Anesthesia in Mowat-Wilson syndrome: information on 11 Italian patients.

ABSTRACT: Mowat-Wilson syndrome is a genetic disease caused by heterozygous mutations or deletions of the ZEB2 gene and characterized by typical clinical features. The congenital malformations typical of this syndrome call for early diagnostic and surgical procedures requiring general anesthesia, but few information about the anesthesiology management of such patients is available. We enrolled 11 families of patients with Mowat-Wilson syndrome who had undergone surgical or diagnostic procedures requiring general anesthesia, and sent them a retrospective questionnaire including 16 open questions about the procedures. They were further contacted by phone for a semistructured interview. A total of 37 procedures requiring general anesthesia was reported in 11 patients. Only two patients reported anesthe

SUBMITTER: Spunton M 

PROVIDER: S-EPMC5907729 | biostudies-literature | 2018 Mar

REPOSITORIES: biostudies-literature