biliary atresia/hypoxia

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Systems Analysis of Biliary Atresia Through Integration of High-Throughput Biological Data

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Ielogoties Reģistrēties

Biliary atresia (BA), blockage of the proper bile flow due to loss of extrahepatic bile ducts, is a rare, complex disease of the liver and the bile ducts with unknown etiology. Despite ongoing investigations to understand its complex pathogenesis, BA remains the most common cause of liver failure

Late complications of biliary atresia: hepatopulmonary syndrome and portopulmonary hypertension.

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Ielogoties Reģistrēties

Children with biliary atresia (BA) following Kasai portoenterostomy have a high risk for portal hypertension, however, while variceal and hemorrhagic complications have been more commonly studied, less frequent but no less possibly devastating complications of hepatopulmonary syndrome (HPS) and

Hypoxic-ischemic gene expression profile in the isolated variant of biliary atresia.

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Ielogoties Reģistrēties

BACKGROUND Biliary atresia (BA) includes a sclerosing cholangiopathy whose nature is not fully deciphered. Aiming to evaluate the role of an arteriopathy as an etiologic factor in BA, we investigated hypoxia and the correlated angiogenic response in livers from affected patients. METHODS Gene

Effects of hypoxemia on early postoperative course of liver transplantation in pediatric patients with intrapulmonary shunting.

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Ielogoties Reģistrēties

Nine pediatric patients (mean age, 10 years) with biliary atresia, who had hypoxemia related to intrapulmonary shunting, underwent living related liver transplantation. The effects of hypoxemia during the early postoperative period after liver transplantation on cardiopulmonary and renal function,

Pediatric living donor liver transplantation for biliary atresia with hepatopulmonary syndrome: the gift of a second wind.

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Ielogoties Reģistrēties

OBJECTIVE Hepatopulmonary syndrome (HPS) is a progressive, deteriorating complication of end-stage liver disease (ESLD) that occurs in 13-47% of liver transplant candidates. Although LT is the only therapeutic option for HPS, it has a high morbidity and mortality, especially in patients with severe

Successful liver transplantation in a child with biliary atresia and hepatopulmonary syndrome.

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Ielogoties Reģistrēties

Hepatopulmonary syndrome (HPS) is characterized by hypoxemia in patients with severe chronic liver disease and pulmonary vasodilatation in the absence of primary cardiac or pulmonary disease. Severe hypoxemia resulting from HPS is generally considered a contraindication to liver transplantation. We

Immunolocalization of VEGF A and its receptors, VEGFR1 and VEGFR2, in the liver from patients with biliary atresia.

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Ielogoties Reģistrēties

In biliary atresia (BA), a cholangiopathy of elusive etiology invariably leads to cirrhosis, and a disturbed angiogenesis may be involved. We evaluated the hepatobiliary immunolocalization of vascular endothelial growth factor (VEGF) A, VEGF receptor 1 (R1), and R2 in BA. We analyzed biopsies

Extracorporeal Membrane Oxygenation in a Pediatric Patient with Hepatopulmonary Syndrome and Interrupted Inferior Vena Cava After Living Related Liver Donation.

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Ielogoties Reģistrēties

Extracorporeal membrane oxygenation (ECMO) is used for cardiopulmonary dysfunction. Hepatopulmonary syndrome (HPS) occurs in the setting of liver failure and may cause hypoxemia. Previous reports have described the use of ECMO for HPS after liver transplant. Our patient is a 19-month-old female with

Reversal of intrapulmonary shunting in cirrhosis after liver transplantation demonstrated by perfusion lung scan.

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Ielogoties Reģistrēties

A young girl with biliary atresia leading to cirrhosis developed respiratory complications with hypoxemia. Intrapulmonary shunting was diagnosed with a 99mTc-MAA perfusion lung scan, which showed marked systemic activity. The shunting resolved after liver transplantation. The perfusion lung scan

Hepatopulmonary syndrome in children - is conventional liver transplantation always needed?

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Ielogoties Reģistrēties

BACKGROUND Hepatopulmonary syndrome (HPS) is the association of liver disease, hypoxemia, and intrapulmonary vascular dilatations. There are little data on the management of HPS in children other than conventional orthotopic liver transplantation (OLT). OBJECTIVE To describe the patient

Early onset conjugated hyperbilirubinemia in newborn infants.

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Ielogoties Reģistrēties

OBJECTIVE To determine the causes and related outcomes of early onset conjugated hyperbilirubinemia in a group of newborn infants and to determine the incidence of sepsis in these neonates. METHODS The charts of 42 babies with conjugated hyperbilirubinemia were retrospectively reviewed. RESULTS The

Relationship between vasoactive intestinal peptide and intrapulmonary vascular dilatation in children with various liver diseases.

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Ielogoties Reģistrēties

OBJECTIVE To evaluate the potential of vasoactive intestinal peptide (VIP) as a pathogenic factor of intrapulmonary vascular dilatation (IVD) in hepatopulmonary syndrome (HPS). BACKGROUND HPS comprises a triad comprising liver dysfunction, IVD and hypoxaemia. Although the pathogenesis of the process

Pulmonary arteriovenous shunting in children with liver disease.

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Ielogoties Reģistrēties

Pulmonary arteriovenous shunting (PAVS) with hypoxemia is a severe complication of cirrhosis that may regress after liver transplantation. We report PAVS in 25 children with cirrhosis and in 1 with portal vein obstruction; proof of shunting was obtained by technetium Tc 99m microaggregated albumin

Improvement of hepatopulmonary syndrome after transjugular intrahepatic portasystemic shunting: case report and review of literature.

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Ielogoties Reģistrēties

The hepatopulmonary syndrome has been described in as many as 5-29% of patients with liver disease. Patients with this syndrome may suffer from chronic hypoxemia, and mortality rates of liver patients with this syndrome are as high as 41%. Early diagnosis of such patients is essential. Currently,

Resolution of cirrhosis-related pulmonary shunting in two children with a transplanted liver.

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Ielogoties Reģistrēties

We treated two children with hypoxemia caused by intrapulmonary shunting associated with cirrhosis secondary to extrahepatic biliary atresia. Following orthotopic liver transplantation, digital clubbing and intrapulmonary shunting were resolved, as demonstrated by normalization of room air arterial

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