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.
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.
Authors: Cristina Woellner; E Michael Gertz; Alejandro A Schäffer; Macarena Lagos; Mario Perro; Erik-Oliver Glocker; Maria C Pietrogrande; Fausto Cossu; José L Franco; Nuria Matamoros; Barbara Pietrucha; Edyta Heropolitańska-Pliszka; Mehdi Yeganeh; Mostafa Moin; Teresa Español; Stephan Ehl; Andrew R Gennery; Mario Abinun; Anna Breborowicz; Tim Niehues; Sara Sebnem Kilic; Anne Junker; Stuart E Turvey; Alessandro Plebani; Berta Sánchez; Ben-Zion Garty; Claudio Pignata; Caterina Cancrini; Jiri Litzman; Ozden Sanal; Ulrich Baumann; Rosa Bacchetta; Amy P Hsu; Joie N Davis; Lennart Hammarström; E Graham Davies; Efrem Eren; Peter D Arkwright; Jukka S Moilanen; Dorothee Viemann; Sujoy Khan; László Maródi; Andrew J Cant; Alexandra F Freeman; Jennifer M Puck; Steven M Holland; Bodo Grimbacher Journal: J Allergy Clin Immunol Date: 2010-02 Impact factor: 10.793
Authors: Waleed Al-Herz; Aziz Bousfiha; Jean-Laurent Casanova; Helen Chapel; Mary Ellen Conley; Charlotte Cunningham-Rundles; Amos Etzioni; Alain Fischer; Jose Luis Franco; Raif S Geha; Lennart Hammarström; Shigeaki Nonoyama; Luigi Daniele Notarangelo; Hans Dieter Ochs; Jennifer M Puck; Chaim M Roifman; Reinhard Seger; Mimi L K Tang Journal: Front Immunol Date: 2011-11-08 Impact factor: 7.561