title: Safety of major abdominal surgical procedures in patients with hyperimmunoglobulinemia E (Job's syndrome): a changing paradigm?
abstract: BACKGROUND
  Autosomal dominant hyperimmunoglobulinemia E syndrome (HIES), also called Job's syndrome, is a primary immunodeficiency characterized by the triad of elevated immunoglobulin E levels, eczema, and infections. Its clinical course manifests as recurrent skin and pulmonary infections, and variable skeletal, connective tissue, and vascular abnormalities. There is evidence of abnormal tissue remodeling with pneumatocoeles frequently complicating pyogenic pneumonias and leading to secondary infections that cause the majority of morbidity and mortality. Complications are known to occur after lung surgery with a high frequency of bronchopleural fistulae, but little has been reported concerning abdominal surgeries.
  
  
  CONCLUSIONS
  Here, we report on the outcome and safety of two separate complex cases (hepatectomy and subtotal gastrectomy) and document our entire experience with abdominal surgical procedures performed on patients with HIES. Despite initial complications, all patients eventually made a full recovery.
  
  
  CONCLUSIONS
  As HIES patients now frequently live beyond the third and fourth decade, surgical issues similar to those in the general population may increase. Complex surgical procedures can be performed safely and benefit select patients with HIES, but benefit strongly from multidisciplinary teams and awareness of complications related to abnormal healing. We discuss current treatment and potential complications post-operatively in patients with HIES.
authors.affiliation: Surgery Branch, National Cancer Institute, National Institutes of Health, Bethesda, MD, USA. Russell.Langan@gmail.com
authors.forename: Russell C
authors.initials: RC
authors.lastname: Langan
authors.forename: Richard M
authors.initials: RM
authors.lastname: Sherry
authors.forename: Itzhak
authors.initials: I
authors.lastname: Avital
authors.forename: Theo
authors.initials: T
authors.lastname: Heller
authors.forename: Carolyn
authors.initials: C
authors.lastname: Henderson
authors.forename: Steven M
authors.initials: SM
authors.lastname: Holland
authors.forename: Alexandra F
authors.initials: AF
authors.lastname: Freeman
authors.forename: Udo
authors.initials: U
authors.lastname: Rudloff
mesh_terms: D000293:Adolescent; D000328:Adult; D002675:Child, Preschool; D005260:Female; D005743:Gastrectomy; D006498:Hepatectomy; D006801:Humans; D007223:Infant; D007589:Job Syndrome / Q000150:complications*; D008297:Male; D008875:Middle Aged; D061214:Patient Safety; D011183:Postoperative Complications / Q000453:epidemiology*; D014481:United States / Q000453:epidemiology