scholarly journals Extrahepatic Biliary Atresia: The Importance of Early Referral

JMS SKIMS ◽  
2014 ◽  
Vol 17 (1) ◽  
pp. 39-40
Author(s):  
Wani Sajad ◽  
Bhat Nisar ◽  
Aejaz Baba ◽  
Gowhar Mufti ◽  
Khursheed Ahmad Sheikh
Keyword(s):  
Liver Transplantation ◽  
Liver Cirrhosis ◽  
Portal Hypertension ◽  
Biliary Atresia ◽  
Bile Ducts ◽  
Early Referral ◽  
Diffusion Of Information ◽  
Extrahepatic Biliary Atresia ◽  
Extrahepatic Bile Ducts ◽  
And Diffusion

Extrahepatic biliary atresia (EHBA), characterized by obliteration or discontinuity of extrahepatic bile ducts, is still the major cause for liver transplantation among children nowadays [1]. All untreated children eventually die due to complications resulting from portal hypertension and liver cirrhosis. The exchange and diffusion of information that can make the diagnosis of EHBA easier is of utmost importance, since prognosis is improved when patients are surgically treated by portoenterostomy in the first 2 months of life. JMS 2014;17(1):39-40

scholarly journals A young adult with biliary atresia and liver decompensation

2019 ◽  
Vol 2 (1) ◽  
pp. 38-40
Author(s):  
Panyavee Pitisuttithum ◽  
Piyawat Komolmit
Keyword(s):  
Liver Transplantation ◽  
Liver Cirrhosis ◽  
Lymph Node ◽  
Portal Hypertension ◽  
Biliary Atresia ◽  
Liver Decompensation ◽  
Native Liver ◽  
Peribiliary Cysts ◽  
Collateral Vessels ◽  
Paraesophageal Lymph Node

Biliary atresia is a progressive fibro-obliterative disease of the bile duct, commonly diagnosed within a few months of life. After hepatoportoenterostomy was successfully performed, about one fourth of biliary atresia patients have grown up to the age of twenty with their native liver. However, progression to liver cirrhosis, development of portal hypertension, or cholangitis are the major problems of adult with biliary atresia. Monitoring of these complications and listing for liver transplantation whenever indicated are the key when caring adult with biliary atresia.   Figure 1  แสดง CT abdomen พบว่ามีตับโต และมีลักษณะของตับแข็งและ ภาวะความดันของระบบหลอดเลือด portal สูงขึ้น (collateral vessels, splenomegaly, ascites) พบการขยายตัวของท่อน้ำดีในตับ (multifocal dilated ducts and peribiliary cysts along bilateral intrahepatic ducts) และต่อมน้ำเหลืองโตในช่องท้อง (mesenteric, gastrohepatic, hepatoduodenal, aortocaval, para aortic, right anterior diaphragmatic, lower paraesophageal lymph node ขนาดสูงสุด 1.8 ซม.)

Biliary Atresia

1989 ◽  
Vol 11 (2) ◽  
pp. 57-62
Author(s):  
Elizabeth A. Wanek ◽  
Frederick M. Karrer ◽  
Carlos T. Brandt ◽  
John R. Lilly
Keyword(s):  
Biliary Atresia ◽  
Biliary Obstruction ◽  
Bile Flow ◽  
Bile Ducts ◽  
Current Treatment ◽  
Histopathologic Examination ◽  
The Past ◽  
The World ◽  
Hepatic Portoenterostomy ◽  
Extrahepatic Bile Ducts

Biliary atresia is a pathologic entity in which there is obliteration of some portion of the extrahepatic bile ducts. In the past, occlusion of the proximal ducts (at the liver hilus) was referred to as "noncorrectable" (Fig 1). If only the distal duct is occluded (and the proximal duct is patent), the lesion was referred to as "correctable." The distinction is academic because current treatment and prognosis are identical. The disease is panductular, ie, both extrahepatic and intrahepatic ducts are involved. Early in the disease, however, occlusion is complete only in the extrahepatic system. Without intervention, intrahepatic biliary obstruction and, subsequently, cirrhosis supervene. In the past, except for a few cases of the correctable variant, surgical procedures were unsuccessful until Morio Kasai performed a hepatic portoenterostomy, which was first reported in English in 1968. Bile flow was effectively reestablished in both correctable and noncorrectable forms of biliary atresia. The operation was only successful when done before the patient was 4 months of age. Subsequent confirmation of Kasai's results were reported throughout the world. ETIOLOGY/PATHOLOGY Biliary atresia was originally thought to be a congenital malformation. Careful histopathologic examination of excised surgical specimens indicate that this is not the case; instead, the disease is a dynamic, progressive panductular sclerotic process that may continue in the intrahepatic ducts even after surgical relief of biliary obstruction.

Liver transplantation in babies and children with extrahepatic biliary atresia

1993 ◽  
Vol 28 (8) ◽  
pp. 1044-1047 ◽  
Author(s):  
S. Beath ◽  
G. Pearmain ◽  
D. Kelly ◽  
P. McMaster ◽  
A. Mayer ◽  
...  
Keyword(s):  
Liver Transplantation ◽  
Biliary Atresia ◽  
Extrahepatic Biliary Atresia

scholarly journals Complex ultrasonography in the assessment of intrarenal hemodynamic impairments in patients with portal hypertension

2021 ◽  
Vol 13 (1) ◽  
pp. 25-32
Author(s):  
L. V. Donova ◽  
M. S. Novruzbekov ◽  
V. E. Syutkin
Keyword(s):  
Liver Transplantation ◽  
Liver Cirrhosis ◽  
Portal Hypertension ◽  
Renal Dysfunction ◽  
Liver Diseases ◽  
Hepatorenal Syndrome ◽  
Resistive Index ◽  
Vein Thrombosis ◽  
Relationship Of ◽  
Diffuse Liver Diseases

Rationale. Hepatorenal syndrome is a threatening complication in patients with liver cirrhosis and portal hypertension. The occurrence of renal dysfunction associated with hepatorenal syndrome manifestations significantly affects the condition severity, the disease duration, and the survival time during the waiting period for liver transplantation.The study purpose was to investigate the potential of a complex ultrasonography examination in the assessment of intrarenal hemodynamic impairments in patients with various diffuse liver diseases.Material and methods. The ultrasound examination results of 167 patients were analyzed. The 1st group included 28 patients with confirmed diffuse liver diseases of viral etiology who did not have signs of cirrhosis formation, the 2nd group included 139 patients with liver cirrhosis due to diffuse liver diseases of various etiologies, and the 3-rd group included 137 patients who had previously been in the 2nd group in whom orthotopic liver transplantation was performed.Results. The study revealed a statistically significant increase in the incidence of secondary hemodynamic impairments in kidney function in patients with liver cirrhosis and no relationship of their severity and incidence to the disease etiology, and also to such markers of the portal hypertension severity as splenomegaly, ascites, and portal vein thrombosis.Conclusions. The resistive index measured on the renal arterial branches by Doppler ultrasound, has a certain predictive value in relation to hepatorenal syndrome in patients with liver cirrhosis of various origins. This also makes it possible to timely identify a group of patients at a high risk of developing severe renal dysfunction and to assess the efficacy of the treatment that has been given.

scholarly journals Screening of targeted genes in extrahepatic bile ducts of mice with experimental biliary atresia

2015 ◽  
Vol 12 (3) ◽  
pp. 4326-4331 ◽  
Author(s):  
ZHEN YOU ◽  
JING WEN ◽  
LIPING CHENG ◽  
HUI YE ◽  
BEI LI
Keyword(s):  
Biliary Atresia ◽  
Bile Ducts ◽  
Extrahepatic Bile Ducts
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