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Chronic intestinal pseudoobstruction

MedGen UID:
536759
•Concept ID:
C0238062
•
Disease or Syndrome
Synonym: Intestinal pseudoobstruction, chronic
SNOMED CT: Chronic intestinal pseudo-obstruction (235828008)
 
Monarch Initiative: MONDO:0017574
Orphanet: ORPHA2978

Definition

Chronic intestinal pseudo-obstruction (CIPO) is a rare gastrointestinal motility disorder characterized by recurring episodes resembling mechanical obstruction in the absence of organic, systemic, or metabolic disorders, and without any physical obstruction being detected by X-ray or during surgery. CIPO develops predominantly in children and may be present at birth. [from ORDO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • Chronic intestinal pseudoobstruction
Follow this link to review classifications for Chronic intestinal pseudoobstruction in Orphanet.

Professional guidelines

PubMed

Diagnosis and management of adult patients with chronic intestinal pseudoobstruction.
Sutton DH, Harrell SP, Wo JM
Nutr Clin Pract 2006 Feb;21(1):16-22. doi: 10.1177/011542650602100116. PMID: 16439766
The medical management of intestinal failure: methods to reduce the severity.
Nightingale JM
Proc Nutr Soc 2003 Aug;62(3):703-10. doi: 10.1079/PNS2003283. PMID: 14692605
The surgeon's role in the treatment of chronic intestinal pseudoobstruction.
Murr MM, Sarr MG, Camilleri M
Am J Gastroenterol 1995 Dec;90(12):2147-51. PMID: 8540505

Recent clinical studies

Etiology

Assessment and outcome of children with intestinal failure referred for intestinal transplantation.
Ganousse-Mazeron S, Lacaille F, Colomb-Jung V, Talbotec C, Ruemmele F, Sauvat F, Chardot C, Canioni D, Jan D, Revillon Y, Goulet O
Clin Nutr 2015 Jun;34(3):428-35. Epub 2014 Apr 30 doi: 10.1016/j.clnu.2014.04.015. PMID: 25015836
Stomal prolapse in children with chronic intestinal pseudoobstruction: a frequent complication?
Irtan S, Bellaïche M, Brasher C, El Ghoneimi A, Cézard JP, Bonnard A
J Pediatr Surg 2010 Nov;45(11):2234-7. doi: 10.1016/j.jpedsurg.2010.06.022. PMID: 21034950
Chronic intestinal pseudoobstruction: report of four pediatric patients.
Dalgiç B, Sari S, Doğan I, Unal S
Turk J Gastroenterol 2005 Jun;16(2):93-7. PMID: 16252200
Chronic intestinal pseudoobstruction syndrome: clinical analysis, outcome, and prognosis in 105 children. French-Speaking Group of Pediatric Gastroenterology.
Faure C, Goulet O, Ategbo S, Breton A, Tounian P, Ginies JL, Roquelaure B, Despres C, Scaillon M, Maurage C, Paquot I, Hermier M, De Napoli S, Dabadie A, Huet F, Baudon JJ, Larchet M
Dig Dis Sci 1999 May;44(5):953-9. doi: 10.1023/a:1026656513463. PMID: 10235603
Intestinal motility disorders of infants and children: classification, clinical manifestations and roentgenology.
Franken EA Jr, Smith WL, Frey EE, Sato Y, Anuras S
Crit Rev Diagn Imaging 1987;27(3):203-36. PMID: 2959451

Diagnosis

Pharmacological and nutritional therapy of children and adults with chronic intestinal pseudo-obstruction.
Di Nardo G, Zenzeri L, Guarino M, Molfino A, Parisi P, Barbara G, Stanghellini V, De Giorgio R
Expert Rev Gastroenterol Hepatol 2023 Apr;17(4):325-341. Epub 2023 Mar 26 doi: 10.1080/17474124.2023.2193887. PMID: 36939480
Small Bowel Dysmotility, Pseudoobstruction, and Functional Correlation with Histopathology: Lessons Learned.
Gonzalez Z, McCallum R
Curr Gastroenterol Rep 2020 Feb 20;22(3):14. doi: 10.1007/s11894-020-0748-8. PMID: 32078071
Stomal prolapse in children with chronic intestinal pseudoobstruction: a frequent complication?
Irtan S, Bellaïche M, Brasher C, El Ghoneimi A, Cézard JP, Bonnard A
J Pediatr Surg 2010 Nov;45(11):2234-7. doi: 10.1016/j.jpedsurg.2010.06.022. PMID: 21034950
Diagnosis and management of adult patients with chronic intestinal pseudoobstruction.
Sutton DH, Harrell SP, Wo JM
Nutr Clin Pract 2006 Feb;21(1):16-22. doi: 10.1177/011542650602100116. PMID: 16439766
Chronic intestinal pseudoobstruction.
Faulk DL, Anuras S, Christensen J
Gastroenterology 1978 May;74(5 Pt 1):922-31. PMID: 346432

Therapy

Pharmacological and nutritional therapy of children and adults with chronic intestinal pseudo-obstruction.
Di Nardo G, Zenzeri L, Guarino M, Molfino A, Parisi P, Barbara G, Stanghellini V, De Giorgio R
Expert Rev Gastroenterol Hepatol 2023 Apr;17(4):325-341. Epub 2023 Mar 26 doi: 10.1080/17474124.2023.2193887. PMID: 36939480
Nutritional Interventions in Chronic Intestinal Pseudoobstruction.
Kirby DF, Raheem SA, Corrigan ML
Gastroenterol Clin North Am 2018 Mar;47(1):209-218. Epub 2017 Dec 6 doi: 10.1016/j.gtc.2017.09.005. PMID: 29413013
Use of buprenorphine in children with chronic pseudoobstruction syndrome: case series and review of literature.
Prapaitrakool S, Hollmann MW, Wartenberg HC, Preckel B, Brugger S
Clin J Pain 2012 Oct;28(8):722-5. doi: 10.1097/AJP.0b013e318243f609. PMID: 22688601
Stomal prolapse in children with chronic intestinal pseudoobstruction: a frequent complication?
Irtan S, Bellaïche M, Brasher C, El Ghoneimi A, Cézard JP, Bonnard A
J Pediatr Surg 2010 Nov;45(11):2234-7. doi: 10.1016/j.jpedsurg.2010.06.022. PMID: 21034950
Chronic intestinal pseudoobstruction: report of four pediatric patients.
Dalgiç B, Sari S, Doğan I, Unal S
Turk J Gastroenterol 2005 Jun;16(2):93-7. PMID: 16252200

Prognosis

Small Bowel Dysmotility, Pseudoobstruction, and Functional Correlation with Histopathology: Lessons Learned.
Gonzalez Z, McCallum R
Curr Gastroenterol Rep 2020 Feb 20;22(3):14. doi: 10.1007/s11894-020-0748-8. PMID: 32078071
Stomal prolapse in children with chronic intestinal pseudoobstruction: a frequent complication?
Irtan S, Bellaïche M, Brasher C, El Ghoneimi A, Cézard JP, Bonnard A
J Pediatr Surg 2010 Nov;45(11):2234-7. doi: 10.1016/j.jpedsurg.2010.06.022. PMID: 21034950
Chronic intestinal pseudoobstruction: report of four pediatric patients.
Dalgiç B, Sari S, Doğan I, Unal S
Turk J Gastroenterol 2005 Jun;16(2):93-7. PMID: 16252200
The medical management of intestinal failure: methods to reduce the severity.
Nightingale JM
Proc Nutr Soc 2003 Aug;62(3):703-10. doi: 10.1079/PNS2003283. PMID: 14692605
The colon in the pseudoobstructive syndrome.
Anuras S, Baker CR Jr
Clin Gastroenterol 1986 Oct;15(4):745-62. PMID: 3536207

Clinical prediction guides

Assessment and outcome of children with intestinal failure referred for intestinal transplantation.
Ganousse-Mazeron S, Lacaille F, Colomb-Jung V, Talbotec C, Ruemmele F, Sauvat F, Chardot C, Canioni D, Jan D, Revillon Y, Goulet O
Clin Nutr 2015 Jun;34(3):428-35. Epub 2014 Apr 30 doi: 10.1016/j.clnu.2014.04.015. PMID: 25015836
The role of immunohistochemistry in idiopathic chronic intestinal pseudoobstruction (CIPO): a case-control study.
Amiot A, Cazals-Hatem D, Joly F, Lavergne-Slove A, Peuchmaur M, Bouhnik Y, Bedossa P, Messing B
Am J Surg Pathol 2009 May;33(5):749-58. doi: 10.1097/PAS.0b013e31819b381a. PMID: 19252433
Peripheral neuropathy with hypomyelination, chronic intestinal pseudo-obstruction and deafness: a developmental "neural crest syndrome" related to a SOX10 mutation.
Pingault V, Guiochon-Mantel A, Bondurand N, Faure C, Lacroix C, Lyonnet S, Goossens M, Landrieu P
Ann Neurol 2000 Oct;48(4):671-6. PMID: 11026454
Congenital markers for chronic intestinal pseudoobstruction.
Pulliam TJ, Schuster MM
Am J Gastroenterol 1995 Jun;90(6):922-6. PMID: 7771422
Long-term efficacy of oral cisapride in symptomatic upper gut dysmotility.
Abell TL, Camilleri M, DiMagno EP, Hench VS, Zinsmeister AR, Malagelada JR
Dig Dis Sci 1991 May;36(5):616-20. doi: 10.1007/BF01297028. PMID: 2022163

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