XXXX : A Large, Single-center US Experience.

@entity1 @entity799 @entity55 @entity1112 @entity794 @entity532 @entity281 @entity465 @entity733 @entity74 @entity4005 @entity1117 @entity3298 @entity5638
BACKGROUND: @entity1117 ( @entity1117 ) is an uncommon @entity465 that can be inherited in an @entity1112 ( @entity1112 ) or an @entity74 ( @entity74 ) manner. We reviewed our large, single-center US experience with @entity1117 . METHODS: We reviewed 27 @entity1 at Ann _ Robert H. Lurie @entity1 '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. @entity281 were defined as those requiring intravenous antibiotics or hospitalization. RESULTS: There were 23 males and 4 females; 19 were @entity1112 and 8 were @entity74 . The average age at diagnosis was 3.0 years; 2.1 years for @entity1112 and 5.3 years for @entity74 inheritance (P = 0.02). There were 128 serious @entity281 . The most frequent infectious agents were @entity794 (n = 13), Serratia (n = 11), @entity4005 (n = 7), Aspergillus (n = 6) and Burkholderia (n = 4). The most common serious @entity281 were @entity55 (n = 38), @entity733 (n = 32) and @entity3298 (n = 29). Thirteen @entity1 had @entity532 . Five @entity1 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 @entity1 were compliant and maintained on interferon-y, @entity799 and an @entity5638 . The serious infection rate was 0.62 per @entity1 -year. Twenty-three @entity1 are alive (1 was lost to follow-up). CONCLUSIONS: We present a large, single-center US experience with @entity1117 . Twenty-three of 27 @entity1 are alive after 3276 @entity1 -months of follow-up (1 has been lost to follow-up), and our serious infection rate was 0.62 per @entity1 -year.
