title: Pediatric chronic intestinal pseudo-obstruction is a rare, serious, and intractable disease: a report of a nationwide survey in Japan.
abstract: OBJECTIVE
  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.
authors.affiliation: Department of Pediatric Surgery, Kagoshima University, Japan. Electronic address: mitsuru@m3.kufm.kagoshima-u.ac.jp.
authors.forename: Mitsuru
authors.initials: M
authors.lastname: Muto
authors.affiliation: Department of Pediatric Surgery, Kagoshima University, Japan.
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Matsufuji
authors.affiliation: PAL Children's Clinic, Japan.
authors.forename: Takeshi
authors.initials: T
authors.lastname: Tomomasa
authors.affiliation: Division of Gastroenterology, Yokohama City University School of Medicine, Japan.
authors.forename: Atsushi
authors.initials: A
authors.lastname: Nakajima
authors.affiliation: Department of Pediatric Surgery, Hamamatsu University School of Medicine, Japan.
authors.forename: Hisayoshi
authors.initials: H
authors.lastname: Kawahara
authors.affiliation: Department of Pediatric Gastroenterology, Nutrition and Endocrinology, Osaka Medical Center and Research Institute for Maternal and Child Health, Japan.
authors.forename: Shinobu
authors.initials: S
authors.lastname: Ida
authors.affiliation: Kurume University Medical Center, Japan.
authors.forename: Kosuke
authors.initials: K
authors.lastname: Ushijima
authors.affiliation: Second Department of Surgery, Wakayama Medical University, Japan.
authors.forename: Akio
authors.initials: A
authors.lastname: Kubota
authors.affiliation: Department of Pediatrics, Nara Hospital Kinki University, Japan.
authors.forename: Sotaro
authors.initials: S
authors.lastname: Mushiake
authors.affiliation: Department of Pediatric Surgery, Kyushu University, Japan.
authors.forename: Tomoaki
authors.initials: T
authors.lastname: Taguchi
mesh_terms: D000293:Adolescent; D000328:Adult; D002648:Child; D002675:Child, Preschool; D002908:Chronic Disease; D005260:Female; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D007418:Intestinal Pseudo-Obstruction / Q000175:diagnosis / Q000453:epidemiology*; D007564:Japan / Q000453:epidemiology; D008297:Male; D011159:Population Surveillance; D015995:Prevalence; D011379:Prognosis; D012189:Retrospective Studies; D011795:Surveys and Questionnaires; D055815:Young Adult
keywords: CIPO; Children; Chronic intestinal pseudo-obstruction; Idiopathic; Nationwide survey