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一名患有急性重症胰腺炎且SPINK 1基因杂合突变的斯里兰卡患者的急性肺栓塞非典型病例。

An Atypical Case of Acute Pulmonary Embolism in a Sri Lankan Patient With Acute Severe Pancreatitis With Heterozygosity for the SPINK 1 Mutation.

作者信息

Aponso Tilan, Wanninayake Athri, Peiris Ranjith

机构信息

Gastroenterology and Hepatology, National Hospital of Sri Lanka, Colombo, LKA.

Medicine, National Hospital of Sri Lanka, Colombo, LKA.

出版信息

Cureus. 2024 Nov 16;16(11):e73800. doi: 10.7759/cureus.73800. eCollection 2024 Nov.

DOI:10.7759/cureus.73800
PMID:39687810
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC11648039/
Abstract

Acute pancreatitis is a disease characterized by local destruction of the pancreatic gland due to premature activation of pancreatic enzymes within the acinar cells. Tissue damage can activate an inflammatory cascade, which can lead to systemic complications. Although vascular complications are uncommon, they significantly contribute to mortality and morbidity. Pulmonary embolism being an exceptionally rare complication of acute pancreatitis is reported in only a few cases, and cases of pulmonary embolism without associated deep vein thrombosis in the lower limbs are extraordinarily rare and unreported in the literature. This article presents the case of a 22-year-old Sri Lankan woman diagnosed with severe acute pancreatitis. On the fifth day of her illness, she developed sudden-onset dyspnea, and imaging revealed a large pulmonary artery embolism affecting the right pulmonary artery and segmental branches in the right lower lobe, with a normal venous duplex scan of the lower limbs. We started her on anticoagulation and supportive care for her acute severe pancreatitis. Her dyspnea and pancreatitis improved with time. She was later diagnosed as heterozygous for the serine peptidase inhibitor Kazal type 1 (SPINK 1) mutation.

摘要

急性胰腺炎是一种由于腺泡细胞内胰酶过早激活导致胰腺局部破坏的疾病。组织损伤可激活炎症级联反应,进而导致全身并发症。尽管血管并发症并不常见,但它们对死亡率和发病率有显著影响。肺栓塞是急性胰腺炎极其罕见的并发症,仅有少数病例报道,而无下肢相关深静脉血栓形成的肺栓塞病例极为罕见,文献中未见报道。本文介绍了一名22岁斯里兰卡女性被诊断为重症急性胰腺炎的病例。在患病的第五天,她突然出现呼吸困难,影像学检查显示右肺动脉及右下叶节段性分支有一大块肺动脉栓塞,下肢静脉双功超声扫描正常。我们开始对她进行抗凝治疗并对其急性重症胰腺炎进行支持治疗。随着时间推移,她的呼吸困难和胰腺炎症状有所改善。她后来被诊断为丝氨酸蛋白酶抑制剂Kazal 1型(SPINK 1)突变的杂合子。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d2af/11648039/8cab7096dd47/cureus-0016-00000073800-i02.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d2af/11648039/453a38932169/cureus-0016-00000073800-i01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d2af/11648039/8cab7096dd47/cureus-0016-00000073800-i02.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d2af/11648039/453a38932169/cureus-0016-00000073800-i01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d2af/11648039/8cab7096dd47/cureus-0016-00000073800-i02.jpg

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本文引用的文献

1
Functional Roles of SPINK1 in Cancers.丝氨酸蛋白酶抑制剂Kazal型1(SPINK1)在癌症中的功能作用
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SPINK1/PSTI polymorphisms act as disease modifiers in familial and idiopathic chronic pancreatitis.SPINK1/PSTI基因多态性在家族性和特发性慢性胰腺炎中作为疾病修饰因子发挥作用。
Gastroenterology. 2000 Sep;119(3):615-23. doi: 10.1053/gast.2000.18017.
9
A population-based perspective of the hospital incidence and case-fatality rates of deep vein thrombosis and pulmonary embolism. The Worcester DVT Study.基于人群视角的深静脉血栓形成和肺栓塞的医院发病率及病死率。伍斯特深静脉血栓形成研究
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