Intestinal pseudo-obstruction

Clinical features of IPO can include abdominal pain, nausea, abdominal distension, vomiting, dysphagia, and constipation. Symptoms depend on the portion of the gastrointestinal tract involved == Epidemiology ==

According to a 2013 study conducted in Japan, the estimated prevalence of CIPO was between 0.80 and 1.00 per 100,000 people, while its incidence rate was reported as 0.21 to 0.24 per 100,000. Furthermore, some studies have indicated that CIPO is more prevalent in females, a trend that may be attributable to the higher rates of secondary CIPO from conditions more common in this demographic. In the case of pediatric intestinal pseudo-obstruction (PIPO), the incidence was estimated at approximately 1 per 40,000 live births. ==Causes==

In primary CIPO (the majority of chronic cases) the condition results from disruption of the intestine's ability to move food. These can be broadly classified as myopathic (affecting the smooth muscle), mesenchymopathic (affecting the interstitial cells of Cajal), or neuropathic (of the nervous system) of the gastrointestinal tract. In some cases there appears to be a genetic association. One form has been associated with DXYS154, some associated with defective ACTG2 gene Secondary chronic intestinal pseudo-obstruction can occur as a consequence of a number of other conditions including: • Hirschsprung's disease – the absence of colonic nerve cells • Chagas' disease – a chronic parasitic infection of the colon leading to loss of nerve endings • Kawasaki disease - a rare presentation for this particular autoimmune disorder of the vasculature • Parkinson's disease - related to the neurodegeneration of gastrointestinal tract • Autoimmune conditions — conditions including systemic lupus erythematosus and scleroderma lead to collagen vascular deposition and gastrointestinal motility disruption • Mitochondrial disease - IPO is a known presentation for mitochondrial disease • Endocrine disorders • Certain medications. The term may be used synonymously with enteric neuropathy if a neurological cause is suspected. ==Diagnosis==

The symptoms of IPO are nonspecific. It is not unusual for patients to present repeatedly and to undergo numerous tests. ==Treatment==

Treatment for IPO (acute or chronic) is aimed at removing the disease process and/or managing the complications present. Focus is placed on management of pain, gastrointestinal symptoms, nutritional deficiencies, fluid status, infection control, and improving quality of life. When CIPO is secondary to another disease, treatment is addressed towards the underlying condition. Surgery is sometimes required in severe cases of CIPO. Medical treatment Prucalopride, pyridostigmine, are medications that aim to enhance intestinal motility. Intestinal stasis, which may lead to bacterial overgrowth and subsequently, diarrhea or malabsorption, is treated with antibiotics. Nutritional deficiencies are treated by encouraging patients to avoid foods that increase distention and are difficult to digest (e.g. those high in fat and fibre), consuming small frequent meals (5–6 per day), focusing on liquids and soft food. Reducing intake of poorly absorbed sugar alcohols may be of benefit. Referral to an accredited dietitian is recommended. If dietary changes are unsuccessful in meeting nutritional requirements and energy needs, enteral nutrition is used. Many patients eventually require parenteral nutrition. Procedures Intestinal decompression by tube placement in a small stoma can also be used to reduce distension and pressure within the gut. The stoma may be a gastrostomy, jejunostomy, ileostomy, or cecostomy. These may be used for feed (e.g. gastrostomy and jejunostomy) or to flush the intestines. Colostomy or ileostomy can bypass affected parts if they are distal to (come after) the stoma. For instance, if only the colon is affected, an ileostomy may be helpful. Either of these ostomies are typically placed at or a few centimeters below the patient's navel per doctor recommendation based on the affected area of the intestines as well as concerns for patient comfort and future physical growth for children. One operation involving multi-organ transplant of the pancreas, stomach, duodenum, small intestine, and liver, and was performed by Doctor Kareem Abu-Elmagd on Gretchen Miller. Potential treatments Further research is necessary into other treatments which may alleviate symptoms. These include stem-cell transplantation and fecal microbiota transplantation. has not been studied with regards to CIPO. Any claims to its efficacy for use in CIPO are speculative. ==Related disorders==

• Ogilvie syndrome: acute pseudoobstruction of the colon in severely ill debilitated patients. • Hirschsprung's disease: enlargement of the colon due to lack of development of autonomic ganglia. • Intestinal neuronal dysplasia: a disease of motor neurons leading to the bowels. • Bowel obstruction: mechanical or functional obstruction of the bowel, most commonly due to adhesions, hernias or neoplasms. • Enteric neuropathy: alternative name sometimes used for diagnosis in UK ==See also==