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The loss of VSMCs in the middle layer of aorta, including phenotypic switching and apoptosis, could represent a critical initial stage in AD formation (76)

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Ruxolitinib or tofacitinib have been used successfully to target the STAT hyperresponsiveness in patients with STAT1 and STAT3 GOF ( Key concepts in bridging therapies: Biologic modifiers are available to treat specific diseases Outcome of long-term therapy needs to be studied Optimising patient status prior to HSCT with such agents is useful to improve outcome Conditioning Historically, conditioning therapy prior to HSCT for patients with IEI was based on the alkylating agents busulfan and cyclophosphamide