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小儿极早发型克罗恩病中首次出现绍曼小体病例:病例报告及文献综述

The First Case of Schaumann Bodies in Pediatric Very Early Onset Crohn's Disease: Case Report and Literature Review.

作者信息

Jevtić Jovan, Đuknić Miloš, Popovac Nevena, Ristić Nina, Milovanovich Ivan, Radusinović Milica, Đordjić Irena, Simić Ljubica, Nikolić Gorana, Životić Maja, Mioljević Ana, Bogosavljević Nikola, Janković Radmila

机构信息

Institute of Pathology 'Prof. Dr. Đorđe Joannović', Faculty of Medicine, University of Belgrade, 11000 Belgrade, Serbia.

University Children's Hospital Tiršova, Faculty of Medicine, University of Belgrade, 11000 Belgrade, Serbia.

出版信息

Children (Basel). 2024 Oct 6;11(10):1216. doi: 10.3390/children11101216.

Abstract

Crohn's disease (CD) is a chronic inflammatory bowel condition with increasing global incidence. Diagnosing CD is challenging and requires close collaboration between clinicians and pathologists due to the lack of specific diagnostic criteria. Histologically, CD is characterized by transmural inflammation, crypt distortion, metaplasia, and granulomas, although granulomas are not always present. Schaumann bodies (SB), initially described in sarcoidosis, are rare in CD but have been reported in about 10% of cases. This case report presents a 4-year-old female with chronic hemorrhagic diarrhea, severe anemia, and elevated inflammatory markers. Endoscopic and histological evaluations suggested CD, with the presence of SB in the gastric mucosa. Further investigations ruled out sarcoidosis, confirming a diagnosis of multi-segmental, very early onset CD with atypical histological features. SB are inclusions composed of calcium carbonate crystals and conchoid bodies, typically found within giant cells. The presence of SB in the mucosa is rare, limiting their diagnostic significance in endoscopic biopsies. Differential diagnosis should exclude other granulomatous diseases such as intestinal tuberculosis and sarcoidosis. This case highlights the importance of considering SB in the diagnosis of CD, particularly in pediatric patients.

摘要

克罗恩病(CD)是一种慢性炎症性肠病,全球发病率呈上升趋势。由于缺乏特异性诊断标准,诊断CD具有挑战性,需要临床医生和病理学家密切合作。在组织学上,CD的特征是透壁性炎症、隐窝扭曲、化生和肉芽肿形成,不过肉芽肿并非总是存在。绍曼小体(SB)最初在结节病中被描述,在CD中罕见,但约10%的病例中有报道。本病例报告介绍了一名4岁女性,有慢性出血性腹泻、严重贫血和炎症标志物升高。内镜和组织学评估提示为CD,胃黏膜中有SB存在。进一步检查排除了结节病,确诊为多节段、极早发型CD伴非典型组织学特征。SB是由碳酸钙晶体和贝壳样小体组成的包涵体,通常见于巨细胞内。黏膜中出现SB很罕见,限制了它们在内镜活检中的诊断意义。鉴别诊断应排除其他肉芽肿性疾病,如肠结核和结节病。本病例突出了在CD诊断中考虑SB的重要性,尤其是在儿科患者中。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ad4f/11506799/e94af48e7fac/children-11-01216-g001.jpg

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