Comprehensive Report of Primary Immunodeficiency Disorders from a Tertiary Care Center in India
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.
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.
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 %).
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.
KeywordsPrimary immunodeficiency disorders SCID antibody deficiency phagocytic defects diseases of immune dysregulation
Authors want to acknowledge the constant guidance by Dr Bobby Gaspar, Institute of Child Health, London and Dr Kimberly Gilmour, Great Ormond Street Hospital in establishing facility for diagnosis and management of PID at our institute. Authors also want to acknowledge the inspiration and constant guidance provided by Dr Zenet Currimbhoy, Bai Jerbai Wadia Hospital for Children, Mumbai
MM and KG conceptualized the paper. AM, SM and MG helped in laboratory assessment of the patients. MM and AM prepared the manuscript. MD helped in clinical diagnosis and management of the patients. Finally the manuscript has been approved by all.
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