Mortality rate of patients with asymptomatic primary biliary cirrhosis diagnosed at age 55 years or older is similar to that of the general population

Recent routine testing for liver function and anti-mitochondrial antibodies has increased the number of newly diagnosed patients with primary biliary cirrhosis (PBC). This study investigated the prognosis of asymptomatic PBC patients, focusing on age difference, to clarify its effect on the prognosis of PBC patients. The study was a systematic cohort analysis of 308 consecutive patients diagnosed with asymptomatic PBC. We compared prognosis between the elderly (55 years or older at the time of diagnosis) and the young patients (< 55 years). The mortality rate of the patients was also compared with that of an age- and gender-matched general population. The elderly patients showed a higher aspartate aminotransferase-to-platelet ratio, and lower alanine aminotransferase level than the young patients (P < 0.01 and P = 0.03, respectively). The two groups showed similar values for alkaline phosphatase and immunoglobulin M. Death in the young patients was more likely to be due to liver failure (71%), while the elderly were likely to die from other causes before the occurrence of liver failure (88%; P < 0.01), especially from malignancies (35%). The mortality rate of the elderly patients was not different from that of the age- and gender-matched general population (standardized mortality ratio, 1.1; 95% confidence interval, 0.6-1.7), although this rate was significantly higher than that of the young patients (P = 0.044). PBC often presents as more advanced disease in elderly patients than in the young. However, the mortality rate of the elderly patients is not different from that of an age- and gender-matched general population

Schistosomal portal hypertension. Assessment of portal bood flow before and after surgical treatment

Objetivo: Avaliar o fluxo sanguíneo portal na esquistossomose hepato-esplênica e o efeito tardio do tratamento cirúrgico na hemodinâmica portal. Método: Foram estudados 64 pacientes por Doppler dúplex: grupo I (pacientes com hipertensão portal esquistossomótica); grupo II (pacientes submetidos a desconexão ázigo-portal com esplenectomia) e grupo III (pacientes submetidos derivação esplenorrenal distal). Resultados: O fluxo da veia porta foi maior no grupo I (1954,46 ± 693,73ml/min) e foi menor no grupo III (639,55 ± 285,86ml/min), neste correlacionou-se com o tempo pós-operatório (r=-0,67, p=0,0005). O fluxo sangüíneo portal do grupo II (1097,18 ± 342,12ml/min) foi semelhante ao de indivíduos normais. As mesmas alterações foram verificadas com relação ao diâmetro da veia porta nos grupos I, II, e III (cm): 1,46 ± 0,23; 1,12 ± 0,22; 0,93 ± 0,20, respectivamente. Conclusões: Estes dados sugerem que: 1) Existe hiperfluxo portal na fisiopatologia da hipertensão portal esquistossomótica; 2) o tratamento cirúrgico interferiu na hemodinâmica portal, diminuindo o fluxo sangüíneo da veia porta; 3) Esta redução do fluxo sangüíneo portal correlacionou-se com o tempo de seguimento pós-operatório no grupo III mas não no grupo II. _________________________________________________________________________________________ ABSTRACT: Background: Assessment of the portal blood flow in hepatoesplenic schistosomosis and the late effect of surgical treatment on portal hemodynamics. Method: Were studied 64 patients by duplex scan: group I (patients with schistosomal portal hypertension); group II (patients who underwent esophagogastric devascularization and splenectomy); group III (patients who underwent distal splenorenal shunt). Results: Portal vein blood flow was the highest in group I (1954.46 ± 693.73 ml/min) and the lowest in group III (639.55 ± 285.86 ml/min) which correlated with follow-up time (r=-0.67, p=0.0005). Group II portal flow (1097.18 ± 342.12 ml/min) was similar to control. The same changes were seen in portal vein diameter in groups I, II, III (cm): 1.46 ± 0.23, 1.12 ± 0.22, 0.93 ± 0.20, respectively. Conclusions: Our data suggest that: 1) there is portal overflow in the physiopathology of schistosomal portal hypertension; 2) surgical treatment has interfered in hemodynamic reducing portal venous blood flow; 3) portal venous blood flow reduction correlated with follow-up time in group III but not in group II

Primary biliary cirrhosis

Primary biliary cirrhosis (PBC) is a chronic and slowly progressive cholestatic liver disease of autoimmune etiology characterized by injury of the intrahepatic bile ducts that may eventually lead to liver failure. Affected individuals are usually in their fifth to seventh decades of life at time of diagnosis, and 90% are women. Annual incidence is estimated between 0.7 and 49 cases per million-population and prevalence between 6.7 and 940 cases per million-population (depending on age and sex). The majority of patients are asymptomatic at diagnosis, however, some patients present with symptoms of fatigue and/or pruritus. Patients may even present with ascites, hepatic encephalopathy and/or esophageal variceal hemorrhage. PBC is associated with other autoimmune diseases such as Sjogren's syndrome, scleroderma, Raynaud's phenomenon and CREST syndrome and is regarded as an organ specific autoimmune disease. Genetic susceptibility as a predisposing factor for PBC has been suggested. Environmental factors may have potential causative role (infection, chemicals, smoking). Diagnosis is based on a combination of clinical features, abnormal liver biochemical pattern in a cholestatic picture persisting for more than six months and presence of detectable antimitochondrial antibodies (AMA) in serum. All AMA negative patients with cholestatic liver disease should be carefully evaluated with cholangiography and liver biopsy. Ursodeoxycholic acid (UDCA) is the only currently known medication that can slow the disease progression. Patients, particularly those who start UDCA treatment at early-stage disease and who respond in terms of improvement of the liver biochemistry, have a good prognosis. Liver transplantation is usually an option for patients with liver failure and the outcome is 70% survival at 7 years. Recently, animal models have been discovered that may provide a new insight into the pathogenesis of this disease and facilitate appreciation for novel treatment in PBC