Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0085584 (encephalopathy)
 18,178 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

With a normal and varied food intake, the vitamin supply is often sufficient to avoid vitamin deficiency. Since synthetic vitamins have become available, it has become possible to take with one dose the amount of vitamins normally taken up from food in one year. In these pharmacological doses vitamins must therefore be considered as drugs. And in pharmacological doses, their actions are often different and not directly linked to their physiological activity. Two types of pathologic state are unquestionably the concern of vitaminotherapy: More or less specific and intense vitamin deficiencies: Rickets, scurvy, beri beri, pellagra, vitamin deficiency related to alcohol consumption, polyneuritis, encephalopathy, malabsorption, mucoviscidosis, etc. Genetic defects of vitamin metabolism: Prescriptions for these cases represent only a tiny part of the vitamin pharmaceutic market. The prescription of vitamins as adjuvants in other pathologic states without vitamin deficiency, such as neurological pains, psychosis, prevention of common cold, alopecia, anemia, asthenia, carpal tunnel defect, etc., is frequent. The results may be good; however, in some cases, the efficacy is due to chance or placebo effect, and there is no scientific or experimental evidence of beneficial activity. At the moment, the pharmacological vitamin research is very active. New products with vitamin-like structures are being synthesized for specialized therapeutic applications. They will in the near future probably replace elevated and mega-doses for clinical prescription, except, of course, for the treatment of vitamin deficiency. On the other hand, the use of multivitamin preparations in nutritional dosage will greatly increase.
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PMID:Clinical conditions requiring elevated dosages of vitamins. 250 93

Objections to portal systemic shunting in children with life-threatening hemorrhage from esophageal varices include the high incidence of postshunt encephalopathy with neurologic and psychiatric sequelae and the inability to provide an adequate shunt in very young children. We have operated on eight children in the past 4 years for bleeding varices. The causes were: portal vein thrombosis (3), congenital hepatic fibrosis (2), chronic active hepatitis (2), and cystic fibrosis (1). The ages at operation were between 2 and 17 years. These children underwent various modifications of an operation described by Sugiura. The operation we have developed is done through a single thoracoabdominal incision, dividing and anastomosing the esophagus with a stapler, preserving the vagal innervation to the pylorus and antrum, and wrapping the fundus around the distal esophagus at the site of the anastomosis. The venous drainage of the lower esophagus and of the upper stomach is divided. The operation is therefore shorter and simpler, but adheres to the principles enunciated by Sugiura. Complications include one significant postoperative anastomotic leak and one symptomatic esophageal stricture. Longterm results have been gratifying with no evidence of rebleeding from esophageal varices. We believe that our modification of the original Sugiura operation is the preferred therapy of bleeding esophageal varices when surgical intervention is indicated because it preserves the normal structure and function of the upper gastrointestinal tract as well as the portal venous drainage to the liver.
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PMID:A modified Sugiura operation for bleeding varices in children. 660 31

Ninety children with portal hypertension were treated by portal diversion. Fifty-two had cavernous transformation of the portal vein and 38 had an intrahepatic block from various causes. There were 59 central splenorenal shunts, 19 mesocaval, 11 portacaval and one distal splenorenal. In 61 peripheral shunts the veins used for the anastomosis were less than 10 mm in diameter. There was no operative mortality in children with extrahepatic block. One child with cystic fibrosis died postoperatively. Thrombosis of the shunt occurred in five children (5.6 per cent) and was responsible for recurrent bleeding in two. Four children with a thrombosed shunt underwent succesful reoperation and one is awaiting another anastomosis. No late complications occurred in the 52 children with extrahepatic block, while encephalopathy developed in four children with intrahepatic block. These figures confirm our earlier results in the management of portal hypertension in childhood and suggest that portal diversion is the treatment of choice. Several precautions have permitted lowering of the rate of thrombosis whichever shunt is performed. Portal diversion should be indicated following the first episode of hemorrhage in children with extrahepatic block. In patients with intrahepatic block, congenital hepatic fibrosis and cystic fibrosis are good indications as are in general the hepatic diseases with no or mild activity.
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PMID:Portal diversion for portal hypertension in children. The first ninety patients. 740 58

TIPSS was successfully performed in a 10-year-old female cystic fibrosis (CF) patient with bleeding gastric varices due to portal hypertension; precipitation of portosystemic encephalopathy later unveiled the presence of a latent colonic stricture associated with high potency pancreatic enzymes. The unusual sequence of events resulting from the co-existence of two CF pathologies are described, and the implications of treatment discussed.
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PMID:Colonic stricture in cystic fibrosis unmasked by successful transjugular intrahepatic portosystemic stent shunt (TIPSS). 887 12

The records of 22 patients who received portosystemic shunting for portal hypertension from 1985 to 1995 inclusive at the Royal Alexandra Hospital for Children (RAHC) were retrospectively reviewed. There were 11 girls and 11 boys. The average age at operation was 8 years, 3 months (range, 2 years, 3 months to 16 years, 7 months). The aetiology was idiopathic portal cavernomatous transformation (n = 9), billiary atresia (n = 4), cystic fibrosis (n = 3), documented neonatal portal vein thrombosis (n = 3), congenital hepatic fibrosis (n = 2), and portal vein obstruction after liver transplant (n = 1). The major presenting problem was upper gastrointestinal haemorrhage. Two patients had recurrent melaena from Roux-en-Y jejunal loop and caecal varices, respectively. Before receiving shunts, 12 patients had endoscopic sclerotherapy, 1 had gastric transection, and 2 had gastric varices oversewn. Portal pressure at preoperative splenoportogram averaged 28 mm Hg (range, 20 to 41). Urgent shunts were performed on 13 patients. Two disadvantaged patients had prophylactic shunts for severe hypersplenism. The types of shunts used were reversed splenorenal (n = 13), splenoadrenal (n = 6), inferior mesenteric renal (n = 1), portocaval (n = 1), inferior mesenteric caval (n = 1), and superior and inferior mesenteric caval (n = 1). In all, 22 patients had 23 shunts. The patency rate was 96% on 6 months to 10 years follow-up (average, 5.8 years). No spleen was lost. There were 2 late deaths. Two cystic fibrosis patients and one child with extrahepatic portal hypertension experienced post-shunt encephalopathy. Three patients rebled in the early postoperative period despite a patent shunt. Two patients subsequently received liver transplantation without any additional difficulties. Thus, portosystemic shunting using a method appropriate for the patient is a reliable option for treating children with portal hypertension in whom variceal sclerotherapy is inappropriate or has failed.
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PMID:Portosystemic shunting for paediatric portal hypertension. 909 25

Lung transplantation has recently offered hope for prolonged survival in patients with cystic fibrosis. Patients with cystic fibrosis have a 7% prevalence of associated liver disease and portal hypertension. These patients have been previously excluded from consideration for lung transplantation. The natural history of cystic fibrosis-associated liver disease suggests a benign and protracted course in most cases. At the University of Washington, 14 of 53 patients (26%) have undergone lung transplantation for cystic fibrosis-related respiratory failure. We report the outcome of double lung transplantation in four of these 14 patients who also had cystic fibrosis-associated liver disease and portal hypertension, all of whom were symptom free from their liver disease. All four patients are alive and well without complications 4 to 31 months after transplantation. We conclude that the presence of cystic fibrosis-associated liver disease with portal hypertension, in the setting of good synthetic function (albumin > 3.0 gm/L and normal prothrombin time), normal serum bilirubin, minimal varices, without ascites or encephalopathy, should not be an absolute contraindication to lung transplantation. We recommend that other transplantation centers also include this patient population in consideration for lung transplantation.
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PMID:Successful lung transplantation in spite of cystic fibrosis-associated liver disease: a case series. 932 44

(1) In 1996 the evidence, though limited, suggested that the hydrophilic bile acid ursodeoxycholic acid slowed the progression of primary biliary cirrhosis. Given the absence of an effective alternative, we considered that the treatment was "A real advance" in this setting. (2) The file is far bulkier now. A meta-analysis of 11 randomised placebo-controlled trials of ursodeoxycholic acid in the treatment of primary biliary cirrhosis did not show efficacy. Histologically, the data suggest that the drug lowers the risk of hepatic fibrosis, but this is based on a low level of evidence. Moreover, there was no clinical improvement in the longest randomised trial (median follow-up 3.4 years). (3) Likewise, ursodeoxycholic acid seems to have a biochemical effect but no clear clinical effect in sclerosing cholangitis or in treating the hepatic complications of cystic fibrosis. (4) The main adverse effects are gastrointestinal disorders. (5) There are still no drugs clearly capable of slowing the clinical progression of primary biliary cirrhosis. Nevertheless, given the minor adverse effects of ursodeoxycholic acid, some patients may wish to gamble on a possible benefit in the very long term. (6) When primary biliary cirrhosis becomes symptomatic (with encephalopathy, osteomalacia, etc.) pending liver transplantation, treatment is aimed at controlling symptoms and preventing the rupture of oesophageal varices.
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PMID:Ursodeoxycholic acid: a second look. Primary biliary cirrhosis: dashed hopes. 1206 38

Liver disease is rare in childhood, but important new developments have altered the natural history and outcome. It is important that clinicians are aware of these diseases and their management. Acute liver failure is most often due to viral hepatitis, paracetamol overdose, or inherited metabolic liver disease. The clinical presentation includes jaundice, coagulopathy, and encephalopathy. Early diagnosis is necessary to prevent complications such as cerebral oedema, gastrointestinal bleeding, and renal failure. Early supportive management, in particular intravenous N-acetylcysteine, may be effective but liver transplantation is usually the definitive treatment and thus early referral to a specialist unit for liver transplantation is mandatory. Chronic liver failure may be due to unresolved neonatal liver disease, either inherited biliary hypoplasia or extrahepatic biliary atresia, while in older children, autoimmune liver disease or cystic fibrosis are the commonest causes. Treatment includes specific medication, nutritional support, and liver transplantation, which now has a 90% survival with good quality life.
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PMID:Managing liver failure. 1249 21

Following a pulmonary transplantation for cystic fibrosis, 2 patients exhibited a syndrome associating arterial hypertension, headache, visual trouble and generalized seizures. Cerebral magnetic resonance imaging revealed diffuse cortical and subcortical lesions predominantly in posterior regions. The exclusion of alternate diagnoses and the disappearance of the symptoms when the cyclosporine treatment was stopped confirmed the diagnosis of cyclosporine-related reversible posterior encephalopathy syndrome (PRES). Immediate appropriate management resulted in symptom disappearance and regression of radiological images.
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PMID:[Posterior reversible encephalopathy syndrome: about 2 cases related to the cyclosporine]. 1283 74

A 24 year old woman presented with a sudden excruciating headache mimicking an acute vascular event. She had undergone a lung transplantation because of cystic fibrosis and was receiving maintenance treatment with tacrolimus and prednisone. Ancillary investigation excluded vascular causes. Magnetic resonance imaging demonstrated hyperintense lesions in the infratentorial and parieto-occipital regions consistent with posterior leucencephalopathy syndrome. Both her clinical condition improved and the lesions disappeared completely after withdrawal of tacrolimus, suggesting that her condition could be explained by a tacrolimus encephalopathy.
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PMID:Acute headache as a presenting symptom of tacrolimus encephalopathy. 1287 50


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