基本情報
研究キーワード
7研究分野
1論文
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Endoscopy international open 2024年1月
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Endoscopy international open 11(12) E1110-E1115 2023年12月Background and study aims Small-bowel polyps in patients with Peutz-Jeghers syndrome (PJS) are sometimes difficult to reach using double-balloon enteroscopy (DBE). However, they can induce intussusception, especially when ≧15 mm. This study aimed to review the outcomes of patients with such polyps. Patients and methods All patients with PJS with small-bowel polyps that bidirectional DBE failed to reach and were ≧5 mm as shown by DBE enterography at our institution from May 2006 to April 2022 were retrospectively evaluated. The endpoint was the earliest occurrence of symptomatic intussusception induced by the polyp, endoscopic removal by repeat DBE or intraoperative endoscopy, or the last medical record describing the patient's condition. Results This study included 27 polyps in 13 patients. All patients had extraluminal adhesions. None developed symptomatic intussusception, eight patients underwent endoscopic removal at repeat DBE without surgery, two patients underwent removal with intraoperative endoscopy, two patients were observed without polyp removal, and one patient had a polyp removed at repeat DBE and three unreachable polyps remained. Repeat DBE without surgery was able to remove 14 polyps (52%). Polyps ≧15 mm included 11 lesions in eight patients and were observed for a median of 14 months without symptomatic intussusception. Conclusions The difficult-to-reach polyps may have a low risk of immediate symptomatic intussusception, possibly due to limited bowel mobility by extraluminal adhesions, and the likelihood of reaching them at repeat DBE was substantial. Hence, repeat DBE 1 year later may be proper in such patients with PJS.
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Digestion 104(5) 335-347 2023年4月13日BACKGROUND: Peutz-Jeghers syndrome (PJS) is a rare disease characterized by the presence of hamartomatous polyposis throughout the gastrointestinal tract, except for the esophagus, along with characteristic mucocutaneous pigmentation. It is caused by germline pathogenic variants of the STK11 gene, which exhibit an autosomal dominant mode of inheritance. Some patients with PJS develop gastrointestinal lesions in childhood and require continuous medical care until adulthood and sometimes have serious complications that significantly reduce their quality of life. Hamartomatous polyps in the small bowel may cause bleeding, intestinal obstruction, and intussusception. Novel diagnostic and therapeutic endoscopic procedures such as small-bowel capsule endoscopy and balloon-assisted enteroscopy have been developed in recent years. SUMMARY: Under these circumstances, there is growing concern about the management of PJS in Japan, and there are no practice guidelines available. To address this situation, the guideline committee was organized by the Research Group on Rare and Intractable Diseases granted by the Ministry of Health, Labour and Welfare with specialists from multiple academic societies. The present clinical guidelines explain the principles in the diagnosis and management of PJS together with four clinical questions and corresponding recommendations based on a careful review of the evidence and involved incorporating the concept of the Grading of Recommendations Assessment, Development and Evaluation system. KEY MESSAGES: Herein, we present the English version of the clinical practice guidelines of PJS to promote seamless implementation of accurate diagnosis and appropriate management of pediatric, adolescent, and adult patients with PJS.
MISC
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消化器内視鏡 25(7) 1034-1035 2013年7月25日
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自治医科大学紀要 = Jichi Medical University Journal 35 135-135 2013年3月平成23年度自治医科大学医学部研究奨励金研究成果報告
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JOURNAL OF GASTROENTEROLOGY AND HEPATOLOGY 27 388-389 2012年12月
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Helicobacter Res 14(6) 468-474 2010年12月1日
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消化器内視鏡 22(9) 1390-1396 2010年9月25日
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JOURNAL OF GASTROENTEROLOGY AND HEPATOLOGY 25 A36-A36 2010年9月
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JOURNAL OF GASTROENTEROLOGY AND HEPATOLOGY 24 A134-A135 2009年10月
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Gastroenterol Endosc 50(Supplement 2) 2283 2008年9月5日
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Gastroenterol Endosc 50(Supplement 1) 848 2008年4月15日
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Gastroenterological Endoscopy 50(Suppl.1) 693-693 2008年4月
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GASTROINTESTINAL ENDOSCOPY 67(5) AB274-AB274 2008年4月
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GASTROINTESTINAL ENDOSCOPY 67(5) AB260-AB260 2008年4月
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