title: Chronic Granulomatous Disease: A Large, Single-center US Experience.
abstract: BACKGROUND
  Chronic granulomatous disease (CGD) is an uncommon primary immunodeficiency that can be inherited in an X-linked (XL) or an autosomal recessive (AR) manner. We reviewed our large, single-center US experience with CGD.
  
  
  METHODS
  We reviewed 27 patients at Ann & Robert H. Lurie Children's Hospital of Chicago from March 1985 to November 2013. Fisher exact test was used to compare differences in categorical variables, and Student t test was used to compare means for continuous variables. Serious infections were defined as those requiring intravenous antibiotics or hospitalization.
  
  
  RESULTS
  There were 23 males and 4 females; 19 were XL and 8 were AR. The average age at diagnosis was 3.0 years; 2.1 years for XL and 5.3 years for AR inheritance (P = 0.02). There were 128 serious infections. The most frequent infectious agents were Staphylococcus aureus (n = 13), Serratia (n = 11), Klebsiella (n = 7), Aspergillus (n = 6) and Burkholderia (n = 4). The most common serious infections were pneumonia (n = 38), abscess (n = 32) and lymphadenitis (n = 29). Thirteen patients had granulomatous complications. Five patients were below the 5th percentile for height and 4 were below the 5th percentile for weight. Average length of follow-up after diagnosis was 10.1 years. Twenty-four patients were compliant and maintained on interferon-γ, trimethoprim-sulfamethoxazole and an azole. The serious infection rate was 0.62 per patient-year. Twenty-three patients are alive (1 was lost to follow-up).
  
  
  CONCLUSIONS
  We present a large, single-center US experience with CGD. Twenty-three of 27 patients are alive after 3276 patient-months of follow-up (1 has been lost to follow-up), and our serious infection rate was 0.62 per patient-year.
authors.affiliation: From the *Department of Pediatrics, Northwestern University Feinberg School of Medicine; †Ann & Robert H. Lurie Children's Hospital of Chicago; and ‡Department of Pathology, Northwestern University Feinberg School of Medicine, Chicago, Illinois.
authors.forename: Pietro
authors.initials: P
authors.lastname: Bortoletto
authors.forename: Kyle
authors.initials: K
authors.lastname: Lyman
authors.forename: Andres
authors.initials: A
authors.lastname: Camacho
authors.forename: Marielle
authors.initials: M
authors.lastname: Fricchione
authors.forename: Aaruni
authors.initials: A
authors.lastname: Khanolkar
authors.forename: Ben Z
authors.initials: BZ
authors.lastname: Katz
mesh_terms: D000293:Adolescent; D000328:Adult; D002648:Child; D002675:Child, Preschool; D005260:Female; D006105:Granulomatous Disease, Chronic / Q000175:diagnosis / Q000235:genetics / Q000401:mortality / Q000628:therapy; D006801:Humans; D007223:Infant; D053208:Kaplan-Meier Estimate; D008297:Male; D012189:Retrospective Studies; D055815:Young Adult