Use of external conduit impairs liver function in patients with biliary atresia

An external conduit (stoma) for patients with biliary atresia has been used to prevent postoperative cholangitis. Thirty-two patients with biliary atresia who had hepatic portoenterostomies with external conduits were studied retrospectively with respect to frequency and severity of postoperative cholangitis or stoma bleeding. Changes in their liver enzyme levels, and total bilirubin (TB) levels were measured before and after closure of the stoma. Cholangitis was observed in 20 patients (62.5%), and major hemorrhage from the stoma site was seen in 14 patients (43.8%) prior to closure. Levels of liver enzymes such as glutamic oxaloacetic transaminase (SGOT), glutamic pyruvic transaminase (SGPT), gamma-glutamyl transpeptidase (gamma-GTP), and alkaline phosphatase (ALP) improved significantly within 1 month after closure of the stoma, and remained low thereafter. The TB concentration was the only liver function that did not change significantly following closure. In summary, the authors do not recommend an external conduit in patients with biliary atresia because it is not an effective way of reducing the incidence of postoperative cholangitis, and it may be deleterious to liver function.     

Organic nitrates--new perspectives on an old drug group

A quarter of patients with erythropoietic protoporphyria develop mild to severe cholestatic liver disease. The determination of early indicators of hepatobiliary involvement are of pivotal importance to select patients for choleretic therapy. Porphyrin parameters were studied during ursodeoxycholic acid treatment in eight patients with protoporphyrin-associated liver disease and eight patients with liver failure before and after liver transplantation. The patients with intrahepatic cholestasis exhibited excessive protoporphyrinemia (27 mumol/l) compared with controls (normal < 0.64 mumol/l). Fecal protoporphyrin excretion decreased in patients with deterioration of liver function, whereas urinary coproporphyrin increased up to 2290 nmol/24 h (normal < 119 nmol/24 h). Coproporphyrin isomer I proportion increased to 71 +/- 10% (mean +/- SD, n = 8) in patients with terminal liver failure (normal < 31%). During therapy with ursodeoxycholic acid biochemical improvement occurred but without clinical remission in most cases. Eight patients underwent liver transplantation between 1987 and 1997. One patient died of liver failure. Two transplant recipients are in a good condition since 8 and 9 years, respectively. All explanted livers revealed micronodular cirrhosis and high protoporphyrin levels of about 25,000-fold (mean, n = 3). Immediately after liver transplantation protoporphyrin in erythrocytes decreased to 46-96% of pre-operative values. Coproporphyrin remained moderately elevated due to post-operative cholestasis. A post-operative rise in fecal protoporphyrin elimination reflected sufficient biliary clearence of protoporphyrin by the transplant. In conclusion, moderate coproporphyrinuria with isomer I is the earliest sign of liver complications in erythropoietic protoporphyria. Progression of protoporphyrin induced toxic liver injury is indicated by excessive protoporphyrinemia and coproporphyrinuria with an isomer I proportion > 71 +/- 10%, and reduction of fecal protoporphyrin excretion. Results suggest that therapy of intrahepatic cholestasis with ursodeoxycholic acid is only effective in the initial stages of liver disease in erythropoietic protoporphyria. In patients with severe cholestatic hepatic failure, liver transplantation is the treatment of choice.     

Topiramate efficacy in infancy

Syncytial giant cell hepatitis is a severe form of hepatitis characterized by diffuse giant cell transformation of hepatocytes. The disease may evolve to chronic cholestatic cirrhosis necessitating liver transplantation. We report the case of an adult liver transplant recipient presenting with early recurrent disease without concomitant clinicobiochemical syndrome. Early recurrence of giant cell hepatitis after liver transplantation favors the hypothesis of a transmissible agent as the etiology of the disease. Routine follow-up liver biopsy is necessary in these cases in order to gain more information about the precise incidence and aggressivity of disease recurrence in the allograft.     

Molecular mechanisms of development of multiple endocrine neoplasia 2 by RET mutations

Although ischemic cholangitis is an important cause of early cholestatic graft failure in hepatic allografts, it rarely leads to biliary tract abnormalities in the late postoperative period. We describe a 54-year-old woman who underwent orthotopic liver transplantation for alcoholic liver cirrhosis in 1988 and presented in April of 1995 with malaise, jaundice, dark urine, clay-colored stools and cholestasis. An endoscopic retrograde cholangiopancreatography demonstrated a rapid progressive sclerosing cholangitis. Liver biopsy findings showed mild portal hepatitis, specimens were non-diagnostic with regard to cholangitis, and no infection was found. Duplex ultrasonography suggested obstruction of hepatic artery blood flow and celiac arteriogram confirmed complete hepatic arterial occlusion. Progressive destruction and irregular stricturing and dilatation of the intra- and extrahepatic biliary tree, complicating ascending infectious cholangitis, progressive cholestatic jaundice and insufficient endoscopic biliary drainage made a hepatic retransplantation in 1995 mandatory. Ischemic cholangitis is an important cause of cholestatic graft failure, but this type of cholangitis is difficult to diagnose because of its misleading biopsy manifestations. We conclude that liver transplant recipients who exhibit nonanastomotic strictures on cholangiography should be evaluated for occlusion of the hepatic artery as a possible cause.     

Mirizzi''s syndrome as the cause of intrahepatic lithiasis. A clinical case

The authors report a case of Mirizzi's syndrome that was the cause of intrahepatic lithiasis. The recurrence of acute episodes of cholecystitis may lead to a partial obstruction of hepato-choledochal duct through compression and phlogosis (Type 1 Mirizzi's syndrome); moreover, the compression of calculous material wedged in the cystic duct may also result in ischemic necrosis of the wall, thus causing a cholecystic-choledochal internal biliary fistula (Type 2 Mirizzi's syndrome). The authors analyse problems relating to the complications of gallbladder calculosis with indications for surgery at the first symptomatic manifestation, given that the recurrence of cholecystic inflammatory episodes provokes pathological conditions in the biliary tract that require major surgery with a consequent increase in mortality and morbidity, above all in elderly patients. The authors recommended performing a through intraoperative study to ensure the correct identification of intrahepatic lithiasis, given the difficulty of preoperative diagnosis. The objective of treatment is to suppress the lithogenic focus and ensure good biliary drainage.     

Infection complicating percutaneous liver biopsy in liver transplant recipients

There is controversy about the frequency of and risk factors for infectious complications of percutaneous liver biopsy in liver transplant recipients. The aim of this study was to identify the incidence and nature of complications associated with liver biopsy after orthotopic liver transplantation (OLT), with particular emphasis on infection. The medical records of all patients undergoing OLT between January 1990 and August 1994 were reviewed retrospectively to identify complications requiring hospitalization within one week of percutaneous liver biopsy. The nature and severity of complications were recorded and possible risk factors for infectious complications were examined. One hundred ninety-eight patients underwent 1,136 percutaneous liver biopsies. There were eleven complications (0.96%), including as follows: 7 infections, 3 bleeding episodes, and 1 vasovagal reaction. Infections after percutaneous liver biopsy included fever and bacteremia (n = 6), and fever without bacteremia (n = 1). All infections developed only in patients with underlying biliary tract abnormalities; the frequency of infection was higher (9.8%) in patients with choledochojejunostomy when compared with those with choledochocholedochostomy (1.4%). Bacteremia was more likely caused by skin flora in patients with choledochocholedochostomy (CDC) and by enteric bacteria in patients with choledochojejunostomy (CDJ). All infections were treated successfully with parenteral antibiotics. We conclude that biliary tract abnormalities are the primary risk factors for infection after percutaneous liver biopsy, although the risk is higher in patients with CDJ than with CDC. These data support the use of antibiotic prophylaxis before percutaneous liver biopsy in OLT recipients with biliary tract abnormalities.     

Effect of morphine on gastroesophageal reflux and transient lower esophageal sphincter relaxation

BACKGROUND: Results from animal studies and preliminary data from pilot studies in patients with primary biliary cirrhosis suggest that tauro-ursodeoxycholic acid has metabolic properties that may favour its long-term use as an alternative to ursodeoxycholic acid for patients with chronic cholestatic liver diseases. No direct comparison of tauro-ursodeoxycholic and ursodeoxycholic acids have yet been carried out in primary biliary cirrhosis. METHODS: The effects of ursodeoxycholic and tauro-ursodeoxycholic acids were compared in 23 patients with primary biliary cirrhosis according to a crossover design. Both drugs were administered at the daily dose of 500 mg. in a randomly assigned sequence for two 6-month periods separated by a 3-month wash-out period. RESULTS: Serum liver enzymes related to cholestasis and cytolysis consistently improved, as compared to baseline values, during the administration of both ursodeoxycholic and tauro-ursodeoxycholic acids, but no significant difference between these two bile acids was found. Both treatments were well tolerated and no patient complained of side effects. CONCLUSION: In the short-term, tauro-ursodeoxycholic acid appears to be safe and at least as effective as ursodeoxycholic acid for the treatment of primary biliary cirrhosis.     

Magnetic resonance cholangiography for evaluation of cholestatic jaundice in neonates and infants

Distinguishing extrahepatic biliary atresia from other causes of cholestasis in neonates and infants is important because surgical intervention before 2 months of age allows for long-term survival. The purpose of this prospective study was to evaluate the usefulness of magnetic resonance (MR) cholangiography in differentiating biliary atresia from other causes of cholestatic jaundice in neonates and infants. Nine anicteric infants (control group) aged 10 to 224 days (mean +/- SD, 8 +/- 65 days) and 15 neonates and infants with cholestatic jaundice, aged 22 to 142 days (mean +/- SD, 71 +/- 37) underwent MR cholangiography. The final diagnosis of extrabiliary atresia (6 patients) was based on laparotomy findings (4 patients) or autopsy (2 patients), while neonatal hepatitis (9 patients) was diagnosed according to the liver biopsy findings and clinical recovery during follow-up. Percutaneous liver biopsies were performed in all 15 patients. Results showed that the gall bladder and common bile duct (CBD) could be visualized using MR cholangiography in all patients in the control group. Nonvisualization of the CBD (6/6 patients) and demonstration of a small gall bladder (6/6 patients) characterized MR cholangiography findings in patients with biliary atresia. MR cholangiography failed to depict the CBD in one infant with hepatitis. We conclude that demonstration of the CBD by MR cholangiography in neonates and infants with cholestasis can be used to exclude the diagnosis of biliary atresia. In patients with cholestatic jaundice considered for exploratory laparotomy, preoperative MR cholangiography is recommended to avoid unnecessary surgery.     

Bread staling. Simultaneous effect of bacterial alpha-amylase and emulsifier on firmness and pasting properties of bread crumbs++

Late recurrent primary biliary cirrhosis (PBC) following orthotopic liver transplant remains a controversial topic. The first documented case of recurrence occurring in 16 patients transplanted for PBC and followed at the authors' institution for longer than one year is presented. A 54-year-old man transplanted for PBC developed a cholestatic pattern of enzyme elevation on post-transplant day (PTD) 1305. Repeat antimitochondrial antibody was strongly positive (1:300 to 1:400). A liver biopsy revealed severe bile duct damage, lymphocytic cholangitis, focal periductal noncaseating granuloma and minimal endotheliitis. Recurrent PBC was diagnosed. At the time of orthotopic liver transplant this patient received induction immunosuppression with OKT3 crossed over to cyclosporine (CsA), azathioprine (AZA) and prednisone. AZA was discontinued early and maintenance CsA tapered to a target trough level of 150 to 200 ng/mL by PTD 365. Prednisone was withdrawn by PTD 664. CsA levels during PTDs 1225 to 1305 (before elevation of hepatobiliary enzymes) were below target at 114 to 166 ng/mL. Of the 16 patients, all but three were maintained on CsA, AZA and prednisone. One was on CsA (trough levels on target) and AZA; the other two, including the patient with recurrent PBC, were on CsA only. The trough CsA level of the patient without recurrent PBC has been within the target range. The authors speculate that the underlying defect in immunoregulation in PBC persists post-transplant and that in the patient without recurrent PBC this defect was unmasked by lowered maintenance immunosuppression--allowing recurrence of PBC in a previously stable liver allograft.     

Evidence for a luteinizing hormone surge center in the hypothalamus of the pig

Lipoprotein-X (Lp-X) is an abnormal low-density lipoprotein frequently found in liver disease. It is regarded as the most sensitive and specific biochemical parameter for the diagnosis of intra- and extrahepatic cholestasis. Moreover, Lp-X is supposed to contribute to the development of hypercholesterolemia in cholestatic liver disease, because it fails to inhibit de novo cholesterol synthesis. This investigation will focus on the relationship between the presence of Lp-X and serum lipid concentrations in cirrhosis. The significance of Lp-X in the diagnosis of cholestasis, compared with alkaline phosphatase (AP), gamma-glutamyl transferase (GGT), and bilirubin levels, will be assessed as well. The present cross-sectional study includes 212 patients with histopathologically proven cirrhosis. The detection of Lp-X and the quantification of -, beta-, and pre-beta-cholesterol was based on agar gel electrophoresis and polyanion precipitation. For the characterization of liver function, the concentrations of albumin and bilirubin, the activities of liver enzymes, and coagulation times were assessed. In a subgroup of 40 individuals, liver biopsies were re-evaluated to confirm or exclude intrahepatic cholestasis. As a result, there was no association between the appearance of Lp-X and total cholesterol concentrations. While all patients with Lp-X showed intrahepatic cholestasis (predictive value of the positive test = 1), only 16 of 28 patients with cholestasis formed Lp-X (sensitivity = 0.57). The activities of AP and of GGT, as well as the concentrations of bilirubin, were strongly elevated in most patients, with and without cholestasis. The predictive values of AP, GGT, and bilirubin were 0.77, 0.69, and 0.74 for the positive test and 0.5, 0, and 0.6 for the negative test, respectively. We conclude that Lp-X is not related to hypercholesterolemia in cirrhosis. The positive, but not the negative, Lp-X test has high predictive value for the diagnosis of cholestasis in cirrhosis. The biochemical parameters traditionally used for the assessment of extrahepatic cholestasis, AP, GGT, and bilirubin, do not support the diagnosis of intrahepatic cholestasis caused by cirrhosis.     

Percutaneous transhepatic endoscopic electrohydraulic lithotripsy of biliary tract calculi after orthotopic liver transplantation

The development of biliary tract calculi after orthotopic liver transplantation presents a unique clinical problem. Previously described techniques for removing biliary stones by shock wave lithotripsy, litholytic therapy with oral bile acids, and endoscopic mechanical extraction may be ineffective or contraindicated in liver transplant patients. For this reason, percutaneous transhepatic electrohydraulic lithotripsy (EHL) was performed using an 11 French flexible ureteroscope in two pediatric patients who developed biliary tract calculi following orthotopic liver transplant. There were no complications and postoperative follow-up over 4 years has been uneventful. To our knowledge, these represent the first reported cases of percutaneous transhepatic endoscopic EHL to fragment biliary tract stones in a transplanted liver, which for us has been a safe and effective therapeutic option.     

Surgical treatment of the paroxysmal tachycardia in Wolff-Parkinson-White syndrome

22 patients with radiolucid stones and functioning gallbladder were grouped to establish ursodeoxycholic acid efficacy (i.e. gallstones dissolution) and innocuousness for gallbladder lithiasis. Methodological aspects were detailed, the dose determined (8 and 10 mg/k/d.) and the patients evaluated after a six months' treatment. 11 patients received "day-time" doses and the other 11 "overnight" dose. Only 7 subjects turned out to be evaluated for the study of biliary lipids, to the other 15 the development of their gallstones was followed up. Successful gallstone dissolution was achieved in 8 patients (53,3%), 2 reduced size and number. 5 out of these 8 patients received "day-time" doses and the other 3 "overnight" doses. From an attendance point of view, we do not consider biliary lipids study (bile cholesterol saturation rate) to be necessary.     

Role of nuclear medicine in liver transplantation

Orthotopic liver transplantation is now a very well-established technique for treating patients with end-stage liver disease. Since 1967, more than 26,000 liver transplants have been performed, including 15,000 in the United States. The overall 1-year survival rate is approximately 80% and 5-year survival is 70%. Nuclear imaging plays an important role in the management of liver transplant recipients before and after liver transplantation. The evaluation of candidates potentially includes liver-spleen scan for liver volume, multiple gated acquisition scan, adenosine or stress thallium study, bone scan, and quantitative ventilation perfusion scan for hepatopulmonary syndrome. In the post-transplant phase, the deconvolution analysis (which corrects for the problem of recirculation) is a promising tool for diagnosing rejection, although its role in the transplant population has to be established. A variety of nuclear medicine techniques are helpful in the postoperative diagnosis of biliary complications. By performing a semiquantitative analysis to discriminate hepatocyte dysfunction from biliary disease and measuring hepatocyte extraction fraction by deconvolution analysis and excretion, (T1/2 values measured by the nonlinear list squares technique) have been very promising.     

Cyclosporine therapy after cardiac transplantation causes hypertension and renal vasoconstriction without sympathetic activation

BACKGROUND: Hypertension frequently complicates the use of cyclosporine A (CyA) therapy, and it has been suggested that sympathoexcitation may be the underlying mechanism in this form of hypertension. METHODS AND RESULTS: To further investigate the possibility of a neurogenic mechanism for this hypertensive effect, we studied the effects of CyA on renal blood flow (n = 11), forearm blood flow (n = 8), and sympathetic nervous system activity, assessed by renal and whole-body radiolabeled norepinephrine plasma kinetics and muscle sympathetic nerve firing (using microneurography) in cardiac transplant recipients receiving CyA and a reference group of healthy age-matched control subjects (n = 17). In 11 cardiac transplant patients (2 hours after cyclosporine dose), renal blood flow was significantly lower than that in 8 control subjects (680 +/- 88 vs 1285 +/- 58 mL/min, P < .001). In 5 of these transplant patients, renal blood flow was measured before and for 2 hours after oral cyclosporine and fell progressively over this period, by 37% (P < .01). Total body and renal norepinephrine spillover rates in transplant patients were similar to those in control subjects (3070 +/- 538 vs 2618 +/- 313 pmol/min and 579 +/- 124 vs 573 +/- 95 pmol/min, respectively), and there was no progressive effect in the 2 hours after cyclosporine dosing. Forearm blood flow was increased 2 hours after CyA administration (1.74 +/- 0.31 to 3.12 +/- 0.50 mL x 100 mL-1 x min-1, P < .001), whereas mean arterial blood pressure and noninvasively determined cardiac output (indirect Fick method) were unchanged. Muscle sympathetic nerve discharge rates recorded in 6 of these transplant patients were not different from those in 9 healthy control subjects (37.9 +/- 10.1 vs 41.3 +/- 2.3 bursts per 100 beats per minute). During 90 to 120 minutes of recording after cyclosporine dosing, nerve firing rates remained unchanged. CONCLUSIONS: CyA therapy causes acute renal vasoconstriction without accompanying systemic hemodynamic effects. These renal effects are nonneural, not being attributable to sympathoexcitation.     

Sclerosing cholangitis

Primary sclerosing cholangitis (PSC) is a chronic, progressive cholestatic liver disease whose aetiopathogenesis is unknown. PSC is frequently associated with inflammatory bowel disease, in particular chronic ulcerative colitis, is most commonly observed in young males and is clinically characterized by fatigue, pruritus and jaundice. The diagnosis is supported by a cholestatic biochemical profile and histological abnormalities, and confirmed by visualization of an abnormal biliary tree. The natural history of the disease is currently being evaluated but is generally recognized to be slowly progressive, leading to complications of chronic cholestasis, portal hypertension and biliary cirrhosis. There is no specific medical treatment, and orthotopic liver transplantation remains the only definitive treatment for patients with end-stage PSC. A more rational approach to medical therapy will ensue upon a better understanding of the aetiopathogenesis of this disease.     

Bilateral discoid medial menisci: an adult patient with symmetrical radial tears in both knees

Inflammation of the gallbladder is known to occur in patients with primary sclerosing cholangitis (PSC). However, the histological features of this form of cholecystitis have not been adequately defined. The aim of this study was to compare the inflammatory lesions of PSC-associated cholecystitis with those present in other cholecystopathies. The cases consisted of 11 gallbladders from patients with PSC who underwent liver transplantation. As controls, gallbladders from liver transplant patients with primary biliary cirrhosis (n = 4) and other chronic nonbiliary hepatopathies (n = 8), and 13 cholecystectomies from patients with chronic cholecystitis with (n = 10) and without (n = 3) lithiasis, were studied. The following histological features were tabulated on coded slides: presence, depth of involvement, and distribution of the inflammatory infiltrate, predominant cell type, presence of lymphoid aggregates, epithelial damage, metaplastic changes (pyloric or intestinal), fibrosis, smooth muscle hypertrophy, and presence of Rokitansky-Aschoff sinuses. At variance with the wide range of histological abnormalities present in other forms of chronic cholecystitis, most PSC-related cholecystitis showed a diffuse infiltrate (6 of 11) rich in plasma cells (6 of 11) predominantly confined to the lamina propria (9 of 11). The combination of these three features was present exclusively in PSC (5 of 11 PSC cholecystitis compared with 0 of 25 controls; P = .001). In conclusion, this study suggests that a characteristic form of cholecystitis may develop in patients with PSC.     

Relationship between changes in factors of nonspecific resistance during traumatic and burn shock

Kaposi's sarcoma (KS) is a common malignancy in patients with acquired immunodeficiency syndrome (AIDS), classically appearing as red to purple plaques containing small papules and nodules. We report our experience with an adolescent orthotopic liver transplant recipient who presented with an unusual presentation of KS. The patient had a protracted multisystem illness that began with hemolytic anemia, fevers, and fatigue and progressed to pancreatitis, sinusitis, lymphadenopathy, and mouth ulcers. The diagnosis was made by a lymph node biopsy that was performed to evaluate for Epstein-Barr virus. The classical subcutaneous nodules characteristic of KS did not become evident until shortly before the patient died. We present this case to emphasize that KS in pediatric liver transplant patients can present as a multisystem disease that progresses to disseminated organ involvement before the characteristic subcutaneous manifestations are evident.     

Intrahepatic bile duct injury and nodular regenerative hyperplasia of the liver in a patient with polyarteritis nodosa

Although involvement of the hepatic vasculature in patients with polyarteritis nodosa is not unusual, biliary manifestations are very rare. We describe a patient with polyarteritis nodosa presenting with a febrile cholestatic anicteric syndrome. Histological examination of the liver revealed necrotizing arteritis of small hepatic arteries associated with significant lesions of intrahepatic bile ducts of the sclerosing cholangitis type, i.e. fibrous collar around the ducts, periductal inflammation and ductal proliferation. Concomitant nodular regenerative hyperplasia was found, a condition which has rarely been described in association with polyarteritis nodosa. We think that hepatic arteritis compromising arterial blood flow to the liver was responsible for the most likely ischemic nature of the bile duct injury and the nodular regenerative hyperplasia seen in our patient.     

Physiologic studies of spinal inhibitory circuits in patients with stiff-person syndrome

Human protoporphyria results from mutations in the ferrochelatase gene. Heritable deficiency of ferrochelatase causes overproduction of protoporphyrin IX, principally in the erythron. Photosensitivity is a universal feature of protoporphyria but hepatic clearance of the hydrophobic protoporphyrin molecule with excretion in bile may lead to precipitation within biliary pathways. Thus cholestatic injury and protoporphyrin gallstones occur. Minor hepatic abnormalities are frequent, but at least 30 patients have been reported with a progressive liver disease that requires transplantation. Fulminant hepatic disease appears to be recessively inherited in some pedigrees. Hazards of liver transplantation include tissue photolysis, hemolysis, and an unexplained neurological syndrome, but most of the 15 patients reported after transplantation have survived for several months to > 6 years. Aspects of protoporphyria, its pathogenesis and contemporary therapeutic strategies are considered, with emphasis on hepatic sequelae.     

Quality of life before and after liver transplantation for cholestatic liver disease

Liver transplantation (LT) is an established therapy for patients with end-stage primary biliary cirrhosis (PBC) or primary sclerosing cholangitis (PSC). In this report, we describe the health status and quality of life (QOL) in patients with these cholestatic liver diseases before and after LT. A QOL questionnaire was completed by 157 adult patients with PBC or PSC before and 1 year after liver transplantation at the Mayo Clinic or Baylor University Medical Center. This questionnaire measured four aspects of QOL, including symptoms; physical, social, and emotional functioning; health perceptions; and overall QOL. Changes in these QOL parameters before and after LT were described, and regression analysis was used to assess the relationships between clinical and QOL factors. There were no differences in QOL parameters between patients with PBC and PSC. QOL following transplantation was substantially better than before transplantation. This was observed in all four aspects of QOL. The degree of improvement as measured by effect size (difference in mean scores divided by the pretransplantation standard deviation) was 0.53 for symptoms (P <.01), 1.16 for function (P <.01), 2.37 for health satisfaction (P <.01), and 1.16 for overall QOL (P <.01). Patients' overall QOL before transplantation was significantly related to subjective and objective health status indicators and clinical factors such as ascites and renal dysfunction. QOL at 1-year follow-up, however, could not be adequately predicted by the pretransplantation subjective health status and clinical factors. Patients with end-stage cholestatic disease undergoing LT experience substantial improvement in all aspects of QOL addressed in this study. The patients' QOL 1 year after LT could not be predicted by pretransplantation variables used in this study.