title: Anaesthesiologic protocol for kidney transplantation in two patients with Fabry Disease: a case series.
abstract: Fabry's Disease is a rare genetic syndrome, with a classic X-linked alpha -galactosidase A deficiency phenotype, responsible for glico-sphyngolypids metabolism impairment with clinical effects in several organs and functions. We describe the anaesthesiologic implications of two patients with Fabry disease who underwent a kidney transplantation from a deceased donor. We recommend careful preoperative evaluation, including cardiac sonography study and spirometry for Fabry disease patients, and according to our experience, we recommend advanced haemodynamic monitoring during surgery. Careful airway examination should be further performed, with particular attention to patient ventilability prediction and available alternative strategies for airway management in case of difficulties. A nephroprotective strategy and a particular care to the associated end-stage organ disease may significantly improve the long-term outcome of patients with Fabry Disease.
authors.affiliation: Department of Surgery, Transplantation and Advanced Technologies, Vascular Surgery and Organ Transplant Unit, University Hospital of Catania, Via Santa Sofia, 86 95123 Catania, Italy. veroux@unict.it.
authors.forename: Massimiliano
authors.initials: M
authors.lastname: Sorbello
authors.forename: Massimiliano
authors.initials: M
authors.lastname: Veroux
authors.forename: Melania
authors.initials: M
authors.lastname: Cutuli
authors.forename: Gianluigi
authors.initials: G
authors.lastname: Morello
authors.forename: Annalaura
authors.initials: A
authors.lastname: Paratore
authors.forename: Mirko Tindaro
authors.initials: MT
authors.lastname: Sidoti
authors.forename: Jessica Giuseppina
authors.initials: JG
authors.lastname: Maugeri
authors.forename: Massimiliano
authors.initials: M
authors.lastname: Gagliano
authors.forename: Giuseppe
authors.initials: G
authors.lastname: Giuffrida
authors.forename: Daniela
authors.initials: D
authors.lastname: Corona
authors.forename: Pierfrancesco
authors.initials: P
authors.lastname: Veroux