• 대한소화기학회지
• /
• 제72권6호
• /
• pp.277-280
• /
• 2018

Although small bowel the mainly occupies the most part of the gastrointestinal tract, small intestine tumors are rare, insidious in clinical presentation, and frequently represent a diagnostic and management challenge. Small bowel tumors are generally classified as epithelial, mesenchymal, lymphoproliferative, or metastatic. Familial adenomatous polyposis and Peutz-Jeghers syndrome are the most common inherited intestinal polyposis syndromes. Until the advent of capsule endoscopy (CE) and device-assisted enteroscopy (DAE) coupled with the advances in radiology, physicians had limited diagnostic examination for small bowel examination. CE and new radiologic imaging techniques have made it easier to detect small bowel tumors. DAE allows more diagnosis and deeper reach in small intestine. CT enteroclysis/CT enterography (CTE) provides information about adjacent organs as well as pictures of the intestinal lumen side. Compared to CTE, Magnetic resonance enteroclysis/enterography provides the advantage of soft tissue contrast and multiplane imaging without radiation exposure. Treatment and prognosis are tailored to each histological subtype of tumors.

• Imaging Science in Dentistry
• /
• 제46권4호
• /
• pp.267-272
• /
• 2016

Gardner syndrome is known as a variant of familial adenomatous polyposis. This syndrome is characterized by multiple intestinal polyposes, osteomas, and epidermoid cysts. In addition, dental abnormalities include an increased frequency of multiple odontomas, as well as supernumerary and impacted teeth. The authors report the case of a 7-year-old male patient with Gardner syndrome. Radiographic findings revealed multiple osteomas in both sides of the maxilla, multiple diffuse enostoses in both jaws, and a complex odontoma in the left mandibular body. Two years later, multiple epidermoid cysts on the scalp were found. Since this patient was suspected to have Gardner syndrome, the authors recommended gastrointestinal endoscopy to check for intestinal polyposis. Gastrointestinal endoscopic examination revealed multiple polyposes in the upper gastrointestinal tract and fundus of the stomach. As a result, the final diagnosis was Gardner syndrome.

• Clinical Endoscopy
• /
• 제57권3호
• /
• pp.402-406
• /
• 2024

Cronkhite-Canada syndrome is a rare gastrointestinal polyposis syndrome with distinctive clinical features and endoscopic findings. Diagnosis can be challenging without suspicion, and the disease carries high mortality due to complications such as infection, gastrointestinal bleeding, and malignancies. This paper presents two cases of Cronkhite-Canada syndrome occurring after coronavirus disease 2019 (COVID-19) mRNA vaccination. Both cases exhibited typical clinical findings, including hypogeusia, onychodystrophy, alopecia, and weight loss. Typical polyposis in the gastrointestinal tract was confirmed through endoscopies. As symptomatic treatment did not improve the symptoms, corticosteroids were administered, and symptoms and laboratory test results improved immediately. The patients improved upon corticosteroids tapering. These cases illustrate typical presentations of Cronkhite-Canada syndrome and the course of the disease following corticosteroid treatment. Additionally, they suggest the possibility that Cronkhite-Canada syndrome may be triggered by COVID-19 mRNA vaccination.

• Pediatric Gastroenterology, Hepatology & Nutrition
• /
• 제13권2호
• /
• pp.154-163
• /
• 2010

목 적: 대장용종증은 소아에서는 드문 질환군으로 다수의 용종으로 인한 출혈, 복통, 장중첩증 등이 반복될 수 있고 용종의 악성화나 장외종양이 발생할 수 있으나 아직은 이에 관한 연구가 많지 않다. 본 연구에서는 소아 대장용종증의 임상 양상과 내시경적, 조직학적 특징을 살펴보고자 하였다. 방 법: 서울대병원 어린이병원에서 1987년부터 2009년까지 대장내시경을 시행 받은 2,956명의 소아 환자중에서 대장용종증으로 진단받은 37명의 환자를 대상으로 의무기록 분석을 시행하였다. 대장용종증 환자들의 진단 시 평균나이는 8세였다. 결 과: Peutz-Jeghers 증후군이 22예로 가장 많았으며 연소성 용종증 7예, 가족성 선종성 용종증 6예, 림프성용종증 2예이었다. 내원 시 가장 흔한 주소는 혈변이었다. 50% 이상의 환자에서 혈변과 복통이 동반되었고 일부에서 항문종괴, 설사, 변비가 동반되었다. 용종의 수와 크기는 다양하였고 위장과 소장에 용종이 동반된 환자는 각각 21명, 17명이었다. Peutz-Jeghers 증후군 환자에서는 주로 다엽성의 목이 있는 용종이 관찰되었다. 연소성 용종증 환자에서는 둥글고 목이 있는 용종이 대부분이었다. 가족성 선종성 용종증 환자에서는 작고 둥글며 목이 없는 용종이 관찰되었다. 림프성 용종증 환자에서는 목이 없는 용종이 관찰되었다. 모든 환자는 내시경적 용종절제술을 시행받았고 14명(38%)은 수술적 용종절제술을 시행받았다. 부분장절제술을 시행받은 환자는 13명(35%)이었고, 가족성 선종성 용종증 환자 4명은 전대장절제술을 시행받았다. Peutz-Jeghers 증후군 환자 중 일부에서 장외 종양이 발생하였으나 용종의 악성화는 없었다. 결 론: 소아의 대장용종증 환자는 출혈, 복통 등의 증상을 보이며 장중첩증 등의 합병증이 발생할 수 있어 조기 진단과 치료가 필요하며 정기적인 대장내시경 검사를 통해 합병증을 예방하고 용종의 악성화나 장외 종양 여부를 확인해야 한다.

• BMB Reports
• /
• 제50권8호
• /
• pp.391-392
• /
• 2017

Overexpression of mammalian 2-Cys peroxiredoxin (Prx) enzymes is observed in most cancer tissues. Nevertheless, their specific roles in colorectal cancer (CRC) progression has yet to be fully elucidated. Here, a novel molecular mechanism by which PrxII/Tankyrase (TNKS) interaction mediates survival of adenomatous polyposis coli (APC)-mutant CRC cells was explored. In mice with an inactivating APC mutation, a model of spontaneous intestinal tumorigenesis, deletion of PrxII reduced intestinal adenomatous polyposis and thereby increased survival. In APC-mutant human CRC cells, PrxII depletion hindered PARP-dependent Axin1 degradation through TNKS inactivation. $H_2O_2-sensitive$ Cys residues in the zinc-binding domain of TNKS1 was found to be crucial for PARsylation activity. Mechanistically, direct binding of PrxII to ARC4/5 domains of TNKS conferred vital redox protection against oxidative inactivation. As a proof-of-concept experiment, a chemical compound targeting PrxII inhibited the growth of tumors xenografted with APC-mutation-positive CRC cells. Collectively, the results provide evidence revealing a novel redox mechanism for regulating TNKS activity such that physical interaction between PrxII and TNKS promoted survival of APC-mutant colorectal cancer cells by PrxII-dependent antioxidant shielding.

• Journal of Digestive Cancer Research
• /
• 제9권2호
• /
• pp.60-67
• /
• 2021

Tumors of the small intestine are rare and generally asymptomatic or with nonspecific symptoms. The small intestine is difficult to approach using conventional endoscopy, and early diagnosis of the small intestinal tumors is difficult. Therefore, many of the small intestinal tumors are diagnosed at an advanced stage, which makes the prognosis poor. Premalignant lesions of the small intestine or known risk factors of small bowel cancer are sporadic adenoma, adenoma associated with familial adenomatous polyposis, hamartomatous polyp associated with Peutz-Jeghers syndrome, Crohn's disease, and celiac disease. Therefore, it is necessary to recognize that the small bowel cancer can occur in these patients with premalignant lesions or risk factors of small bowel cancer. To reduce the possibility of small bowel cancer or to detect at an earlier stage, attention should be paid to screening and surveillance of these patients with premalignant lesions or risk factors of the small bowel cancer.

• Pediatric Gastroenterology, Hepatology & Nutrition
• /
• 제13권1호
• /
• pp.1-6
• /
• 2010

The small bowel is the most difficult intestinal segment to examine. Radiologic tests are mostly insensitive and double-balloon enteroscopy is unsuitable for the younger child. Capsule endoscopy is a novel wireless method of investigation of the small bowel. The primary indications for capsule endoscopy include evaluation of obscure gastrointestinal bleeding, small bowel Crohn's disease, and polyposis syndromes. Capsule endoscopy offers an accurate and effective means of investigating the small bowel in children. Capsule retention is a potential complication of capsule endoscopy. This review provides the indications, safety, and limitations of wireless capsule endoscopy in children.

• Investigative Magnetic Resonance Imaging
• /
• 제24권2호
• /
• pp.85-89
• /
• 2020

Amyloidosis is an all-inclusive disease of deposition of amyloid proteins in the extracellular spaces, which in localized or systemic form cause tissue damage and dysfunction. Herein, we report a case of small bowel involvement of systemic amyloidosis presenting with multiple polypoid wall thickening mimicking small bowel polyposis syndrome in an age 75 male. Interestingly, polypoid wall thickening and amyloidoma showed hypointensity on T2-weighted images. To our knowledge, there has been no literature describing MRI findings of poylpoid wall thickening and amyloidoma. Although the underlying mechanisms are unclear and need validation, hypointensity on T2-weighted images could be valuable in diagnosing small bowel involvement of amyloidosis in patients presenting with poylpoid wall thickening and amyloidoma.

• 한국수의병리학회:학술대회논문집
• /
• 한국수의병리학회 2005년도 Proceedings of The 2nd Asian Society of Veterinary Pathology Symposium(Vol.2) and 2005 Annual Meeting of The Korean Society of Veterinary Pathology(Vol.9)
• /
• pp.92-93
• /
• 2005

• Maxillofacial Plastic and Reconstructive Surgery
• /
• 제34권5호
• /
• pp.363-366
• /
• 2012

Peutz-Jeghers syndrome is a rare syndrome with characteristic features of multiple hamartomatous polyps and mucocutaneous pigmentation. This syndrome is an autosomal dominant disease, and has complications related with polyps of the gastrointestinal tract, such as small bowel obstruction, iron deficiency anemia associated with bleeding, and intussusceptions. Many studies have reported about higher cancer risk of patients with this syndrome than those with no syndrome in the gastrointestinal tract, including gastric, duodenal, jejunal and the extragastrointestinal organs, such as gallbladder, breast and reproductive system. There are guidelines for periodic test for early detection and treatment for higher risk organs. We report a case of Peutz-Jeghers syndrome patient in the emphasis of Oral and Maxillofacial surgeon's role with review of the literature.