Newcomers in paediatric GI pathology: childhood enteropathies including very early onset monogenic IBD.
Newcomers in paediatric GI pathology: childhood enteropathies including very early onset monogenic IBD.
复制标题
DOI：
10.1007/s00428-017-2197-9
复制
发表时间：
2018-01
期刊：
影响因子：
--
通讯作者：
Russo P
中科院分区：
文献类型：
--
作者：
Ensari A;Kelsen J;Russo P
儿童肠病是一组导致严重慢性（>2-3周）腹泻的疾病，通常在出生后第一周开始，可能给受影响的婴儿带来致命的并发症。因此，儿童肠病的早期识别和准确分类对于制定治疗决策以预防危及生命的并发症至关重要。根据潜在的病理学，儿童肠病可分为四组：(i) 与营养物质和电解质的消化、吸收和运输缺陷有关的病症； (ii) 与肠上皮细胞分化和极化相关的疾病； (iii)肠内分泌细胞分化缺陷； (iv) 与肠道免疫反应调节缺陷相关的疾病。虽然在与先天性运输或酶缺乏相关的肠病中，肠粘膜通常是正常的，但其他疾病的肠活检可能会显示多种异常，从正常绒毛结构到绒毛萎缩和/或炎症，或潜在疾病的特有特征，包括上皮异常、肠上皮细胞中的脂质空泡化、浆细胞缺失、淋巴管扩张、微生物和粘膜嗜酸性粒细胞或组织细胞浸润。本综述旨在为儿童肠病（“新来者”，包括极早发型单基因炎症性肠病（IBD））的小肠活检结果提供最新信息，特别是针对执业病理学家。
Childhood enteropathies are a group of diseases causing severe chronic (>2–3 weeks) diarrhoea often starting in the first week of life with the potential for fatal complications for the affected infant. Early identification and accurate classification of childhood enteropathies are, therefore, crucial for making treatment decisions to prevent life-threatening complications. Childhood enteropathies are classified into four groups based on the underlying pathology: (i) conditions related to defective digestion, absorption and transport of nutrients and electrolytes; (ii) disorders related to enterocyte differentiation and polarization; (iii) defects of enteroendocrine cell differentiation; and (iv) disorders associated with defective modulation of intestinal immune response. While the intestinal mucosa is usually normal in enteropathies related to congenital transport or enzyme deficiencies, the intestinal biopsy in other disorders may reveal a wide range of abnormalities varying from normal villous architecture to villous atrophy and/or inflammation, or features specific to the underlying disorder including epithelial abnormalities, lipid vacuolization in the enterocytes, absence of plasma cells, lymphangiectasia, microorganisms, and mucosal eosinophilic or histiocytic infiltration. This review intends to provide an update on small intestinal biopsy findings in childhood enteropathies, the “newcomers”, including very early onset monogenic inflammatory bowel disease (IBD), in particular, for the practicing pathologist.
登录
查看更多内容
影响因子：
29.4
作者：
Folwaczny, C;Noehl, N;Fricke, H
通讯作者：
Fricke, H
影响因子：
5.6
作者：
Groisman, GM;Amar, M;Livne, E
通讯作者：
Livne, E
影响因子：
8.6
作者：
Cheng, Laurence E.;Kanwar, Bittoo;Wara, Diane W.
通讯作者：
Wara, Diane W.
影响因子：
7.5
作者：
de Serre, Natacha Patey-Mariaud;Canioni, Danielle;Brousse, Nicole
通讯作者：
Brousse, Nicole
影响因子：
1.2
作者：
Egritas, Odul;Sari, Sinan;Ensari, Arzu
通讯作者：
Ensari, Arzu