Improving the outcome of children with congenital athymia.

The thymus gland produces white blood cells, called T-cells, an essential part of the immune system. Infants born without a thymus (athymia) lack T-cells, are extremely vulnerable to infections and may die. Thymus tissue necessarily removed from otherwise healthy infants during surgery for heart defects can be grown in the laboratory and transplanted into athymic infants. Although the tissue type of these infants is not matched with the thymus they receive, transplanted patients can fight infections and survive. However, T-cell numbers rarely reach normal levels and sometimes these cells mistakenly attack the patient's own body causing autoimmune complications. This may be due to lack of tissue-type matching. Using new research techniques to investigate how non-matched thymuses can support T-cell development and the mechanisms underlying complications, I aim to gain fundamental insights into thymus functioning, paving the way for the development of alternative treatment approaches to improve patient outcomes.