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Wilson disease

Wilson disease is a very rare inherited multisystemic disease presenting non-specific neurological, hepatic, psychiatric or osseo-muscular manifestations due to excessive copper deposition in the body.

Cirrhosis

A chronic disorder of the liver in which liver tissue becomes scarred and is partially replaced by regenerative nodules and fibrotic tissue resulting in loss of liver function.

Total: 12

(per page)

PMID (PMCID)
27915967	FEMALE	Middle Aged
	Liver Transplant Can Resolve Severe Neuropsychiatric Manifestations of Wilson Disease: A Case Report.
	Walker G, Hussaini T, Stowe R, Cresswell S, Yoshida EM. Exp Clin Transplant. 2018;16(5):620-624.
	Although liver transplant for decompensated cirrhosis secondary to Wilson disease is well accepted, the use of transplant for patients with severe neurologic manifestations of this condition remains controversial, and these can be perceived as a contraindication.
30701819	OTHER	Middle Aged
	The first experience of non-interferon therapy of HCV infection in patients with Wilson-Konovalov's disease.
	Rozina TP, Ignatova TM, Fastovets SV, Starostina EE, Samokhodskaia LM, Krasnova TN. Ter Arkh. 2018;90(11):74-78.
	In the article we present three clinical observations demonstrating that HCV infection in patients with remission of Wilson disease causes an recrudescence of the disease, in one of the observations - decompensation of liver cirrhosis.
29190228 (5704733)	MALE	Child
	Hepatic Venous Outflow Stenosis After Auxiliary Left Hemiliver Transplantation Diagnosed by Ultrasonic Shear Wave Elastography Combined With Doppler Ultrasonography.
	Li JW, Lu Q, Luo Y. Ultrasound Q. 2017;33(4):289-292.
	We report the case of an 11-year-old male patient with cirrhosis due to hepatolenticular degeneration who received an auxiliary left hemiliver graft from his uncle.
25016221 (4203706)	MIXED_SAMPLE	Infant, Newborn
	Individual exome analysis in diagnosis and management of paediatric liver failure of indeterminate aetiology.
	Vilarinho S, Choi M, Jain D, Malhotra A, Kulkarni S, Pashankar D, Phatak U, Patel M, Bale A, Mane S, Lifton RP, Mistry PK. J Hepatol. 2014;61(5):1056-63.
	Patient 1 was a 10 year-old female diagnosed with Wilson disease but no detectable ATP7B mutations, and decompensated liver cirrhosis who underwent liver transplant and subsequently developed onset of neurodegenerative disease.
23441660	MIXED_SAMPLE	Adult
	Tacrolimus-induced thrombotic microangiopathy in orthotopic liver transplant patients: case series of four patients.
	Nwaba A, MacQuillan G, Adams LA, Garas G, Delriviere L, Augustson B, DeBoer B, Moody H, Jeffrey GP. Intern Med J. 2013;43(3):328-33.
	The indications for OLTX in the four patients were fulminant hepatic failure in three (Wilson disease, paracetamol overdose and post-partum thrombotic thrombocytopenic purpura) and hepatitis C virus-related cirrhosis.
22261259	MIXED_SAMPLE
	[Diagnosis and care of Wilson disease with neurological revelation].
	Wagner S, Brunet AS, Bost M, Lachaux A, Broussolle E, Des Portes V, Lion-Francois L. Arch Pediatr. 2012;19(3):271-6.
	Wilson disease is an autosomal recessive disease that produces a copper accumulation in many organs, initially in the liver, progressing to liver cirrhosis, and in the brain, with different neurologic symptoms.
21453233	MIXED_SAMPLE	Child
	Leukoencephalopathy syndrome after living-donor liver transplantation.
	Umeda Y, Matsuda H, Sadamori H, Shinoura S, Yoshida R, Sato D, Utsumi M, Yagi T, Fujiwara T. Exp Clin Transplant. 2011;9(2):139-44.
	The underlying diseases were alcoholic cirrhosis in 3 cases, viral cirrhosis in 2, biliary atresia in 1, and Wilson disease in 1.
21453233	MIXED_SAMPLE	Child
	Leukoencephalopathy syndrome after living-donor liver transplantation.
	Umeda Y, Matsuda H, Sadamori H, Shinoura S, Yoshida R, Sato D, Utsumi M, Yagi T, Fujiwara T. Exp Clin Transplant. 2011;9(2):139-44.
	The underlying diseases were alcoholic cirrhosis in 3 cases, viral cirrhosis in 2, biliary atresia in 1, and Wilson disease in 1.
19207220	MIXED_SAMPLE	Child
	New-onset diabetes mellitus presenting with diabetic ketoacidosis after pediatric liver transplantation.
	Dehghani SM, Nikeghbalian S, Eshraghian A, Haghighat M, Imanieh MH, Bahador A, Kazemi K, Malek-Hosseini SA. Pediatr Transplant. 2009;13(5):536-9.
	The underlying diseases leading to transplantation were cryptogenic liver cirrhosis, Wilson disease, and congenital hepatic fibrosis.
10714666	MALE	Adult
	Effect of liver transplantation on neurological manifestations in Wilson disease.
	Stracciari A, Tempestini A, Borghi A, Guarino M. Arch Neurol. 2000;57(3):384-6.
	Liver transplantation (LT) is the sole resolutive therapy for Wilson disease (WD) and is the treatment of choice for patients with WD who have fulminant hepatic failure or end-stage cirrhosis.
3681585	MALE	Child
	Idiopathic hepatic copper toxicosis in a child.
	Maggiore G, De Giacomo C, Sessa F, Burgio GR. J Pediatr Gastroenterol Nutr. 1987;6(6):980-3.
	This report suggests that a disease akin to Indian childhood cirrhosis but different from Wilson disease can be found in non-Indian children probably representing a new, possibly inherited, disease of copper metabolism leading to copper overload in the liver.
3912072	MIXED_SAMPLE	Infant
	[Liver transplantation in children].
	Otte JB, de Hemptine B, Moulin D, Veyckemans F, Carlier MA, Buts JP, Claus D, Reynaert M, de Ville de Goyet J, Rahier J, et al.. Chir Pediatr. 1985;26(5):261-73.
	b) the metabolic diseases resulting either in cirrhosis with liver failure (alpha-1-antitrypsin deficiency, Wilson disease, glycogen storage disease type I and IV, protoporphyria) or in extrahepatic complications of enzymatic deficiency of an otherwise normally functioning liver (Crigler-Najjar syndrome type I, familial hypercholesterolemia and perhaps oxalosis).