[Primary sclerosing cholangitis in children and adolescents]. / Colangite esclerosante primária em crianças e adolescentes.

Primary sclerosing cholangitis is a disease usually with a poor prognosis with a mean survival of 10-12 years, starting from the time of diagnosis. Among the different populations studied, males predominate in a ratio 2:1 with ages ranging from 20-78 (mean 42 years). This article describes four patients with this syndrome, a child of eight and three adolescents, thirteen and fourteen years old. It is possibly of autoimmune etiology, characterized by stenosis, dilatation and fibrosis involving the intrahepatic and extrahepatic biliary tree. We analyzed laboratory assays, clinical, immunological, laboratory assays, histological and cholangiographic aspects. In conclusion, in this age group perhaps of the small population sample there was no sex predominance, and 50% progressed without jaundice and without hepatomegaly. The serum levels of gamma glutamyltransferases and especially alkaline phosphatase were elevated compared to aminotransferases without increase of the serum auto antibodies. Only one patients displayed the typical histological appearance of primary sclerosing cholangitis. The diagnosis in al was confirmed by typical cholangiographic signs, restricted only to the intrahepatic biliary tree, identified in all those included in this study.

[Diffuse primary malignant mesothelioma in abdominal cavity]. / Mesotelioma maligno primário difuso em cavidade abdominal.

Two cases of diffuse malignant mesothelioma of abdominal cavity were analysed. These tumors arise from the peritoneum and are also found in the parietal and visceral pleura, pericardium and in vaginal tunic. All of them, infra or supra-diaphragmatic, are associated with asbestos exposure in at least 80% of cases. It is difficult to explain how inhaled asbestos induces peritoneal neoplasms. This aspects become very important in the diagnostic, basically why it is done at laparotomy or laparoscopy. When was proceed the biopsy of the lesions, and occasionally by identification of malignant mesothelial cells in ascitic fluid. In this two cases exposed considerations about the advanced phase of diagnostic are made, the diagnostic was performed in the majority of the collected cells, showing the advanced stage of the disease. At that time of diagnosis we observed poor evolution. We call attention to the importance of precancer diagnosis, the best chance to treatment options, always based on surgical resections, radiation or chemotherapy alone or combined. If the radical surgery is not possible, this patients must be treated by chemotherapy or radiotherapy, defined after complete staging of the disease.

[Treatment of hemorrhagic congestive gastropathy with TIPS implantation. Pilot study]. / Tratamento da gastropatia congestiva hemorrágica através do implante do TIPS. Estudo piloto.

Minimally invasive percutaneous procedures has an important place in internal medicine. In this concept is included a transjugular intrahepatic portosystemic shunts (TIPS). In this pilot study we describe the indication of this therapeutic modality in three cirrhotic patients with hemorrhagic congestive gastropathy unresponsive to infusion of drugs that reduce the portal pressure. All of them were treated by TIPS and control of gastric bleeding occurred in all. One of these showed partial stenosis of the wallstent, treated by prosthesis dilatation. Another developed portosystemic encephalopathy controlled by usual therapeutic measures in the other we implanted two TIPS to obtain a low pressure level of the hepatic-portal gradient. They had no further necessity of blood transfusion, with better hemodynamic conditions and biochemical tests with reduction of the values of the GHP. We conclude that TIPS may be useful to treat hemorrhagic congestive gastropathy acting like a bridge for subsequent elective liver transplantation under good clinical and hemodynamic conditions.

[Reversal of hepatorenal syndrome after liver transplantation. Considerations about 3 patients]. / Reversão da síndrome hepatorrenal após transplante de fígado. Considerações sobre três pacientes.

Hepatorenal syndrome was observed in 25% of the patients with end stage liver disease. In this situation they stayed longer time in intensive unit treatment, hemodialysis. This evolution was observed in 67% of them with this complication after surgery. In this report we describe three patients with hepatorenal syndrome reversible after liver transplant. We define the form of management of these patients after surgery, regard treatment with cyclosporine A, only 72 h after surgery, without administration of aminoglycosides and amphoterycin. This good evaluation also depends of absence of arterial hypotension or infection episodes during and after surgery. It also depends of the good conditions of the implanted liver.

[Transjugular intrahepatic portosystemic shunts (TIPS) as a bridge for liver transplantation]. / Anastomose portossistêmica intra-hepática (TIPS) como ponte para o transplante de fígado.

Transjugular intrahepatic portosystemic shunts (TIPS) represents a new surgical technique minimally invasive utilized in the treatment of portal hypertension. Such technique avoid the risks of general anesthesia, and major surgery like portocava anastomosis, reducing the hepatic-portal gradient, and help bleeding esophagogastric varices, hemorrhagic congestive gastropathy and refractary ascites. Certainly diminishes the intensity of intraperitoneal colorectal circulation and the necessity of blood transfusion during surgery for liver transplantation. In this report we reported the first case in Brazil of the implant of TIPS like preparation for liver transplantation in cirrhotic alcoholic patient. We made consideration about techniques aspects and surgery evolution. This new interesting technique applied for this type of patients is indicated as a bridge for candidates for liver transplantation.

[Ultrasonically guided needle biopsy in malignant focal solid nodules of the liver]. / Biopsia percutânea guiada por ultra-som em lesões nodulares sólidas focais malignas do fígado.

In the most of the cases, the diagnosis of focal solid hepatic lesions are performed by ultrasonography, computed tomography, magnetic resonance and hepatic angiography imagig. However, the distinction between benign and malignant neoplasias, sometimes is made after liver biopsy. This report is about 32 of these lesions, diagnosed after guided liver biopsy by ultrasonography. The efficacy of this propedeutic method, minimally invasive, is defined emphasizing that there are no mortality and low levels of morbidity.

Cholelithiasis in cirrhotic patients. (Analysis of cholelithiasis among patients with liver cirrhosis in São Paulo, Brazil).

During the past 20 years, several authors have reported increased prevalence of cholelithiasis in liver cirrhosis. This biliary disease has been implicated with the deterioration of liver function, liver disease of alcoholic origin or even the presence of hypersplenism in this patient population. This study analyzes the incidence and possible factors which are responsible for promoting cholelithiasis in cirrhosis. The study included 110 cirrhotic patients of a private center specialized in treating liver diseases. The incidence of cholelithiasis was 27.3% (25.3% in males and 33.3% in females). There was no correlation between liver function defined by Child's classification or through the laboratory examinations (AST, ALT, AP, GGT, PT, Alb, TB, DB, PA) and the presence of gallstones. No evidence was found that the etiology of cirrhosis or the presence/absence of hypersplenism affected the prevalence of cholelithiasis in this population. In conclusion, an increased prevalence of cholelithiasis was verified in this population of cirrhotics but the pathogenesis is still obscure.

[Hepatic hemangioma. Analysis of 103 cases. Clinical considerations and imaging methods]. / Hemangiomas hepáticos. Análise de 103 casos. Considerações clínicas e dos métodos de imagem.

Hemangiomas are the most common benign vascular tumor found in the liver. In this study, 103 cases of hepatic hemangiomas were analysed in a retrospective manner. Image aspects such as those seen in ultrassonography, computed tomography, nuclear magnetic resonance and arteriography are discussed. The incidence was higher amongst the female sex, between individuals from 40 to 60 years old, which presented predominantly without symptoms (66%) and localized mainly in the right hepatic lobe (74%). The meaning of those aspects, when adopted, permits the clinicians to differentiate benign from malignant nodular lesions in the liver.