BACKGROUND/AIMS: [corrected] Hepatobiliary carcinoma (HBC) has been considered to be a late complication of end-stage primary sclerosing cholangitis (PSC). The incidence of HBC is approximately 20% in PSC patients evaluated for liver transplantation. The diagnosis of HBC is difficult, at least at an early stage and the prognosis is poor even after liver transplantation. The aim of the study was to look for signs and risk factors for developing hepatobiliary carcinoma in patients with PSC. METHODS: Thirty-six consecutive patients with PSC and HBC (32 with bile duct carcinoma, BDC, and four with hepatocellular carcinoma, HCC) were pair-matched to control patients referred for liver transplantation because of PSC but who did not have HBC. Gender and age at referral were used as matching factors. Clinical and biochemical data were registered. RESULTS: PSC patients with BDC had a shorter median duration of PSC (1 year) compared with the controls (7 years) and PSC patients with HCC (8 years). There were no statistically significant differences in the liver biochemistry between the patient groups. Varices were more common in patients with PSC and HCC (100%) than in controls (56%) or patients with PSC and HBC (12%) (P < 0.0005). CONCLUSIONS: The relatively short duration of PSC and the absence of varices in patients with BDC suggest that BDC, unlike HCC, is not necessarily a late complication of end-stage PSC, as was previously assumed.

Adolescent↗

PubMed2000

Liver transplantation for primary sclerosing cholangitis--a single-center experience.

The purpose of this study was to analyze the outcome of liver transplantation for primary sclerosing cholangitis (PSC) at our center. The medical records of 47 consecutive patients transplanted during the period 1985-1996 were reviewed. Actuarial patient survival was determined at 1, 5, and 10 years. Data on reasons for retransplantation, incidental carcinoma, biliary complications that required surgical intervention, and signs of possible disease recurrence were collected. The median follow-up period was 3.6 years. Overall patient survival was 75% at 1 year and 66% at 5 and 10 years. Patients transplanted during the period 1994-1996 (n = 24) had a significantly (P < 0.02) better 1 year (88% vs 61%) and 5-year (83% vs 48%) survival than patients transplanted during the period 1985-1993 (n = 23). Six patients (13%) were retransplanted, and 4 are currently alive. Nine patients (19%) had biliary complications that required surgical intervention. Cholangiocellular carcinoma was found in 4 (9%) explanted livers, and all 4 patients succumbed within 2 years of transplantation. Indications of disease recurrence were seen in 4 patients (9%). In conclusion, the results of liver transplantation for PSC at our center are comparable to those of other benign indications, but a relatively high incidence of biliary complications was noted, and a possible disease recurrence was detected in 9% of patients.

Actuarial Analysis↗

PubMed2000

Liver transplantation in patients over 60 years of age.

Liver transplantation was previously only offered to patients under 60 years of age. We have analyzed the outcome after acceptance on the waiting list and after liver transplantation of patients over 60 years old. A total of 150 patients over 60 years old were listed for a first liver transplantation during 1990-1998. The annual number increased throughout the period. Primary biliary cirrhosis, primary sclerosing cholangitis, and acute hepatic failure were the most frequent diagnoses. A total of 119 patients received a first liver allograft. The patient 1-year survival was 75% and 3-year survival 62%, which was not significantly lower (P = 0.21) than that of the younger patients. When correcting for year of transplantation, the survival was, however, moderately but significantly lower than among the younger patients. Survival among those > 65 years (n = 38) did not differ from that of patients 60-65 years of age (n = 81). We conclude that an increasing number of patients over 60 years old can be listed for liver transplantation and receive a liver allograft with highly satisfying results.

Age Distribution↗

PubMed2000

The liver protective effect of ischemic preconditioning may be mediated by adenosine.

We investigated the involvement of adenosine in ischemic preconditioning (IPC) by the unspecific antagonist, 8-phenyltheophylline (8-PT). Anesthetized Wistar rats were treated as follows: 1. non-ischemic controls, 2. ischemic controls: 60 min of clamping of the common hepatic artery followed by 60 min reperfusion, 3. IPC: 10 min ischemia followed by 15 min reperfusion, prior to the identical ischemia-reperfusion (IR) period as in group 2, 4. 8-PT + IPC: 8-PT 10 mg/kg i.v. was given 10 min prior to the identical procedure as in group 3. The peripheral liver blood flow was monitored by laser-Doppler flowmetry. Blood alanine aminotransferase (ALT) was analyzed once every 60 min. IPC significantly reduced impairment of liver blood flow, as well as ALT increase during reperfusion. This effect was abolished by pretreatment with 8-PT. Adenosine appears to be a crucial effector in IPC. Clinical studies need to be undertaken to explore a possible effect of IPC in liver transplantation.

Adenosine↗

PubMed2000

Experiences from the first five-organ multivisceral transplantation in Scandinavia.

Explore the source record for details and available documents.

Child, Preschool↗

PubMed2000

Liver transplantation for hereditary transthyretin amyloidosis.

Transthyretin (TTR) amyloidosis is the most common form of hereditary amyloidosis. It is a systemic amyloidosis caused by an amyloidogenic variant TTR (ATTR), of which the methionine for valine at position 30 (ATTR Val30Met) gives rise to a fatal neuropathic amyloidosis. Because more than 95% of TTR is produced by the liver, a liver transplantation should abolish the liver's production of amyloidogenic mutant TTR and thereby halt amyloid formation. The first liver transplantation for hereditary TTR amyloidosis was performed in Sweden in 1990 on a patient with ATTR Val30Met amyloidosis, and the result was encouraging. Today, liver transplantation for TTR amyloidosis is an established treatment. However, the disease is rarely seen except in a few endemic areas; therefore, most transplantation centers only receive a few cases. Because the disease phenotype varies with different TTR mutations and variability is even encountered for the same mutation, an evaluation of patients for transplantation must include an investigation of all organs that may be affected by the disease and may impact on the morbidity and mortality of the procedure. The aim of this review is to present the results of liver transplantation for TTR amyloidosis and give recommendations for patient evaluation and selection based on the literature and our experience with the disease.

Amyloid Neuropathies↗

PubMed2000

Interventional treatment of gastrointestinal neuroendocrine tumours.

Neuroendocrine (NE) tumours of the gastrointestinal tract (carcinoids and endocrine pancreatic tumours) are rare diseases. In the presence of liver metastases these patients may suffer from disabling symptoms due to hormone overproduction. Patients with localized disease can be resected for cure and also patients with liver metastases can undergo potentially curative tumour resection. However, long-term follow-up of the latter cases indicates frequent recurrence of tumour. Using close biochemical monitoring of tumour markers combined with newer techniques for tumour visualization, these recurrences can often be diagnosed at an early stage so that repeat surgical procedures can be performed. During the last years very active surgery has been recommended for NE tumours, many of which have a relatively slow growth. Even in patients not amenable to curative liver surgery, debulking can be considered if the main tumour burden can be safely excised. The primary aim of this type of treatment is palliation of hormonal symptoms. An important question is whether the aggressive treatment actually prolongs survival. No prospective studies have been performed. Such studies are hampered by the lack of strict surgical programs running over long periods and the relative rarity of NE tumours. Liver transplantation may be another treatment modality in selected cases.

Biomarkers, Tumor↗

PubMed1999

[Liver transplantation in neuroendocrine tumors prolongs symptom-free period, might also be a cure].

Several neuroendocrine tumours, such as carcinoids and pancreatic endocrine tumours, may manifest relatively slow tumour growth. The patients may suffer from severe hormonal symptoms, largely due to liver metastases which sometimes are amenable to cytoreductive surgery. If residual tumour after primary tumour resection is multilobar, liver transplantation may be one way to treat hormonal symptoms and possibly prolonging survival. Early long-term outcome of liver transplantation in patients with neuroendocrine tumours suggests prognosis to be more favourable for carcinoids than for endocrine pancreatic tumours. It is suggested that liver transplantation may be appropriate for patients with isolated hepatic tumour disease in the following situations: 1, tumour recurrence after liver surgery for cure; 2, non-resectable liver disease, especially in cases of severe hormonal symptoms; and 3, disease progression after hepatic arterial embolisation and medical therapy. These indications are discussed in the light of three case reports.

Adult↗

PubMed1999

[Liver transplantation from a living donor is now possible. The first cases in Sweden completed with good results].

Liver transplantation is the sole treatment option for children with chronic liver failure, a patient category previously characterised by high mortality due to the lack of suitable organs. The introduction of new surgical techniques used in 'in situ splitting' of a donor liver, and in living related donor transplants has enabled mortality to be reduced. The article consists gives an account of the first five procedures where the in situ split technique was used in cadaveric livers, and of the first living related liver transplant performed in Sweden. The technique will improve the outcome of liver transplantation in children, and reduce the risk of death due to long waiting lists.

Adult↗