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.)

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

Long-term outcome and necessity of liver transplantation in infants with biliary atresia are independent of cytokine milieu in native liver and serum

Cytokine ◽  
2018 ◽  
Vol 111 ◽  
pp. 382-388 ◽  
Author(s):  
Omid Madadi-Sanjani ◽  
Joachim F. Kuebler ◽  
Stephanie Dippel ◽  
Anna Gigina ◽  
Christine S. Falk ◽  
...  
Keyword(s):  
Liver Transplantation ◽  
Biliary Atresia ◽  
Term Outcome ◽  
Long Term Outcome ◽  
Native Liver ◽  
Cytokine Milieu

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.

Variceal Bleeds in Patients with Biliary Atresia

2017 ◽  
Vol 28 (05) ◽  
pp. 439-444 ◽  
Author(s):  
Mauri Witt ◽  
Niels Bax ◽  
Henkjan Verkade ◽  
René Scheenstra ◽  
Ruben de Kleine ◽  
...  
Keyword(s):  
Liver Transplantation ◽  
Portal Hypertension ◽  
Biliary Atresia ◽  
Variceal Bleeding ◽  
Laboratory Tests ◽  
Subsequent Development ◽  
Kasai Portoenterostomy ◽  
Receive Treatment ◽  
Related Mortality

Introduction Portal hypertension often occurs in biliary atresia (BA). The subsequent development of esophageal varices and bleeding from these varices are a well-known complication. We aim to describe the incidence and severity of variceal bleeding in patients with BA. In addition, we describe the characteristics of patients who experienced variceal bleeds. Materials and Methods We included all infants treated for BA at our center between March 1987 and August 2015. Variceal bleeding was defined as hematemesis and/or melena with presence of varices at endoscopy. Findings at endoscopy and ultrasound, laboratory tests, clearance of jaundice, fibrosis-grade at Kasai portoenterostomy, and several varices prediction scores were documented. Routine endoscopies were not performed. Results In this study, 74 patients were included. During follow-up, 18 out of 74 patients (24%) developed variceal bleeding at an age of 9 months (range, 4–111). Twelve patients were listed for liver transplantation at the time of bleeding. Patients who did not clear their jaundice developed variceal bleeds more often and earlier in life. Bleeds were treated with sclerotherapy, banding, or octreotide. Four patients did not receive treatment. No bleeding-related mortality occurred. Conclusion One-fourth of the children diagnosed with BA experience variceal bleeds during follow-up. Most of these children are younger than 1 year and often already listed for transplantation. Major complications did not occur after variceal bleeding.

scholarly journals Splenectomy in Liver Cirrhosis with Splenomegaly and Hypersplenism

2020 ◽  
Author(s):  
Adianto Nugroho
Keyword(s):  
Liver Transplantation ◽  
Liver Cirrhosis ◽  
Portal Hypertension ◽  
Human Body ◽  
Elevated Risk ◽  
Risk Of Infection ◽  
Pneumococcal Sepsis ◽  
Cirrhotic Patients

Spleen is a “mysterious” organ since with unique functions, and might be related to other pathology in the human body. Splenomegaly and hypersplenism can manifest following the development of portal hypertension in liver cirrhosis through fibrogenesis, immune and microenvironment dysregulation. Cirrhotic patients are generally considered as immunocompromised and prone to infections. Splenectomy in cirrhotic patients has produced concern over decrease immunity and elevated risk of infection, namely overwhelming post splenectomy pneumococcal sepsis. This review discus the splenectomy effect to the liver and how it can play a role in cirrhotic patients with portal hypertension without readily available access to liver transplantation.

Indication of liver transplantation for jaundice-free biliary atresia with portal hypertension

2011 ◽  
Vol 16 (4) ◽  
pp. 7-11 ◽  
Author(s):  
Yukihiro Sanada ◽  
Koichi Mizuta ◽  
Taizen Urahashi ◽  
Yoshiyuki Ihara ◽  
Taiichi Wakiya ◽  
...  
Keyword(s):  
Liver Transplantation ◽  
Portal Hypertension ◽  
Biliary Atresia

Assessment of native liver fibrosis using ultrasound elastography and serological fibrosis indices in children with biliary atresia after the Kasai procedure

2020 ◽  
pp. 028418512094848
Author(s):  
Jisun Hwang ◽  
Hee Mang Yoon ◽  
Kyung Mo Kim ◽  
Seak Hee Oh ◽  
Jung-Man Namgoong ◽  
...  
Keyword(s):  
Liver Cirrhosis ◽  
Liver Fibrosis ◽  
Liver Function ◽  
Diagnostic Accuracy ◽  
Biliary Atresia ◽  
Non Invasive ◽  
Kasai Procedure ◽  
Serologic Markers ◽  
Native Liver ◽  
Us Elastography

Background Validated non-invasive examinations are necessary to monitor liver fibrosis in children with biliary atresia (BA) after the Kasai procedure. Purpose To evaluate the diagnostic accuracy of two-dimensional shear wave elastography (2D-SWE), transient elastography (TE), and the serologic biomarkers of aspartate transaminase-to-platelet ratio index (APRI) and Fibrosis-4 (FIB-4) score for evaluating native liver fibrosis in children with BA. Material and Methods We retrospectively reviewed same-day 2D-SWE and TE liver stiffness (LS) measurements of 63 patients with BA who underwent the Kasai procedure. The APRI and FIB-4 score were computed. Hepatic fibrosis was categorized into three clinical categories based on the ultrasound (US) hepatic morphology and clinical manifestations of liver cirrhosis: I, pre-cirrhotic liver state (n = 15); II, US and/or clinical signs of liver cirrhosis with compensated liver function (n = 27); and III, liver cirrhosis with decompensated liver function (n = 21). We compared area under the receiver operating characteristic curve (AUC) data among 2D-SWE, TE, APRI, and FIB-4 score. Combined evaluation of serologic fibrosis indices and US elastography was conducted and AUCs of combinations were analyzed. Results 2D-SWE, TE, APRI, and FIB-4 score showed good to excellent diagnostic accuracy for differentiating clinical categories (AUCs 0.779–0.955). AUC values were significantly increased after adding TE to FIB-4 score for detecting liver cirrhosis ( P = 0.02). Conclusion 2D-SWE, TE, APRI, and FIB-4 score are accurate non-invasive markers for monitoring native liver fibrosis in patients with BA. Combined use of serologic markers and US elastography could yield more accurate diagnoses of liver fibrosis than serologic markers alone.

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