LIVER TRANSPLANTATION IN CHILDREN WITH GLYCOGEN STORAGE DISEASES: RISK ASSESSMENT AND NECESSITY OF THIS PROCEDURE
https://doi.org/10.15825/1995-1191-2013-1-67-74
Abstract
Glycogen storage diseases I, III and IV types are congenital disorders, which are commonly associated with severe liver diseases. Liver transplantation has been proposed as a treatment of choise for these disorders. While liver transplantation corrects the primary hepatic enzyme defect, the extrahepatic manifestations of glycogenoses often complicate the posttransplant management. Upon review of the English-language literature, 42 children under 18 years old were discovered to have undergone liver transplantation for complications associated with glycogenoses (18 patients with Ia type, 6 – with Ib type, one patient – with III type, 17 – with IV type). This artic- le represents the pediatric liver transplantation for complications associated with glycogenosis Ia type, analyzed posttransplant period in this recipient.
About the Authors
S. V. Gautier
Academician V.I. Shumakov Federal Research Center of Transplantology and Artificial Organs, Moscow
I.M. Sechenov First Moscow State Medical University, Department of Transplantology and Artificial Organs, Moscow
O. M. Tsirulnikova
Academician V.I. Shumakov Federal Research Center of Transplantology and Artificial Organs, Moscow
I.M. Sechenov First Moscow State Medical University, Department of Transplantology and Artificial Organs, Moscow
D. S. Mnatsakanyan
Academician V.I. Shumakov Federal Research Center of Transplantology and Artificial Organs, Moscow
I. M. Ilyinsky
Academician V.I. Shumakov Federal Research Center of Transplantology and Artificial Organs, Moscow
I.M. Sechenov First Moscow State Medical University, Department of Transplantology and Artificial Organs, Moscow
N. P. Mozheiko
Academician V.I. Shumakov Federal Research Center of Transplantology and Artificial Organs, Moscow
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For citations:
Gautier S.V., Tsirulnikova O.M., Mnatsakanyan D.S., Ilyinsky I.M., Mozheiko N.P. LIVER TRANSPLANTATION IN CHILDREN WITH GLYCOGEN STORAGE DISEASES: RISK ASSESSMENT AND NECESSITY OF THIS PROCEDURE. Russian Journal of Transplantology and Artificial Organs. 2013;15(1):67-74. (In Russ.) https://doi.org/10.15825/1995-1191-2013-1-67-74
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