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Chronic Idiopathic Intestinal Pseudo-Obstruction (CIIP)

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Hirschsprung’s Disease and the Allied Disorders

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Abstract

Chronic idiopathic intestinal pseudo-obstruction (CIIP) is a rare and intractable disease with an unknown etiology for which effective treatment methods have not yet been established. A key to the diagnosis of CIIP is the recognition of nonmechanical obstructive symptoms, which last for ≥2 months for cases of neonatal onset and ≥6 months for cases of postneonatal onset, including adults, and the exclusion of pathological abnormalities in the ganglion plexus of the affected intestine by a full-thickness biopsy. More than half of childhood cases of CIIP develop during the neonatal period. Peristaltic disorders occur at single or multiple sites of the gastrointestinal tract. Remission and exacerbation of the pseudo-obstructive symptoms are repeated with the progress of the condition. However, no treatment modality has been clearly shown to be effective in relieving symptoms. While the prognosis of CIIP with respect to the survival is good, this outcome is deemed unsatisfactory given the restriction of patients’ long-term quality of life due to daily requirements, including intravenous nutrition support and management of enterostomy. The number of adult patients with CIIP transitioning from childhood is expected to increase in the future.

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Authors and Affiliations

  1. Department of Neonatology, Perinatal Medical Center, Kagoshima City Hospital, Kagoshima, Japan

    Mitsuru Muto

  2. Department of Pediatric Surgery, Research Field in Medicine and Health Sciences, Medical and Dental Sciences Area, Research and Education Assembly, Kagoshima University, Kagoshima, Japan

    Mitsuru Muto

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  1. Mitsuru Muto
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Correspondence to Mitsuru Muto .

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Editors and Affiliations

  1. Department of Pediatric Surgery, Kyushu University, Fukuoka, Fukuoka, Japan

    Tomoaki Taguchi

  2. Department of Pediatric Surgery, St. Luke’s International Hospital, Chuo-Ku, Tokyo, Japan

    Hiroshi Matsufuji

  3. Department of Pediatric Surgery, Kagoshima University, Kagoshima, Kagoshima, Japan

    Satoshi Ieiri

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Muto, M. (2019). Chronic Idiopathic Intestinal Pseudo-Obstruction (CIIP). In: Taguchi, T., Matsufuji, H., Ieiri, S. (eds) Hirschsprung’s Disease and the Allied Disorders. Springer, Singapore. https://doi.org/10.1007/978-981-13-3606-5_42

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  • DOI: https://doi.org/10.1007/978-981-13-3606-5_42

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