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Fig. 1 | Pediatric Rheumatology

Fig. 1

From: An unusual presentation of purine nucleoside phosphorylase deficiency mimicking systemic juvenile idiopathic arthritis complicated by macrophage activation syndrome

Fig. 1

Immunological functional tests of the patient. Intracellular expression of perforin in CD56+ NK cells was assessed by flow cytometry (a). Degranulation potential of CD56+ NK cells was assessed by flow cytometry after overnight co-culture with target cells K562. Plots show the surface expression of CD107a on unstimulated and stimulated NK cells (b). Lymphocytes subsets were analysed by flow cytometry: T cells were identified as CD3+ CD56 CD19; within the CD3+ gate, γδ-T cells were gated as CD3+ γδ-TCR+ αβ-TCR (c). T cell proliferation was assessed by a standard [3H]-thymidine incorporation assay following stimulation with anti-CD3 (OKT3) or PHA (d). The results are shown for first determination while on 2 mg/Kg of methylprednisolone. They were repeated on a lower dose of oral prednisone (0.25 mg/Kg) with similar results

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