Podgaetz E, Chan C

Language: English
References: 13
Page: 131-134
PDF size: 40.59 Kb.

Text Extraction

Background/Aims: Orthotopic liver transplantation is being used with more frequency as the treatment for Wilson´s disease. The experience at the Instituto Nacional de Ciencias Medicas y Nutricion Salvador Zubiran with orthotopic liver transplantation for Wilson´s disease is reported. We perform an extensive literature review for this treatment modality. Methods: Between january 2000 and june 2003, 23 orthotopic liver transplants were performed at our institution, 2 of them for Wilson´s disease. Both the patients presented with chronic advanced liver disease and one presented neurologic dysfunction. Results: Both the patients were trasplanted without any major complication and are alive 43 and 22 months after the transplant respectively. To our knowledge 370 liver transplants have been reported in the international literature since 1994 for the treatment of Wilson´s disease. Conclusions: Currently, orthotopic liver transplantation should be considered as a major option for the treatment of chronic liver disease in patients with Wilson´s disease. Although it is well known that the transplant only partially corrects the defective metabolism in patients with Wilson´s disease, it does convert the copper kinetics of a homozygous to that of a heterozigote, thus, providing an effective phenotypic cure.

REFERENCES

1. Bellary S, Hassanein T, Van Thiel H. Liver transplantation for Wilson´s disease. Journal of Hepatology 1995; 23: 373-381.

2. Reuben: Not too little, not too much, but just right. Hepatology 2002; 1555-1558.

3. Macedo G, Maia JC, et al. Wilson´s disease: Challenging diagnosis, management, and liver transplantation timing. Transplantation Proceedings 2000; 32: 2668.

4. Robles R, Parrilla P, Sicilia J, Bueno FS, et al. Indications and results of liver transplants in wilson´s disease. Transplantation Proceedings 1999; 31: 2453-2454.

5. Geissler J, Heinemann K, Rohm S, et al. Liver transplantation for hepatic and neurological Wilson´s disease. Transplantation Proceedings 2003; 35: 1445-1446.

6. Sutcliffe RP, Maguire DD, Muiesan P, et al. Liver transplantation for Wilson´s disease: long-term results and quality-of-life assessment. Transplantation 2003; 75(7): 1003-6.

7. Eghtesad B, Nezakatgoo N, Geraci L, et al. Liver transplantation for Wilson´s disease: a single center experience. Liver Transpl Surg 1999; 5: 467-474.

8. Schilsky ML, Scheinberg IH, Sternlieb I. Liver transplantation for Wilson´s disease: indications and outcome. Hepatology 1994; 19(3): 583-7.

9. Emre S, Atillasoy EO, Ozdemir S, et al. Othotopic liver transplantation for Wilson´s disease: a single center experience. Transplantation 2001; 72(7): 1232-6.

10. Hermann W, Eggers B, Wagner A. The indication for liver transplant to improve neurological symptoms in a patient with Wilson´s disease. J Neurol 2002; 249: 1733-1734.

11. Cummings JN. The effects of B.A.L in hepatolenticular degeneration. Brain 1951; 74: 10-22.

12. Cummings JN. The copper and iron content of brain and liver in the normal and in hepatolenticular degeneration. Brain 1948; 71: 410-415.

13. Walshe JM. Copper: not too little, not too much, but just right. J R Coll Physicians 1995; 20: 280-288.