title: [Combined heart and kidney transplantation in Fabry's disease: Long-term outcomes in two patients].
abstract: BACKGROUND
  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.
  
  
  METHODS
  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.
  
  
  CONCLUSIONS
  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.
authors.affiliation: Service de médecine interne-rhumatologie, hôpital de la Croix-Saint-Simon, 125, rue d'Avron, 75020 Paris, France.
authors.forename: S-N
authors.initials: SN
authors.lastname: Tran Ba
authors.affiliation: Service de médecine interne-rhumatologie, hôpital de la Croix-Saint-Simon, 125, rue d'Avron, 75020 Paris, France; Centre de référence des maladies lysosomales, site Avron, 125, rue d'Avron, 75020 Paris, France. Electronic address: olidove@hopital-dcss.org.
authors.forename: O
authors.initials: O
authors.lastname: Lidove
authors.affiliation: Chirurgie cardiaque, hôpital Bichat-Claude-Bernard, Paris, France.
authors.forename: R
authors.initials: R
authors.lastname: Dorent
authors.affiliation: Chirurgie cardiaque, institut mutualiste Montsouris, Paris, France.
authors.forename: M
authors.initials: M
authors.lastname: Debauchez
authors.affiliation: Chirurgie cardiaque, hôpital Bichat-Claude-Bernard, Paris, France.
authors.forename: P
authors.initials: P
authors.lastname: Nataf
authors.affiliation: Néphrologie, hôpital Foch, Suresnes, France.
authors.forename: M
authors.initials: M
authors.lastname: Delahousse
authors.affiliation: Néphrologie, HEGP, 20, rue Leblanc, 75015 Paris, France.
authors.forename: A
authors.initials: A
authors.lastname: Karras
authors.affiliation: Néphrologie, hôpital Bichat-Claude-Bernard, Paris, France.
authors.forename: L
authors.initials: L
authors.lastname: Azeroual
authors.affiliation: Cardiologie, institut mutualiste Montsouris, Paris, France.
authors.forename: P
authors.initials: P
authors.lastname: De Lentdecker
authors.affiliation: Médecine interne, hôpital Bichat-Claude-Bernard, Paris, France.
authors.forename: M P
authors.initials: MP
authors.lastname: Chauveheid
authors.affiliation: Service de médecine interne-rhumatologie, hôpital de la Croix-Saint-Simon, 125, rue d'Avron, 75020 Paris, France.
authors.forename: T
authors.initials: T
authors.lastname: Sené
authors.affiliation: Service de médecine interne-rhumatologie, hôpital de la Croix-Saint-Simon, 125, rue d'Avron, 75020 Paris, France.
authors.forename: J-M
authors.initials: JM
authors.lastname: Ziza
mesh_terms: D000328:Adult; D000795:Fabry Disease / Q000150:complications / Q000628:therapy*; D005500:Follow-Up Studies; D016027:Heart Transplantation / Q000379:methods*; D006801:Humans; D007676:Kidney Failure, Chronic / Q000150:complications / Q000628:therapy*; D016030:Kidney Transplantation / Q000379:methods*; D008297:Male; D008875:Middle Aged; D035781:Siblings; D013997:Time Factors; D016896:Treatment Outcome
keywords: Fabry disease; Heart transplantation; Kidney transplantation; Maladie de Fabry; Transplantation cardiaque; Transplantation rénale