Long-Term Follow-Up After Successful Transjugular Intrahepatic Portosystemic Shunt Placement in a Pediatric Patient with Budd-Chiari Syndrome

Orthotopic liver transplantation is the standard of care in patients with Budd-Chiari syndrome (BCS), and transjugular intrahepatic portosystemic shunt (TIPS) has become an important adjunct procedure while the patient is waiting for a liver. No long-term follow up of TIPS in BCS patients has been published in children. We report successful 10-year follow-up of a child with BCS and iatrogenic TIPS dysfunction caused by oral contraceptive use.

Hepatic Veins and Inferior Vena Cava Thrombosis in a Child Treated by Transjugular Intrahepatic Portosystemic Shunt

We report the case of a 9-year-old boy with portal hypertension, due to Budd-Chiari syndrome, and retrohepatic inferior vena cava thrombosis, submitted to a transjugular intrahepatic portosystemic shunt (TIPS) by connecting the suprahepatic segment of the inferior vena cava directly to the portal vein. After 3 months, the withdrawal of anticoagulants promoted the thrombosis of the TIPS. At TIPS revision, thrombosis of the TIPS and the main portal vein and clots at the splenic and the superior mesenteric veins were found. Successful angiography treatment was performed by thrombolysis and balloon angioplasty of a severe stenosis at the distal edge of the stent.

Management of Uncommon Hernias in Cirrhotic Patients

Background. Abdominal hernias are a common disease among cirrhotic patients, because of malnutrition and persistently high intra-abdominal pressure due to ascites. When tense ascites is present, life-threatening complications are likely to occur. In such cases, the morbidity and mortality rates are high. Objective. We describe 3 cirrhotic patients with rare complicated hernias that needed surgical repair. We discuss optimal timing for surgical approaches and the necessity of ascites control before surgery, as well as the technical details of the procedures. Method. Review of hospital charts of selected rare cases of herniae in cirrhotic patients. Conclusion. Elective surgical approaches can treat even uncommon hernias in cirrhotic patients with good results.

Test of a Modular Fault Current Limiter for 220 V Line Using YBCO Coated Conductor Tapes With Shunt Protection

A modular superconducting fault current limiter (SFCL) consisting of 16 elements was constructed and tested in a 220 V line for a fault current between 1 kA to 7.4 kA. The elements are made up of second generation (2G) YBCO-coated conductor tapes with stainless steel reinforcement. For each element four tapes were electrically connected in parallel with effective length of 0.4 m per element, totaling 16 elements connected in series. The evaluation of SFCL performance was carried out under DC and AC tests. The DC test was performed through pulsed current tests and its recovery characteristics under load current were analysed by changing the shunt resistor value. The AC test performed using a 3 MVA/220 V/60 Hz transformer has shown the current limiting ratio achieved a factor higher than 10 during fault of up to five cycles without conductor degradation. The measurement of the voltage for each element during the AC test showed that in this modular SFCL the quench is homogeneous and the transition occurs similarly in all the elements.

Extrahepatic portal vein thrombosis after umbilical catheterization: is it a good choice for Rex shunt?

Background: Extrahepatic portal vein thrombosis (EHPVT) is an important cause of portal hypertension in children. Rex shunt has been used successfully to treat these patients. Methods: We report our experience in 19 infants and children (5 months to 14 years) with HPVT eligible for a mesenteric-portal surgical shunt with left internal jugular vein autograft. Eight children had idiopathic EHPVT, nine had post-umbilical catheterization EHPVT, one had portal vein agenesis, and one had posttransplant EHPVT. Results: It was possible to perform the Rex shunt in all patients except for 8 of 9 cases in the post-umbilical catheterization EHPVT group. A Warren procedure was performed in 4 of those patients and a proximal splenorenal shunt in 1. Current follow-up ranges from 3 to 26 months. Shunt thrombosis occurred in one patient with portal vein agenesis and associated cardiac anomaly. Portal hypertension has significantly improved after surgery. None of our patients have experienced new bleeding episodes until now. Conclusions: The Rex shunt should be considered in the treatment of children with idiopathic EHPVT experiencing repeated gastrointestinal bleeding episodes refractory to endoscopic treatment. Nevertheless, the role of this operation for children with post-umbilical catheterization EHPVT is yet to be clearly evaluated. (C) 2011 Elsevier Inc. All rights reserved.

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.

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.

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.

The ventriculoomental bursa shunt

Object The aim of this study is to access the efficacy of the omental bursa (lesser sac) as a receptacle of cerebrospinal fluid (CSF) and to use it as an alternative to the ventriculoatrial or ventriculopleural shunts when the peritoneum reduces or loses its CSF absorption capacity. Methods Three patients with hydrocephalus presented with malfunctioning of ventriculoperitoneal shunts, secondary to peritoneal blockage caused by previous episodes of shunt infections in two and peritonitis in one patient. All patients underwent previous shunt revisions due to ventriculitis and shunt obstruction ranging from three to eight times. In order to keep the peritoneal cavity as the main receptacle of CSF absorption site, the distal catheter was inserted in the omental bursa, through the foramen of Winslow, jointly by a pediatric surgeon. We denominated this new technique of CSF diversion as ventriculoomental bursa (VOB) shunting. The children have been followed at least for 1 year (range 12 to 28 months) with no recurrence of shunt. Conclusions VOB shunting may be considered an acceptable technique to CSF shunting when the anterior peritoneum loses or decreases its CSF absorption capacity.

Blood gases and cardiovascular shunt in the South American lungfish (Lepidosiren paradoxa) during normoxia and hyperoxia

The South American lungfish (Lepidosiren paradoxa) has an arterial P(O2), (Pa(O2)) as high as 70-100 mm Hg, corresponding to Hb-O(2) saturations from 90% to 95%, which indicates a moderate cardiovascular right to left (R-L) shunt. In hyperoxia (50% O(2)), we studied animals in: (1) aerated water combined with aerial hyperoxia, which increased Pa(O2) from 78 +/- 2 to 114 +/- 3 mm Hg and (2) and aquatic hyperoxia (50% O(2)) combined room air, which gradually increased Pa(O2) from 75 +/- 4 mm Hg to as much as 146 +/- 10 mm Hg. Further, the hyperoxia (50%) depressed pulmonary ventilation from 58 +/- 13 to 5.5 +/- 3.0 mLBTPS kg h(-1), and Pa(CO2) increased from 20 +/- 2 to 31 +/- 4 mm Hg, while pHa became reduced from 7.56 +/- 0.03 to 7.31 +/- 0.09. At the same time, venous P(O2) (Pv(O2)) rose from 40.0 +/- 2.3 to 46.4 +/- 1.2 mm Hg and, concomitantly, Pvco, increased from 23.2 +/- 1.1 to 32.2 +/- 0.5 mm Hg. R-L shunts were estimated to about 19%, which is moderate when compared to most amphibians. (C) 2010 Elsevier B.V. All rights reserved.

Physiological implications of the regulation of vacuolar H+-ATPase by chloride ions

Vacuolar H+-ATPase is a large multi-subunit protein that mediates ATP-driven vectorial H+ transport across the membranes. It is widely distributed and present in virtually all eukaryotic cells in intracellular membranes or in the plasma membrane of specialized cells. In subcellular organelles, ATPase is responsible for the acidification of the vesicular interior, which requires an intraorganellar acidic pH to maintain optimal enzyme activity. Control of vacuolar H+-ATPase depends on the potential difference across the membrane in which the proton ATPase is inserted. Since the transport performed by H+-ATPase is electrogenic, translocation of H+-ions across the membranes by the pump creates a lumen-positive voltage in the absence of a neutralizing current, generating an electrochemical potential gradient that limits the activity of H+-ATPase. In many intracellular organelles and cell plasma membranes, this potential difference established by the ATPase gradient is normally dissipated by a parallel and passive Cl- movement, which provides an electric shunt compensating for the positive charge transferred by the pump. The underlying mechanisms for the differences in the requirement for chloride by different tissues have not yet been adequately identified, and there is still some controversy as to the molecular identity of the associated Cl--conducting proteins. Several candidates have been identified: the ClC family members, which may or may not mediate nCl-/H+ exchange, and the cystic fibrosis transmembrane conductance regulator. In this review, we discuss some tissues where the association between H+-ATPase and chloride channels has been demonstrated and plays a relevant physiologic role.

Efeito da oxigenoterapia hiperbárica: relato de caso de paciente com síndrome hepato-pulmonar em lista de espera para transplante hepático

INTRODUÇÃO: As doenças hepáticas apresentam índices de morbidade e mortalidade elevados e quando em estágio avançado têm o transplante do fígado como forma de tratamento potencialmente curativo e eficaz, embora este não possa ser oferecido a todos os pacientes. Isso faz com que essas doenças sejam consideradas problema de saúde pública em todo o mundo. Os cuidados clínicos para manter o paciente com condições de esperar e suportar o transplante continua um desafio. RELATO DO CASO: Mulher com 65 anos de idade, procedente do Recife, com diagnóstico de cirrose hepática secundária a vírus C apresentava dispnéia importante aos mínimos esforços tendo PaO2 de repouso de 60 mmHg e O2 de 90%, com espirometria normal. Realizou eco-Doppler que evidenciou shunt pulmonar importante. Durante a triagem em lista de transplante (MELD de 16 em agosto de 2006) foi optado pelo início de sessões de oxigenioterapia em câmara hiperbárica a fim de melhorar a sintomatologia respiratória da síndrome hepato-pulmonar. Apresentava melhora substancial da tolerância ao exercício após a terapia hiperbárica, assim como os valores do PaO2 à gasometria. Realizou 10 sessões de oxigenioterapia em câmara hiperbárica. Realizou transplante hepático em outubro de 2007 e vem em acompanhamento ambulatorial com boa evolução e melhora substancial da dispnéia. CONCLUSÃO: Constatou-se melhora da condição hepato-pulmonar após oxigenoterapia hiperbárica. Desta forma, ela surge como mais uma ferramenta para o tratamento das doenças hepáticas, devendo ser realizados outros estudos que avaliem sua utilização clínica.

Weight loss associated with exercise training restores ventilatory efficiency in obese children

The purpose of this study was to test the hypothesis that in obese children: 1) Ventilatory efficiency (VentE) is decreased during graded exercise; and 2) Weight loss through diet alone (D) improves VentE, and 3) diet associated with exercise training (DET) leads to greater improvement in VentE than by D. Thirty-eight obese children (10 +/- 0.2 years; BMI > 95(th) percentile) were randomly divided into two Study groups: D (n=17; BMI = 30 +/- 1 kg/m(2)) and DET (n = 21; 28 +/- 1 kg/m(2)). Ten lean children were included in a control group (10 +/- 0.3 years; 17 +/- 0.5 kg/m(2)). All children performed maximal treadmill testing with respiratory gas analysis (breath-by-breath) to determine the ventilatory anaerobic threshold (VAT) and peak oxygen consumption (VO(2) peak). VentE was determined by the VE/VCO(2) method at VAT. Obese children showed lower VO(2) peak and lower VentE than controls (p < 0.05). After interventions, all obese children reduced body weight (p < 0.05). D group did not improve in terms of VO(2) peak or VentE (p > 0.05). In contrast, the DET group showed increased VO(2) peak (p = 0.01) and improved VentE(Delta VE/VCO(2) = -6.1 +/- 0.9; p = 0.01). VentE is decreased in obese children, where weight loss by means of DET, but not D alone, improves VentE and cardiorespiratory fitness during graded exercise.

Fault current test of a bifilar Bi-2212 bulk coil

A bifilar Bi-2212 bulk coil with parallel shunt resistor was tested under fault current condition using a 3 MVA single-phase transformer in a 220 V-60 Hz line achieving fault current peak of 8 kA. The fault current tests are performed from steady state peak current of 200 A by applying controlled short circuits up to 8 kA varying the time period from one to six cycles. The test results show the function of the shunt resistor providing homogeneous quench behavior of the HTS coil besides its intrinsic stabilizing role. The limiting current ratio achieves a factor 4.2 during 5 cycles without any degradation.

Offline Detection, Identification, and Correction of Branch Parameter Errors Based on Several Measurement Snapshots

This paper proposes a three-stage offline approach to detect, identify, and correct series and shunt branch parameter errors. In Stage 1 the branches suspected of having parameter errors are identified through an Identification Index (II). The II of a branch is the ratio between the number of measurements adjacent to that branch, whose normalized residuals are higher than a specified threshold value, and the total number of measurements adjacent to that branch. Using several measurement snapshots, in Stage 2 the suspicious parameters are estimated, in a simultaneous multiple-state-and-parameter estimation, via an augmented state and parameter estimator which increases the V - theta state vector for the inclusion of suspicious parameters. Stage 3 enables the validation of the estimation obtained in Stage 2, and is performed via a conventional weighted least squares estimator. Several simulation results (with IEEE bus systems) have demonstrated the reliability of the proposed approach to deal with single and multiple parameter errors in adjacent and non-adjacent branches, as well as in parallel transmission lines with series compensation. Finally the proposed approach is confirmed on tests performed on the Hydro-Quebec TransEnergie network.