Long-term outcome of liver transplantation in patients with glycogen storage disease type Ia.

摘要: Liver transplantation may be indicated in patients with GSD type Ia when dietary treatment fails or hepatic adenomas develop, because they carry a risk of liver cancer severe intratumoral haemorrhage. Published reports on the results include 10 and provide little information long-term outcome. In particular, it is not known whether prevents renal failure due to focal segmental glomerulosclerosis. We report here 3 whom was performed at 15, 17 23 years age multiple all fear malignant transformation, poor metabolic balance growth retardation youngest one. Renal function normal patients. During 6-8 following transplantation, quality life has initially greatly improved, none previous restraints spectacular increase height. However, complications included chronic hepatitis C one patient, gouty attacks another glomerulosclerosis progressive insufficiency third. These results: (1) confirm that restores Ia, allows catch-up improves life; (2) suggest considered teenagers unresectable lack clear-cut criteria detect transformation early; (3) does prevent associated Ia.