MELD / PELD and the allocation of deceased donor livers for status 1 recipients with acute fulminant hepatic failure, primary nonfunction, hepatic artery thrombosis, and acute Wilson's disease

2004 ◽  
Vol 10 (S10) ◽  
pp. S17-S22 ◽  
Author(s):  
Russell H. Wiesner
Keyword(s):  
Hepatic Artery ◽  
Fulminant Hepatic Failure ◽  
Hepatic Failure ◽  
Wilson’S Disease ◽  
Deceased Donor ◽  
Wilson's Disease ◽  
Hepatic Artery Thrombosis ◽  
Primary Nonfunction ◽  
Artery Thrombosis

scholarly journals Rescue Thrombectomy for Early Hepatic Artery Thrombosis Using Stent Retriever in a Child Post Combined Deceased Donor Liver and Renal Transplant

2021 ◽  
Author(s):  
Manish Kumar Yadav ◽  
Madhavan Unni ◽  
U T Shabeer Ali ◽  
Shiraz Ahmed Rather ◽  
B Venugopal
Keyword(s):  
Renal Transplant ◽  
Hepatic Artery ◽  
Primary Hyperoxaluria ◽  
Deceased Donor ◽  
Stent Retriever ◽  
Hepatic Artery Thrombosis ◽  
Donor Liver ◽  
Post Intervention ◽  
Artery Thrombosis ◽  
Tissue Plasminogen

AbstractWe report this case of a 5-year-old child post combined liver and renal transplant for primary hyperoxaluria. Patient developed hepatic artery thrombosis on day 3 posttransplant that was managed by reexploration and reanastomosis of the hepatic artery. On day 4, the patient again developed hepatic artery thrombosis that failed to revascularize by surgical exploration and reanastomosis. Tissue plasminogen activator was injected into the hepatic artery intraoperatively to lyse any clot; however, no revascularization could be achieved. Subsequently, catheter angiogram confirmed no flow in the hepatic artery. A4 × 22 mm revive stent retriever was deployed across the site of occlusion and retrieved after 5 minutes of indwell time. Two such passes were made and complete recanalization of the hepatic artery was achieved. The hepatic artery remained patent as confirmed on serial Doppler images post intervention.

Inherited diseases of copper metabolism: Wilson’s disease and Menkes’ disease

2020 ◽  
pp. 2115-2120
Author(s):  
Michael L. Schilsky ◽  
Pramod K. Mistry
Keyword(s):  
Liver Disease ◽  
Fulminant Hepatic Failure ◽  
Hepatic Failure ◽  
Wilson’S Disease ◽  
Menkes Disease ◽  
Wilson's Disease ◽  
Copper Transport ◽  
Loss Of Function ◽  
Copper Excretion ◽  
P Type

Copper is an essential metal that is an important cofactor for many proteins and enzymes. Two related genetic defects in copper transport have been described, each with distinct phenotypes. Wilson’s disease—an uncommon disorder (1 in 30 000) caused by autosomal recessive loss-of-function mutations in a metal-transporting P-type ATPase (ATP7B) that result in defective copper excretion into bile and hence copper toxicity. Typical presentation is in the second and third decade of life with liver disease (ranging from asymptomatic to acute fulminant hepatic failure or chronic end-stage liver disease) or neurological or psychiatric disorder (dystonia, dysarthria, parkinsonian tremor, movement disorder, a spectrum of psychiatric ailments). While no single biochemical test or clinical finding is sufficient for establishing the diagnosis, typical findings include low serum ceruloplasmin, high urinary copper excretion, and elevated liver copper content. Corneal Kayser–Fleischer rings may be seen. Treatment is with copper chelating agents and zinc. Liver transplantation is required for fulminant hepatic failure and decompensated liver disease unresponsive to medical therapy. Menkes’ disease—a rare disorder (1 in 300 000) caused by X-linked loss-of-function mutations in a P-type ATPase homologous to ATP7B (ATP7A) that result in defective copper transport across intestine, placenta, and brain and hence cellular copper deficiency. Clinical presentation is in infancy with facial dimorphism, connective tissue disorder, hypopigmentation, abnormal hair, seizures, and failure to thrive, usually followed by death by age 3 years (although some variants with a milder phenotype result from milder mutations, e.g. occipital horn syndrome). Treatment, which is only effective when presymptomatic diagnosis is made in a sibling after florid presentation in a previous affected sibling, is with intravenous copper histidine.

scholarly journals Diagnosis of Wilson's Disease Presenting as Fulminant Hepatic Failure

1983 ◽  
Vol 84 (1) ◽  
pp. 161-167 ◽  
Author(s):  
Arthur J. Mccullough ◽  
C. Richard Fleming ◽  
Johnson L. Thistle ◽  
William P. Baldus ◽  
Jurgen Ludwig ◽  
...  
Keyword(s):  
Fulminant Hepatic Failure ◽  
Hepatic Failure ◽  
Wilson’S Disease ◽  
Wilson's Disease

Fulminant hepatic failure during perinatal period in a pregnant woman with Wilson’s disease

1991 ◽  
Vol 26 (1) ◽  
pp. 69-73 ◽  
Author(s):  
Nobuyuki Shimono ◽  
Hiromi Ishibashi ◽  
Hideyuki Ikematsu ◽  
Jiro Kudo ◽  
Masafumi Shirahama ◽  
...  
Keyword(s):  
Pregnant Woman ◽  
Fulminant Hepatic Failure ◽  
Hepatic Failure ◽  
Wilson’S Disease ◽  
Perinatal Period ◽  
Wilson's Disease

scholarly journals BILIARY COMPLICATIONS AFTER LIVER TRANSPLANTATION

2017 ◽  
Vol 30 (2) ◽  
pp. 127-131 ◽  
Author(s):  
Júlio Cezar Uili COELHO ◽  
Lucas de Oliveira LEITE ◽  
Antonio MOLENA ◽  
Alexandre Coutinho Teixeira de FREITAS ◽  
Jorge Eduardo Fouto MATIAS
Keyword(s):  
Liver Transplantation ◽  
Hepatic Artery ◽  
Balloon Dilation ◽  
Deceased Donor ◽  
Biliary Complications ◽  
High Rate ◽  
Hepatic Artery Thrombosis ◽  
Stent Insertion ◽  
Percutaneous Procedures ◽  
Artery Thrombosis

ABSTRACT Background: Biliary reconstitution has been considered the Achilles’s heel of liver transplantations due to its high rate of postoperative complications. Aim: To evaluate the risk factors for occurrence of biliary strictures and leakages, and the most efficient methods for their treatment. Method: Of 310 patients who underwent liver transplantation between 2001 and 2015, 182 medical records were retrospectively analyzed. Evaluated factors included demographic profile, type of transplantation and biliary reconstitution, presence of vascular and biliary complications, their treatment and results. Results: 153 (84.07%) deceased donor and 29 (15.93%) living donor transplantations were performed. Biliary complications occurred in 49 patients (26.92%): 28 strictures (15.38%), 14 leakages (7.7%) and seven leakages followed by strictures (3.85%). Hepatic artery thrombosis was present in 10 patients with biliary complications (20.4%; p=0,003). Percutaneous and endoscopic interventional procedures (including balloon dilation and stent insertion) were the treatment of choice for biliary complications. In case of radiological or endoscopic treatment failure, surgical intervention was performed (biliodigestive derivation or retransplantation (32.65%). Complications occurred in 25% of patients treated with endoscopic or percutaneous procedures and in 42.86% of patients reoperated. Success was achieved in 45% of patients who underwent endoscopic or percutaneous procedures and in 61.9% of those who underwent surgery. Conclusion: Biliary complications are frequent events after liver transplantation. They often require new interventions: endoscopic and percutaneous procedures at first and surgical treatment when needed. Hepatic artery thrombosis increases the number of biliary complications.

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