Abstract

The genus Helicobacter is classified into two main groups according to its habitat: gastric and enterohepatic. Patients with X-linked agammaglobulinemia (XLA) appear to be associated with invasive infection with enterohepatic non-Helicobacter pylori species (NHPH), mainly H. cinaedi and H. bilis. Such infections are difficult to control and have a high potential for recurrence. The spectrum of illnesses caused by these species includes recurrent fever, bacteremia, arthritis, osteomyelitis, cellulitis, abdominal abscesses, and pyoderma gangrenosum-like ulcer. The presence of these Helicobacters is particularly difficult to diagnose and eradicate, as they are very fastidious bacteria and present resistance to several types of antibiotics. We report two clinical cases of XLA patients infected with H. bilis. These infections were chronic in these patients and could not be eradicated in one of them. We also review the cases of enterohepatic non-Helicobacter pylori species (NHPH) in patients with this inborn error of immunity.

Highlights

  • The genus Helicobacter includes species classified as gastric and enterohepatic

  • It is important to describe that the skin biopsies taken were taken to perform the molecular identification of non-H. pylori species

  • An 18-year-old male diagnosed with X-linked agammaglobulinemia in 2008 (Lopez-Herrera et al, 2008), since he was on regular replacement therapy with IVIG

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Summary

Introduction

The genus Helicobacter includes species classified as gastric and enterohepatic. This genus belongs to a group of microaerophilic, gram-negative spiral-shaped bacteria. H. pylori is the most important, and its natural host is human; other Helicobacter species, both gastric and enterohepatic, have some animals as their natural host but can infect humans. Several publications have shown the importance of both human and animal infection. NHPH Infections in XLA with the so-called non-Helicobacter pylori Helicobacter species (NHPH). Most clinically relevant enterohepatic NHPH are H. cinaedi and H. bilis, both of which colonize the intestine, biliary tree and liver of animals and humans. Sporadic cases of H. fennelliae, H. canis, H. equorum, H. canadiensis and H. pollurum have been reported (Fox et al, 2000; Flahou et al, 2013; Smet & Menard, 2020)

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