title: Thoracic Surgery in Chronic Granulomatous Disease: a 25-Year Single-Institution Experience.
abstract: Chronic granulomatous disease (CGD) is a genetic disorder in which phagocyte dysfunction leads to recurrent infection. Persistent pulmonary infections sometimes require thoracic surgical intervention. We reviewed our 25-year experience to identify outcomes and prognostic factors associated with thoracic surgery in these patients.
  
  
  
  A retrospective single-institution review of all patients with CGD from 1990 through 2015 was performed. Univariate analysis identified prognostic variables to include in a Cox model. Overall survival was estimated by the Kaplan-Meier method.
  
  
  
  We identified 258 patients who had 2221 admissions (both scheduled and emergent). During the period examined, 51 thoracic operations were performed in 13.6 % (35/258) of patients and 2.3 % (35/2221) of overall admissions. Patients undergoing surgery did not have statistically significant differences in disease genotype compared to those that did not require surgery. Pathogens were identified from 67 % (34/51) of specimens. Complications occurred in 27 % (14/51), including 10 % (5/51) with wound and 12 % (6/51) with pulmonary infections. Mortality at 30 and 90 days was 0 and 6 % (3/51), respectively. Overall survival probabilities were 75 and 62 % at 5- and 10-year follow-up (median potential follow-up: 16.5 years), respectively. Undergoing thoracic surgery was associated with an increased hazard ratio for death of 3.71 (p < 0.0001). Both chest wall resection and EBL > 500 mL were negative prognostic factors (p < 0.05).
  
  
  
  A minority of CGD patients required thoracic surgery for infections refractory to antibiotic or antifungal therapy. Patients who had these operations had significant morbidity and relatively poor long-term survival, particularly in the cases of chest wall resection or significant blood loss.
authors: Thoracic and Gastrointestinal Oncology Branch, National Cancer Institute, National Institutes of Health, Bethesda, MD, USA. paul.feingold@nih.gov.
authors: Paul L
authors: PL
authors: Feingold
authors: Thoracic and Gastrointestinal Oncology Branch, National Cancer Institute, National Institutes of Health, Bethesda, MD, USA.
authors: Humair S
authors: HS
authors: Quadri
authors: Biostatistics and Data Management Section, Office of the Clinical Director, Center for Cancer Research, National Cancer Institute, National Institutes of Health, Bethesda, MD, USA.
authors: Seth M
authors: SM
authors: Steinberg
authors: Laboratory of Host Defenses, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, MD, USA.
authors: Harry L
authors: HL
authors: Malech
authors: Laboratory of Host Defenses, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, MD, USA.
authors: John I
authors: JI
authors: Gallin
authors: Laboratory of Clinical Infectious Diseases, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, MD, USA.
authors: Christa S
authors: CS
authors: Zerbe
authors: Laboratory of Clinical Infectious Diseases, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, MD, USA.
authors: Kol A
authors: KA
authors: Zarember
authors: Laboratory of Clinical Infectious Diseases, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, MD, USA.
authors: Beatrice E
authors: BE
authors: Marciano
authors: Laboratory of Clinical Infectious Diseases, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, MD, USA.
authors: Steven M
authors: SM
authors: Holland
authors: Thoracic and Gastrointestinal Oncology Branch, National Cancer Institute, National Institutes of Health, Bethesda, MD, USA.
authors: David S
authors: DS
authors: Schrump
authors: Thoracic and Gastrointestinal Oncology Branch, National Cancer Institute, National Institutes of Health, Bethesda, MD, USA.
authors: Robert T
authors: RT
authors: Ripley
mesh_terms: D015415:Biomarkers; D002648:Child; D002675:Child, Preschool; D015897:Comorbidity; D019468:Disease Management; D005260:Female; D006105:Granulomatous Disease, Chronic / Q000150:complications / Q000175:diagnosis / Q000401:mortality / Q000601:surgery*; D006801:Humans; D007223:Infant; D008297:Male; D009154:Mutation; D000074662:NADPH Oxidase 2 / Q000235:genetics; D012189:Retrospective Studies; D019616:Thoracic Surgical Procedures / Q000379:methods; D016896:Treatment Outcome
chemical_list: D015415:Biomarkers; C056558:CYBB protein, human; D000074662:NADPH Oxidase 2
keywords: Empyema; chronic granulomatous disease; lung abscess; pulmonary resection