Editor-in-Chief, Deputy Editor 2017-2019

 

Editor-in-Chief:

Tom MOREELS

 

Deputy Editor:

Nicolas LANTHIER

 

Symposium



Adult stem cells perspectives in treatment of metabolic diseases


Price: €10,00

There is currently great excitement and expectation concerning the differential potential of adult stem cells or adult cells with capacity of differentiation. As the body of work concerning transdifferentiation of somatic stem cells and bone marrow derived stem cells grows, the number of critics increases steadily questioning the reliability of reported findings. So scientists are now challenged more and more to prove that resulting differentiated somatic cells originated from somatic adult stem cell through a transdifferentiation process. Phenomenons such as fusion of cells have to be ruled out and the origin of the differentiated cell has to be determined by specific techniques i.e. in situ hybridisation. Cellular mimicry through uptake of specific factors out of the medium is questioned to be the reason for cells staining positive for Insulin. Some multipotent adult stem cells can cross lineage boundaries and differentiate into somatic cells of other lineages after being relocated. Bone marrow cells have been described to have the greatest plasticity among adult stem cells regenerating damaged liver or myocardium. It has been proposed that the differentiation of bone marrow derived adult stem cells occurs naturally even in healthy organs as a physiologic process of tissue-regeneration. Others believe that organ damage is essential to induce transdifferentiation by release of organ specific microenvironmental factors. We here try to constitute necessary data which should be demonstrated to give substantial evidence for transdifferentiation of newly characterized cells including exclusion of fusion, phagocytosis or DNA uptake, description of the outset cell, differentiation into all three germ layers and functional parameters. [Product Details...]



Crigler Najjar (CN) disease and liver transplantation


Price: €10,00

No Abstract available. [Product Details...]



Hepatocyte transplantation for metabolic disorders, experience at Kings College hospital and review of literature


Price: €10,00

Hepatocyte transplantation is emerging as a potential treatment for liver based metabolic disorders and acute liver failure. To date, clinical studies have shown that hepatocyte transplantation could be used as a bridge to liver transplantation in a few patients with acute liver failure and has changed the phenotype of metabolic patients in terms of reducing the severity of illness. For many years, research studies have been carried out to optimise conditions for a) hepatocyte isolation in order to obtain the highest possible number of viable hepatocytes isolated from various sources of unused donor liver tissues, and b) cryopreservation and storage of hepatocytes for immediate cell availability in emergency cases. In this review, we summarise the worldwide clinical experience in hepatocyte transplantation for liver based metabolic disorders including the experience of our centre at Kings College Hospital, London (United Kingdom). We briefly comment on the possible future developments and improvements needed in this field. [Product Details...]



In vitro tissue expansion perspectives


Price: €10,00

No Abstract available [Product Details...]



Introduction : liver and liver cell transplantation for inborn errors of liver metabolism


Price: €10,00

No Abstract available. [Product Details...]



Liver and liver cell transplantation for glycogen storage disease type IA


Price: €10,00

Glycogen storage disease type Ia (GSDIa) is an inherited disorder of glucose metabolism, due to the selective deficiency of the hepatic enzyme glucose-6-phosphatase. Clinical manifestations include severe hypoglycaemia three to four hours post-prandially, increased production of lactic acid, triglycerides and uric acid, hepatic glycogen storage disease with development of multiple adenomas and kidney disease with proteinuria. Liver transplantation is frequently performed in order to achieve metabolic control and when malignant transformation of adenomas is suspected. Long term outcome following transplantation is good, but immunosuppressive therapy can worsen the progression of associated kidney disease. Hepatocyte transplantation could be considered as a less invasive procedure in such patients. Our experience with hepatocyte transplantation in a 47 year-old woman affected by glycogen storage disease type Ia and suffering of severe fasting hypoglycaemia indicates that the procedure can partially correct some metabolic abnormalities and improve the quality of life in this disease. However, the metabolic improvement was reduced and finally abolished during long term follow-up, probably due to rejection or to senescence of transplanted cells. Moreover, the portal and pulmonary hypertension associated with the disease need to be evaluated for their possible influence on haemodynamic changes associated with cell infusion. Finally, hepatic adenomas need careful monitoring because of the possible risk of malignant transformation [Product Details...]



Liver or liver cell transplantation for phenylketonuria


Price: €10,00

No Abstract available. [Product Details...]



Liver transplantation for inherited metabolic disease


Price: €10,00

Liver Transplantation is indicated for children with inherited metabolic disease because of a primary hepatic enzyme deficiency, which leads to liver failure and/or hepatic cancer, or severe extra hepatic disease. The selection and timing depends on the rate of progression of disease, the success of medical therapy and the extent of reversible extra hepatic disease. Transplant techniques include orthotopic, living related and auxiliary liver transplantation or combined liver and kidney transplantation. Liver transplantation effectively treats structural metabolic hepatic disease with a phenonotypic and functional cure in alpha1 antitrypsin deficiency, Wilsons disease, PFIC and neonatal haemochromatosis. There is only a partial cure in Tyrosinaemia Type 1 as abnormal metabolites are still produced by the kidney, but a complete cure in primary oxalosis and urea cycle defects. Outcome for organic acidaemias remains poor due to technical and metabolic challenges. [Product Details...]



Liver transplantation for neurologic manifestations of Wilsons disease


Price: €10,00

No abstract available. [Product Details...]



Long-term complications of immunosuppression in pediatric liver recipients


Price: €10,00

Liver transplantation (LT) today constitutes a well-standardized and efficient therapy for children with acute and chronic hepatic failure. Appropriate pre-transplant management, organ preservation, adequate surgical techniques, and the progressive introduction of new immunosuppressive regimens have contributed to significantly improve, over the years, the general outcome after LT. Consequently, these good overall results has allowed the constitution of a growing cohort of children, adolescents and young adults submitted to chronic immunosuppression. The long-term complications of immunosuppression administered to transplant recipients include the adverse effects secondary to the depression of the immune system, the toxicities specifically related to the individual immunosuppressive drugs, and the sense of lack of rehabilitation for the transplant patient, with, secondarily, the question of non-adherence to the medications. This review will essentially focus on these three issues in the particular context of pediatric liver transplantation [Product Details...]



Long-term outcome of urea cycle disorders


Price: €10,00

Evaluation of long-term outcome of patients with urea cycle diseases (UCD) is needed for medical decisions and counselling. Own data comparing outcome of UCD patients with the old treatment limited to protein restriction (i.e. close to the natural history) with that of patients on the modern conservative treatment have shown that gains in survival occur at the cost of more mentally retarded surviving patients. We discuss the possible bias in long-term outcome studies of those rare inheritable disorders where non-predictable environmental factors leading to catabolic crises have a crucial impact on prognosis. A combination of peak or initial ammonia value combined with the duration of coma is discussed as a criterion for prognosis of handicap. The neglect of dietary compensation of branched chain amino acid deficiency worsened by phenylbutyrate treatment in some published protocols could well be an additional cause of the non satisfactory long- term results of conservative treatment which in our view - mainly aim at bridging optimally the period of late neonatal presentation until liver transplantation in patients with CPS and OTC deficiency (except for mild forms). [Product Details...]



Medical progress and pitfalls of the treatment of inborn errors involving the liver


Price: €10,00

No Abstract available [Product Details...]



Medical versus surgical management of progressive familial intrahepatic cholestasis PFIC


Price: €10,00

No Abstract available [Product Details...]



Preemptive liver transplantation for primary hyperoxaluria type 1


Price: €10,00

No Abstract available. [Product Details...]



Progrees and pifalls in organic aciduria


Price: €10,00

No Abstract available [Product Details...]



Report of liver transplantation in organic aciduria


Price: €10,00

No Abstract available [Product Details...]



Results of liver cell transplantation in urea cycle disorders


Price: €10,00

No Abstract available. [Product Details...]


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