[Combined heart and kidney transplantation in Fabry's disease: Long-term outcomes in two patients]. / Transplantation combinée cÅurrein au cours de la maladie de Fabry : suivi à long terme de deux patients.
Rev Med Interne
; 38(2): 137-142, 2017 Feb.
Article
en Fr
| MEDLINE
| ID: mdl-27241078
ABSTRACT
INTRODUCTION:
Fabry disease is a lysosomal storage disorder linked to an alpha-galactosidase A deficiency that can lead to heart and kidney failure. There is little data about the prognosis of patients who undergo a combined heart and kidney transplantation. CASE REPORTS Two brothers who were diagnosed with Fabry disease after the age of 30 years underwent a combined heart and kidney transplantation at respectively 49 and 42 years of age because of a severe hypertrophic cardiomyopathy with end stage renal failure. They are alive respectively 4 and 9 years after the transplantation. No recurrence of the disease in the transplanted organs has been found.CONCLUSION:
Combined heart and kidney transplantation in Fabry disease is an efficient therapy for the cardiomyopathy and kidney failure. Its prognosis can be good when the patients are carefully selected. However, an early diagnosis is critical in order to avoid a procedure associated with a high perioperative mortality.Palabras clave
Texto completo:
1
Base de datos:
MEDLINE
Asunto principal:
Trasplante de Corazón
/
Trasplante de Riñón
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Enfermedad de Fabry
/
Fallo Renal Crónico
Tipo de estudio:
Observational_studies
/
Prognostic_studies
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Risk_factors_studies
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Screening_studies
Idioma:
Fr
Revista:
Rev Med Interne
Año:
2017
Tipo del documento:
Article