CIPO is the very " tip of the iceberg" of functional gastrointestinal disorders, being a rare and frequently misdiagnosed condition characterized by an overall poor outcome. Diagnosis should be based on clinical features, natural history and radiologic findings. There is no cure for CIPO and management strategies include a wide array of nutritional, pharmacologic, and surgical options which are directed to minimize malnutrition, promote gut motility and reduce complications of stasis (ie, bacterial overgrowth). Pain may become so severe to necessitate major analgesic drugs. Underlying causes of secondary CIPO should be thoroughly investigated and, if detected, treated accordingly. Surgery should be indicated only in a highly selected, well characterized subset of patients, while isolated intestinal or multivisceral transplantation is a rescue therapy only in those patients with intestinal failure unsuitable for or unable to continue with TPN/HPN. Future perspectives in CIPO will be directed toward an accurate genomic/proteomic phenotying of these rare, challenging patients. Unveiling causative mechanisms of neuro-ICC-muscular abnormalities will pave the way for targeted therapeutic options for patients with CIPO.

Chronic intestinal pseudo-obstruction (CIPO) is a rare and severe condition characterized by a marked impairment of gut propulsive motility mimicking a mechanical obstruction in the absence of any demonstrable mechanical cause of luminal constriction of the gastrointestinal tract. Abnormalities of the enteric neuromuscular control systems may account for the gut dysmotility observed in CIPO. Patients with CIPO usually experience disabling symptoms and potentially life-threatening complications which may go unrecognized. Nutritional support is often required and is a cornerstone of the management of patients with CIPO. Medical and surgical therapeutic options are generally unsatisfactory and long-term outcome is poor in most cases.

Chronic intestinal pseudo-obstruction: clinical features, diagnosis, and therapy

DE GIORGIO, Roberto
;
2011

Abstract

CIPO is the very " tip of the iceberg" of functional gastrointestinal disorders, being a rare and frequently misdiagnosed condition characterized by an overall poor outcome. Diagnosis should be based on clinical features, natural history and radiologic findings. There is no cure for CIPO and management strategies include a wide array of nutritional, pharmacologic, and surgical options which are directed to minimize malnutrition, promote gut motility and reduce complications of stasis (ie, bacterial overgrowth). Pain may become so severe to necessitate major analgesic drugs. Underlying causes of secondary CIPO should be thoroughly investigated and, if detected, treated accordingly. Surgery should be indicated only in a highly selected, well characterized subset of patients, while isolated intestinal or multivisceral transplantation is a rescue therapy only in those patients with intestinal failure unsuitable for or unable to continue with TPN/HPN. Future perspectives in CIPO will be directed toward an accurate genomic/proteomic phenotying of these rare, challenging patients. Unveiling causative mechanisms of neuro-ICC-muscular abnormalities will pave the way for targeted therapeutic options for patients with CIPO.
DE GIORGIO, Roberto; Cogliandro, Rf; Barbara, G; Corinaldesi, R; Stanghellini, V.
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Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/11392/2374850
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