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先天性巨结肠症中肠神经丛的嗜酸性粒细胞浸润。

Eosinophilic infiltration of the enteric neural plexuses in Hirschsprung's disease.

作者信息

Lowichik A, Weinberg A G

机构信息

Department of Pathology, Children's Medical Center of Dallas, Texas, USA.

出版信息

Pediatr Pathol Lab Med. 1997 Nov-Dec;17(6):885-91.

PMID:9353828
Abstract

Inflammatory infiltrations of the enteric plexuses are uncommon and are usually lymphoplasmacytic. Within the past 15 years, nine pediatric cases in which a predominantly eosinophilic infiltrate of the gastrointestinal wall with a predilection for the myenteric (Auerbach's) and deep submucosal (Henle's) plexuses were seen at our institution. Two were neonates without gastrointestinal abnormalities who expired shortly after birth. Seven were patients with short-segment Hirsch-sprung's disease. There was a mild increase in mucosal eosinophils in the overlying mucosa and only one patient had peripheral eosinophilia. Follow-up data obtained 1 month to 7 1/2 years after biopsy revealed no development of inflammatory bowel disease, connective tissue disease, malignancy, allergic disorder, or intestinal dysmotility. The proximal location of the infiltrate suggests that it may represent a secondary finding rather than a primary cause of aganglionosis.

摘要

肠神经丛的炎性浸润并不常见,通常为淋巴浆细胞性。在过去15年里,我们机构有9例儿科病例,其胃肠道壁以嗜酸性粒细胞浸润为主,且好发于肌间(奥尔巴赫氏)神经丛和深层黏膜下(亨勒氏)神经丛。2例为无胃肠道异常的新生儿,出生后不久死亡。7例为短段型先天性巨结肠病患者。其上方黏膜中的黏膜嗜酸性粒细胞轻度增多,仅有1例患者外周血嗜酸性粒细胞增多。活检后1个月至7年半的随访数据显示,未发生炎症性肠病、结缔组织病、恶性肿瘤、过敏性疾病或肠道运动障碍。浸润的近端位置表明,它可能是一种次要发现,而非神经节缺失的主要原因。

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引用本文的文献

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Case Report: Eosinophilic Myenteric Ganglionitis in a Child With Hirschsprung's Disease: A Challenge in Pseudo-Obstruction.病例报告:一名患有先天性巨结肠症儿童的嗜酸性肌间神经节炎:假性肠梗阻的一个挑战
Front Pediatr. 2021 Feb 4;8:617309. doi: 10.3389/fped.2020.617309. eCollection 2020.
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The relationship of eosinophilia with outcomes of Hirschsprung disease in children.嗜酸性粒细胞增多与儿童先天性巨结肠症预后的关系。
Pediatr Surg Int. 2019 Apr;35(4):425-429. doi: 10.1007/s00383-018-04430-9. Epub 2019 Jan 21.
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Waardenburg syndrome with isolated deficiency of myenteric ganglion cells at the sigmoid colon and rectum.
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Pediatr Rep. 2018 May 24;10(2):7500. doi: 10.4081/pr.2018.7500.
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Myotonic dystrophy type 1 and pseudo-obstruction in a child with smooth muscle α-actin deficiency and eosinophilic myenteric plexitis.1型强直性肌营养不良与一名患有平滑肌α-肌动蛋白缺乏和嗜酸性肠肌丛炎儿童的假性肠梗阻
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