Mitsuru Muto1, Hiroshi Matsufuji2, Takeshi Tomomasa3, Atsushi Nakajima4, Hisayoshi Kawahara5, Shinobu Ida6, Kosuke Ushijima7, Akio Kubota8, Sotaro Mushiake9, Tomoaki Taguchi10. 1. Department of Pediatric Surgery, Kagoshima University, Japan. Electronic address: mitsuru@m3.kufm.kagoshima-u.ac.jp. 2. Department of Pediatric Surgery, Kagoshima University, Japan. 3. PAL Children's Clinic, Japan. 4. Division of Gastroenterology, Yokohama City University School of Medicine, Japan. 5. Department of Pediatric Surgery, Hamamatsu University School of Medicine, Japan. 6. Department of Pediatric Gastroenterology, Nutrition and Endocrinology, Osaka Medical Center and Research Institute for Maternal and Child Health, Japan. 7. Kurume University Medical Center, Japan. 8. Second Department of Surgery, Wakayama Medical University, Japan. 9. Department of Pediatrics, Nara Hospital Kinki University, Japan. 10. Department of Pediatric Surgery, Kyushu University, Japan.
Abstract
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.
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.
Authors: Eric A Sparks; Cristine S Velazco; Brenna S Fullerton; Jeremy G Fisher; Faraz A Khan; Amber M Hall; Tom Jaksic; Leonel Rodriguez; Biren P Modi Journal: Gastroenterol Res Pract Date: 2017-09-18 Impact factor: 2.260