动力学稳定,脸色苍白,营养不良,腹部膨胀。检测CD、抗TTG IgA 10、抗TTG IgG 13(阳性>10)。为确认乳糜泻,行食管胃十二指肠镜检查(EGD),符合乳糜泻的诊断(MARSH 3a型)。建议无麸质饮食。12天后,患者再次出现黄疸、发热、厌食、尿色深和烦躁。他因暴发性肝功能衰竭入院,他的检查显示直接高胆红素血症,ANA +ve和高IgG。肝脏活检证实自身免疫性肝炎进一步贫血检查显示网织红细胞计数7.1,LDH 423,直接和间接coombs试验阳性,证实自身免疫性溶血性贫血。患儿对硫唑嘌呤和泼尼松龙治疗反应良好,临床改善。我们报告一例罕见的乳糜泻合并多重自身免疫的病例。自身免疫性肝炎合并乳糜泻患者的病例报告已在成人患者中报道。乳糜泻与自身免疫性溶血性贫血和自身免疫性肝炎的关联是一种独特而罕见的疾病。
Celiac Disease With Autoimmune Hemolytic Anemia and Autoimmune Hepatitis in a Young Child: Case Report and Literature Review.
Celiac disease (CD) is a chronic autoimmune condition with intestinal and extra-intestinal features. Extra intestinal features including hematological, neurological, and endocrine symptoms are seen more frequently in elder children. A 4 years 7 months old male child presented in clinic with history of abdominal pain and diarrhea on and off for 1 year. On examination, he was hemodynamically stable, pale, and malnourished with distended abdomen. He was investigated for CD, Anti TTG IgA <0.1 (positive >10), Anti TTG IgG 13 (positive >10). To confirm celiac disease, Esophagogastroduodenoscopy (EGD) was done which was consistent with diagnosis of Celiac disease (MARSH Type 3a). Gluten free diet was advised. Later, after 12 days he again presented with jaundice, fever, anorexia, and dark colored urine and irritability. He was admitted for fulminant hepatic failure, his workup revealed direct hyperbilirubenemia, ANA +ve, and hyper IgG. Liver biopsy confirmed autoimmune hepatitis. Further workup for anemia showed reticulocyte count 7.1, LDH 423, direct and indirect coombs test was positive confirming autoimmune hemolytic anemia. Child responded well to Azathioprine and prednisolone with clinical improvement. We report a rare presentation of celiac disease with polyautoimmunity in a young child. Case reports of autoimmune hepatitis with CD patients have been reported in adult patients. Association of celiac disease with autoimmune hemolytic anemia and autoimmune hepatitis is a distinct and rare condition.