biliary atresia/hypoxia

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

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

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

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,

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

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

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 (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

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

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

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

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 (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

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,

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