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Nude severe combined immunodeficiency is a rare inherited disease caused by autosomal recessive loss-of-function mutations in FOXN1. This gene encodes a transcription factor essential for the development of the thymus, the primary lymphoid organ that supports T-cell development and selection. To date nine cases have been reported presenting with the clinical triad of absent thymus resulting in severe T-cell immunodeficiency, congenital alopecia universalis and nail dystrophy. Diagnosis relies on testing for FOXN1 mutations, which allows genetic counselling and guides therapeutic management. Options for treating the underlying immune deficiency include HLA-matched genoidentical haematopoietic cell transplantation containing mature donor T-cells or thymus tissue transplantation.
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INTROIT All things are in thy will, O Lord: and there is none that can resist thy will: for thou hast made all things, heaven and earth, and all things that are under the cope of heaven: thou art Lord of all. Blessed are the undefiled in the way: who walk in the law of the Lord. Glory etc. Brethern, Be strengthened in the Lord, and in the might of his power. Put you on the armor of God, that you may be able to stand against the deceits of the devil: for our wrestling is not against flesh and blood, but against principalities and powers, against the rulers of the world of this darkness, against the spirits of wickedness in high places.
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Frenchman33 16.10.2019
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