Acute liver failure (ALF) in children differs from that observed in adults in both the etiologic ... more Acute liver failure (ALF) in children differs from that observed in adults in both the etiologic spectrum and the clinical picture. Children, particularly very young ones, do not demonstrate classical features of encephalopathy and the definition of ALF has been revised to include patients with advanced coagulopathy, regardless of mental status. A significant number of these children will go on to require transplant or die. Etiologies vary by age with metabolic and infectious diseases prominent in the first year of life and acetaminophen overdose and Wilson's disease occurring in adolescents. In almost 50% of cases, however, the child has an indeterminate cause for ALF. Management requires a multidisciplinary approach and is directed at establishing the etiology where possible and monitoring, anticipating, and managing the multisystem complications that occur in children with ALF. Overall, short-term outcomes are better in children than adults but are dependent upon the degree of encephalopathy and diagnosis.
Early portal vein thrombosis (PVT) represents a serious complication after liver transplantation ... more Early portal vein thrombosis (PVT) represents a serious complication after liver transplantation (OLTx). From October 1997 through July 2004, 260 OLTx were performed in 231 children, including 189 of left lateral segments (LLS). We retrospectively analyzed the incidence and the outcome of early PVT in this group. A daily doppler US scan was performed during the first week after transplantation. Early PVT occurred in 14 patients (8%), 10 males and four females of median age 0.77 years. The main indication for primary transplantation was biliary atresia (10), followed by Byler's disease (2), acute liver failure on cryptogenetic cirrhosis (1), and Alagille syndrome (1). Four children underwent retransplantation; three cases of thrombectomy and revision of the anastomosis, two children were treated with beta blockers, one of whom had a later failed attempt at percutaneous revascularization and eventually a meso-caval shunt. Five patients were followed with observation and no treatment. Among the four patients who died, three were in the retransplantation group and one in the thrombectomy and revision of the anastomosis group; the overall mortality was 28%. With a median follow up of 399 days, 10 patients are alive with an actuarial survival at 1 and 5 years of 72%, and graft survival rates at 1 and 5 years of 64%. PVT represents a serious complication after pediatric OLTx with LLS grafts. Prompt detection and aggressive surgical treatment in selected cases are required to reduce the mortality and graft loss.
Tumors of the liver are rare in infancy and childhood. Some are peculiar to the pediatric age, e.... more Tumors of the liver are rare in infancy and childhood. Some are peculiar to the pediatric age, e.g., hepatoblastoma, infantile hemangioendothelioma, mesenchymal hamartoma, embryonal rhabdomyosarcoma. This review is based upon personal experience with a series of 32 cases. On the basis of the histological features it is proposed that focal modular hyperplasia (FNH) and mesenchymal hamartoma be considered as tumor-like lesions rather than true neoplasms. A few benign epithelial lesions (FNH, adenoma) were associated with inborn error of metabolism. In half of the patients, hepatocellular carcinoma (HCC) developed in perinatally hepatitis B virus (HBV)-infected children. HCC developed in a noncirrhotic liver in a single patient, in whom HBV-DNA integration had occurred.
Acute liver failure (ALF) in children differs from that observed in adults in both the etiologic ... more Acute liver failure (ALF) in children differs from that observed in adults in both the etiologic spectrum and the clinical picture. Children, particularly very young ones, do not demonstrate classical features of encephalopathy and the definition of ALF has been revised to include patients with advanced coagulopathy, regardless of mental status. A significant number of these children will go on to require transplant or die. Etiologies vary by age with metabolic and infectious diseases prominent in the first year of life and acetaminophen overdose and Wilson's disease occurring in adolescents. In almost 50% of cases, however, the child has an indeterminate cause for ALF. Management requires a multidisciplinary approach and is directed at establishing the etiology where possible and monitoring, anticipating, and managing the multisystem complications that occur in children with ALF. Overall, short-term outcomes are better in children than adults but are dependent upon the degree of encephalopathy and diagnosis.
Early portal vein thrombosis (PVT) represents a serious complication after liver transplantation ... more Early portal vein thrombosis (PVT) represents a serious complication after liver transplantation (OLTx). From October 1997 through July 2004, 260 OLTx were performed in 231 children, including 189 of left lateral segments (LLS). We retrospectively analyzed the incidence and the outcome of early PVT in this group. A daily doppler US scan was performed during the first week after transplantation. Early PVT occurred in 14 patients (8%), 10 males and four females of median age 0.77 years. The main indication for primary transplantation was biliary atresia (10), followed by Byler's disease (2), acute liver failure on cryptogenetic cirrhosis (1), and Alagille syndrome (1). Four children underwent retransplantation; three cases of thrombectomy and revision of the anastomosis, two children were treated with beta blockers, one of whom had a later failed attempt at percutaneous revascularization and eventually a meso-caval shunt. Five patients were followed with observation and no treatment. Among the four patients who died, three were in the retransplantation group and one in the thrombectomy and revision of the anastomosis group; the overall mortality was 28%. With a median follow up of 399 days, 10 patients are alive with an actuarial survival at 1 and 5 years of 72%, and graft survival rates at 1 and 5 years of 64%. PVT represents a serious complication after pediatric OLTx with LLS grafts. Prompt detection and aggressive surgical treatment in selected cases are required to reduce the mortality and graft loss.
Tumors of the liver are rare in infancy and childhood. Some are peculiar to the pediatric age, e.... more Tumors of the liver are rare in infancy and childhood. Some are peculiar to the pediatric age, e.g., hepatoblastoma, infantile hemangioendothelioma, mesenchymal hamartoma, embryonal rhabdomyosarcoma. This review is based upon personal experience with a series of 32 cases. On the basis of the histological features it is proposed that focal modular hyperplasia (FNH) and mesenchymal hamartoma be considered as tumor-like lesions rather than true neoplasms. A few benign epithelial lesions (FNH, adenoma) were associated with inborn error of metabolism. In half of the patients, hepatocellular carcinoma (HCC) developed in perinatally hepatitis B virus (HBV)-infected children. HCC developed in a noncirrhotic liver in a single patient, in whom HBV-DNA integration had occurred.
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Papers by D. Alberti