Pediatric chronic intestinal pseudo-obstruction is a rare, serious, and intractable disease: A report of a nationwide survey in Japan

Mitsuru Muto, Hiroshi Matsufuji, Takeshi Tomomasa, Atsushi Nakajima, Hisayoshi Kawahara, Shinobu Ida, Kosuke Ushijima, Akio Kubota, Sotaro Mushiake, Tomoaki Taguchi

研究成果: ジャーナルへの寄稿学術誌査読

44 被引用数 (Scopus)

抄録

Background/Purpose A nationwide survey was conducted to identify the clinical presentation of pediatric chronic intestinal pseudo-obstruction (CIPO) in Japan. Methods Data were collected via a questionnaire, ensuring patient anonymity, from facilities that treat pediatric gastrointestinal diseases in Japan. Results Ninety-two responses were collected from forty-seven facilities. Sixty-two patients (28 males, 34 females) met formal diagnostic criteria for CIPO. The estimated pediatric prevalence was 3.7 in 1 million individuals. More than half the children (56.5%) developed CIPO in the neonatal period. Full-thickness intestinal specimens were available for histopathology assessment in forty-five patients (72.6%). Forty-one (91.1%) had no pathological abnormalities and were considered to be idiopathic. Patients were treated according to the local protocol of each facility. Forty-one patients (66.1%) had restricted oral intake of ordinary diets, and twenty-nine (46.8%) depended on parenteral nutrition. No therapeutic intervention, including medication and surgery, successfully improved oral food intake or obstructive symptoms. Only three patients (4.8%) died from enteritis or sepsis. Conclusions In Japan, pediatric CIPO is a rare, serious, and intractable disease. The prognosis with respect to survival is good, but unsatisfactory because of the need for prolonged parenteral nutrition and associated potential for restricted quality of life.

本文言語英語
ページ(範囲)1799-1803
ページ数5
ジャーナルJournal of Pediatric Surgery
49
12
DOI
出版ステータス出版済み - 12月 1 2014

!!!All Science Journal Classification (ASJC) codes

  • 小児科学、周産期医学および子どもの健康
  • 外科

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