About the book
Primary immunodeficiencies (PID) are the genetic anomalies/disorders which are characterized by the jeopardized immune system in organisms. Primary immune deficiencies are genetic in origin and may result from a single genetic defect. These may be caused by recessive or dominant inheritance. Some are latent and require a certain environmental trigger to become manifest, like the presence in the environment of a reactive allergen. Other problems become apparent due to the aging of bodily and cellular maintenance processes. Most of the primary immune deficiencies are genetically ablated and diagnosed in children who are in their initial span of age. So far more than several hundred PIDs have been identified until recently which are associated with one of other genetic/somatic factors. Interestingly, the majority of them are very rare. According to WHO data about 1 in 1000 children is born with primary immunodeficiency.
As mentioned earlier, genetically ablated or functionally poor immune profile of the immune-deficient person/patient predisposes them highly susceptible to recurring infections, auto-immunity, tumors and malfunctioning of organs. Although most of PID's are having no stable treatment, due to advancement in the biotechnological approach, new treatment modalities are emerging which has shown promise for the clinical management of these inevitable disorders. Because of this, there is a dire need for newer research modality like the use of stem cell (HSCT) and/or gene therapies which are paramount for the modification of null immune system in PID patients.
One of the common manifestations of primary immunodeficiency includes recurrent or persistent infections or delay in the resolution of the infection which might inflict PID. Among various organs which are affected by PID is the skin, heart, facial and skeletal system.
Given the above, the main purpose of this book is to discuss various immune genetic and molecular aspects of PID. The emphasis of this book is to discuss various interventional modalities for the management of PID and cover the translational spectrum of disease management.