Related liver fragment transplantation for type 1 glycogenosis: the first Russian experience The paper describes two clinical examples of successful related liver fragment transplantation for type 1 glycogenosis in a one-year-old boy and a 6-year-old girl. The given first successful clinical experience suggests that liver fragments for type 1 glycogenoses can be transplanted from live related donors and offers promise for the radical treatment of patients with this pathology.
https://doi.org/10.23873/2074-0506-2011-0-2-3-24-28
Abstract
The paper describes two clinical examples of successful related liver fragment transplantation for type 1 glycogenosis in a one-year-old boy and a 6-year-old girl. The given first successful clinical experience suggests that liver fragments for type 1 glycogenoses can be transplanted from live related donors and offers promise for the radical treatment of patients with this pathology.
About the Authors
A. V. Filin
Acad. B.V. Petrovsky Russian Surgery Research Center, Russian Academy of Medical Sciences; Research Institute of Nutrition, Russian Academy of Medical Sciences
Russian Federation
Russian Federation
A. V. Semenkov
Acad. B.V. Petrovsky Russian Surgery Research Center, Russian Academy of Medical Sciences; Research Institute of Nutrition, Russian Academy of Medical Sciences
Russian Federation
Russian Federation
N. A. Koroteyeva
Acad. B.V. Petrovsky Russian Surgery Research Center, Russian Academy of Medical Sciences; Research Institute of Nutrition, Russian Academy of Medical Sciences
Russian Federation
Russian Federation
A. Ya. Keligova
Acad. B.V. Petrovsky Russian Surgery Research Center, Russian Academy of Medical Sciences; Research Institute of Nutrition, Russian Academy of Medical Sciences
Russian Federation
Russian Federation
A. V. Metelin
Acad. B.V. Petrovsky Russian Surgery Research Center, Russian Academy of Medical Sciences; Research Institute of Nutrition, Russian Academy of Medical Sciences
Russian Federation
Russian Federation
E. F. Kim
Acad. B.V. Petrovsky Russian Surgery Research Center, Russian Academy of Medical Sciences; Research Institute of Nutrition, Russian Academy of Medical Sciences
Russian Federation
Russian Federation
D. S. Dzhamanchin
Acad. B.V. Petrovsky Russian Surgery Research Center, Russian Academy of Medical Sciences; Research Institute of Nutrition, Russian Academy of Medical Sciences
Russian Federation
Russian Federation
N. E. Khartukova
Acad. B.V. Petrovsky Russian Surgery Research Center, Russian Academy of Medical Sciences; Research Institute of Nutrition, Russian Academy of Medical Sciences
Russian Federation
Russian Federation
Yu. R. Kamalov
Acad. B.V. Petrovsky Russian Surgery Research Center, Russian Academy of Medical Sciences; Research Institute of Nutrition, Russian Academy of Medical Sciences
Russian Federation
Russian Federation
E. Yu. Kryzhanovskaya
Acad. B.V. Petrovsky Russian Surgery Research Center, Russian Academy of Medical Sciences; Research Institute of Nutrition, Russian Academy of Medical Sciences
Russian Federation
Russian Federation
V. A. Khovrin
Acad. B.V. Petrovsky Russian Surgery Research Center, Russian Academy of Medical Sciences; Research Institute of Nutrition, Russian Academy of Medical Sciences
Russian Federation
Russian Federation
T. N. Galyan
Acad. B.V. Petrovsky Russian Surgery Research Center, Russian Academy of Medical Sciences; Research Institute of Nutrition, Russian Academy of Medical Sciences
Russian Federation
Russian Federation
B. S. Kaganov
Acad. B.V. Petrovsky Russian Surgery Research Center, Russian Academy of Medical Sciences; Research Institute of Nutrition, Russian Academy of Medical Sciences
Russian Federation
Russian Federation
E. N. Kutyreva
Acad. B.V. Petrovsky Russian Surgery Research Center, Russian Academy of Medical Sciences; Research Institute of Nutrition, Russian Academy of Medical Sciences
Russian Federation
Russian Federation
A. I. Zubovich
Acad. B.V. Petrovsky Russian Surgery Research Center, Russian Academy of Medical Sciences; Research Institute of Nutrition, Russian Academy of Medical Sciences
Russian Federation
Russian Federation
References
1. Ozen H. Glycogen storage diseases: New perspectives. World J Gastroenterol 2007;13(18):2541–53.
2. Эндокринология (под ред. Н. Лавина) М.: Практика, 1999.
3. Von Gierke E. Hepato-nephromegalia glykogenica (Glykogenspeicherkrankheit der Leber und Nieren). Beitr Path Anat 1929;82:497–513.
4. Cori G.T., Cori C.F. Glucose-6-phosphatase of the liver in glycogen storage disease. J Biol Chem. Dec 1952;199(2):661–7.
5. Chen Y.T., Burchell A. Glycogen storage disease. In: C.R. Scriver, A.L. Beaudet, W.S. Sly, D. Valle, eds. The Metabolic Basis of Inherited Disease. 8th ed. New York: McGraw-Hill, 2001;1521–51.
6. Ekstein J., Rubin B.Y., Anderson S.L. et al. Mutation frequencies for glycogen storage disease Ia in the Ashkenazi Jewish population. Am J Med Genet A 2004;129:162–4.
7. Bhattacharya K., Heaton N., Rela M. et al. The benefits of liver transplantation in glycogenosis type 1b. J Inherit Metab Dis 2004;27:539–40.
8. Matern D., Starzl T.E., Arnaout W. et al. Liver transplantation for glycogen storage disease types I, III and IV. Eur J Pediatr 1999;158 (suppl 2):43–8.
9. Olmos M.A., Sanroman L.A., Vaquero M.P. et al. Liver transplantation for type Ib glycogenesis with reversal of cyclic neutropenia. Clin Nutr 2001;20:375–7.
10. Martin A.P., Bartels M., Schreiber S. et al. Successful staged kidney and liver transplantationfor glycogen storage disease type 1b: a case report.Transplant Proc 2006;38:3615–9.
11. Morioka D., Kasahara M., Takada Y. et al. Living donor liver transplantationfor pediatric patients with inheritable metabolic disorders. Am J Transplant 2005;5:2754–63.
12. Готье С.В., Константинов Б.А., Цирульникова О.М. Трансплантация печени. Руководство для врачей. М.: МИА, 2008.
13. Ким Э.Ф. Клинические и хирургические аспекты прижизненного донорства фрагментов печени. Дис. … д-ра мед. наук. М., 2008.
14. Семенков А.В. Родственные доноры для трансплантации печени: отбор и хирургическая тактика. Дис. … канд. мед. наук. М., 2003.
15. Филин А.В. Родственная пересадка в хирургическом лечении заболеваний печени. Дис. … д-ра мед. наук. М., 2008.
Review
For citations:
Filin A.V., Semenkov A.V., Koroteyeva N.A., Keligova A.Ya., Metelin A.V., Kim E.F., Dzhamanchin D.S., Khartukova N.E., Kamalov Yu.R., Kryzhanovskaya E.Yu., Khovrin V.A., Galyan T.N., Kaganov B.S., Kutyreva E.N., Zubovich A.I. Related liver fragment transplantation for type 1 glycogenosis: the first Russian experience The paper describes two clinical examples of successful related liver fragment transplantation for type 1 glycogenosis in a one-year-old boy and a 6-year-old girl. The given first successful clinical experience suggests that liver fragments for type 1 glycogenoses can be transplanted from live related donors and offers promise for the radical treatment of patients with this pathology. Transplantologiya. The Russian Journal of Transplantation. 2011;(2-3):24-28. (In Russ.) https://doi.org/10.23873/2074-0506-2011-0-2-3-24-28
Views:
690
Email this article 