Chronic Granulomatous Disease: A Large, Single-Center U.S. Experience.
Pediatr Infect Dis J. 2015 Jul 15;
Authors: Bortoletto P, Lyman K, Camacho A, Fricchione M, Khanolkar A, Katz BZ
Abstract
BACKGROUND: 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 U. S. experience with CGD.
MATERIAL AND METHODS: We reviewed 27 patients seen from March 1985 to November 2013. Fisher’s exact test was used to compare differences in categorical variables, and Student’s 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 (13), Serratia (11), Klebsiella (7), Aspergillus (6) and Burkholderia (4). The most common serious infections were pneumonia (38), abscess (32) and lymphadenitis (29). Thirteen patients had granulomatous complications. Five patients were below the 5 percentile for height and 4 were below the 5 percentile for weight.Average length of follow-up after diagnosis was 10.1 years. Twenty-four patients were compliant and maintained on interferon gamma, trimethoprim-sulfa and an azole. The serious infection rate was 0.62 per patient-year. Twenty-three patients are alive (one was lost to follow-up).
CONCLUSIONS: We present a large U.S. single center 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.
PMID: 26181896 [PubMed – as supplied by publisher]
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