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Pediatric-onset Chronic Nonspecific Multiple Ulcers of Small Intestine: A Nationwide Survey and Genetic Study in Japan

Uchida, Keiichi; Nakajima, Atsushi; Ushijima, Kosuke; Ida, Shinobu; Seki, Yoshitaka; Kakuta, Fumihiko; Abukawa, Daiki; Tsukahara, Hisayuki; Maisawa, Shun-ichi; Inoue, Mikihiro; Araki, Toshimitsu; Umeno, Junji; Matsumoto, Takayuki; Taguchi, Tomoaki

Journal of Pediatric Gastroenterology & Nutrition: April 2017 - Volume 64 - Issue 4 - p 565–568
doi: 10.1097/MPG.0000000000001321
Short Communication: Gastroenterology

ABSTRACT: We performed a Japanese nationwide survey of pediatric-onset chronic nonspecific multiple ulcers of the small intestine between January 2000 and July 2013 in 176 institutions of pediatric surgery or pediatric gastroenterology and clarified the clinical features associated with genetic abnormalities in the Solute Carrier Organic Anion Transporter Family, Member 2A1 (SLCO2A1) gene. A total of 4 cases (3 girls and 1 boy) were diagnosed in this series, which had to be differentiated from Crohn disease, Behçet disease, tuberculosis, or drug-induced enteropathy. Clinical symptoms appeared in infants and accurate diagnosis required several years. Medical therapies for inflammatory bowel disease were administered in all patients; however, 2 of the 4 patients had mutation in the SLCO2A1 gene which are responsible for primary hypertrophic osteoarthopathy, and underwent strictureplasty or ileal resection after long-term follow-up. Pediatric gastroenterologists should include this new entity in the differential diagnosis of small intestinal ulcers and inflammatory bowel disease.

*Department of Gastrointestinal and Pediatric Surgery, Mie University Graduate School of Medicine, Mie

Department of Gastroenterology and Hepatology, Yokohama City University Graduate School of Medicine, Kanagawa

Department of Pediatrics, Kurume University, Fukuoka

§Department of Pediatric Gastroenterology, Nutrition and Endocrinology, Osaka Medical Center and Research Institute for Maternal and Child Health, Osaka

||Department of General Pediatrics, Miyagi Children's Hospital, Miyagi

Department of Pediatrics, Morioka Children's Hospital, Iwate

#Department of Medicine and Clinical Science, Graduate School of Medical Sciences, Kyushu University, Fukuoka

**Division of Gastroenterology and Hepatology, Department of Internal Medicine, Iwate Medical University, Morioka

††Department of Pediatric Surgery, Graduated School of Medical Sciences, Kyushu University, Fukuoka, Japan.

Address correspondence and reprint requests to Keiichi Uchida, MD, PhD, Department of Gastrointestinal and Pediatric Surgery, Mie University Graduate School of Medicine, 2-174 Edobashi, Tsu, Mie 514-8507, Japan (e-mail: ucchie@clin.medic.mie-u.ac.jp).

Received 30 March, 2016

Accepted 19 June, 2016

This study was supported by the Practical Research Project for Rare/Intractable Diseases from the Japan Agency for Medical Research and Development, (AMED) (15ek0109053h0002, to T.M.) and by grants from the Japanese Ministry of Health, Labour, Sciences Research Grants for Research on intractable disease (H24-Nanchi-Ippan-037, to T.T.) and the Japan Society for the Promotion of Science (JSPS) KAKENHI (25460953, to J.U. and T.M.). The funders had no role in study design, data collection and analysis, decision to publish, or preparation of the manuscript.

The authors report no conflicts of interest.

© 2017 by European Society for Pediatric Gastroenterology, Hepatology, and Nutrition and North American Society for Pediatric Gastroenterology,