期刊
JOURNAL OF PEDIATRIC SURGERY
卷 49, 期 12, 页码 1799-1803出版社
W B SAUNDERS CO-ELSEVIER INC
DOI: 10.1016/j.jpedsurg.2014.09.025
关键词
Chronic intestinal pseudo-obstruction; CIPO; Children; Nationwide survey; Idiopathic
资金
- Ministry of Health, Labor and Welfare of Japan
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. (C) 2014 Elsevier Inc. All rights reserved.
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