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[胆汁淤积与呕吐:黑斑息肉综合征一例的罕见鉴别诊断]

[Cholestasis and vomiting: unusual differential diagnosis in a case of Peutz-Jeghers syndrome].

作者信息

Mandraka F, Zuelke C, Lock G

机构信息

Klinik und Poliklinik für Innere Medizin I, Klinikum der Universität Regensburg.

出版信息

Dtsch Med Wochenschr. 2003 May 2;128(18):984-8. doi: 10.1055/s-2003-38955.

DOI:10.1055/s-2003-38955
PMID:12721876
Abstract

HISTORY AND CLINICAL FINDINGS

A 54-year-old patient with painless jaundice and vomiting had been diagnosed with a Peutz-Jeghers syndrome 20 years before.

INVESTIGATIONS

The blood analysis showed a cholestatic constellation as well as increased transaminases. Sonographic, radiological, endoscopic and histological findings indicated multiple hamartomatous polyps of the Peutz-Jeghers' type in the entire small and large bowel with occlusion of the papilla of Vater and the superior gastrointestinal tract by a big polyp.

TREATMENT AND COURSE

After an initial percutaneous transhepatic cholangiographic drainage, Whipple's operation and a segmental resection of small and large bowel were performed. A highly differentiated adenocarcinoma of the duodenum was found in the resected specimen.

CONCLUSION

This case demonstrates the potentially severe complications of a Peutz-Jeghers syndrome that had been neglected for years.

摘要

病史及临床检查结果

一名54岁无痛性黄疸和呕吐患者20年前被诊断为佩-吉综合征。

检查

血液分析显示胆汁淤积及转氨酶升高。超声、放射学、内镜及组织学检查结果表明,全小肠和大肠存在多发佩-吉型错构瘤性息肉,一个大息肉阻塞了 Vater 壶腹和上消化道。

治疗及病程

最初行经皮经肝胆管造影引流,之后进行了惠普尔手术及小肠和大肠部分切除术。切除标本中发现十二指肠高分化腺癌。

结论

该病例显示了多年来被忽视的佩-吉综合征可能引发的严重并发症。

相似文献

1
[Cholestasis and vomiting: unusual differential diagnosis in a case of Peutz-Jeghers syndrome].[胆汁淤积与呕吐:黑斑息肉综合征一例的罕见鉴别诊断]
Dtsch Med Wochenschr. 2003 May 2;128(18):984-8. doi: 10.1055/s-2003-38955.
2
Solitary Peutz-Jeghers type polyp of the duodenum containing a focus of adenocarcinoma.十二指肠孤立性黑斑息肉病型息肉,伴有腺癌灶。
Ital J Gastroenterol. 1996 Feb-Mar;28(2):95-7.
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Duodenal and ampullary obstruction by a Peutz-Jeghers polyp.十二指肠和壶腹受黑斑息肉病息肉梗阻。
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[Peutz-Jeghers' syndrome with malignant development in a hamartomatous polyp: report of one case and review of the literature].[黑斑息肉综合征合并错构瘤性息肉恶变:1例报告及文献复习]
Gastroenterol Clin Biol. 2008 Mar;32(3):250-4. doi: 10.1016/j.gcb.2008.01.009. Epub 2008 Mar 4.
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Peutz-Jeghers syndrome with polyps in the stomach, duodenum, and small and large intestine: a case report.胃、十二指肠、小肠和大肠多发性息肉的 Peutz-Jeghers 综合征:病例报告。
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Endoscopic snare papillectomy for a solitary Peutz-Jeghers-type polyp in the duodenum with ingrowth into the common bile duct: Case report.十二指肠单发的具有向胆总管内生长的黑斑息肉综合征样息肉的内镜圈套息肉切除术:病例报告
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Peutz-Jeghers syndrome manifesting complete intussusception of the appendix and associated with a focal cancer of the duodenum and a cystadenocarcinoma of the pancreas: report of a case.表现为阑尾完全套叠并伴有十二指肠局灶性癌和胰腺囊腺癌的黑斑息肉综合征:病例报告
Dis Colon Rectum. 1998 Apr;41(4):517-21. doi: 10.1007/BF02235769.
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A solitary Peutz-Jeghers type hamartomatous polyp in the duodenum--a case report.
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引用本文的文献

1
Extra-ampullary Peutz-Jeghers polyp causing duodenal intussusception leading to biliary obstruction: a case report.引起十二指肠套叠并导致胆道梗阻的壶腹外黑斑息肉病:一例报告
J Med Case Rep. 2016 Jul 15;10:196. doi: 10.1186/s13256-016-0990-8.