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)

The authors performed 20 liver transplantations from living related donors between June 1990 and July 1991. The 20 pediatric patients (14 biliary atresia, two Budd-Chiari syndrome, one liver cirrhosis after hepatitis C viral infection (HCV hepatitis), 1 progressive intrahepatic cholestasis, 1 liver cirrhosis, 1 protoporphyria) were transplanted with 11 left lobes, eight left lateral segments, and one right lobe. The choice of donors was restricted to the parents of the recipients. The immunosuppressive treatment consisted of FK 506 and steroids. Seventeen recipients are alive, 15 of whom are well and at home. Two recipients, who underwent emergency transplantation, died of postoperative complications. Another recipient died of accidental asphyxia at 6 months after the transplantation. All 20 donors had uneventful postoperative courses and were able to resume their normal social lives. The arterial ketone body ratio (AKBR) increased to above 1.0 within 2 days after the transplantation in all cases. Relatively mild rejection episodes were encountered in only two cases transplanted with ABO-compatible grafts, and these were treated successfully with steroids and FK 506.
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PMID:An appraisal of pediatric liver transplantation from living relatives. Initial clinical experiences in 20 pediatric liver transplantations from living relatives as donors. 128 74

Over a period of 10 years, 65 cases of hepatic cirrhosis and cirrhogenic disease have been observed in five Pediatric Centers in mid Tunisia. The age of the patients ranged from 30 days to 14 years. The main etiology was biliary cirrhosis (24 cases) followed by post-hepatic cirrhosis (15 cases). Eight cases had a metabolic origin, which was Wilson's diseases in five cases. Three children had cirrhosis of a pre-hepatic origin. In 15 children, the liver biopsy showed the presence of cirrhosis but the etiology could not be found. Preventive measures are needed in order to reduce the frequency of cirrhosis among Tunisian children: 1) early recognition of biliary atresia, 2) vaccination against hepatitis B virus of at risk neonates and children, 3) Genetic counselling and search for familial cases when cirrhosis of metabolic origin is identified, particularly Wilson's disease.
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PMID:[Cirrhosis and cirrhogenic diseases in Tunisian children. Multicenter study of 65 cases]. 133 67

Increased portal pressure is the product of both increased resistance to splanchnic flow through the liver and increased blood flow in the portal circuit. Although portal hypertension in children is less common than in adults, the important clinical end results are the same, ie, esophageal variceal hemorrhage, ascites, and hypersplenism. The etiology of portal hypertension in children is very different from adults in whom cirrhosis (most commonly secondary to alcohol) is the predominant cause. In children, extrahepatic obstruction due to portal vein thrombosis is the most common cause. However, as children survive longer with biliary atresia, cystic fibrosis, and other liver diseases, the incidence of intrahepatic obstruction causing portal hypertension is increasing. The treatment has also undergone a dramatic evolution over the last decade with the near extinction of portosystemic shunt procedures and their replacement with endoscopic treatment of esophageal varices and liver transplantation.
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PMID:Portal hypertension. 134 80

The histological and immunohistochemical characteristics of the liver in 44 children (28 boys, 16 girls) with extrahepatic biliary atresia at different stages of the clinical course were studied. Thirty-four wedge liver biopsy specimens taken during Kasai operations (25 specimens) and relaparotomy (9 specimens) and 20 hepatectomy explants taken at the time of transplantation were examined. Routine histological stains and monoclonal antibodies against different molecular weight cytokeratins and HLA-DR were used. The histopathological changes and the pattern of cytokeratin expression observed during the course of the disease were suggestive of persistent or recurrent extrahepatic biliary obstruction that occurred despite the Kasai operation and eventually led to cirrhosis and liver failure. Quantitative studies showed a progressive loss of intrahepatic bile ducts over the time course of the disease. This destruction of bile ducts had a geographic anatomical distribution in hepatectomy specimens, and in two livers it occurred predominantly in only one lobe. This geographic distribution of the vanishing bile ducts probably indicates an unpredictable and uneven obliteration of bile ducts in the porta hepatis during portoenterostomy wound healing and scarring.
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PMID:Intrahepatic bile duct loss in biliary atresia despite portoenterostomy: a consequence of ongoing obstruction? 137 80

Hypoxemia in liver cirrhosis has been attributed to increased pulmonary perfusion; lung function abnormalities have rarely been found in adults. In infants, however, smaller airways and the disproportion in size between the enlarged liver and abdominal cavity leading to lung compression by elevated diaphragms may well suggest that ventilation disturbances play an important role in the development of hypoxemia. We examined lung functions, ventilation-perfusion scans, chest radiographs, and blood gases in air and 80% oxygen in 19 infants with biliary atresia (mean age 14 months) and found maximum flows at functional residual capacity (VmaxFRC) markedly decreased [48% +/- 29% (mean +/- SD)] and thoracic gas volume (TGV) elevated (156% +/- 30.2%). PO2 was less than 9.3 kPa in seven of 19 patients, in whom TGV was higher compared with the other patients (182% vs. 141%, p less than 0.005). However, the decrease in PO2, was much more closely correlated to the amount of shunting (r = 0.62, p less than 0.05) than to the reduced airway patency (VmaxFRC/TGV, r = 0.41, p = 0.08). We conclude that airway narrowing probably by lung compression is present more frequently in infants than in adults with liver disease. We found some evidence that hyperinflation contributes to the observed low PO2 values, possibly aggravated by inadequate vasoconstriction to hypoxic stimuli. However, pulmonary shunting independent of ventilatory disturbances more readily explained hypoxemia already present in these infants.
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PMID:Hypoxemia in infants with biliary atresia: the role of airway obstruction. 140 65

Liver biopsy in the diagnosis of the results of a retrospective analysis of percutaneous liver biopsy in the differential diagnosis of prolonged cholestasis in infancy are reported. We compare the clinical features, serum bilirubin levels, hepatobiliary scintigraphy and histology in two groups of patients. One group of 56 patients had extrahepatic biliary obstruction (biliary atresia: 42; choledochal cyst: 9; extrinsic obstruction: 4; Caroli's disease: 1). Another group of 54 children had intrahepatic cholestasis with patent biliary tract (hepatitis: 38; non-specific cholestasis: 14; cirrhosis: 2). The percutaneous liver biopsy was better than the other procedures to differentiate biliary atresia from hepatitis. We conclude that percutaneous liver biopsy should be carried out in children with prolonged cholestasis when other non-invasive procedures have not ruled out extrahepatic biliary obstruction and before any surgical exploration of the biliary tract is performed.
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PMID:[Role of liver biopsy in the diagnosis of prolonged cholestasis in infants]. 143 8

Ursodeoxycholic acid (UDCA) allows symptomatic treatment of cholestatic liver diseases such as primary biliary cirrhosis, primary sclerosing cholangitis, intrahepatic biliary atresia, and cholestasis of cystic fibrosis. Patients should be treated at an early stage of the disease in order to prevent progression to cirrhosis. Since UDCA has no toxic effects longterm treatment with this substance is possible without the risk of undesired side effects. In patients with primary biliary cirrhosis and rapid progression of the disease, UDCA may be combined with an immunosuppressive substance (i.e. cyclosporin). In primary sclerosing cholangitis, biliary atresia and cholestasis of cystic fibrosis, UDCA at present seems the only treatment of which a benefit for the patients can be expected. In endstage disease liver transplantation is indicated. The role of UDCA in chronic hepatitis and alcohol induced liver disease needs to be clarified in further studies. Whether the improvement of laboratory tests in such patients indicates amelioration of the course of disease, still is unclear.
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PMID:[Treatment of cholestatic liver diseases; the role of ursodeoxycholic acid]. 144 78

The extracellular matrix (ECM) is a complex of macromolecules that includes collagens, proteoglycans, and complex glycoproteins. In fibrotic liver tissue there is an increase in all of these matrix components, and they increase in serum in the patients with alcoholic hepatitis or liver cirrhosis. These ECM components have been used as a serum marker of hepatic fibrosis. Prolonged obstruction of bile flow results in morphologic and biochemical changes and the development of secondary biliary cirrhosis. In congenital biliary atresia (CBA) there is a close correlation between the degree of the hepatic fibrosis and bile flow after the operation. We estimated that, in CBA, ECM increased in serum, and it would reflect the degree of the hepatic fibrosis. To clarify this we examined the serum procollagen-III-peptide (P-III-P) and laminin in CBA patients. P-III-P was elevated in all preoperative patients but in two of the three postoperative patients whose jaundice disappeared P-III-P was in the normal range. In the all 3 patients whose jaundice continued, P-III-P was in normal range. Serum laminin was elevated in 12 preoperative patients with CBA, but there is no correlation between day of diagnosis and level of laminin. Mean concentration in CBA without jaundice after operation was 3.18 U/mL, 3.226 U/mL in CBA with jaundice and 3.3 U/mL in infantile hepatitis. There were no significant differences among three groups. With the elevation of serum alanine aminotransferase, aspartate aminotransferase, and total bilirubin, serum laminin level was also increased.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Laminin and procollagen-III-peptide as a serum marker for hepatic fibrosis in congenital biliary atresia. 150 Oct 26

According to our recent study, 38 of 93 patients (40.9%) who underwent portoenterostomy at Juntendo University Hospital between 1977 and 1986 survived for more than 5 years. In order to learn the future improvement of the prognosis of biliary atresia patients who undergo portoenterostomy, we investigated the relationship between the prognosis of biliary atresia patients and the age and the histological changes of liver at the time of surgery. Twenty-seven of 30 patients (90%) who survived for more than 5 years with no jaundice and no findings of liver cirrhosis had a mild degree of liver fibrosis (F1 or F2) and a mild degree of degeneration of intrahepatic bile ducts (B1 or B2) at the time of surgery, in spite of the difference of size of intrahepatic bile ducts at the porta hepatis. On the other hand, all 8 patients who showed severe liver fibrosis (F3) and severe degeneration of intrahepatic bile ducts (B3) did not survive for more than 5 years. Accordingly, we can expect promising prognoses in patients who undergo refined portoenterostomy procedures and receive proper postoperative treatment before they have severe histological changes of liver. The portoenterostomy will still play an important role as treatment of biliary atresia in the future.
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PMID:The future role of hepatic portoenterostomy as treatment of biliary atresia. 150 Oct 28

We retrospectively studied the clinical use of MR angiography, a gradient-echo technique susceptible to motion, in the preoperative evaluation of the hepatic vasculature in children with liver disease. Twenty imaging examinations were performed in 18 patients 3 months to 10 years old (mean, 2.3 years). Eleven patients had liver tumors and seven had cirrhosis associated with biliary atresia, cystic fibrosis, and short-bowel syndrome. Respiratory motion artifacts were present in most patients, but all MR angiographic studies were technically adequate. Comparing MR angiograms with spin-echo images, we found that vascular visualization was better with MR angiography in 12 cases (60%), equal in five (25%), and worse in three (15%). In 10 of 20 cases, MR angiography provided additional vascular information not present on spin-echo images. MR angiography is a useful adjunct to spin-echo imaging for assessing hepatic vascular anatomy in children with liver disease.
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PMID:Diagnosis of liver disease in children: value of MR angiography. 150 37


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