Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0023890 (cirrhosis)
 42,195 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Anatomical abnormalities of the small bowel that cause intestinal stagnation result in bacterial overgrowth and a blind loop syndrome (BLS). Bacterial breakdown of bile salts and deamination of protein lead to malabsorption, steatorrhea, and fat-soluble vitamin deficiencies. Four children developed BLS as a complication of necrotizing enterocolitis, jejunal atresia, gastroschisis, and biliary atresia. BLS was suggested by abdominal pain, feculent vomiting, steatorrhea, and hypoalbuminemia. Dilated, stagnant bowel loops were demonstrated in each instance by upper gastrointestinal contrast study. Positive intestinal bacterial aspirates were confirmatory. Antibiotic treatment in two patients improved symptomatology but all children ultimately required surgery. Surgical procedures consisted of blind loop resection, intestinal plication, and catheterization of the bilioenteric conduit. All patients are now asymptomatic but one child suffers from parenteral nutrition-related cirrhosis and another requires chronic antibiotic therapy.
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PMID:The blind loop syndrome in children. 240 46

One hundred twenty-five infants underwent surgical intervention for necrotizing enterocolitis between 1972 and 1984. Sixty-three infants, who survived more than 30 days postoperatively, were evaluated for long-term complications. There were 28 girls and 35 boys (mean birth weight 1,725 +/- 890 g; gestational age 32 +/- 4 weeks). Associated problems included hyaline membrane disease (43), cardiac anomalies (25), and trisomy 21(2). Thirty-six survivors required long-term ventilatory support. Fifty-nine infants underwent bowel resection and enterostomy, 3 decompressing enterostomies without resection, and 1, exploratory laparotomy only. Enterostomies were closed at four months. Twenty four had short bowel syndrome. Fifteen infants subsequently died for a late mortality rate of 23%. Mortality was related to sepsis (3), respiratory failure (5), cardiac anomalies (3), cardio-respiratory arrest (2), and TPN related liver failure (2), and was common with gestational age less than 31 weeks and birth weight less than 1,000 g. Medical problems included cholestasis (17), TPN induced cirrhosis (3), meningitis (3), seizures (8), and nutritional rickets (6). Significant developmental and intellectual delays were observed.
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PMID:Long-term follow-up after surgical management of necrotizing enterocolitis: sixty-three cases. 372 6

We report a 58-year-old woman with pneumatosis intestinalis and necrotizing enterocolitis associated with liver cirrhosis. She was receiving treatment for liver cirrhosis and hepatic failure when sudden severe right upper abdominal pain and fever developed. Abdominal radiograph disclosed pneumatosis intestinalis, involving the ascending colon, and small collections of free air along the right hemidiaphragm. The pneumatosis intestinalis appeared in both cystic and linear form. Autopsy revealed necrosis and multiple gas-filled mural cysts in the ascending colon. To our knowledge, this is the first case to be reported of pneumatosis intestinalis and necrotizing enterocolitis associated with liver cirrhosis.
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PMID:Pneumatosis intestinalis and necrotizing enterocolitis associated with liver cirrhosis. 884 85

Nutritional support to patients in neonatal and pediatric intensive care units is critical not only to minimize negative nitrogen balance but also to promote growth and development. Continuous technological and logistical advances in the Western countries have improved the efficacy and reduced the complications of parenteral nutrition (PN) to the extent that despite the constraints of cost and infrastructure, PN is now fast growing in India. Although widespread availability is very much desired, it is important that the technique is developed with considerable expertise and used judiciously with full knowledge of its indications, limitations, dangers and benefits. Indications for PN include surgical conditions (short gut syndrome), very low birth weight infants (particularly with necrotizing enterocolitis and surgical anomalies), malabsorption syndromes, conditions requiring bowel rest (acute pancreatitis, severe ulcerative colitis and necrotizing enterocolitis) and several non-gastrointestinal indications (end stage liver disease, renal failure, multiple trauma and extensive burns). Provision of PN is associated with significant and sometimes life threatening complications. The possible complications are technical (thrombosis, perforation of vein, thrombophlebitis), infections, metabolic disturbances, hepatobiliary stenosis, cholestasis, fibrosis, cirrhosis or cholelithiasis and bone related complications like osteopenia and fractures. Meticulous monitoring is necessary not only to detect complications but also to document clinical benefit.
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PMID:Pediatric parenteral nutrition in India. 1113 60

Necrotising enterocolitis (NEC) remains a common and severe condition affecting both preterm and term infants. Treatment when NEC involves a large proportion of the gastrointestinal tract remains controversial. We present one surgeon's experience of high jejunostomy (HJ) as the primary procedure in this group of children. HJ was performed in the presence of severe panintestinal disease. In those who survived, a second-look procedure and reconstruction was performed after 6 to 8 weeks. Over a 16-year period, 10 of 113 patients with NEC had a HJ constructed. Two died within 1 day due to persisting instability; the others survived to undergo a second-look laparotomy. Intestinal continuity was restored in all cases with one to five anastomoses. Three patient's died within 1 year from total parenteral nutrition (TPN) related cholestasis and cirrhosis. Five became long-term TPN-free survivors. The HJ as an initial procedure is a useful surgical option in neonates with severe NEC affecting the majority of the intestine. In this high-risk group, we achieved 50% survival from NEC.
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PMID:Is there a role for high jejunostomy in the management of severe necrotising enterocolitis? 1131 69

The risk of hemorrhage in infants with severe coagulopathies unresponsive to fresh frozen plasma (FFP) infusions may preclude therapeutic invasive interventional procedures. We describe the successful use of recombinant factor VIIa (rFVIIa) in two such infants, the first with cirrhosis requiring paracentesis and the second with necrotizing enterocolitis requiring laparotomy. This report reviews the current concepts on the mechanism of action of the drug rFVIIa and considers its expanded use in infants unresponsive to FFP replacement.
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PMID:Use of recombinant factor VIIa in infants with severe coagulopathy. 1511 25

Necrotizing enterocolitis is typically found in premature neonates, but it also has been described in adults, particularly those with cirrhosis and immunocompromised. Radiographic findings of diffuse pneumatosis intestinalis and portal venous gas are characteristic of necrotizing enterocolitis. Here, we provide radiographic findings of a serious adult case of necrotizing enterocolitis. To the best of our knowledge, a wide range of portal vein and vena cava gas has never been reported.
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PMID:Inferior vena cava gas, portal venous system gas, and pneumatosis intestinalis. 2012 98