J.Jpn. Surg. Soc.
ISSN 1880-1129

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J.Jpn. Surg. Soc.. 119(6): 616-622, 2018

Feature topic

PATHOGENESIS OF AND REGENERATIVE THERAPY FOR HIRSCHSPRUNG’S DISEASE AND ALLIED DISORDERS

1) Department of Pediatric Surgery, Reproductive and Developmental Medicine, Faculty of Medical Sciences, Kyushu University, Fukuoka, Japan
2) Department of Molecular Cell Biology and Oral Anatomy, Kyushu University Graduate School of Dental Science, Fukuoka, Japan

Koichiro Yoshimaru1), Kosuke Kirino1), Yusuke Yanagi1), Yuki Kawano1), Jun Kono1), Keiko Irie1), Yoshiaki Takahashi1), Satoshi Obata1), Tomoko Izaki1), Toshiharu Matsuura1), Takayoshi Yamaza2), Tomoaki Taguchi1)

Hirschsprung’s disease (HD) and its allied disorders (ADHD) develop in childhood and cause peristaltic dysfunction of the intestinal tract. Despite the absence of obvious obstructive lesions, bowel obstruction symptoms occur. Among these disorders, the prognosis for HD patients with hypoganglionosis, megacystis microcolon intestinal hypoperistalsis syndrome, and chronic idiopathic intestinal pseudo-obstruction is poor. Although various genetic/nongenetic backgrounds have been discussed as causes of these diseases, their pathogenesis has not yet been clarified. Because outcomes remain unsatisfactory, further investigation of the pathogenesis and the establishment of curative treatment are required. This article explains the outline of HD and ADHD along with their genetic/nongenetic backgrounds and explores the possibility of regenerative medicine for their treatment.

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