title: Lung parenchyma surgery in autosomal dominant hyper-IgE syndrome.
abstract: OBJECTIVE
  Autosomal dominant hyper-IgE syndrome (AD-HIES) due to heterozygous STAT3 mutation is a primary immunodeficiency characterized by eczema, elevated serum IgE, recurrent infections, and connective tissue and skeletal findings. Healing of pneumonias is often abnormal with formation of pneumatoceles and bronchiectasis. We aimed to explore whether healing after lung surgery is also aberrant.
  
  
  METHODS
  We retrospectively analyzed the medical records of 32 patients with AD-HIES who received lung surgery for the management of pulmonary infections from 1960 to 2011. We collected information including patient demographics, STAT3 mutation status, clinical history, surgical and medical procedures performed, complications, related medical treatments, and outcomes.
  
  
  RESULTS
  More than 50% of lung surgeries had associated complications, with the majority being prolonged bronchopleural fistulae. These fistulae often led to empyemas that necessitated additional interventions including prolonged antibiotics, prolonged thoracostomy tube drainage and re-operations.
  
  
  CONCLUSIONS
  Lung surgery in AD-HIES patients is associated with high complication rates. STAT3 mutations likely lead to abnormalities in tissue remodelling that are further exacerbated by infection.
authors.affiliation: Laboratory of Clinical Infectious Diseases, NIAID, NIH, NIH Building 10 Room 11 N234, 9000 Rockville Pike, Bethesda, MD 20892, USA. freemaal@mail.nih.gov
authors.forename: Alexandra F
authors.initials: AF
authors.lastname: Freeman
authors.forename: Ellen D
authors.initials: ED
authors.lastname: Renner
authors.forename: Carolyn
authors.initials: C
authors.lastname: Henderson
authors.forename: Anne
authors.initials: A
authors.lastname: Langenbeck
authors.forename: Kenneth N
authors.initials: KN
authors.lastname: Olivier
authors.forename: Amy P
authors.initials: AP
authors.lastname: Hsu
authors.forename: Beate
authors.initials: B
authors.lastname: Hagl
authors.forename: Annette
authors.initials: A
authors.lastname: Boos
authors.forename: Joie
authors.initials: J
authors.lastname: Davis
authors.forename: Beatriz E
authors.initials: BE
authors.lastname: Marciano
authors.forename: Lisa
authors.initials: L
authors.lastname: Boris
authors.forename: Pamela
authors.initials: P
authors.lastname: Welch
authors.forename: Julie
authors.initials: J
authors.lastname: Sawalle-Belohradsky
authors.forename: Bernd H
authors.initials: BH
authors.lastname: Belohradsky
authors.forename: King F
authors.initials: KF
authors.lastname: Kwong
authors.forename: Steven M
authors.initials: SM
authors.lastname: Holland
mesh_terms: D000293:Adolescent; D000328:Adult; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D007589:Job Syndrome / Q000276:immunology* / Q000503:physiopathology*; D008168:Lung / Q000276:immunology / Q000503:physiopathology / Q000601:surgery; D008171:Lung Diseases / Q000235:genetics / Q000276:immunology / Q000503:physiopathology* / Q000601:surgery*; D008297:Male; D008875:Middle Aged; D009154:Mutation; D012189:Retrospective Studies; D050796:STAT3 Transcription Factor / Q000235:genetics; D014945:Wound Healing / Q000235:genetics / Q000502:physiology*; D055815:Young Adult
chemical_list: D050796:STAT3 Transcription Factor; C494086:STAT3 protein, human