• The Korean Journal of Gastroenterology
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• v.72 no.6
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• pp.277-280
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• 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
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• v.46 no.4
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• pp.267-272
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• 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.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.13 no.2
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• pp.154-163
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• 2010

Purpose: Colonic polyposis is less common in children than in adults. The clinical data pertaining to colonic polyposis in children are limited. Children with colonic polyposis have complications associated with numerous polyps, malignant transformation of the polyps, and extraintestinal neoplasms. We studied the clinical spectrum, endoscopic characteristics, and histologic findings of colonic polyposis in Korean children. Methods: We reviewed the clinical data of 37 children with multiple colonic polyps between 1987 and 2009. The mean age at the time of diagnosis of colonic polyposis was 8.0${\pm}$3.2 years. Results: Peutz-Jeghers syndrome, juvenile polyposis syndrome, familial adenomatous polyposis (FAP), and lymphoid polyposis was diagnosed in 22, 7, 6, and 2 children, respectively. The most common clinical presentation in children with colonic polyposis was hematochezia. A family history of colonic polyposis was noted in 7 children. The colonoscopic findings of colonic polyposis varied with the size and number of polyps. The majority of polyps were multi-lobulatd and pedunculated in children with Peutz-Jeghers syndrome. The polyps in children with juvenile polyposis syndrome were primarily round and pedunculated. For the children with FAP, the colon was carpeted with small, sessile polyps. There were multiple sessile polyps in the patients with lymphoid polyposis. Surgical polypectomy was performed in 14 children (38%). Intestinal segmental resection was performed in 13 children (35%). Four patients with FAP underwent total colectomy. Four children with Peutz-Jeghers syndrome had extraintestinal neoplasms. No malignant transformation of polyp was identified. Conclusion: Children with colonic polyposis should undergo a careful initial evaluation and require periodic re-evaluation.

• BMB Reports
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• v.50 no.8
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• pp.391-392
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• 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 Reports
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• v.9 no.2
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• pp.60-67
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• 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
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• v.13 no.1
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• pp.1-6
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• 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
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• v.24 no.2
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• pp.85-89
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• 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.

• Proceedings of the Korean Society of Veterinary Pathology Conference
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• 2005.11a
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• pp.92-93
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• 2005

• Maxillofacial Plastic and Reconstructive Surgery
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• v.34 no.5
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• pp.363-366
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• 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.

• BMB Reports
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• v.55 no.12
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• pp.609-614
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• 2022

Mutation of the gene for adenomatous polyposis coli (APC), as seen in Apc^Min/+ mice, leads to intestinal adenomas and carcinomas via stabilization of β-catenin. Transmembrane 4 L six family member 5 (TM4SF5) is involved in the development of non-alcoholic fatty liver disease, fibrosis, and cancer. However, the functional linkage between TM4SF5 and APC or β-catenin has not been investigated for pathological outcomes. After interbreeding Apc^Min/+ with TM4SF5-overexpressing transgenic (Tg^TM4SF5) mice, we explored pathological outcomes in the intestines and livers of the offspring. The intestines of 26-week-old dual-transgenic mice (Apc^Min/+:Tg^TM4SF5) had intramucosal adenocarcinomas beyond the single-crypt adenomas in Apc^Min/+ mice. Additional TM4SF5 overexpression increased the stabilization of β-catenin via reduced glycogen synthase kinase 3β (GSK3β) phosphorylation on Ser9. Additionally, the livers of the dualtransgenic mice showed distinct sinusoidal dilatation and features of hepatic portal hypertension associated with fibrosis, more than did the relatively normal livers in Apc^Min/+ mice. Interestingly, TM4SF5 overexpression in the liver was positively linked to increased GSK3β phosphorylation (opposite to that seen in the colon), β-catenin level, and extracellular matrix (ECM) protein expression, indicating fibrotic phenotypes. Consistent with these results, 78-week-old Tg^TM4SF5 mice similarly had sinusoidal dilatation, immune cell infiltration, and fibrosis. Altogether, systemic overexpression of TM4SF5 aggravates pathological abnormalities in both the colon and the liver.