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Report of the Tunisian Registry of Primary Immunodeficiencies: 25-Years of Experience (1988-2012).
Mellouli, Fethi; Mustapha, Imen Ben; Khaled, Monia Ben; Besbes, Habib; Ouederni, Monia; Mekki, Najla; Ali, Meriem Ben; Larguèche, Beya; Hachicha, Mongia; Sfar, Tahar; Gueddiche, Neji; Barsaoui, Siheme; Sammoud, Azza; Boussetta, Khadija; Becher, Saida Ben; Meherzi, Ahmed; Guandoura, Najoua; Boughammoura, Lamia; Harbi, Abdelaziz; Amri, Fethi; Bayoudh, Fethi; Jaballah, Najla Ben; Tebib, Neji; Bouaziz, Asma; Mahfoudh, Abdelmajid; Aloulou, Hajer; Mansour, Lamia Ben; Chabchoub, Imen; Boussoffara, Raoudha; Chemli, Jalel; Bouguila, Jihène; Hassayoun, Saida; Hammami, Saber; Habboul, Zakia; Hamzaoui, Agnès; Ammar, Jamel; Barbouche, Mohamed-Ridha; Bejaoui, Mohamed.
Afiliación
  • Mellouli F; Service d'immuno hématologie pédiatriques, Centre National de Greffe de Moelle Osseuse de Tunis, 13 Rue du Djebel Lakhdhar, 1006, Tunis, Tunisia. melloulif@yahoo.fr.
  • Mustapha IB; Faculté de medicine de Tunis, Université El Manar II, Tunis, Tunisia. melloulif@yahoo.fr.
  • Khaled MB; Faculté de medicine de Tunis, Université El Manar II, Tunis, Tunisia.
  • Besbes H; Laboratoire d'immunologie, Institut Pasteur de Tunis, Tunis, Tunisia.
  • Ouederni M; Service d'immuno hématologie pédiatriques, Centre National de Greffe de Moelle Osseuse de Tunis, 13 Rue du Djebel Lakhdhar, 1006, Tunis, Tunisia.
  • Mekki N; Faculté de medicine de Tunis, Université El Manar II, Tunis, Tunisia.
  • Ali MB; Service d'immuno hématologie pédiatriques, Centre National de Greffe de Moelle Osseuse de Tunis, 13 Rue du Djebel Lakhdhar, 1006, Tunis, Tunisia.
  • Larguèche B; Faculté de medicine de Tunis, Université El Manar II, Tunis, Tunisia.
  • Hachicha M; Service d'immuno hématologie pédiatriques, Centre National de Greffe de Moelle Osseuse de Tunis, 13 Rue du Djebel Lakhdhar, 1006, Tunis, Tunisia.
  • Sfar T; Faculté de medicine de Tunis, Université El Manar II, Tunis, Tunisia.
  • Gueddiche N; Faculté de medicine de Tunis, Université El Manar II, Tunis, Tunisia.
  • Barsaoui S; Laboratoire d'immunologie, Institut Pasteur de Tunis, Tunis, Tunisia.
  • Sammoud A; Faculté de medicine de Tunis, Université El Manar II, Tunis, Tunisia.
  • Boussetta K; Laboratoire d'immunologie, Institut Pasteur de Tunis, Tunis, Tunisia.
  • Becher SB; Faculté de medicine de Tunis, Université El Manar II, Tunis, Tunisia.
  • Meherzi A; Laboratoire d'immunologie, Institut Pasteur de Tunis, Tunis, Tunisia.
  • Guandoura N; Service de pédiatrie, hôpital de Sfax, Sfax, Tunisia.
  • Boughammoura L; Service de pédiatrie, hôpital de Mehdia, Mehdia, Tunisa.
  • Harbi A; Service de pédiatrie, hôpital de Monastir, Monastir, Tunisia.
  • Amri F; Service de pédiatrie A, hôpital Bechir Hamza de Tunis, Tunis, Tunisia.
  • Bayoudh F; Service de pédiatrie B, hôpital Bechir Hamza de Tunis, Tunis, Tunisia.
  • Jaballah NB; Service de pédiatrie C, hôpital Bechir Hamza de Tunis, Tunis, Tunisia.
  • Tebib N; Service de pédiatrie PUC, hôpital Bechir Hamza de Tunis, Tunis, Tunisia.
  • Bouaziz A; Service de pédiatrie, hôpital Mongi Slim La Marsa, La Marsa, Tunisia.
  • Mahfoudh A; Service de pédiatrie, hôpital de Bizerte, Bizerte, Tunisia.
  • Aloulou H; Service de pédiatrie, hôpital Farhat Hached de Sousse, Sousse, Tunisia.
  • Mansour LB; Service de pédiatrie, hôpital Sahloul de Sousse, Sousse, Tunisia.
  • Chabchoub I; Service de pédiatrie, hôpital de Kairouan, Kairouan, Tunisia.
  • Boussoffara R; Service de pédiatrie, hôpital Militaire de Tunis, Tunis, Tunisia.
  • Chemli J; Service de réanimation pédiatrique, hôpital Bechir Hamza de Tunis, Tunis, Tunisia.
  • Bouguila J; Service de pédiatrie, hôpital La Rabta de Tunis, Tunis, Tunisia.
  • Hassayoun S; Service de pédiatrie, hôpital de Nabeul, Nabeul, Tunisia.
  • Hammami S; Service de pédiatrie, hôpital de Sfax, Sfax, Tunisia.
  • Habboul Z; Service de pédiatrie, hôpital de Sfax, Sfax, Tunisia.
  • Hamzaoui A; Service de pédiatrie, hôpital de Sfax, Sfax, Tunisia.
  • Ammar J; Service de pédiatrie, hôpital de Sfax, Sfax, Tunisia.
  • Barbouche MR; Service de pédiatrie, hôpital de Mehdia, Mehdia, Tunisa.
  • Bejaoui M; Service de pédiatrie, hôpital Sahloul de Sousse, Sousse, Tunisia.
J Clin Immunol ; 35(8): 745-53, 2015 Nov.
Article en En | MEDLINE | ID: mdl-26464197
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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Texto completo: 1 Colección: 01-internacional Banco de datos: MEDLINE Asunto principal: Linfocitos B / Linfocitos T / Sistema de Registros / Síndromes de Inmunodeficiencia / Anticuerpos Tipo de estudio: Prevalence_studies / Risk_factors_studies Límite: Female / Humans / Infant / Male País/Región como asunto: Africa Idioma: En Revista: J Clin Immunol Año: 2015 Tipo del documento: Article País de afiliación: Túnez

Texto completo: 1 Colección: 01-internacional Banco de datos: MEDLINE Asunto principal: Linfocitos B / Linfocitos T / Sistema de Registros / Síndromes de Inmunodeficiencia / Anticuerpos Tipo de estudio: Prevalence_studies / Risk_factors_studies Límite: Female / Humans / Infant / Male País/Región como asunto: Africa Idioma: En Revista: J Clin Immunol Año: 2015 Tipo del documento: Article País de afiliación: Túnez