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在Peutz-Jeghers综合征中,急性胰腺炎与周围脉性哈马托马斯多相关
Edson Kenzo Mizushima1, Augusto Ricken Siqueira1, Luis Carlos Anflor1
1Radiology, Hospital de Clínicas de Porto Alegre, Brazil.
Revista espanola de enfermedades digestivas
|January 29, 2026
概括
皮茨-杰格斯综合征 (PJS) 的一种罕见并发症发生在一名11岁的女孩身上,该女孩因十二指肠的大型哈马托马图斯多而患有阻塞性胰腺炎. 手术切除解决了胰腺炎,突出了PJS作为胰腺问题的原因.
科学领域:
- 胃肠病学 胃肠病学
- 儿科手术 儿科手术
- 遗传学 遗传学 是一个
背景情况:
- 皮茨-杰格斯综合征 (PJS) 是一种罕见的遗传性疾病,其特征是胃肠道中的哈马托马斯多,以及各种癌症的风险增加.
- PJS的并发症可能包括内,出血和阻塞,但胰腺炎是不常见的表现.
研究的目的:
- 报告一种罕见的急性胰腺炎病例,该病例是由皮特兹-杰格斯综合征患者的十二指肠周边哈马托马托斯多体引起的.
- 强调考虑PJS作为阻塞性胰腺炎的病因的重要性,特别是在有已知的综合征史的儿科患者中.
主要方法:
- 一个11岁女孩患有PJS和卵巢切除病史的病例报告.
- 诊断工作包括实验室检测,成像 (CT/MRI) 和上部内镜.
- 手术涉及十二指肠切除术,多角切除术和胆囊切除术.
主要成果:
- 这名患者呈现急性胰腺炎症状,成像显示出一个大型的周十二指肠多,导致胆道和胰腺管道扩张.
- 组织病理学证实了与PJS一致的哈马托马托斯多体.
- 手术治疗导致胰腺炎和症状完全消失.
结论:
- 在皮茨-杰格斯综合征中,周边囊性哈马托马托型息肉可以导致阻塞性胰腺炎,这是一个罕见但显著的并发症.
- 通过成像和内镜及时诊断,然后进行手术干预,对于管理这种情况至关重要.
- 这一案例强调了在诊断和管理PJS患者的胃肠道并发症时需要保持警.
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