Long-term follow-up of children with extrahepatic portal vein obstruction: impact of an endoscopic sclerotherapy program on bleeding episodes, hepatic function, hypersplenism, and mortality

Background: Endoscopic sclerotherapy (ES) has been the standard treatment for children with idiopathic extrahepatic portal vein obstruction (EHPVO). Portosystemic shunts are indicated when variceal bleeding cannot be controlled by ES. Recently, mesenteric left portal vein bypass was indicated as a surgical intervention and preventative measure for hepatic dysfunction in children with long-term EHPVO. Nevertheless, there is a lack Of published data confirming the extent of hepatic dysfunction, hypersplenism, and physical development in children with long-term follow-up. Method: We retrospectively verified the long-term outcomes in 82 children with EHPVO treated with ES protocol, focusing on mortality, control of bleeding, hypersplenism, and consequent hepatic dysfunction. Results: Of the children, 56% were free from bleeding after the initiation of ES. The most frequent cause of rebleeding was gastric varices (30%). Four patients had recurrent bleeding from esophageal varices (4.6%). Four patients underwent surgery as a consequence of uncontrolled gastric varices. There were no deaths. Most patients showed good physical development. We observed a mild but statistically significant drop in factor V motion, as well as leukocyte and platelet count. Conclusion: Endoscopic sclerotherapy is an efficient treatment for children with EHPVO. The incidence of rebleeding is low, and there was no mortality. Children develop mild liver dysfunction and hypersplenism with long-term follow-up. Only a few patients manifest symptoms of hypersplenism, portal biliopathy, or liver dysfunction before adolescence. (C) 2009 Elsevier Inc. All rights reserved.

Long-term results of the percutaneous transhepatic venoplasty of portal vein stenoses after pediatric liver transplantation

This paper has the objective to evaluate retrospectively the long-term results of transhepatic treatment of PV stenoses after pediatric LT. During an eight-yr period, 15 children with PV stenoses underwent PTA with balloon dilation or stent placement in case of PTA failure after LT. Patients` body weights ranged from 9.3 to 46 kg (mean, 15.5 kg). PV patency was evaluated in the balloon dilation and in the stent placement groups. Technical and clinical successes were achieved in all cases with no complication. Eleven patients (11/15; 73.3%) were successfully treated by single balloon dilation. Four patients (4/15; 26.7%) needed stent placement. One patient was submitted to stent placement during the same procedure because of PTA failure. The other three developed clinical signs of portal hypertension because of PV restenoses two, eight, and twenty-eight months after the first PTA. They had to be submitted to a new procedure with stent placement. The follow-up time ranged from 3 to 8.1 yr (mean, 6.3 yr). In conclusion, transhepatic treatment of PV stenoses after pediatric LT with balloon dilation or stent placement demonstrated to be a safe and effective treatment that results in long-term patency.

Effects of the administration of pentoxifylline and prednisolone on the evolution of portal fibrogenesis secondary to biliary obstruction-an experimental study in growing animals

Background: Many chronic liver diseases lead to progressive hepatic fibrosis, a condition that can ultimately result in loss of organ function and severe portal hypertension necessitating hepatic transplantation. Within the last few decades, studies have been conducted to demonstrate the possibility of drug modulation of hepatic fibrogenesis. Regarding biliary obstruction, it has been suggested that administration of corticosteroids could promote better late outcomes for children with biliary atresia submitted to Kasai`s portoenterostomy. Models used to test potential antifibrogenic drugs such as pentoxifylline (PTX) have not included growing animals. Methods: In this experimental study, 119 young rats (21st or 22nd days) were submitted to laparotomy and common bile duct ligation (CBDL) or to sham surgery (SHAM). Animals were allocated into 5 groups, according to surgical procedure, and administered the following solutions: (1) CBDL + distilled water, (2) SHAM + distilled water, (3) CBDL + PTX, (4) CBDL + prednisolone (PRED), and (5) CBDL + PTX + PRED (PTX + PRED). Each group was further divided into 2 subgroups according to the length of the experiment (15 or 30 days). At the end of the defined period, animals were weighed, and a hepatic fragment was collected from each one for analyses. Results: The PTX animals exhibited increased weight gain compared to animals in the PRED or PTX + PRED groups. Animals from the 3 therapeutic groups (PTX, PRED, and PTX + PRED) showed diminished collagen-filled area in portal spaces. Total portal space area was increased in the PTX group. Conclusions: Hepatic fibrosis induced by bile duct ligation in young rats could be modulated by pharmacologic interventions. Administration of PTX or PRED, or the combination of both, resulted in diminished collagen-filled areas in portal spaces. (C) 2009 Elsevier Inc. All rights reserved.

Rex Shunt for Acute Portal Vein Thrombosis After Pediatric Liver Transplantation in Children With Biliary Atresia

Background/Purpose. Posttransplantation portal vein thrombosis (PVT) can have severe health consequences, and portal hypertension and other consequences of the long-term privation of portal inflow to the graft may be hazardous, especially in young children. The Rex shunt has been used successfully to treat PVT patients since 1998. In 2007, we started to perform this surgery in patients with idiopathic PVT and late posttransplantation PVT. Herein we have reported our experience with this technique in acute posttransplantation PVT. Methods. Three patients of ages 12, 15, and 18 months underwent cadaveric (n = 1) or living donor (n = 2) orthotopic liver transplantation (OLT). All patients had biliary atresia with portal vein hypoplasia; they developed acute PVT on the first postoperative day. They underwent a mesenteric-portal surgical shunt (Rex shunt) using a left internal jugular vein autograft (n = 2) or cadaveric iliac vein graft (n = 1) on the first postoperative day. Results. The 8-month follow-up has confirmed shunt patency by postoperative Doppler ultrasound. There have been no biliary complications to date. Conclusions. The mesenteric-portal shunt (Rex shunt) using an autograft of the left internal jugular or a cadaveric vein graft should be considered for children with acute PVT after OLT. These children usually have small portal veins; reanastomosis is often unsuccessful. In addition, this technique has the advantage to avoid manipulation of the hepatic hilum and biliary anastomosis. Although this study was based on a limited experience, we concluded that this technique is feasible, with great benefits to and low risks for these patients.

Absence of bifurcation of the portal vein

Absence of the horizontal segment of the left portal vein (PV) or absence of bifurcation of the portal vein (ABPV) is extremely rare anomaly. The aim of this study was to study the extra-hepatic PV demonstrating the importance of its careful assessment for the purpose of split-liver transplantation. Human cadaver livers (n = 60) were obtained from routine autopsies. The cutting plane of the liver consisted of a longitudinal section made immediately on the left of the supra-hepatic inferior vena cava through the gallbladder bed preserving the arterial, portal and biliary branches in order to obtain two viable grafts (right lobe-segments V, VI, VII, and VIII and left lobe-segments II, III, and IV) as defined by the main portal scissure. The PV was dissected out and recorded for application of the liver splitting. The PV trunk has been divided into right and left branch in 50 (83.3%) cases. A trifurcation of the PV was found in 9 (15.2%) cases, 3 (5%) was a right anterior segmental PV arising from the left PV and 6 (10%) a right posterior segmental PV arising from the main PV. ABPV occurred in 1 (1.6%) case. Absence of bifurcation of the portal vein is a rare anatomic variation, the surgeon must be cautious and aware of the existence of this exceptional PV anomaly either pre or intra-operatively for the purpose of hepatectomies or even split-liver transplantation.

Rex Shunt for the Treatment of Portal Vein Thrombosis After Pediatric Liver Transplantation: A Case Report

Background and Purpose. Late portal vein thrombosis (PVT) can be extremely well tolerated, although portal hypertension and other consequences of the long-term deprivation of portal inflow to the graft may be hazardous, especially in young children. Recently, the ""Rex shunt"" has been used successfully to treat these patients. We now report the initial experience with this novel technique. Methods. A 3-year-old girl with PVT at 7 months after whole organ cadaveric liver transplant displayed portal hypertension with an episode of gastrointestinal bleeding, requiring a mesenteric-portal surgical shunt (""Rex shunt"") using a left internal jugular vein autograft. Results. Upon current follow-up of 6 months, postoperative Doppler ultrasound confirmed shunt patency. Endoscopic status was significantly improved after surgery with resolution of portal hypertension. There was no recurrence of bleeding. Conclusions. The mesenteric-portal shunt (""Rex shunt""), using a left internal jugular vein autograft, should be considered for children with late PVT after liver transplantation. Although this is an initial experience, we may conclude that this technique is feasible, with great potential benefits and low risks for these patients.

Hepatoportal Sclerosis and Extrahepatic Portal Venous Obstruction Associated with Anti-phospholipid Antibody Syndrome in Child

We report a 2-year-old child with extrahepatic portal venous obstruction, hepatoportal sclerosis and pulmonary thromboembolism whose sole hypercoagulability factor was the presence of anti-phospholipid antibodies.

Portal do software p??blico brasileiro

O Portal do Software P??blico Brasileiro consolida-se como uma iniciativa que conseguiu criar um ambiente comum para compartilhar solu????es de software no setor p??blico, racionalizar a gest??o dos recursos de inform??tica, reaproveitar as solu????es de software existentes para diminuir custos e atividades redundantes, estabelecer parcerias e a????es cooperadas, refor??ar a pol??tica p??blica de estimular o uso de software livre e definir uma forma de licenciamento de software que sustente o compartilhamento de solu????es entre os ??rg??os do setor p??blico de acordo com as prerrogativas legais brasileiras e a Constitui????o Federal. Esta iniciativa trouxe como inova????o o compartilhamento de ???software p??blico???, que oficializou um novo modelo de licenciamento e de gest??o das solu????es desenvolvidas na administra????o p??blica, estabelecendo parceria entre diversos ??rg??os, institui????es, empresas e cidad??os, e hoje conta com mais de 100 mil participantes

Portal de peri??dicos da Capes

O Portal de Peri??dicos da Capes disponibiliza ??s institui????es de ensino superior e de pesquisa, conte??do atualizado e de alta qualidade permitindo consultas e acesso aos textos completos de artigos publicados em 23.925 revistas internacionais, nacionais e estrangeiras e mais de 124 bases de dados com resumos de documentos em todas as ??reas do conhecimento. Tem como finalidade oferecer ??s universidades, institutos de pesquisa e organiza????es governamentais, n??o governamentais e particulares, onde se desenvolve a p??sgradua????o e a pesquisa do pa??s, informa????o cient??fica necess??ria ?? forma????o de recursos humanos de alto n??vel e desenvolvimento do conhecimento, tecnologia e inova????o. O acesso ?? biblioteca eletr??nica se d?? via internet, por meio do site www.periodicos.capes.gov.br, de forma gratuita ??s organiza????es autorizadas. Em 2009, foram baixados mais de 65 milh??es de documentos, entre textos completos e abstracts

Portal corporativo da Escola Nacional de Sa??de P??blica Sergio Arouca, da Funda????o Oswaldo Cruz (Fiocruz)

O portal da ENSP foi criado como ferramenta de apoio ?? gest??o do conhecimento e para garantir a qualidade da informa????o gerada e organizada pela institui????o. Oferece um conjunto de aplicativos que consolida, gerencia, analisa e distribui informa????es, n??o s?? internamente, mas tamb??m para o p??blico externo. A integra????o desses aplicativos permite que os usu??rios possam ter um ??nico ponto de acesso ??s informa????es, permitindo ler, gravar e atualizar dados pessoais e institucionais. Nessa vers??o do portal da ENSP, os usu??rios devem-se cadastrar para ter acesso a alguns servi??os, como postagem de documentos na Biblioteca Multim??dia, coment??rios no Informe ENSP e participa????o em redes sociais, documentos internos e informa????es departamentais

Sistema e-Mec:reinven????o processual da educa????o superior em tr??s dimens??es da gest??o p??blica: jur??dica, organizacional e tecnol??gica

Com o objetivo de elevar a qualidade do ensino superior do pa??s, em 2006 o Minist??rio da Educa????o (MEC) iniciou um movimento de ???reinven????o processual??? em tr??s dimens??es: i) jur??dica, com a reformula????o da legisla????o aplic??vel aos processos regulat??rios da educa????o superior; ii) organizacional, implementando mudan??as na din??mica e no desenho das rotinas de trabalho; e iii) tecnol??gica, com a implementa????o do sistema eletr??nico e-MEC de operacionaliza????o dos procedimentos de regula????o. A tr??plice iniciativa permitiu, a um s?? tempo, trazer seguran??a jur??dica, transpar??ncia, credibilidade e celeridade ao processo. Isso possibilitou a cria????o de ambiente prop??cio ao salto qualitativo que o MEC pretendia dar em rela????o tanto ao condicionamento do funcionamento de institui????es e cursos superiores quanto ?? comprova????o da excel??ncia de sua opera????o. O objetivo final??stico foi atingido de maneira satisfat??ria, podendo-se dizer que est?? em curso a institucionaliza????o de uma cultura da qualidade da educa????o superior

Portal da transpar??ncia

O Portal da Transpar??ncia, criado em novembro de 2004, tem por objetivo promover a transpar??ncia da gest??o p??blica e estimular a participa????o e o controle social. O Portal viabiliza o acompanhamento efetivo da execu????o financeira de todos os programas e a????es do Governo Federal, em linguagem simples, navega????o amig??vel e sem necessidade de senhas, de modo que qualquer pessoa possa ter ampla no????o sobre como ?? aplicado o dinheiro p??blico. Est??o dispon??veis informa????es exatas, tempestivas e completas sobre recursos transferidos pelo Governo Federal aos outros entes p??blicos e diretamente ao cidad??o; gastos diretos realizados pelo Governo Federal em compras ou contrata????o de obras e servi??os; bem como gastos realizados por meio de cart??es de pagamentos do Governo Federal. O Portal da Transpar??ncia ?? hoje o mais abrangente banco de dados sobre os gastos do Governo Federal aberto a toda sociedade, representando, assim, iniciativa in??dita de transpar??ncia p??blica no Brasil e no mundo