Treatment and prognostic factors in patients with hepatocellular carcinoma.

INTRODUCTION: Hepatocellular carcinoma is a leading cause of death from cancer worldwide. Survival of patients depends on tumor extension and liver function, but yet there is no consensual prognostic model. AIMS: To evaluate the influence on survival of pretreatment parameters (clinico-laboratorial, liver function, tumor extension, Okuda and Cancer of the Liver Italian program (CLIP) staging) and treatment modalities. METHODS: We retrospectively analyzed 207 patients, diagnosed between 1993 and 2003. The initial treatment was: surgery--six patients; radiofrequency ablation--21; percutaneous ethanol injection--29; transarterial chemoembolization--49; tamoxifen--49; supportive care alone--53. Factors determining survival were assessed by Kaplan-Meier method and Cox regression models. RESULTS: Median survival was 24 months. In univariate analysis, Child-Pugh classification and Model for end-stage liver disease (MELD) score, portal vein thrombosis (PVT), tumor size, number of lesions, Okuda and CLIP scores were all associated with prognosis (P < 0.001). Alpha-fetoprotein levels were not predictive of survival. Independent predictors of survival were ascites, bilirubin, PVT and therapeutic modalities (P < 0.001). In early stage hepatocellular carcinoma (HCC), survival was similar for both percutaneous ablation modalities, either radiofrequency or ethanol injection (P = NS). In advanced HCC, survival was better in patients receiving tamoxifen than supportive care alone (P < 0.001). CONCLUSION: This study reinforces the importance of baseline liver function (Child-Pugh classification and MELD score) in the survival of patients with HCC, although staging systems allowed the stratification of patients in different prognostic groups. Ascites, bilirubin and PVT were independent pretreatment predictors of survival. All treatments influenced the patient's outcome, whether in early or advanced stages.

Hemobilia in hereditary hemorrhagic telangiectasia: an unusual complication of endoscopic retrograde cholangiopancreatography.

Hepatobiliary manifestations of hereditary hemorrhagic telangiectasia (HHT) are rare, but often involve cholestasis. We report here a case of HHT associated with cholestasis due to common bile duct stenosis. Attempted balloon dilation of the stenosis during endoscopic retrograde cholangiopancreatography (ERCP) resulted in hemobilia. Hemostasis was achieved by adjusting the nasobiliary drain. The aim of this report is to highlight the biliary manifestations of HHT and draw attention to an unusual complication of ERCP in this setting.

Enterocutaneous fistulas: an unusual solution.

Enterocutaneous fistulas are not a minor problem in gastrointestinal tract surgery. Significant reduction of mortality and morbidity has been attained but they still remain high. The authors report three clinical cases in which they sealed and treated enterocutaneous, chronic, iatrogenic fistulas by injecting biological glue (N-Butil 2-Cyanoacrylate-Histoacryl) into the internal opening and fistulous tract. We believe that the technic we propose here, in the proper setting, may be an important contribution to the management of iatrogenic (postoperative) enterocutaneous fistulas.

Autosomal dominant polycystic kidney disease: evidence for the existence of a third locus in a Portuguese family.

Autosomal dominant polycystic kidney disease is characterized by clinical and genetic heterogeneity. Two loci implicated in the disease have previously been mapped (PKD1 on chromosome 16 and PKD2 on chromosome 4). By two point and multipoint linkage analysis, negative lod scores have been found for both chromosome 16 and chromosome 4 markers in a large Portuguese family, indicating that a third PKD locus is involved in the development of the disease.

Takayasu's disease presenting as a nephrotic syndrome due to amyloidosis.

We report an 18 year old black woman who presented with nephrotic syndrome in whom the investigations led to the diagnosis of diffuse Takayasu's disease, renal amyloidosis of AA type and interstitial lung disease. Proteinuria in Takayasu's disease is usually ascribed to hypertension or more rarely to glomerulonephritis. This case suggests that amyloidosis should be considered also in the investigation of proteinuria in these patients in view of the serious prognostic implications. This case represents further evidence that Takayasu's disease can be the cause of systemic reactive amyloidosis which may also be the presenting feature.

[Acute pancreatitis: inflammatory effusions--course and prognosis]. / Pancreatite aguda: colecções inflamatórias--evolução e prognóstico.

Ten to twenty per cent of Acute Pancreatitis (AP) attacks are severe and their evolution depends on regional or systemic complications and medical of surgical management applied. Three hundred and twelve cases of AP were retrospectively studied, 51 of which had pancreatic or peripancreatic effusions (early fluid effusion--14%; pseudocyst--14%; phlegmon--43% abscess--29%). Mortality in this group (33%) was greater than in the overall population (5.9%): Alcoholic etiology, concomitant systemic complications and early surgery were determinant factors. Prognostic criteria evaluated had a predictive value of about 80%. Fifty per cent of patients with phlegmon had spontaneous resolution after medical intensive care management. Diagnosis of secondary infection of pancreatic and peripancreatic necrosis is determinant for optimal surgical management. Mortality in abscess was 40%, greater when surgical drainage was done in the first two weeks of disease.