Helicobacter cinaedi -Associated Refractory Cellulitis in Patients with X-Linked Agammaglobulinemia
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ORIGINAL ARTICLE
Helicobacter cinaedi-Associated Refractory Cellulitis in Patients with X-Linked Agammaglobulinemia Kento Inoue 1 & Saeko Sasaki 2 & Takahiro Yasumi 3 & Kohsuke Imai 4 & Takashi Kusunoki 2 & Tomohiro Morio 1 & Hirokazu Kanegane 5 Received: 24 March 2020 / Accepted: 20 July 2020 # Springer Science+Business Media, LLC, part of Springer Nature 2020
Abstract X-linked agammaglobulinemia (XLA) is characterized by severe or recurrent infections, hypogammaglobulinemia, and circulating B cell deficiency. The frequent pathogens seen in patients with XLA include Streptococcus pneumoniae, Haemophilus influenzae, Pseudomonas aeruginosa, and enterovirus as well as Campylobacter and Helicobacter species. Here, we describe two patients with XLA who developed cellulitis and bacteremia caused by Helicobacter cinaedi even when administered an appropriate immunoglobulin replacement therapy. H. cinaedi may be difficult to isolate using a conventional blood culture system and could be identified by sequence analysis and mass spectrometry. H. cinaedi infection causes recurrent symptoms frequently, and patients require a long course of antibiotic treatment. Recently, the case of non-H. pylori Helicobacter (NHPH) infection such as H. cinaedi and H. bilis infection is increasing in number in patients with XLA. Systemic NHPH infection should be suspected, and extensive microbiological analysis should be performed to appropriately treat patients with XLA who present with fever and skin lesions. Keywords Bacteremia . cellulitis . Helicobacter cinaedi . X-linked agammaglobulinemia
Abbreviations GNR Gram-negative rod HIV Human immunodeficiency virus Kento Inoue and Saeko Sasaki contributed equally to this work. Electronic supplementary material The online version of this article (https://doi.org/10.1007/s10875-020-00830-6) contains supplementary material, which is available to authorized users. * Hirokazu Kanegane [email protected] 1
Department of Pediatrics and Developmental Biology, Graduate School of Medical and Dental Sciences, Tokyo Medical and Dental University (TMDU), Tokyo, Japan
2
Department of Pediatrics, Shiga Medical Center for Children, Moriyama, Shiga, Japan
3
Department of Pediatrics, Kyoto University Graduate School of Medicine, Kyoto, Japan
4
Department of Community Pediatrics, Perinatal and Maternal Medicine, Graduate School of Medical and Dental Sciences, Tokyo Medical and Dental University (TMDU), Tokyo, Japan
5
Department of Child Health and Development, Graduate School of Medical and Dental Sciences, Tokyo Medical and Dental University (TMDU), 1-5-45 Yushima, Bunkyo-ku, Tokyo 113-8519, Japan
IVIg MS NHPH PBMC PCR SCIg XLA
Intravenous immunoglobulin Mass spectrometry Non-Helicobacter pylori Helicobacter Peripheral blood mononuclear cell Polymerase chain reaction Subcutaneous immunoglobulin X-linked agammaglobulinemia
Introduction X-linked agammaglobulinemia (XLA) was originally described by Bruton in 1952 [1]. He reported about an 8-year-old boy with recurrent bacterial infections and hyp
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