Report of the Tunisian Registry of Primary Immunodeficiencies: 25-Years of Experience (1988-2012) - RIIP - Réseau International des Instituts Pasteur Access content directly
Journal Articles Journal of Clinical Immunology Year : 2015

Report of the Tunisian Registry of Primary Immunodeficiencies: 25-Years of Experience (1988-2012)

Tahar Sfar
  • Function : Author
Siheme Barsaoui
  • Function : Author
Azza Sammoud
  • Function : Author
Khadija Boussetta
  • Function : Author
Saida Ben Becher
  • Function : Author
Najoua Guandoura
  • Function : Author
Fethi Amri
  • Function : Author
Asma Bouaziz
  • Function : Author
Hajer Aloulou
  • Function : Author
Raoudha Boussoffara
  • Function : Author
Jamel Ammar
  • Function : Author
Mohamed-Ridha Barbouche
  • Function : Author
  • PersonId : 917302

Abstract

Primary immunodeficiencies (PIDs) are a large group of diseases characterized by susceptibility to not only recurrent infections but also autoimmune diseases and malignancies. The aim of this study was to describe and analyze the distribution, clinical features and eventual outcome of PID among Tunisian patients. We reviewed the record of 710 patients diagnosed with Primary Immunodeficiency Diseases (PIDs) from the registry of the Tunisian Referral Centre for PIDs over a 25-year period. The male-to-female ratio was 1.4. The median age at the onset of symptoms was 6 months and at the time of diagnosis 2 years. The estimated prevalence was 4.3 per 100,000 populations. The consanguinity rate was found in 58.2 % of families. According to the International Union of Immunological Societies classification, spectrums of PIDs were as follows: combined T-cell and B-cell immunodeficiency disorders account for the most common category (28.6 %), followed by congenital defects of phagocyte (25.4 %), other well-defined immunodeficiency syndromes (22.7 %), predominant antibody deficiency diseases (17.7 %), diseases of immune dysregulation (4.8 %), defect of innate immunity (0.4 %) and complement deficiencies (0.4 %). Recurrent infections, particularly lower airway infections (62.3 %), presented the most common manifestation of PID patients. The overall mortality rate was 34.5 %, mainly observed with combined immunodeficiencies. The distribution of PIDs was different from that reported in Western countries, with a particularly high proportion of Combined Immunodeficiencies and phagocyte defects in number and/or function. More is needed to improve PID diagnosis and treatment in our country.
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pasteur-01375025 , version 1 (01-06-2017)

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Fethi Mellouli, Imen Ben Mustapha, Monia Ben Khaled, Habib Besbes, Monia Ouederni, et al.. Report of the Tunisian Registry of Primary Immunodeficiencies: 25-Years of Experience (1988-2012). Journal of Clinical Immunology, 2015, 35 (8), pp.745-753. ⟨10.1007/s10875-015-0206-9⟩. ⟨pasteur-01375025⟩

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