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Peutz-Jeghers综合征

Peutz-Jeghers syndrome

定义 英文原文(暂无中文)

A genetic intestinal polyposis syndrome characterized by development of characteristic hamartomatous polyps throughout the gastrointestinal (GI) tract, and by mucocutaneous pigmentation. This disorder carries a considerably increased risk of GI and extra-GI malignancies.

别名

错构瘤样肠息肉病

基本事实

遗传方式
常染色体显性
发病年龄
青少年期、成年期、儿童期
患病率
1-9 / 100 000

相关基因 1

基因名称关联类型
STK11serine/threonine kinase 11Disease-causing germline mutation(s) in

临床表型 33

极常见 99–80%5

  • 口腔粘膜色素沉着异常 HP:0100669
  • 胃肠道异常 HP:0011024
  • 胃肠道癌 HP:0002672
  • 斑疹 HP:0012733
  • 多发性雀斑样痣 HP:0001003

常见 79–30%1

  • 胃肠道出血 HP:0002239

偶见 29–5%27

  • 腹痛 HP:0002027
  • 胆囊异常 HP:0005264
  • 鼻异常 HP:0000366
  • 呼吸系统异常 HP:0002086
  • 输尿管异常 HP:0000069
  • 贫血 HP:0001903
  • 胆管新生物 HP:0100574
  • 乳腺癌 HP:0003002
  • 宫颈癌 HP:0030079
  • 增大的多囊卵巢 HP:0008675
  • 食管肿瘤 HP:0100751
  • 胃肠道梗塞 HP:0005244
  • 肠梗阻 HP:0005214
  • 黑甲 HP:0100644
  • 多发性肾囊肿 HP:0005562
  • 鼻息肉 HP:0100582
  • 肿瘤 HP:0002664
  • 结肠新生物 HP:0100273
  • 肺部肿瘤 HP:0100526
  • 鼻肿瘤 HP:0012720
  • 直肠肿瘤 HP:0100743
  • 小肠新生物 HP:0100833
  • 胰腺腺癌 HP:0006725
  • 直肠脱垂 HP:0002035
  • 肾细胞癌 HP:0005584
  • 胃癌 HP:0012126
  • 呕吐 HP:0002013

近两年的全球研究 655L2

2024/10 起在 Europe PMC 检索所得,按发表时间倒序显示最近 20 篇。标题未译成中文——自动翻译需要接入 LLM 服务,尚未引入。

  • 2026-10
    Abstracts
    JPGN reports
  • 2026-10开放获取
    Outcomes of 'in-house' genetic testing within a specialist hereditary colorectal cancer registry
    Colorectal disease : the official journal of the Association of Colopr · DOI · Europe PMC
  • 2026-09开放获取
    Developmental experiences of female patients diagnosed with Peutz-Jeghers syndrome in childhood: a qualitative study based on the social ecological model in China
    BMJ open · DOI · Europe PMC
  • 2026-09综述开放获取
    The Gut-Skin Axis in Atopic Dermatitis and Inflammatory Bowel Disease: Mechanisms, Microbiota, and Therapeutic Implications
    Journal of personalized medicine · DOI · Europe PMC
  • 2026-09开放获取
    Conserved Epithelial Remodeling Programs Underlie Pediatric Juvenile Colorectal Polyps Associated with Allergic Sensitization
    International journal of molecular sciences · DOI · Europe PMC
  • 2026-09综述开放获取
    Closing the Diagnostic Gap in Early-Onset Colorectal Cancer: Red-Flag Symptoms, Screening, and Inherited Risk
    Cancers · DOI · Europe PMC
  • 2026-09综述开放获取
    <i>STK11</i>/LKB1 Loss in Cancer: From Developmental Constraint to Stress-Adapted Malignancy
    Cancers · DOI · Europe PMC
  • 2026-09
    STK11 adnexal tumor mimicking a female adnexal tumor of probable Wolffian origin (FATWO) associated with Peutz-Jeghers syndrome: A brief review
    Indian journal of pathology & microbiology · DOI · Europe PMC
  • 2026-09综述开放获取
    Oral Pigmentation in Children: A Narrative Review and Practical Diagnostic Framework
    Cureus · DOI · Europe PMC
  • 2026-09综述开放获取
    Cronkhite-Canada syndrome: An elusive gastrointestinal disorder with multisystem involvement-pathogenesis, diagnosis, and therapeutic strategies
    World journal of gastrointestinal pathophysiology · DOI · Europe PMC
  • 2026-09开放获取
    Presumed Intraductal Papillary Mucinous Neoplasms in Individuals at High Risk for Pancreatic Cancer
    JAMA network open · DOI · Europe PMC
  • 2026-09开放获取
    Real‐Time AI‐Assisted Detection of Colonic Lesions Using the iIDEAS Intelligent‐C Module: A Prospective Single‐Center Validation Study
    JGH open : an open access journal of gastroenterology and hepatology
  • 2026-09综述开放获取
    Current insights into the diagnosis and management of small bowel bleeding
    The Korean journal of internal medicine · DOI · Europe PMC
  • 2026-09综述开放获取
    Optimizing multiple diagnostic modalities for small bowel evaluation: a strategic approach
    The Korean journal of internal medicine · DOI · Europe PMC
  • 2026-09开放获取
    STK11 Mutations and Deletions Define an Aggressive Molecular Subgroup of Cervical Adenocarcinoma
    Cancer medicine · 被引 1 · DOI · Europe PMC
  • 2026-09开放获取
    Pathological lead points in pediatric secondary intussusception: A single-center retrospective observational study of five common etiologies
    Medicine · DOI · Europe PMC
  • 2026-08开放获取
    Targeting LKB1-inactivated lung cancers with a stapled peptide inhibitor of the CRTC-CREB pathway
    Molecular therapy. Oncology · DOI · Europe PMC
  • 2026-08开放获取
    Exploring Traditional Breast Cancer Risk Genes Among Aboriginal and Torres Strait Islander Women
    Life (Basel, Switzerland) · DOI · Europe PMC
  • 2026-08
    Fibroblast heterogeneity in Peutz-Jeghers syndrome: identifying the polyp-driving subset<sup>†</sup>
    The Journal of pathology · DOI · Europe PMC
  • 2026-08开放获取
    Automatic Conversion of NICE Guidelines to an Executable Computational Model Using Large Language Models
    Learning health systems · DOI · Europe PMC

在中国开展的临床试验 4L2

按病名在 ClinicalTrials.gov 检索、并校验研究中心含中国大陆而来。登记状态不等于现在真的能入组——务必按 NCT 号到原站核实,并与主治医生商量。

登记为可入组 1

  • 招募中NCT06722534
    Celecoxib for Prevention of Progression in Peutz-Jeghers Syndrome
    不适用 · 干预性 · 2025/02/01Air Force Military Medical University, China
    中国研究中心 1 个:Xi'an
其他状态的试验(3 项)
  • 状态未知NCT03806075
    Study of Accurate Diagnosis and Treatment of Peutz-Jeghers Syndrome
    不适用 · 干预性 · 2018/03/01Yiqi Du
    中国研究中心 1 个:Shanghai
  • 状态未知NCT03781050
    Efficacy of Rapamycin (Sirolimus) in the Treatment of Peutz-Jeghers Syndrome
    IV 期 · 干预性 · 2018/09/16Peking Union Medical College Hospital
    中国研究中心 1 个:Beijing
  • 状态未知NCT06001476
    Cold Snare Polypectomy for Small Bowel Polyps in Patients With Peutz-Jeghers Syndrome
    IV 期 · 干预性 · 2023/08/20Shandong University
    中国研究中心 1 个:Jinan

中国境外的在招试验 5L2

这些试验在中国没有研究中心,通常无法直接报名——入组一般要求在当地居住并接受随访。列在这里是因为它另有用处:看清楚全世界正在试哪些药、做到了哪一期、由谁在做。把药名和 NCT 编号记下来去问主治医生,或据此进一步查该药是否已在境外获批、是否有拓展性用药(expanded access)通道。

美国3意大利1

CT.gov 报告命中 5 项,此处取回并展示最近的 4 项。

  • 招募中NCT04095195
    Registry of Subjects at Risk of Pancreatic Cancer
    观察性 · 2019/08/20Associazione Italiana per lo Studio del Pancreas
    意大利
  • 招募中NCT03050268
    Familial Investigations of Childhood Cancer Predisposition
    观察性 · 2017/04/06St. Jude Children's Research Hospital
    美国
  • 招募中NCT02000089
    The Cancer of the Pancreas Screening-5 CAPS5)Study
    III 期 · 干预性 · 2014/01/06Johns Hopkins University
    美国
  • 招募中NCT02775461
    Pancreas Registry and High Risk Registry
    观察性 · 2013/03/01Icahn School of Medicine at Mount Sinai
    美国

外部标识与链接

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本页数据来源

  • 疾病定义、同义词、基因、表型、流行病学:Orphanet(CC BY 4.0)
  • 中文病名:Orphanet 中文包,冻结于 2020-06-01
  • 表型中文标签:HPO 简体中文翻译(CHPO 上游成果)