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Titolo:
Treatment of primary biliary cirrhosis
Autore:
Szalay, F;
Indirizzi:
Semmelweis Univ Med, Dept Med 1, H-1083 Budapest, Hungary Semmelweis Univ Med Budapest Hungary H-1083 1, H-1083 Budapest, Hungary
Titolo Testata:
JOURNAL OF PHYSIOLOGY-PARIS
fascicolo: 1-6, volume: 95, anno: 2001,
pagine: 407 - 412
SICI:
0928-4257(200101/12)95:1-6<407:TOPBC>2.0.ZU;2-K
Fonte:
ISI
Lingua:
ENG
Soggetto:
URSODEOXYCHOLIC-ACID THERAPY; RANDOMIZED CONTROLLED TRIALS; CHOLESTATIC LIVER-DISEASE; DOUBLE-BLIND TRIAL; ORAL BUDESONIDE; MEDICAL-TREATMENT; QT-INTERVAL; TRANSPLANTATION; PLACEBO; NEUROPATHY;
Keywords:
primary biliary cirrhosis; ursodeoxycholic acids; budesonide; osteoporosis; liver transplantation;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Life Sciences
Citazioni:
49
Recensione:
Indirizzi per estratti:
Indirizzo: Szalay, F Semmelweis Univ Med, Dept Med 1, Koranyi S 2A, H-1083 Budapest, Hungary Semmelweis Univ Med Koranyi S 2A Budapest Hungary H-1083 ungary
Citazione:
F. Szalay, "Treatment of primary biliary cirrhosis", J PHYSL-PAR, 95(1-6), 2001, pp. 407-412

Abstract

Primary biliary cirrhosis (PBC) is a presumed autoimmune disease of the liver, which predominantly affects middle age women. Most patients are diagnosed when asymptomatic. The disease is characterised by chronic. granulomatous inflammation of the small bile ducts, which leads to progressive ductopenia, cholestasis, fibrosis, cirrhosis and eventual liver failure. All PBC patients with abnormal liver biochemistry should be considered for therapy. Ursodeoxycholic acid (URSO) treatment reduces intracellular hydrophobic bile acid levels and thereby may have a cytoprotective effect on cell membranes. URSO may also act as an immun-modulating agent. Multicenter randomised controlled trials proved that the treatment is associated with a marked improvement in serum biochemical markers of cholestasis, i.e. bilirubin, ALP, GGT, including fall in serum cholesterol levels. Treatment does not seem to benefit the symptoms of fatigue, pruritus, and osteoporosis. UDCA has been shown when given in a dose of 15mg/kg daily for up to 4 years to prolong the time to liver transplantation or death. Immunosuppressive therapy: based on the immunological abnormalities, several immunosuppresive drugs have been tested. Neither azathioprine nor cyclosporine was found in large enough trials to show beneficial effect on survival. D-penicillamine, cholchicin, methotrexat, prednisolone were round without significant long-term benefit. Combination therapy with URSO and budenoside appears to add some benefit toURSO monotherapy, but further studies are needed. Liver transplantation. The most crucial question is the timing. Serum bilirubin, Mayo risk score and some other factors such as uncontrollable pruritus and severe osteoporosis influence the decision. Recurrence of PBC in allograft is rare, the progress is slow. and is no reason for not recommending transplantation. Symptomatic treatment of pruritus, sicca syndrome and preventive treatment of osteoporosis. neuropathy and fat soluble vitamin deficiency is also important. (C) 2001 Elsevier Science Ltd. All rights reserved.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 02/12/20 alle ore 13:12:13