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

Many disorders are capable of producing cholestasis in infancy. Primary hepatobiliary diseases and systemic infectious, toxic, and metabolic insults may present clinically as conjugated hyperbilirubinemia. A careful, organized diagnostic evaluation allows early identification of potentially treatable lesions. Recent success in the surgical management of biliary atresia, previously a uniformly fatal disorder, has emphasized the need for early diagnosis. Medical management of the complications of chronic cholestasis remains a major challenge. Liver transplantation currently offers the only chance for long-term survival for infants with progressive cirrhosis.
 ...
PMID:Cholestasis in infancy and childhood. 675 96

One hundred and three infants with prolonged cholestasis beginning before 3 months were classified as having alpha-1-antitrypsin deficiency (17 patients), scanty interlobular bile ducts (16 patients), or "neonatal hepatitis" (70 patients). Twenty-two gradually developed chronic liver disease and the remaining 81 recovered within a few months. Prognosis was found to be poor for infants with alpha-1-antitrypsin deficiency, scanty interlobular bile ducts, and familial "idiopathic" hepatitis. Patients who developed cirrhosis often presented with severe and persistent neonatal cholestasis, mimicking extrahepatic biliary atresia and leading to laparotomy. Thus, a high-risk group of infants-defined by aetiology, family history, and degree of cholestasis-can be recognised in the first months of life.
 ...
PMID:Long-term prognosis for infants with intrahepatic cholestasis and patent extrahepatic biliary tract. 697 22

One hundred and twenty-five children with chronic liver disease were seen in Pune in 13 months. Fifty-nine of them, aged 8-39 months, had Indian childhood cirrhosis histologically diagnosed. Their characteristics included an insidious onset of symptoms, a geographical clustering of cases in rural areas north-east of Pune, a high rate of parental consanguinity and affected siblings, and a very high hepatic copper concentration (790-6654 micrograms/g dry weight). Only 8 survived for 6 months, adverse prognostic features being jaundice, ascites, enlargement of the gall bladder, and severe anaemia at presentation. Clinical differentiation from other liver disorders in the same age group was clear in advanced cases but unreliable in earlier cases. Four asymptomatic siblings with hepatomegaly had a benign course. The need for non-invasive methods to diagnose early cases in the community is demonstrated. The other major diagnostic categories were: unresolved hepatitis (12); chronic active hepatitis (7); cryptogenic cirrhosis (6); neonatal hepatitis and biliary atresia (8).
 ...
PMID:Liver disease in India. 718 21

Failure of early diagnosis of biliary atresia results in the development of cirrhosis and death. Commonly used hepatobiliary agents are not ideal for follow-up studies because of their unfavorable physical properties or short half-life. The excellent physical properties of Ru-97 should overcome these limitations. Therefore, Ru-97 PIPIDA (N,alpha-(p-isopropyl acetanilide) iminoacetic acid) is being investigated as a potential hepatobiliary agent that would allow an improved diagnosis of the disease. Ruthenium-97 PIPIDA and Tc-99m PIPIDA showed similar blood clearance rates in dogs. Ru-97 PIPIDA scintigrams in dogs showed early uptake in liver and gallbladder and slow excretion through the gastrointestinal tract. Biodistribution studies were performed in normal rats and rats with biliary obstruction. The findings suggest that Ru-97 PIPIDA should be useful for delayed studies (1-3 days) of the biliary tract.
 ...
PMID:Ruthenium-97 hepatobiliary agents for delayed studies of the biliary tract. I. ru-97 PIPIDA: concise communication. 720 82

The portal vein pressure was measured and biopsies of the liver were taken during the corrective operation in 31 patients with biliary atresia and during relaparotomy in 16 patients free from jaundice 4 mo to 9 yr after a successful corrective operation. Because the portal vein pressure was higher than 200 mmH2O in about 70% of patients during the corrective operation, portal hypertension appears to have already developed in most of the patients with biliary atresia at 2-4 mo of age. In the patients who had had frequent episodes of postoperative cholangitis, the portal vein pressure was elevated and the amount of interstitial tissue in the liver was markedly increased at reoperation compared with those at the corrective operation. These results showed that postoperative cholangitis aggravated portal hypertension and fibrosis of the liver. On the contrary, the portal vein pressure declined in patients in whom active bile drainage had persisted and cholangitis had not been complicated after operation. An early corrective operation and prevention of postoperative cholangitis are of the greatest important for prevention of development of the portal hypertension and cirrhosis of the liver in long-term survivors after surgery for biliary atresia.
 ...
PMID:Changes of portal vein pressure and intrahepatic blood vessels after surgery for biliary atresia. 724 16

Choledochal cyst is a rare surgical condition that requires prompt surgical treatment to prevent biliary cirrhosis and portal hypertension. The results in this series indicate that the preferred surgical management is: (A) cyst excision and anastomosis of the common bile duct to a Roux-en-Y jejunal loop or (B) direct anastomosis of the "cyst" to a Roux-en-Y jejunal loop. A higher long-term complication rate was seen in patients after direct anastomosis of the duodenum to the adjacent "cyst." Choledochal cyst with biliary atresia probably represents an operable form of extrahepatic bile duct atresia. Of 22 patients with biliary atresia, 11 are living and well without liver disease. The importance of early surgical attention cannot be over emphasized.
 ...
PMID:Choledochal cyst: survey by the surgical section of the American Academy of Pediatrics. 725 48

We measured plasma concentration of endothelin-1 in three children with Byler's disease, five with biliary atresia after portoenterostomy, and nine controls. No patients had ascites or hepatorenal syndrome. Plasma endothelin-1 levels were significantly higher in patients with Byler's disease than in the controls (5.19 +/- 0.90 versus 1.81 +/- 0.19 pg/ml, respectively; p < 0.01), but were normal in operated biliary atresia. Urinary concentrations of N-acetyl-beta-D-glucosaminidase (NAG) were significantly higher in the patients with Byler's disease than in controls. Plasma endothelin-1 level correlated significantly with serum concentration of bile acid (r = 0.91; p < 0.01) and urinary concentration of NAG (r = 0.92; p < 0.01). We conclude that plasma endothelin-1 levels are high in patients with severe biliary cirrhosis and that endothelin-1 may partially contribute to development of renal injury in cirrhosis.
 ...
PMID:Plasma endothelin-1 levels of children with cirrhosis. 747 10

We reviewed 37 living related liver transplantations (LRLT) performed by our department during the last 27 months on children with end-stage liver disease. The patients were 15 boys and 22 girls aged 7 months to 15 years with biliary atresia (27), cryptogenic cirrhosis (3), Budd-Chiari syndrome (2), progressive intrahepatic cholestasis (2), protoporphyria (1), Wilson's disease (1), and fulminant hepatitis (1). The donors were 14 fathers and 23 mothers. Grafts were made from the left lateral segment (19), left lateral segment with partial S4 (11), left lobe (6), and right lobe (1). After graft harvesting all donors resumed normal liver function and normal life. The recipient underwent total hepatectomy with preservation of the inferior vena cava. FK506 and low-dose steroids were used for immunosuppression. The survival rate was 90% (27/30) in elective cases and 57% (4/7) in emergency cases. Six recipients had functioning grafts but died of extrahepatic complications. Hepatic vein stenosis occurred in 3 cases at 3 months after LRLT and was successfully treated by balloon dilatation. Portal vein stenosis occurred in 1 case at 8 months after LRLT and was also safely dilated. We incurred no hepatic artery thrombosis after introducing microsurgery techniques. Among 12 viral, 5 bacterial, and 3 fungal postoperative infections, 1 Candida pneumonia and 1 EBV-associated lymphoma were lethal. Three patients with ABO-blood group compatible grafts and one with an incompatible graft developed acute rejection, which was controlled in evey case by steroid bolus and/or increasing the dose of FK506. There were no definite episodes of rejection in ABO-identical cases. Children with moderate growth retardation (> or = -1.5 SD of normal growth) caught up in growth soon after LRLT, but those with severe retardation (<-1.5 SD) were slow to attain age-normal height. Appropriate timing, meticulous surgical procedures, and comprehensive management of complications are crucial for successful outcome with LRLT. LRLT is a promising option for alleviating the shortage of livers for pediatric transplantation and may be regarded as an independent modality to supplement cadaver donation.
 ...
PMID:Living related liver transplantation in children. 751 49

The survival of infants with biliary atresia has improved significantly during the past two decades as a result of modification of the Kasai hepatoportoenterostomy procedure complemented by advances in liver transplantation. Recent reports suggest that the long-term success rate of the Kasai procedure is 40%. Failures are salvaged by liver transplantation. Advances in organ preservation, the use of reduced-sized grafts, and newer immunosuppressive agents (cyclosporine, FK 506) have strongly influenced these improved results. Unfortunately, liver transplantation is associated with a high complication rate, the risk of opportunistic infection, and an increased rate of malignancy due to immunosuppression. Until immunotolerance can be achieved, the Kasai procedure remains the procedure of choice for infants with biliary atresia. Liver transplantation is a life-saving complementary procedure for patients who fail to drain bile following the Kasai procedure, who are older than 3 to 4 months of age at diagnosis, or who have advanced cirrhosis. In the current era, the overall survival should exceed 80%.
 ...
PMID:Is there a place for the Kasai procedure in biliary atresia? 758 57

Pulmonary arteriovenous shunting (PAVS) with hypoxemia is a severe complication of cirrhosis that may regress after liver transplantation. We report PAVS in 25 children with cirrhosis and in 1 with portal vein obstruction; proof of shunting was obtained by technetium Tc 99m microaggregated albumin pulmonary scanning or a high alveoloarterial O2 gradient or both. Cyanosis or dyspnea or both occurred at ages ranging from 6 months to 14 years, earlier in children with biliary atresia and polysplenia syndrome (p < 0.01). Mean arterial oxygen tension (PaO2) was 57 mm Hg (range, 42 to 81 mm Hg) during breathing of 21% O2 and 367 mm Hg (range, 179 to 535 mm Hg) in 100% O2. Cardiac index was always raised, significantly more in children with biliary atresia and polysplenia syndrome (p < 0.01). Seven untreated children died 3 months to 8 years after the diagnosis of PAVS. Eleven underwent liver transplantation: seven are alive (follow-up, 1 to 4 years) and have no signs of PAVS. The PaO2 value during breathing of 100% O2 was > 300 mm Hg in the survivors and < 200 mm Hg in the four nonsurvivors (p < 0.01). These results indicate (1) that PAVS can occur at any age in children with portal hypertension, and that the risk is highest and earliest in children with biliary atresia and polysplenia syndrome, (2) that early liver transplantation allows regression of PAVS, and (3) that the prognosis may in part be related to the level of PaO2 while the patient is breathing 100% O2. The results indicate that systematic screening for PAVS should be part of the examination of these children.
 ...
PMID:Pulmonary arteriovenous shunting in children with liver disease. 769 35


<< Previous 1 2 3 4 5 6 7 8 9 10 Next >>