Abstract
Anesthesia management of an infant with artemis severe combined immune deficiency
Highlights
Artemis is a nuclear protein that plays an essential role in DNA repair and in V, D and J recombination: a process by which B cell antibody genes and T cell receptor genes are assembled
Artemis was first identified as the gene defective in a subset of severe combined immune deficiency (SCID) patients that were unusually sensitive to radiation [1,2,3,4]
Sedation was requested for a full term 5-month-old infant, 6.3 kg, as a late afternoon add on case for neck and chest magnetic resonance imaging (MRI)
Summary
Artemis is a nuclear protein that plays an essential role in DNA repair and in V (variable), D (diversity) and J (joining) recombination: a process by which B cell antibody genes and T cell receptor genes are assembled. In Artemis-deficient individuals, no mature B or T cells are produced, a condition known as severe combined immune deficiency (SCID). Artemis was first identified as the gene defective in a subset of SCID patients that were unusually sensitive to radiation [1,2,3,4].
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