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

• Главные авторы: Alessia Arduini (Автор), Emiliano Marasco (Автор), Giulia Marucci (Автор), Manuela Pardeo (Автор), Antonella Insalaco (Автор), Ivan Caiello (Автор), Gian Marco Moneta (Автор), Giusi Prencipe (Автор), Fabrizio De Benedetti (Автор), Claudia Bracaglia (Автор)
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• Опубликовано: BMC, 2019-05-01T00:00:00Z.
• Предметы: article
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Abstract Background Systemic juvenile idiopathic arthritis (sJIA) is an inflammatory condition that presents with fever, rash and arthritis. At onset systemic features are predominant and the diagnosis may be a challenge. Secondary hemophagocytic lymphohistiocytosis (sHLH) forms may be associated wi...