Thoracic Surgery in Chronic Granulomatous Disease: a 25-Year Single-Institution Experience

• Published in: Journal of clinical immunology 2016-10, Vol.36 (7), p.677-683
• Main Authors: Feingold, Paul L., Quadri, Humair S., Steinberg, Seth M., Malech, Harry L., Gallin, John I., Zerbe, Christa S., Zarember, Kol A., Marciano, Beatrice E., Holland, Steven M., Schrump, David S., Ripley, Robert T.
• Format: Article
• Language: English
• Subjects: Biomarkers; Biomedical and Life Sciences; Biomedicine; Child; Child, Preschool; Comorbidity; Disease Management; Female; Granulomatous Disease, Chronic - complications; Granulomatous Disease, Chronic - diagnosis; Granulomatous Disease, Chronic - mortality; Granulomatous Disease, Chronic - surgery; Humans; Immunology; Infant; Infectious Diseases; Internal Medicine; Male; Medical Microbiology; Mutation; NADPH Oxidase 2 - genetics; Original Article; Retrospective Studies; Thoracic Surgical Procedures - methods; Treatment Outcome
• ISSN: 0271-9142; 1573-2592
• DOI: 10.1007/s10875-016-0319-9

Introduction 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. Methods 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. Results 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   500 mL were negative prognostic factors ( p