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Biomedical subjects

B G Ericzon

Publications and source records attributed to B G Ericzon.

At least 55 records · Page 3Linked to original sources
PubMed1995

Liver transplantation in familial amyloidotic polyneuropathy. Follow-up of the first 20 Swedish patients.

Familial amyloidotic polyneuropathy (FAP) is an inherited fatal form of amyloidosis caused by mutant transthyretin. The disease is characterized by progressive peripheral and autonomic neuropathy. Most of the transthyretin is produced by the liver, and we have shown previously that the metabolic deficiency can be corrected by liver transplantation. In the present study, the clinical results from the first 20 patients who underwent liver transplantation for FAP in Sweden are evaluated. Three of the patients suffered from renal failure and underwent a simultaneous kidney transplantation. Fourteen of the 20 patients (70%) are alive 10-52 months after transplantation. The patients' nutritional status at the time of transplantation had a significant impact on mortality and morbidity (P < 0.007). Long-standing disease was another negative prognostic factor (P < 0.02). One year after transplantation, the nutritional status had improved (P < 0.02). Improvements were also noted in walking capacity and for gastrointestinal and urogenital symptoms. The results show that liver transplantation offers an effective means to treat patients with FAP. The procedure should preferably be performed before the nutritional status is poor and advanced organ dysfunction has developed.

Adult↗
PubMed1995

Change in variant transthyretin levels in patients with familial amyloidotic polyneuropathy type I following liver transplantation.

Three patients with familial amyloidotic polyneuropathy (FAP) type I underwent liver transplantation from heart-beating cadaveric donors. Since 2 patients underwent blood transfusion during the operation, variant transthyretin (TTR) levels in the plasma did not decrease time dependently. However, in 1 patient without blood transfusion variant TTR levels decreased in a time dependent manner and plasma half life of variant TTR was calculated to be 2.1 days. Total protein, normal, and variant TTR levels in cerebrospinal fluid (CSF) remained unchanged after liver transplantation.

Adult↗
PubMed1995

Liposomal amphotericin B prevents invasive fungal infections in liver transplant recipients. A randomized, placebo-controlled study.

Eighty-six consecutive liver transplant recipients were prospectively randomized in a double-blind, placebo-controlled antifungal prophylaxis study. Seventy-seven patients received 5 days of prophylaxis starting during the transplantation with either liposomal amphotericin B (AmBisome) 1 mg/kg/day or placebo. Among 40 AmBisome-treated patients, no invasive Candida infection was seen during the first month, compared with 5 invasive Candida albicans infections among 37 control patients (P < 0.05). Furthermore, 1 placebo patient experienced Aspergillus niger pneumonia. Thus, the overall incidence of invasive fungal infections was 0/40 (0%) in the AmBisome group versus 6/37 (16%) in the placebo group (P < 0.01). Patient survival at 30 days was 92% versus 94% for AmBisome- and placebo-treated patients, respectively. One patient experienced backache related to AmBisome infusion. Two patients had transient thrombocytopenia possibly caused by AmBisome treatment. AmBisome was otherwise well tolerated. The total cost for all antifungal drugs used in both groups was equal. However, prophylaxis with AmBisome was $5000 less expensive than treatment of proven invasive fungal infections among placebo patients.

Adolescent↗
PubMed1995

Influence of orthotopic liver transplantation on serum vitamin A levels in patients with chronic liver disease.

BACKGROUND: Low serum vitamin A levels are observed in many liver diseases, such as primary biliary cirrhosis (PBC) and alcoholic liver disease. The aim of this study was to investigate serum vitamin A in patients with advanced liver diseases before and after orthotopic liver transplantation (OLT). METHODS: Serum vitamin A (retinol) concentrations were investigated in 54 patients before (OLT) and in 21 patients 1, 2, 3, and 4 weeks and 2 and 3 months after OLT. Ten healthy subjects and 19 patients with inflammatory bowel disease (IBD) served as control groups. RESULTS: The mean serum retinol concentration before OLT was 0.64 +/- 0.1 mumol/l in patients with alcoholic and postnecrotic cirrhosis (n = 24), 1.06 +/- 0.48 mumol/l in patients with PBC (n = 14), 0.96 +/- 0.64 mumol/l in sclerosing cholangitis (n = 7), and 1.02 +/- 0.73 mumol/l in liver cancer (n = 9). These results were significantly lower than in healthy controls (2.34 +/- 0.54 mumol/l) and patients with IBD (2.7 +/- 0.74 mumol/l) (p < 0.001). CONCLUSIONS: After OLT, serum retinol levels increased significantly already after 1 week (1.4 +/- 0.1 mumol/l) (p < 0.001), normalized after 2 weeks (2.2 +/- 0.4 mumol/l), and remained normal during the observation.

Adult↗
PubMed1995

Bone mineral status in end-stage liver disease and the effect of liver transplantation.

BACKGROUND: The aim of this study was to determine bone mass at different skeletal sites in patients with end-stage liver disease and the effect of liver transplantation on bone mineralization. METHODS: Bone mineral density in different skeletal regions was measured by photon absorptiometry in 25 patients with chronic liver disease, and the measurements were repeated in nine patients after orthotopic liver transplantation. RESULTS: In patients with liver failure bone mass values were not significantly different from those of controls. After liver transplantation bone mass decreased significantly during the first 6 posttransplant months at the distal radius, lumbar spine, and femur (p < 0.01) and was still below pretransplant values at the 12th posttransplant month. Serum osteocalcin increased significantly from the 3rd month after transplantation (from 6.9 +/- 4.4 to 12.0 +/- 6.5 micrograms/l; p < 0.0001) and remained increased throughout the first posttransplant year. CONCLUSION: Early and accelerated bone loss occurred after liver transplantation. This bone reduction seems to be mainly the result of increased bone resorption, possibly related to corticosteroid therapy.

Absorptiometry, Photon↗
PubMed1994

A randomized trial of OKT3-based versus cyclosporine-based immunoprophylaxis after liver transplantation. Long-term results of a European and Australian multicenter study.

A multicenter randomized trial was performed to compare two immunosuppressive protocols after first ABO-compatible liver transplantation. Forty six patients were randomized to a 14-day treatment with Orthoclone (OKT3) in association with steroids and azathioprine, cyclosporine being progressively introduced on day 11 posttransplant. Fifty patients were randomized to a standard protocol of cyclosporine with steroids and azathioprine. Minimum follow-up was 1 year and graft and patient survivals were updated for the purpose of the study. The cumulative 1-year incidence of acute rejection tended to be greater in the cyclosporine group (75%) than in the OKT3 group (67%), especially when patients who did not receive full-course treatment with OKT3 were excluded (59%). Renal function was better preserved during the first two postoperative weeks in the OKT3 group than in the control group but plasma creatinine levels were comparable in both groups thereafter. The incidence of severe infections was lower in the OKT3 group (13.6%) than in the cyclosporine group (32%). The 4-year incidences of patient and graft survival in the OKT3 group (69% and 61%, respectively) were not different from those in the cyclosporine group (62% versus 54%, respectively). Thus this prospective trial shows that OKT3 immunoprophylaxis is a safe alternative to cyclosporine immunoprophylaxis in unselected recipients of a first liver graft.

Adolescent↗
PubMed1994

Treatment of a Japanese patient with familial amyloidotic polyneuropathy with orthotopic liver transplantation.

A 28-year-old male patient with familial amyloidotic polyneuropathy (FAP) underwent a liver transplantation from a heart-beating cadaveric donor in Sweden. He had suffered from the disease for 2.5 years. It took 5.5 hours to carry out the operation without blood transfusion. After the liver transplantation, serum amyloidgenic variant transthyretin (TTR) levels became extremely low and diarrhea stopped after the 7th day. On day 13, the patient was discharged from the hospital and one month after the transplantation, his general condition remained quite good. This is the first case of a Japanese patient with congenital metabolic disorders as well as FAP to receive a liver transplantation from a heart-beating cadaveric donor.

Adult↗