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Familial amyloidotic polyneuropathy type 1 (FAP-1) is a type of systemic amyloidosis caused by mutant transthyretin (mTTR) that is mainly produced in the liver. Most patients have progressive peripheral and autonomic neuropathy. Ten patients with FAP underwent orthotopic liver transplantation (OLT) at the Queensland Liver Transplant Service (Princess Alexandra Hospital, Brisbane, Australia). Nine patients are still alive, and one patient died of cardiac failure 10 days after OLT. Some symptoms of FAP were alleviated in some of the patients. OLT seems to be a worthwhile treatment for FAP, because it halts the progression of symptoms and achieves improvement in some patients. [ABSTRACT FROM AUTHOR]

Whether primary sclerosing cholangitis (PSC) occurs after orthotopic liver transplantation is controversial, largely because the pre-transplant diagnosis of PSC is based on nonspecific radiological and histological findings. We reviewed clinical, radiological, and histological records of 53 patients who underwent liver transplantation for PSC between 1985 and 1998. Three patients with patent hepatic arteries and no evidence of chronic rejection had radiological and histological findings that may have been due to recurrent PSC. Bile duct stricturing in these patients proved permanent and progressive and affected both the quality of life and graft survival. The first patient, who is 110 months after transplantation, has had repeated episodes of cholangitis for the last year. The second patient underwent excision of a strictured hepatic duct 45 months after transplantation and was ultimately retransplanted 95 months after initial transplantation. The third patient underwent left hemihepatectomy of an atrophied lobe 50 months after transplantation. Although the patient population assessed in this study is limited, putative recurrent PSC in the allografts has led either to graft loss or to clinically significant hepatobiliary complications of the graft. [ABSTRACT FROM AUTHOR]

We investigated the long-term results and ongoing management issues of 39 Japanese children who underwent liver transplantation in Brisbane, Australia. Whole liver grafts were used in 15 patients (Wh group) and reduced-size grafts were used in 24 patients (Re group). The 1-year and 3-year survival rates were 74% and 60%, respectively, and all cases of late mortality which occurred after 6 months were due to infection. Statistical analysis showed no differences between the Wh and Re groups with regard to late mortality or liver function tests, although 4 of 24 (16.7%) patients from the Re group developed a recurrence of esophageal varices. Three patients treated with cyclosporine developed lymphoproliferative disorders following transplantation, but none of the patients developed severe nephrotoxicity or hypertension. Although a 'cathch-up' gain in weight was observed, poor growth in height was displayed, and there were no differences between the Wh and Re groups in this regard. Thus, we conclude that late complications of liver transplantation in children are common and further studies are necessary to evaluate the ongoing growth problems. [ABSTRACT FROM AUTHOR]

To characterize the potential utility of the University of Wisconsin (UW) solution, 37 grafts preserved in UW solution (UW group) were compared with 38 grafts preserved in Euro-Collins (EC) solution (EC group). Patients in both groups underwent similar perioperative management. Donor and recipient data in the two groups were similar. The mean cold ischemia time (CIT) of the UW group was 6.51±2.06 h (range: 3.17-14.65 h); this was significantly longer than that of the EC group, of 4.80±1.68 h (range: 1.67-8.83h) ( P<0.05). There were no significant differences in actuarial patient or graft survival between the two groups. Blood concentrations of aspartate aminotransferase (AST) within 12 h and lactic dehydogenase (LDH) within 6 h of reperfusion were significantly lower in the UW group than in the EC group, each being ( P<0.05). The total bilirubin (TBR) concentration was lower in the UW group than in the EC group, but the difference was not significant. Alkaline phosphatase (ALP), gamma glutamyl transpeptidase (GGTP), and prothrombin time (PT) showed no significant differences between the two groups. There was no difference in the frequency of complications or rejection episodes between the two groups. However, gender identical grafts had a lower rejection than gender non-identical grafts in the UW group ( P<0.05). Our data suggest that the UW solution reduces transplantation-related graft injury compared with the EC solution. [ABSTRACT FROM AUTHOR]

A prospective study of renal function was undertaken on an unselected group of 8 children with chronic progressive liver disease on whom a renal biopsy was performed subsequently at the time of orthotopic liver transplantation. Two patients had abnormal urinalyses and 2 elevated urinary albumin/creatinine ratios. The remainder had no clinical evidence of renal dysfunction. All had normal serum creatinine concentrations. Glomerular abnormalities were present in all renal biopsies and were of two types: hepatic glomerulosclerosis ( n=5) and minor glomerular abnormalities ( n=3). IgM immunofluorescence was present in all biopsies and IgA in 6. Elevated serum immunoglobulin levels were observed in all patients, with IgM elevation in 6, IgA in 4 and IgG in 6. C3 and/or C4 were reduced in 5 patients and increased circulating immune complexes containing IgM were noted in 4. The clinical significance of these cirrhosis-associated glomerular abnormalities can only be established by long-term follow-up studies after orthotopic liver transplantation. [ABSTRACT FROM AUTHOR]

Presents a letter to the editor about the use of stronger neo minophagen C for the improvement of hepatitis B after liver transplantation.