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Chronic intestinal pseudo-obstruction
Chronic intestinal pseudo-obstruction (CIPO) is a rare disease characterized by repetitive episodes or continuous symptoms of bowel obstruction when no blockage exists
Prevalence
Exact prevalence unknown
N/A
US Estimated
N/A
Europe Estimated
Age of Onset
All ages
ICD-10
K59.8
Inheritance Pattern
Autosomal dominant
Autosomal recessive
X-linked recessive
5 Facts you should know
FACT
CIPO is characterized by recurrent symptoms of bowel obstruction without a mechanical blockage, caused by impaired enteric nerve or smooth muscle function
FACT
Can be primary (idiopathic or genetic) or secondary to systemic diseases such as mitochondrial disorders, connective tissue diseases, or neurologic conditions
FACT
Clinical manifestations include abdominal pain, distension, nausea, vomiting, constipation, malnutrition, and failure to thrive, with onset possible at any age, from infancy to adulthood
FACT
Diagnosis relies on a combination of clinical history, radiographic and motility studies, and exclusion of mechanical obstruction, with full-thickness intestinal biopsy sometimes required to assess neuromuscular abnormalities
FACT
Management is supportive and multidisciplinary, focusing on nutritional support (enteral or parenteral nutrition), prokinetic agents, infection control, and in severe cases, intestinal transplantation
Interest over time
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Common signs & symptoms
Abnormal nervous system morphology
Intestinal malrotation
Pyloric stenosis
Constipation alternating with diarrhea
Malnutrition and growth failure
Current treatments
Nutritional support
enteral feeds, parenteral nutrition when needed
Prokinetic agents
(e.g., erythromycin, metoclopramide) with limited benefit