Comprehensive report of primary immunodeficiency disorders from a tertiary care center in India

M Madkaikar, A Mishra, M Desai, M Gupta… - Journal of clinical …, 2013 - Springer
M Madkaikar, A Mishra, M Desai, M Gupta, S Mhatre, K Ghosh
Journal of clinical immunology, 2013Springer
Objectives There is paucity of data on Primary immunodeficiency disorders (PID) from India.
Here we describe the frequency of different primary immunodeficiency disorders, their
clinical features and disease complications of 159 patients with PID diagnosed in a tertiary
care center from India over the last 3 years. Methods We retrospectively reviewed the
records of all the patients identified to have specific PID from 2008 to 2011. The diagnosed
patients were classified according to guidelines of International Union of Immunological …
Objectives
There is paucity of data on Primary immunodeficiency disorders (PID) from India. Here we describe the frequency of different primary immunodeficiency disorders, their clinical features and disease complications of 159 patients with PID diagnosed in a tertiary care center from India over the last 3 years.
Methods
We retrospectively reviewed the records of all the patients identified to have specific PID from 2008 to 2011. The diagnosed patients were classified according to guidelines of International Union of Immunological Society (IUIS) into eight different sub groups.
Results
The distribution pattern was as follows: diseases of immune dysregulation (29 %), phagocytic defects (29 %), predominant antibody deficiency (13 %), combined T and B cell deficiency (19 %) and other well defined diseases (10 %).
Conclusion
The distribution pattern of PID varied significantly from those reported by western studies. This study highlights the need for development of more advanced facilities for diagnosis and management of PID in India and also the need for establishing population and hospital based registries.
Springer
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