Lancet
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X-linked severe combined immunodeficiency (SCID-X1) is caused by mutations in the common cytokine-receptor gamma chain (gamma(c)), resulting in disruption of development of T lymphocytes and natural-killer cells. B-lymphocyte function is also intrinsically compromised. Allogeneic bone-marrow transplantation is successful if HLA-matched family donors are available, but HLA-mismatched procedures are associated with substantial morbidity and mortality. We investigated the application of somatic gene therapy by use of a gibbon-ape-leukaemia-virus pseudotyped gammaretroviral vector. ⋯ Gene therapy for SCID-X1 is a highly effective strategy for restoration of functional cellular and humoral immunity.
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Neuromyelitis optica is an inflammatory demyelinating disease with generally poor prognosis that selectively targets optic nerves and spinal cord. It is commonly misdiagnosed as multiple sclerosis. Neither disease has a distinguishing biomarker, but optimum treatments differ. The relation of neuromyelitis optica to optic-spinal multiple sclerosis in Asia is uncertain. We assessed the capacity of a putative marker for neuromyelitis optica (NMO-IgG) to distinguish neuromyelitis optica and related disorders from multiple sclerosis. ⋯ NMO-IgG is a specific marker autoantibody of neuromyelitis optica and binds at or near the blood-brain barrier. It distinguishes neuromyelitis optica from multiple sclerosis. Asian optic-spinal multiple sclerosis seems to be the same as neuromyelitis optica.
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This article presents an introduction to the causes and characteristics of armed conflicts. It reviews some of the key humanitarian crises that broke new ground in terms of the technologies and practices that developed at the field level in response to each new complex emergency, with particular focus on the health sector. ⋯ Here, we term humanitarian governance to include the use of international humanitarian law and human rights instruments to govern the behaviour of state and non-state organisations in conflict zones in a way that protects the lives and livelihoods of affected populations. We note, however, that terrorist concerns appear to be replacing humanitarian logic in the network of worldwide governance.