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

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Abstract

Purpose

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.

Methods

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.

Results

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.

Conclusion

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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Author information

Author notes

    Authors and Affiliations

    1. Service d’immuno hématologie pédiatriques, Centre National de Greffe de Moelle Osseuse de Tunis, 13 Rue du Djebel Lakhdhar, 1006, Tunis, Tunisia

      Fethi Mellouli, Monia Ben Khaled, Habib Besbes, Monia Ouederni & Mohamed Bejaoui

    2. Faculté de medicine de Tunis, Université El Manar II, Tunis, Tunisia

      Fethi Mellouli, Imen Ben Mustapha, Monia Ben Khaled, Habib Besbes, Monia Ouederni, Najla Mekki, Meriem Ben Ali, Beya Larguèche, Mohamed-Ridha Barbouche & Mohamed Bejaoui

    3. Laboratoire d’immunologie, Institut Pasteur de Tunis, Tunis, Tunisia

      Imen Ben Mustapha, Najla Mekki, Meriem Ben Ali, Beya Larguèche & Mohamed-Ridha Barbouche

    4. Service de pédiatrie, hôpital de Sfax, Sfax, Tunisia

      Mongia Hachicha, Abdelmajid Mahfoudh, Hajer Aloulou, Lamia Ben Mansour & Imen Chabchoub

    5. Service de pédiatrie, hôpital de Mehdia, Mehdia, Tunisa

      Tahar Sfar & Raoudha Boussoffara

    6. Service de pédiatrie, hôpital de Monastir, Monastir, Tunisia

      Neji Gueddiche, Saber Hammami & Zakia Habboul

    7. Service de pédiatrie A, hôpital Bechir Hamza de Tunis, Tunis, Tunisia

      Siheme Barsaoui

    8. Service de pédiatrie B, hôpital Bechir Hamza de Tunis, Tunis, Tunisia

      Azza Sammoud

    9. Service de pédiatrie C, hôpital Bechir Hamza de Tunis, Tunis, Tunisia

      Khadija Boussetta

    10. Service de pédiatrie PUC, hôpital Bechir Hamza de Tunis, Tunis, Tunisia

      Saida Ben Becher

    11. Service de pédiatrie, hôpital Mongi Slim La Marsa, La Marsa, Tunisia

      Ahmed Meherzi

    12. Service de pédiatrie, hôpital de Bizerte, Bizerte, Tunisia

      Najoua Guandoura

    13. Service de pédiatrie, hôpital Farhat Hached de Sousse, Sousse, Tunisia

      Lamia Boughammoura & Jihène Bouguila

    14. Service de pédiatrie, hôpital Sahloul de Sousse, Sousse, Tunisia

      Abdelaziz Harbi, Jalel Chemli & Saida Hassayoun

    15. Service de pédiatrie, hôpital de Kairouan, Kairouan, Tunisia

      Fethi Amri

    16. Service de pédiatrie, hôpital Militaire de Tunis, Tunis, Tunisia

      Fethi Bayoudh

    17. Service de réanimation pédiatrique, hôpital Bechir Hamza de Tunis, Tunis, Tunisia

      Najla Ben Jaballah

    18. Service de pédiatrie, hôpital La Rabta de Tunis, Tunis, Tunisia

      Neji Tebib

    19. Service de pédiatrie, hôpital de Nabeul, Nabeul, Tunisia

      Asma Bouaziz

    20. Service de pneumologie, hôpital l’Ariana de Tunis, Tunis, Tunisia

      Agnès Hamzaoui & Jamel Ammar

    Authors
    1. Fethi Mellouli
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    2. Imen Ben Mustapha
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    3. Monia Ben Khaled
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    4. Habib Besbes
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    5. Monia Ouederni
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    6. Najla Mekki
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    7. Meriem Ben Ali
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    8. Beya Larguèche
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    9. Mongia Hachicha
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    10. Tahar Sfar
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    11. Neji Gueddiche
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    12. Siheme Barsaoui
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    13. Azza Sammoud
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    14. Khadija Boussetta
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    15. Saida Ben Becher
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    16. Ahmed Meherzi
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    17. Najoua Guandoura
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    18. Lamia Boughammoura
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    19. Abdelaziz Harbi
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    20. Fethi Amri
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    21. Fethi Bayoudh
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    22. Najla Ben Jaballah
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    23. Neji Tebib
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    24. Asma Bouaziz
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    25. Abdelmajid Mahfoudh
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    26. Hajer Aloulou
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    28. Imen Chabchoub
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    29. Raoudha Boussoffara
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    30. Jalel Chemli
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    31. Jihène Bouguila
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    32. Saida Hassayoun
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    33. Saber Hammami
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    34. Zakia Habboul
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    35. Agnès Hamzaoui
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    36. Jamel Ammar
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    37. Mohamed-Ridha Barbouche
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    38. Mohamed Bejaoui
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    Corresponding author

    Correspondence to Fethi Mellouli.

    Additional information

    Fethi Mellouli, Imen Ben Mustapha, Mohamed-Ridha Barbouche and Mohamed Bejaoui contributed equally to this work.

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    Mellouli, F., Mustapha, I.B., Khaled, M.B. et al. Report of the Tunisian Registry of Primary Immunodeficiencies: 25-Years of Experience (1988–2012). J Clin Immunol 35, 745–753 (2015). https://doi.org/10.1007/s10875-015-0206-9

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    • DOI: https://doi.org/10.1007/s10875-015-0206-9

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