An Overview on Severe Combined Immunodeficiency Disorders
Keywords:Severe Combined Immunodeficiency Disorders, Adenosine Deaminase Deficiency, Bone Marrow Transplant
Severe Combined Immunodeficiency Disorders (SCID) are rare life-threatening inherited conditions. They are characterised by the absence or non-functional immune system [1, 2]. As a result, affected children are unable to fight infections. Living in normal environment usually proves fatal for affected children. They get very sick with recurrent attacks of bacterial, viral and fungal infections and do not survive beyond first year of life. There are many genetic mutations responsible for different types of SCID. T-lymphocytes, B-lymphocytes and natural killer cells (NK-cells) can all be affected; two common types are the X-linked and the
Adenosine Deaminase Deficiency (ADA deficiency). Affected kids develop symptoms usually within the first few months of life. Commonly affected body systems are; lungs, meninges, blood stream, skin, gut and liver. In the absence of efficient immune system, causative organisms do not easily respond to anti-microbial agents. Recurrent episodes of serious and life-threatening infections like pneumonia, meningitis, septicaemia, chronic skin infections, diarrhoea, and hepatitis result in death within first year of life .