Congenital disorders of the liver, biliary tract, and pancreas

2010 ◽  
pp. 2579-2583
Author(s):  
J.A. Summerfield

Congenital disorders of the liver, biliary tract, and pancreas are rare or very rare. Biliary atresias present with cholestatic jaundice starting after the first 2 weeks of life and may eventually cause biliary cirrhosis and liver failure. Prognosis depends on the type of atresia: (1) intrahepatic—both nonsyndromic (with cirrhosis usually developing in late childhood, and fatal without liver transplantation) and syndromic (e.g. Alagille’s syndrome, due to mutation in the ...

2008 ◽  
Vol 49 (6) ◽  
pp. 936-945 ◽  
Author(s):  
Katriina Nikkilä ◽  
Markku J. Nissinen ◽  
Helena Gylling ◽  
Helena Isoniemi ◽  
Tatu A. Miettinen

2020 ◽  
pp. 1-5
Author(s):  
Olivier Zerbib ◽  
Benjamin Zribi ◽  
David Dahan ◽  
Eviatar Nesher ◽  
Jonathan Cohen ◽  
...  

Objective: To describe the experience with a multimodal therapeutic approach in a patient who developed toxic liver syndrome and fulminant hepatic failure following orthotopic liver transplantation (OLT) as a result of occlusion of the portal vein. Setting: Department of Intensive Care. Patient: A patient with liver cirrhosis secondary to autoimmune hepatitis and primary biliary cirrhosis who underwent orthotopic liver transplantation (OLT). Interventions: Transplant hepatectomy, plasmapheresis and retransplantation. Case Report: A 39-year-old man underwent an elective OLT. A routine postoperative doppler ultrasound examination a few hours after surgery revealed portal vein thrombosis. Attempts at recanalization failed, and the patient developed acute fulminant liver failure, which remained resistant to supportive therapy. A transplant hepatectomy was performed 9 hours later and plasmapheresis started. Following a 10-hour anhepatic period, the patient received a second liver, from an ABO-incompatible donor. The patient underwent column plasmapheresis and subsequent splenectomy to remove anti-B antibody to preserve the incompatible transplanted liver from immunogenic complications. The patient spent a total of 21 days in the Intensive Care Unit (ICU) before being discharged to a step-down ward. Conclusion: Our experience suggests that multimodal therapy, including transplant hepatectomy, plasmapheresis and retransplantation of an even non-ABO compatible liver may result in the successful outcome in patients with acute fulminant liver failure complicating OLT.


2021 ◽  
Vol 11 (1) ◽  
Author(s):  
Yuji Suzuki ◽  
Keisuke Kakisaka ◽  
Takuro Sato ◽  
Ryouichi Mikami ◽  
Hiroaki Abe ◽  
...  

AbstractPatients with severe acute liver injury (SLI) usually recover spontaneously. However, some SLI patients progress to acute liver failure with varying degrees of hepatic encephalopathy. Acute liver failure is associated with high mortality and can be substantially reduced by liver transplantation. Therefore, distinguishing SLI patients who might progress to acute liver failure and are at a risk of death is important when evaluating patients needing liver transplantation. The present study aimed to determine whether technetium-99m-diethylenetriaminepentaacetic acid galactosyl human serum albumin (Tc-99m GSA) scintigraphy can predict the prognosis of patients with SLI. This prospective observational study included 69 SLI patients. The accuracy of Tc-99m GSA for predicting death or liver transplantation for 6 months was assessed. Between the two groups of patients stratified based on the cut-off values from the receiver operating characteristic curves, 6-month transplant-free survival was compared. Sixteen (23.2%) patients died or underwent liver transplantation from admission (poor outcome). The hepatic accumulation index was calculated by dividing the radioactivity of the liver region of interest by that of the liver-plus-heart region of interest at 15 min (i.e., LHL15). The LHL15 in the 16 patients (0.686) was significantly lower than that in survivors (0.836; P < 0.0001). The optimal LHL15 cut-off for distinguishing poor outcome and survival was 0.737 with a sensitivity of 81.3%, specificity of 88.7%, and area under the curve of 0.907 (95% CI, 0.832–0.981). When patients were divided into two groups based on the LHL15 cut-off value, the 6-month transplant-free survival was significantly lower in patients with an LHL15 level ≤ 0.737. Tc-99m GSA scintigraphy may help predict the prognosis of patients with SLI.


2021 ◽  
Vol 11 (01) ◽  
pp. e145-e147
Author(s):  
Nida Mirza ◽  
Ravi Bharadwaj ◽  
Smita Malhotra ◽  
Anupam Sibal

AbstractWilson disease (WD) is a disorder of copper metabolism resulting in accumulation of copper in vital organs of the human body, predominantly in the liver and the brain. Acute liver failure in WD has a bad prognosis, especially with a score ≥11 in the revised WD prognostic index; emergency liver transplantation is considered the only life-saving option in this scenario. Here, we reported a girl patient with WD-induced liver failure and poor prognostic score who was rescued by plasmapheresis. She also manifested severe Coombs negative hemolytic anemia and acute kidney injury. This case report highlights the utility of an adjunctive modality besides liver transplantation for the management of fulminant liver failure caused by WD.


Sign in / Sign up

Export Citation Format

Share Document