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[佩-杰二氏综合征]

[Peutz-Jeghers syndrome].

作者信息

Rubio Tapia Alberto, Ramírez Arias Francisco, Angeles Angeles Arturo, Uscanga Luis

机构信息

Departamento de Gastroenterología, INCMNSZ, Tlalpan, México, D.F.

出版信息

Rev Gastroenterol Mex. 2005 Jul-Sep;70(3):291-5.

PMID:17063785
Abstract

OBJECTIVE

To describe the demographic, clinical characteristics and associated diseases in eight patients with the Peutz-Jeghers syndrome seen at the INCMNSZ and literature review.

SETTING

National Institute of Health.

DESIGN

Description of eight cases of Peutz-Jeghers syndrome and literature review.

MATERIAL AND METHODS

A retrospective review of all the discharge diagnosis was doing between January 1987 to February 2004. The diagnosis of Peutz-Jeghers syndrome was made on clinical and anatomical grounds. The clinical features, follow-up, treatment and last visit clinical status were analyzed in all the patients.

PRESENTATION OF CASES

Eight patients with the Peutz-Jeghers syndrome were investigated, five women and three men. The median of time at diagnosis was 31 years-old (range, 26-37). All the patients had mucocutaneous pigmentation and hamartomatous polyps. The ileum and large bowel were the most frequent sites of the polyps, and there were generally sessile and pedunculated. The major abdominal symptoms were abdominal pain, GI bleeding, intestinal obstruction, weight loss and intussusception. One case of small-bowel cancer and one of serous cystadenoma of the ovary were detected. Surgical interventions were doing in five patients; the most frequent indication was polyp-induced bowel obstruction. All the patients are alive to date.

摘要

目的

描述在墨西哥社会保险和服务国家机构(INCMNSZ)就诊的8例黑斑息肉综合征患者的人口统计学、临床特征及相关疾病,并进行文献综述。

地点

国家卫生研究所。

设计

8例黑斑息肉综合征病例描述及文献综述。

材料与方法

对1987年1月至2004年2月期间所有出院诊断进行回顾性研究。根据临床和解剖学依据做出黑斑息肉综合征的诊断。分析所有患者的临床特征、随访情况、治疗方法及末次就诊时的临床状态。

病例介绍

对8例黑斑息肉综合征患者进行了研究,其中5名女性,3名男性。诊断时的年龄中位数为31岁(范围26 - 37岁)。所有患者均有黏膜皮肤色素沉着和错构瘤性息肉。息肉最常见于回肠和大肠,通常有广基和带蒂两种类型。主要腹部症状为腹痛、胃肠道出血、肠梗阻、体重减轻和肠套叠。发现1例小肠癌和1例卵巢浆液性囊腺瘤。5例患者接受了手术干预;最常见的指征是息肉引起的肠梗阻。所有患者至今均存活。

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[Peutz-Jeghers syndrome].[佩-杰二氏综合征]
Rev Gastroenterol Mex. 2005 Jul-Sep;70(3):291-5.
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Solitary Peutz-Jeghers Type Polyp of Jejunum with Gastric Fundic and Antral Gland Lining Mucosa: A Case Report and Review of Literature.孤立性空肠型肠息肉病伴胃底和贲门黏膜腺体:病例报告及文献复习。
Int J Surg Pathol. 2022 Aug;30(5):539-542. doi: 10.1177/10668969211067760. Epub 2021 Dec 27.
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Peutz-Jeghers syndrome: a clinical study of a large family in two generations.佩-吉二氏综合征:对一个两代大家庭的临床研究
Z Kinderchir. 1986 Dec;41(6):364-8. doi: 10.1055/s-2008-1043380.
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[Peutz-Jeghers syndrome: an account of 3 cases in the same family and a review of the literature].[黑斑息肉综合征:同一家庭中的3例病例报告及文献复习]
Chir Ital. 2005 Jul-Aug;57(4):425-36.
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Clinical features, diagnosis, and treatment of Peutz-Jeghers syndrome: Experience with 566 Chinese cases.临床特征、诊断和治疗 Peutz-Jeghers 综合征:566 例中国病例的经验。
World J Gastroenterol. 2023 Mar 14;29(10):1627-1637. doi: 10.3748/wjg.v29.i10.1627.

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