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Purpose X-linked agammaglobulinemia is a primary humoral immunodeficiency characterized by hypogammaglobulinemia and increased susceptibility to infection. Although there is increased awareness of autoimmune and inflammatory complications in XLA, the spectrum of gastrointestinal manifestations has not previously been fully explored. Methods We present a case report of a family with two affected patients with XLA. Given the gastrointestinal involvement of the grandfather in this family, we performed a retrospective descriptive analysis of XLA patients with reported diagnoses of GI manifestations and inflammatory bowel disease (IBD) or enteritis registered at the USIDNet, a national registry of primary immunodeficiencies. Results In this cohort of patients with XLA, we found that up to 35% had concurrent gastrointestinal manifestations, and 10% had reported diagnoses of IBD or enteritis. The most commonly reported mutations were missense, which have been associated with a less severe XLA phenotype in the literature. The severity of symptoms were wide-ranging, and management strategies were diverse and mainly experimental. Conclusions Patients with XLA may require close monitoring with particular attention for GI manifestations including IBD and infectious enteritis. Further studies are needed to improve diagnosis and management of GI conditions in XLA patients.

RATIONALE: Vancomycin is a broad-spectrum antibiotic whose use is limited by adverse drug reactions (ADRs). While vancomycin toxicities are known, there are limited data on vancomycin hypersensitivity reactions (HSRs). We aimed to identify the most commonly reported vancomycin HSRs through systematic review. METHODS: We performed a literature search for English-language case reports and series from 1982 through 2015 (last search July 31) on Ovid MEDLINE and PubMed. Search included subject heading vancomycin with subheading ''adverse effects,'' and separately text word searches for vancomycin with a list of specified HSRs. References of identified articles were reviewed to find additional articles. Each assessed case was reviewed by two investigators. Clinical data were collected and summarized. RESULTS: Of 200 identified articles, 86 were screened, 65 were fully assessed, and 54 cases were included in the analysis. Vancomycin HSRs were immediate (anaphylaxis, n57) and non-immediate (n547). Nonimmediate HSRs included linear IgA bullous dermatosis (LABD, n523), drug rash eosinophilia and systemic symptoms (DRESS) syndrome (n515), acute interstitial nephritis (AIN, n56), and Stevens-Johnson syndrome/toxic epidermal necrolysis (SJS/TEN, n53). Median days of vancomycin therapy prior to HSR onset was 7 [3.5, 10] for LABD, 21 [18,28] for DRESS, 26 [11,28] for AIN, and 9 [9.13] for SJS/TEN. Overall case fatality was 8/54 (14.8%), with no difference between immediate and non-immediate HSRs (p51). CONCLUSIONS: Vancomycin causes a variety of HSRs; cases were most commonly non-immediate with LABD most frequently reported. We observed a high frequency of HSR mortality. Further data are needed to identify the frequency and severity of vancomycin HSRs.

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