Conditions such as Marfan syndrome, Ehler-Danlos syndrome, ataxia telangiectasia, Bruton type agammaglobulinaemia, ankylosing spondylitis, cutis laxa are also associated with secondary tracheobronchial enlargement [5-8].
Comprehensive immunohaematological analysis is therefore used to screen paediatric patients for underlying immune suppression associated with primary immunodeficiency such as X-linked agammaglobulinaemia and severe combined immunodeficiency.
Saraste, "Stucture of the PH domain and Btk motif from Bruton's tyrosine kinase: molecular explanations for X-linked agammaglobulinaemia," The EMBO Journal, vol.
Absence of B cells with normal T cells and NK cells is in keeping with agammaglobulinaemia, while reduced T cells are found in severe combined immunodeficiency syndrome (SCID).