Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0036690 (sepsis)
 59,461 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Chronic intestinal pseudo-obstruction (CIPO) in children may be life-threatening due to the complications of parenteral nutrition (PN) or catheter-related sepsis. Multivisceral transplantation (MVTx) is a lifesaving option but limited experience is available. We report our experience with MVTx in pediatric CIPO patients. Sixteen children with CIPO underwent MVTx at median age of 4 years. Indications for MVTx were liver failure (n = 10), loss of venous access (n = 3), or sepsis (n = 3). Modified MVTx without the liver was performed in six patients. Induction immunosuppression included tacrolimus, steroid with adjunctive agent in period I (April 1996 to December 2000), namely, OKT3 (n = 1), mycophenolate mofetil (n = 4), or daclizumab (n = 2); and in period II (January 2001 to present), Campath 1H (n = 4) or daclizumab (n = 5). The grade of rejection was severe in 12.5% and mild to moderate in 87.5% of cases. Isolated rejection of the transplanted stomach or pancreas was not diagnosed during clinical course or on autopsy. Actuarial patient survival for 1 year/2 years for period, I and II were 57.1%/42.9% and 88.9%/77.8%. None of the long-term survivors is on PN and all tolerate enteral feedings. Pancreatic enzyme supplementation or insulin therapy is not needed in survivors. Gastric emptying was substantially affected in one case. Bladder function did not improve in those with urinary retention problems. MVTx for CIPO offers a lifesaving option with excellent function of the transplanted pancreas and stomach among survivors.
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PMID:Multivisceral transplantation for pediatric intestinal pseudo-obstruction: single center's experience of 16 cases. 1505 Jan 42

Chronic intestinal pseudo-obstruction (CIP) is a gastrointestinal motility disorder characterized by chronic symptoms and signs of bowel obstruction in the absence of a fixed, lumen-occluding lesion. Radiographic findings consist of dilated bowel with air-fluid levels. Pseudo-obstruction is an uncommon condition and can result from primary or secondary causes. The management is primarily focused on symptom control and nutritional support to prevent weight loss and malnutrition. The principles of management of patients with CIP involve 1) establishing a correct clinical diagnosis and excluding mechanical obstruction; 2) differentiating between idiopathic and secondary forms; 3) performing a symptomatic and physiologic assessment of the parts of the gastrointestinal (GI) tract involved by manometric and whole gut transit scintigraphic studies; 4) careful assessment of nutritional status of the patient; and 5) developing a therapeutic plan addressing the patient's symptoms and nutritional status. Treatment of CIP includes frequent small meals with a low-fat, low-fiber diet, liquid nutritional supplements may be needed; prokinetic agents such as metoclopramide may help to reduce upper GI symptoms. Trials of drugs such as erythromycin, domperidone, cisapride, and tegaserod may be considered if there is no response. Subcutaneous octreotide may be helpful to improve small bowel dysmotility especially in patients with scleroderma. In patients with symptoms suggestive of bacterial overgrowth, courses of antibiotics such as metronidazole, ciprofloxacin, and doxycycline may be needed. Nutritional assessment and support is an important aspect of management. Enteral nutrition is usually preferred. In carefully selected patients, feeding jejunostomy with or without decompression gastrostomy may be tried. Long term parenteral nutrition should be reserved for patients who can not tolerate enteral nutrition. Complications associated with total parenteral nutrition include infections, sepsis, and cholestatic hepatic dysfunction.
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PMID:Chronic Intestinal Pseudo-Obstruction. 1562 29

Chronic intestinal pseudo-obstruction(CIPO) is a rare severe digestive disease in which clinical symptoms of intestinal obstruction appear without any mechanical cause. Pathophysiologically, CIPO shows ineffective intestinal propulsion due to an impairment of intestinal smooth muscle, enteric nervous system, and interstitial cells of Cajal(ICC). Sustained increased intra-bowel pressure often causes small intestinal malabsorption and bacterial translocation, and leads to malnutrition and blood stream infection (sepsis). Key points of the medical approach for CIPO are to improve nutritional status and reduce abdominal symptoms. Dietary cure and defecation control are the main options in mild cases, whereas home-parenteral-nutrition(HPN) and decompression therapy are often needed in severe cases. Stimulant laxatives, prokinetics and herbal medicine are usually used but often in fail. Percutaneous endoscopic gastrojejunostomy(PEG-J) tube may be burdenless compared to conventional ileus tube. Most important points in the management of this disease are to make a correct diagnosis as early as possible and avoid unnecessary surgery. However, no clear diagnostic criteria have been established so far. Manometry, scintigraphy, and full-thickness biopsy are the major examination for the CIPO diagnosis in the Western countries; however these specialized examinations are not popular in Japan. Therefore the Research Group(chief investigator, Atsushi Nakajima) proposed Japanese diagnostic criteria in 2009 to facilitate the diagnosis of this rare disease by the general physician. In 2013, we have reported that cine-MRI is a non-invasive diagnostic method for CIPO. Although further data are eagerly awaited, it can become a promising diagnostic tool in CIPO patients. Furthermore the Japanese criteria have been revised, and in 2014, the comprehensive criteria from a child to an adult have been devised. In 2015, CIPO is newly certified as Specified Rare and Intractable Disease which is subsidized from public expense, in Japan. In the future, the diagnostic criteria should be appropriately modified by consultation with additional researchers to make them more practical and internationally applicable.
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PMID:[Chronic intestinal pseudo-obstruction]. 2598 46