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Original article
High IL-18 (interferon-γ inducing factor) concentration in a purine nucleoside phosphorylase deficient patient
  1. Tetsuya Yamamotoa,
  2. Yuji Moriwakia,
  3. Kiyoshi Matsuia,
  4. Sumio Takahashia,
  5. Hiroko Tsutsuib,
  6. Tomohiro Yoshimotob,
  7. Haruki Okamurac,
  8. Kenji Nakanishib,
  9. Yoshihiro Kurosawad,
  10. Shinya Yamaguchie,
  11. Yuko Sasakif,
  12. Kazuya Higashinoa
  1. aThird Department of Internal Medicine, Hyogo College of Medicine, Mukogawa-cho 1–1, Nishinomiya, Hyogo 663–8501, Japan, bDepartment of Immunology and Medical Zoology, Hyogo College of Medicine, cLaboratory of Host Defenses, Institute for Advanced Medical Sciences, Hyogo College of Medicine, dDepartment of Pediatrics, Keio University School of Medicine, Tokyo, Japan, eDepartment of Pediatrics, The Kitasato Institute Hospital, Tokyo, Japan, fDepartment of Safety Research on Biologics, National Institute of Infectious Disease, Tokyo, Japan
  1. Dr Yamamoto.

Abstract

The plasma concentration of IL-18 (interferon-γ inducing factor) was severely increased in a 3 year old boy with purine nucleoside phosphorylase (PNP) deficiency. The presence and activity of IL-18 were confirmed by immunoblotting and bioassay, respectively. These results suggest that IL-18 may be abundantly produced and secreted into plasma by PNP deficient macrophages in PNP deficiency.

  • purine nucleoside phosphorylase deficiency
  • interferon-γ inducing factor
  • cytokines

https://doi.org/10.1136/adc.81.2.179

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