Surgical intervention is the only means available for a definitive diagnosis of biliary atresia (intraoperative cholangiography) and therapy (Kasai portoenterostomy). (medscape.com)
To evaluate the outcome of Scottish children with extra hepatic biliary atresia (EHBA) since rationalisation of Kasai services to three English centres in 2002 (The 'Group A' centres). (nih.gov)
She advocated centralizing the Kasai Portoenterostomy (a surgical procedure) for the treatment of Biliary Atresia in infants, citing its ability to greatly enhance bile drainage along with keeping serum bilirubin levels within range, thus improving the patient's quality of life. (wikipedia.org)
She evaluated the success ratio of kasai portoenterostomy and demonstrated a higher success and overall survival rate within children who were treated at high volume centers compared to those who were treated at low volume centers. (wikipedia.org)
The study involved 148 infants and the results showed that out of 96% of the infants that underwent kasai portoenterostomy, 57% of them recovered from jaundice, while the remaining 37% had to go for a liver transplant. (wikipedia.org)
To evaluate the incidence of portal hypertension (PHT) in biliary atresia (BA) patients and to monitor its progress after Kasai portoenterostomy (KP) by measuring nitric oxide (NO) levels in peripheral blood. (jiaps.com)
In the unusual circumstance of distal patency of the common duct with acceptable proximal luminal caliber, a modified portoenterostomy may be considered in place of the traditional Kasai procedure. (medscape.com)
Understanding the challenges that preclude success of the Kasai portoenterostomy (KPE) is the key to improve the overall outcomes of BA and implementing intervention strategies. (bvsalud.org)
The discovery of metabolic liver diseases, bile acid synthesis defects, cryptogenic neonatal hepatitis syndromes, and the development of the Kasai portoenterostomy procedure for the treatment of biliary atresia, a condition up until then considered untreatable, were all factors that contributed to the foundation of the subspecialty of pediatric hepatology in the 1970s. (abdominalkey.com)
Serum bile acids as a prognostic biomarker in biliary atresia following Kasai portoenterostomy. (ucdenver.edu)
Biliary atresia5
Although histopathologic features of biliary atresia have been extensively studied in surgical specimens from excised extrahepatic biliary systems of infants undergoing portoenterostomy, the pathogenesis of this disorder remains poorly understood. (medscape.com)
Kelly conducted research on pediatric hepatic diseases with a particular focus on biliary atresia. (wikipedia.org)
Hepatic portoenterostomy was performed in 87 patients with biliary atresia, and biopsy alone was performed in 15. (jamanetwork.com)
The radiologic diagnosis of non-biliary atresia with MRC was based on visualization of the common bile duct and common hepatic duct. (e-cep.org)
Common hepa1
Type II: atresia of the common hepatic duct. (findmeacure.com)
Liver transplantation2
Roux-en-Y cholangiojejunostomy is a standard procedure for biliary reconstruction in pediatric living donor liver transplantation (LDLT). (bvsalud.org)
Pediatric liver transplantation, therefore, is positioned at the very birth of the hepatic transplantation surgical specialty, which now has become the standard treatment worldwide for a large range of serious or end-stage liver diseases in adults and children. (abdominalkey.com)
Cirrhosis1
The primary triad of HPS is hypoxemia, intrapulmonary vascular dilatation (IPVD) and hepatic cirrhosis. (transpopmed.org)
Ducts1
90% of patients) involves atresia of the right and left hepatic ducts to the level of the porta hepatis. (medscape.com)
Atresia3
Generalmente la atresia biliar se encuentra en los lactantes y es la causa de la tercera parte de las ICTERICIAS colestásicas neonatales. (bvsalud.org)
In most cases of atresia, dissection into the porta hepatis and creation of a Roux-en-Y anastomosis with a 35-cm to 40-cm retrocolic jejunal segment is the procedure of choice. (medscape.com)
Type III: atresia of the right and left hepatic duct. (findmeacure.com)
Infants1
Hepatic portoenterostomy is the procedure of choice in infants younger than 3 months. (jamanetwork.com)
Therapy1
Reassessing the role of medical therapy in the management of hepatic vein thrombosis. (harvard.edu)
Patients1
hepatic portoenterostomy failed in 36 patients. (jamanetwork.com)
Biliary17
She developed neonatal cholestasis due to biliary atresia and required surgical intervention (portoenterostomy) when 32-d old. (nih.gov)
Surgical intervention is the only means available for a definitive diagnosis of biliary atresia (intraoperative cholangiography) and therapy (Kasai portoenterostomy). (medscape.com)
Although histopathologic features of biliary atresia have been extensively studied in surgical specimens from excised extrahepatic biliary systems of infants undergoing portoenterostomy, the pathogenesis of this disorder remains poorly understood. (medscape.com)
1. Kasai-Like Portoenterostomy for Multiple Biliary Duct Reconstruction After Extended Liver Resection of Perihilar Cholangiocarcinoma. (nih.gov)
3. Robotic Extrahepatic Biliary Resection with Roux-en-Y Hepaticojejunostomy for Type 2 Klatskin Tumor. (nih.gov)
Biliary atresia (BA) is characterized by inflammation and fibrosis of the intra- and extra-hepatic biliary tree. (researchsquare.com)
Kasai portoenterotomy, which is the first choice of treatment for BA [ 1 ] , can facilitate the bile flow, thus prolonging the survival time of the native liver in some children with extra-hepatic biliary obstruction. (researchsquare.com)
[ 9 , 10 ] Complete visualization of the extrahepatic biliary system excludes biliary atresia, whereas nonvisualization of the common or hepatic bile ducts suggests the disease. (medscape.com)
Biliary atresia (BA) is a destructive process affecting both extra- and intra-hepatic bile ducts leading to fibrosis and obliteration of the biliary tree and cirrhosis usually within 2 years. (ijpmonline.org)
The beneficial impact of revision of Kasai portoenterostomy for biliary atresia: an institutional study. (uc.edu)
Methods We retrospectively assessed the outcomes of 256 patients with biliary atresia who underwent Kasai portoenterostomy at our hospital between 1972 and 2014. (elsevierpure.com)
Prednisolone administration monitored by postoperative stool color achieves high jaundice clearance after laparoscopic portoenterostomy for biliary atresia. (amedeo.com)
Laparoscopic versus open portoenterostomy for treatment of biliary atresia: a meta-analysis. (amedeo.com)
Hypoplasia of Extrahepatic Biliary Tree and Intrahepatic Cholangiolopathy in Cystic Fibrosis Imperfectly Mimic Biliary Atresia in 4 Infants with Cystic Fibrosis and Kasai Portoenterostomy. (hanspopperhepatopathologysociety.org)
Bile1
As the image below shows, in the correctable group (10-15% of cases), the proximal common hepatic duct is patent, allowing for primary anastomosis of the extrahepatic bile duct to the bowel. (medscape.com)
Kasai Portoenterostomy2
6. Kasai portoenterostomy--new insights from hepatic morphology. (nih.gov)
Letter to the Editor: Comment on Wickramasekara N, Ignatius J, Lamahewage A. Prognostic factors and outcomes of Kasai portoenterostomy (KPE): nine-year experience from a lower-middle income country. (amedeo.com)
Liver4
In some cases, the condition is correctable that is another form of familial conjugated hyperbilirubinaemia by surgery, while in vast majority the atresia isn't correc with delicate persistent jaundice however differs from Dubin-Johnson table and in such circumstances hepatic portoenterostomy (Kasai syndrome in having no brown pigment within the liver cells. (ehd.org)
Acute hepatic failure is a clinical syndrome that occurs within weeks to a few months of the onset of liver disease in patients in whom liver function is presumed to have been normal before the illness. (musculoskeletalkey.com)
Acute hepatic failure implies the occurrence of either acute massive destruction of liver tissue or other processes that cause rapid deterioration in function. (musculoskeletalkey.com)
Children who present with hepatic failure may have end-stage chronic liver disease or previously undiagnosed liver disease. (musculoskeletalkey.com)
Resection4
2. Right trisectionectomy with principle en bloc portal vein resection for right-sided hilar cholangiocarcinoma: no-touch technique. (nih.gov)
15. En-Bloc Complete Segment 1 Resection and Left Hepatectomy for Klatskin Tumor. (nih.gov)
16. Laparoscopic Resection of Perihilar Cholangiocarcinoma Type IIIb: A Video Demonstration of No-Touch En-Block Technique and Radical Lymphadenectomy. (nih.gov)
19. Left Hepatectomy with Combined Resection and Reconstruction of Right Hepatic Artery for Bismuth Type I and II Perihilar Cholangiocarcinoma. (nih.gov)
Outcome2
Factors influencing the outcome of portoenterostomy (PE) have not been clearly defined. (ijpmonline.org)
Spectrum of hepatic hemangiomas: management and outcome. (uc.edu)
Duct1
Prospective evaluation of common hepatic duct histopathology at the time of choledochal cyst excision ranging from children to adults. (amedeo.com)
Neonatal1
For example, severe neonatal hepatitis may result in decreased hepatic radiotracer uptake and therefore decreased excretion into the bowel. (medscape.com)
Pediatric1
It is the most important cause of acute hepatic failure in children in developed countries, comprising the majority of pediatric acute hepatic failure cases. (musculoskeletalkey.com)
Severe2
This represents the most severe hepatic phenotype of an HNF1B variant recognized thus far. (nih.gov)
Acute hepatic failure in the neonate may result from infection with viruses that characteristically do not cause severe hepatitis in older patients. (musculoskeletalkey.com)
Diagnosis1
Hepatic encephalopathy with hyperammonemia is necessary for establishing the diagnosis of acute hepatic failure, as is coagulopathy with prolongation of the prothrombin time (PT) and partial thromboplastin time (PTT). (musculoskeletalkey.com)
Secondary1
A high case fatality rate is characteristic of acute hepatic failure secondary to non-A-G hepatitis. (musculoskeletalkey.com)
Common1
In children, the three most common drugs that cause acute hepatic failure are acetaminophen, isoniazid, and propylthiouracil. (musculoskeletalkey.com)
Children1
Viral hepatitis accounts for more than 80% of cases of acute hepatic failure in children of all age groups. (musculoskeletalkey.com)
Evaluation1
Additionally, the ML-HVPG score was found to be superior to evaluation of hepatic collagen by morphometry (AUROCs in test set: 0.76 vs. 0.65). (hanspopperhepatopathologysociety.org)
Total1
Comparison of perioperative surgical outcomes following total robotic and total laparoscopic Roux-en Y hepaticojejunostomy for choledochal cyst in paediatric population: a preliminary report from a tertiary referral centre. (amedeo.com)