ISSN:0270-9139    

DOI:10.1002/hep.1840040702

出版商:W.B. Saunders    

年代:1984

数据来源: WILEY

摘要:

AbstractPrognosis of 600 consecutive patients with hepatocellular carcinoma was analyzed in relation to treatment. They were divided into three stages based on four parameters of advanced disease: ascites, tumor greater than 50% of the two‐dimensional size of the liver, serum albumin below 3 gm per dl, and serum bilirubin above 3 mg per dl. Stage I had none of these signs; Stage II one or two signs, and Stage III three or all signs. Of 600 patients, 98 had resection, 333 had nonsurgical treatment (158 treated by intraarterial chemotherapy, 94 systemic chemotherapy, 77 transcatheter embolization, and 4 others) and 169 no treatment.The median survival of untreated patients was only 1.6 months from diagnosis, and no untreated Stage III patient lived more than 3 months; there was a median survival of 0.7 month. Surgically treated patients lived significantly longer than nonsurgical patients of comparable stages; median survival was 19.6 months in the former and 2.8 months in the latter. Whereas Stage I patients did fairly well without treatment, chemotherapy significantly prolonged survival of patients of Stages II and III. These results suggest that early diagnosis and hepatic resection improve prognosis in patients with hepatocellular carcinoma in the areas where this cancer frequently emerges unicentrically. In view of the generally poor prognosis, liver transplantation is recommended when resection is not possible or indicated, and before extrahepatic metastasis occur

ISSN:0270-9139    

DOI:10.1002/hep.1840040703

出版商:W.B. Saunders    

年代:1984

数据来源: WILEY

摘要:

AbstractExtrahepatic biliary atresia is defined as partial or total absence of permeable bile duct between porta hepatis and the duodenum. The incidence varies from 1:8,000 to 1:10,000. Cholestasis is total and permanent. 131I Rose Bengal test and needle liver biopsy allow correct identification of 95% of cases before surgery Before the surgical procedure described by Kasai in 1959, all patients died between 1 and 2 years of age. Through the use of different types of Kasai's procedures by experienced groups, 30 to 35% of patients are successfully operated on. In our group, 248 infants underwent surgery, 121 before 1977: 44 (36.6%) are alive at least 5 years after surgery. Growth is within normal limits in all children still alive, even in those who presented with numerous and severe episodes of cholangitis during the first two postoperative years. Portal hypertension was progressive in 23 children with splenomegaly and esophageal varices developing between 1 and 2 years of age. Five patients bled profusely between 2 and 4 years of age: portal systemic shunts were performed in 4. Surgical liver biopsies were performed in 20 children, 5 to 8 years old: cirrhosis was present in all and was micronodular in 13 and macronodular in 7. The most impressive histologic change was absence of biliary ducts or ductules. This histologic finding can be related with percutaneous transhepatic cholangiographic pictures.Despite the abnormal appearance of intrahepatic bile ducts and the constant presence of cirrhosis with portal hypertension, all 44 patients who are alive at least 5 years after surgery seem to lead almost normal lives.

ISSN:0270-9139    

DOI:10.1002/hep.1840040704

出版商:W.B. Saunders    

年代:1984

数据来源: WILEY

摘要:

AbstractLiver disease related to a–1–antitrypsin deficiency occurs only in Pi ZZ homozygous children. Eleven per cent of Pi ZZ infants present with prolonged neonatal cholestasis. In our group, 25 of 45 Pi ZZ infants with prolonged neonatal cholestasis presented with later cirrhosis. Persistence of jaundice beyond the sixth month of age, early development of splenomegaly, persistence of hard hepatomegaly and liver function abnormalities, and early portal fibrosis have a poor prognostic significance. The most severe course occurs in infants with an early histologic pattern of paucity of interlobular bile ducts. Portal hypertension was present in 19 of 25 children presenting with cirrhosis; 8 of 25 Pi ZZ children with cirrhosis died during childhood.Long‐term protein‐restricted diet and portal systemic shunts were helpful in treatment of four Pi ZZ children with cirrhosis; however, the long‐term course in Pi ZZ children with cirrhosis is unpr

ISSN:0270-9139    

DOI:10.1002/hep.1840040705

出版商:W.B. Saunders    

年代:1984

数据来源: WILEY

摘要:

AbstractThe clinical course of certain patients with Wilson's disease resembles that of patients with viral or drug‐induced fulminant hepatitis lasting only few weeks from recognition of symptoms to severe hepatic insufficiency and death. The disease is complicated by hemolysis and is characterized by hypercupremia. Routine laboratory findings may underestimate the severity of the disease. These patients, as well as patients with decompensated Wilsonian cirrhosis who are not responding to therapy, should be considered as candidates for liver transplant

ISSN:0270-9139    

DOI:10.1002/hep.1840040706

出版商:W.B. Saunders    

年代:1984

数据来源: WILEY

摘要:

AbstractThe liver may be involved in metabolic disorders in two ways, (i) Due to its critical role in several metabolic pathways, the liver may be the major site of expression of a biochemical abnormality. For example, in Type I Crigler‐Najjar syndrome, absence of hepatic bilirubin glucuronyl transferase activity causes severe unconjugated hyperbilirubinemia which invariably leads to death from kernicterus. (ii) The liver may also be structurally damaged by the metabolic disorder. Several patients with protoporphyria have died in hepatic failure due to liver damage caused by protoporphyrin deposition. Numerous other metabolic disorders can be sited in which significant liver involvement occurs. In many of these disorders, there is no effective medical therap

ISSN:0270-9139    

DOI:10.1002/hep.1840040707

出版商:W.B. Saunders    

年代:1984

数据来源: WILEY

摘要:

AbstractMortality of fulminant hepatic failure with standard supportive therapy is high (80 to 85%), but unfortunately most patients present to hospital already in Grade IV encephalopathy with severe liver dysfunction, and many have secondary organ damage. If seen earlier, during Grade III encephalopathy, recent experience with charcoal hemoperfusion can give good results (65% survival). Transplantation has a place in those patients who do not respond, and in cases with early Grade IV encephalopathy. Transplantation should also be considered in patients with subacute hepatic failure in whom mortality is equally high and progression to cirrhosis may occur in those who recover. In both groups, the ability to correct the hemorrhagic diathesis and an adequate supply of donor organs are essential to achievement of a successful transplant at this critical time.

ISSN:0270-9139    

DOI:10.1002/hep.1840040708

出版商:W.B. Saunders    

年代:1984

数据来源: WILEY

摘要:

AbstractChronic hepatitis is defined as diffuse chronic liver disease existing for at least 6 months. Cirrhosis is a sequel. It is of multiple etiology. Liver biopsy is essential for diagnosis and prognosis. Hepatitis B‐related chronic hepatitis is slowly progressive. Corticosteroid therapy is disappointing. Current antiviral therapy converts the hepatitis B e antigen‐positive patient to anti‐HBe in about 50%. Non‐A, non‐B virus hepatitis‐related chronic hepatitis suffers from lack of a diagnostic marker. No current therapy is of proven benefit. Autoimmune lupoid chronic active hepatitis presents a very active biochemical and immunological picture. Prednisolone therapy prolongs life but does not prevent the development of cirrhosis. Drug‐related liver disease is recognized by its associations. Recovery follows withdrawal of the drug. Deaths often follow continuation of the drug. Indications of progression to a terminal state with likelihood of less than a 6–month survival are detailed. These are helpful in deciding on hepatic transplantation before the patient b

ISSN:0270-9139    

DOI:10.1002/hep.1840040709

出版商:W.B. Saunders    

年代:1984

数据来源: WILEY

ISSN:0270-9139    

DOI:10.1002/hep.1840040710

出版商:W.B. Saunders    

年代:1984

数据来源: WILEY

ISSN:0270-9139    

DOI:10.1002/hep.1840040711

出版商:W.B. Saunders    

年代:1984

数据来源: WILEY