Patients with any of the following were excluded from this study: chronic viral hepatitis;
hepatolenticular degeneration (Wilson's disease); alpha antitrypsin deficiency; primary biliary cirrhosis; hemochromatosis; alcoholism; drug use; schistosomiasis; autoimmune liver disease; hypothyroidism or Cushing's syndrome; malignant tumors; acute renal disease (abnormal renal function within [less than or equal to]3 months); or kidney transplant patients.
The etiologic factors of cirrhosis included hepatitis virus infection related-, alcoholic-, cholestatic-, and
hepatolenticular degeneration caused cirrhosis, whereas indications of OLT for noncirrhotic patients included acute liver failure, hepatocellular and cholangiocellular carcinoma.
[USPRwire, Wed Oct 17 2018] Wilson's disease is also known as progressive lenticular degeneration and
hepatolenticular degeneration that causes copper poisoning in the body.
Wilson's disease (
hepatolenticular degeneration) is an autosomal recessive defect in cellular copper transport.
Accumulation of copper in the brain also occurs because of which the disease is sometimes referred to as the
hepatolenticular degeneration.
Wilson's disease, also known as '
Hepatolenticular degeneration' is a disorder of copper handling.
Progressive
hepatolenticular degeneration, or Wilson's disease (WD), is a genetic disorder of copper metabolism [1].
Etiology of cirrhosis Cases Constituent Rank ratio (%) Hepatitis B 58,742 71.15 1 Hepatitis C 9627 11.66 2 Alcoholic liver disease 5517 6.68 3 Autoimmune liver disease 4080 4.94 4 Cryptogenic cirrhosis 2681 3.25 5 Hepatitis B overlapping C 1119 1.36 6 Drug-induced liver injury 548 0.66 7
Hepatolenticular degeneration 128 0.16 8 Vascular obstruction disease 33 0.04 9 Nonalcoholic fatty liver 32 0.04 10 disease Bilharziasis 28 0.03 11 Cardiac cirrhosis 24 0.03 12 Malnutritional cirrhosis 3 0.00 13 Table 2: Age of the cirrhosis groups.
Transplantation of primary (noncultivated) hepatocytes via portal vein proved effective in some animal liver disease models (reviewed in [110, 111]) and treatment ofhuman liver metabolic and genetic disorders like
hepatolenticular degeneration (Wilson-Konovalov disease), tyrosinemia, Crigler-Najjar syndrome, urea cycle disorders, severe dyslipidemia, and others (reviewed in [112, 113]).