43 results on '"Ben Khaled M"'
Search Results
2. An engineered enzyme embedded into PLA to make self-biodegradable plastic
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Guicherd, M., Ben Khaled, M., Guéroult, M., Nomme, J., Dalibey, M., Grimaud, F., Alvarez, P., Kamionka, E., Gavalda, S., Noël, M., Vuillemin, M., Amillastre, E., Labourdette, D., Cioci, G., Tournier, V., Kitpreechavanich, V., Dubois, P., André, I., Duquesne, S., and Marty, A.
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- 2024
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3. Evaluation of graft processing quality indicators for allogeneic bone marrow grafts prepared at the national blood transfusion center of Tunisia
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Lameri, M., primary, Aissa, W., additional, Guettari, M., additional, Turki, I., additional, Ouerghi, R., additional, Ben Khaled, M., additional, Ben Ammar, S., additional, Jemaa, N., additional, Bouhalouene, K., additional, Bejaoui, M.S., additional, Raies, L., additional, Mtaallah, R., additional, Ben Abdeljelil, N., additional, Ouederni, M., additional, Ben Othmen, T., additional, Kaabi, H., additional, Chaabane, M., additional, and Hmida, S., additional
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- 2024
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4. 5613312 PLASMATIC MICROPARTICLES AS POTENTIAL BIOMARKERS IN THE PREVENTIVE DIAGNOSIS OF BETA THALASSEMIA
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Chebbi, M., primary, Khalfaoui, K., additional, Saidani, S., additional, Safra, I., additional, Barmate, M., additional, Chaouechi, D., additional, Ben Khaled, M., additional, Ouederni, M., additional, Mellouli, F., additional, Menif, S., additional, and Moumni, I., additional
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- 2023
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5. Eryptosis and circulating blood cells microparticules in sickle cell diseases
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Moumni, I, primary, Khalfaoui, K, additional, Safra, I, additional, Chebbi, M, additional, Barmate, M, additional, chaouechi, D, additional, Ben khaled, M, additional, Ouederni, M, additional, Mellouli, F, additional, and Menif, S, additional
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- 2020
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6. Rôle de la surcharge en fer post transfusionnelle dans la survenue de l’hypothyroïdie chez les patients porteurs d’hémoglobinopathie
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Guirat, N., primary, Kouki, R., additional, Ouederni, M., additional, Ben Khaled, M., additional, Mellouli, F., additional, and Bejaoui, M., additional
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- 2018
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7. Anomalies pubertaires et béta-thalassémie majeure
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Guirat, N., primary, Kouki, R., additional, Ouederni, M., additional, Ben Khaled, M., additional, Mellouli, F., additional, and Bejaoui, M., additional
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- 2018
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8. Intérêt du dosage du peptide-C chez les patients bêta-thalassémiques majeurs polytransfusés
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Guirat, N., primary, Kouki, R., additional, Ouederni, M., additional, Ben Khaled, M., additional, Mellouli, F., additional, and Bejaoui, M., additional
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- 2018
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9. Évaluation de l’IGF1 chez les patients suivis pour bêta-thalassémie majeure (Étude comparative avec une population drépanocytaire)
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Guirat, N., primary, Kouki, R., additional, Ouederni, M., additional, Ben Khaled, M., additional, Mellouli, F., additional, and Bejaoui, M., additional
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- 2018
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10. Le retard statural et déficits immunitaires primitifs : fréquence et facteurs de risque dans une série tunisienne
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Ben Khaled, M., primary, Ouederni, M., additional, Ben Fradj, I., additional, Rhouma, K., additional, Rekaya, S., additional, Dhouib, N., additional, Mellouli, F., additional, and Bejaoui, M., additional
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- 2016
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11. Évolution des endocrinopathies sous traitement chélateur dans la bêta-thalassémie majeure
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Ouederni, M., primary, Rhouma, K., additional, Ben Khaled, M., additional, Dhouib, N., additional, Ben Fraj, E., additional, Rkaya, S., additional, Mellouli, F., additional, and Bejaoui, M., additional
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- 2016
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12. Association entre les troubles des hydrates de carbones et l’hémochromatose cardiaque dans la bêtathalassémie majeure
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Ouederni, M., primary, Rhouma, K., additional, Ben Khaled, M., additional, Rkaya, S., additional, Dhouib, N., additional, Ben Frej, E., additional, Mellouli, F., additional, and Bejaoui, M., additional
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- 2016
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13. L’hypoparathyroïdie complication rare de l’hémochromatose secondaire : à propos de trois cas tunisiens
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Ben Khaled, M., primary, Rhouma, K., additional, Ouederni, M., additional, Rekaya, S., additional, Ben Fradj, I., additional, Dhouib, N., additional, Mellouli, F., additional, and Bejaoui, M., additional
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- 2016
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14. P-254 – L'indice de masse corporelle chez les patients béta thalassémiques polytransfusés
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Dhouib, N. Guirat, primary, Thraya, S., additional, Ouederni, M., additional, Ben Khaled, M., additional, Oueslati, A., additional, Besbes, H., additional, Laabidi, F., additional, Mellouli, F., additional, and Bejaoui, M., additional
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- 2015
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15. P-256 – Prise en charge du Purpura thrombopénique immunologique persistant ou chronique
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Ouederni, M., primary, Oueslati, A., additional, Ben Khaled, M., additional, Dhouib, N., additional, Jridi, I., additional, Jbebli, I., additional, Mellouli, F., additional, and Bejaoui, M., additional
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- 2015
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16. P-255 – Incidence des effets indésirables transfusionnels dans un service d'hématologie pédiatrique
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Ouederni, M., primary, Jbebli, I., additional, Ben Khaled, M., additional, Dhouib, N., additional, Oueslati, A., additional, Jridi, I., additional, Mellouli, F., additional, and Bejaoui, M., additional
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- 2015
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17. P-252 – Dysmorphie faciale et surcharge en fer chez les enfants bêta-thalassémiques polytransfusés
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Ouederni, M., primary, Jridi, I., additional, Ben Khaled, M., additional, Dhouib, N., additional, Oueslati, A., additional, Jbebli, I., additional, Mellouli, F., additional, and Bejaoui, M., additional
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- 2015
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18. Diabète et bêta-thalassémie majeure
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Guirat Dhouib, N., primary, Hassouna, R., additional, Ouederni, M., additional, Ben Khaled, M., additional, Haoua, A., additional, Thraya, S., additional, Mellouli, F., additional, and Bejaoui, M., additional
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- 2014
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19. L’ostéoporose chez les sujets bêta-thalassémiques tunisiens : étude de 70 cas pédiatriques
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Mellouli, F., primary, Thraya, S., additional, Ouederni, M., additional, Ben Khaled, M., additional, Dhouib, N., additional, Hassouna, R., additional, Haoua, A., additional, and Béjaoui, M., additional
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- 2014
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20. SFP P-039 – Expression phénotypique du syndrome de Wiskott Aldrich (SWA) en Tunisie
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Ben Khaled, M., primary, Labidi, F., additional, Ouederni, M., additional, Bouzidi, L., additional, Dhouib, N., additional, Jridi, I., additional, Mellouli, F., additional, and Bejaoui, M., additional
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- 2014
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21. SFP PC-68 – Insuffisance surrénale et β thalassémie majeure
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Guirat-Dhouib, N., primary, Laabidi, F., additional, Ouederni, M., additional, Ben Khaled, M., additional, Belhadj Hmida, S., additional, Jridi, I., additional, Mellouli, F., additional, and Bejaoui, M., additional
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- 2014
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22. SFP P-040 – Intérêt des indices HOMA chez les β thalassémiques majeurs polytransfusés
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Guirat-Dhouib, N., primary, Jridi, I., additional, Ouederni, M., additional, Ben Khaled, M., additional, Belhadj Hmida, S., additional, Laabidi, F., additional, Mellouli, F., additional, and Bejaoui, M., additional
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- 2014
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23. SFP CO-04 - Manifestations auto-immunes des déficits immunitaires primitifs
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Ouederni, M., primary, Labidi, F., additional, Ben Khaled, M., additional, Dhouib, N., additional, Jeridi, I., additional, Mellouli, F., additional, and Bejaoui, M., additional
- Published
- 2014
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24. SFP PC-67 – Les syndromes myélodysplasiques de l’enfant (SMD): 8 cas tunisiens
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Ouederni, M., primary, Jeridi, I., additional, Ben Khaled, M., additional, Dhouib, N., additional, Labidi, F., additional, Mellouli, F., additional, and Bejaoui, M., additional
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- 2014
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25. SFCE P-15 - Les déficits immunitaires primitifs et cancers
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Ouederni, M., primary, Kchaw, M., additional, Ben Khaled, M., additional, Dhouib, N., additional, Jeridi, I., additional, Labidi, F., additional, Mellouli, F., additional, and Bejaoui, M., additional
- Published
- 2014
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26. 46th Medical Maghrebian Congress. November 9-10, 2018. Tunis
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Alami Aroussi, A., Fouad, A., Omrane, A., Razzak, A., Aissa, A., Akkad, A., Amraoui, A., Aouam, A., Arfaoui, A., Belkouchi, A., Ben Chaaben, A., Ben Cheikh, A., Ben Khélifa, A., Ben Mabrouk, A., Benhima, A., Bezza, A., Bezzine, A., Bourrahouat, A., Chaieb, A., Chakib, A., Chetoui, A., Daoudi, A., Ech-Chenbouli, A., Gaaliche, A., Hassani, A., Kassimi, A., Khachane, A., Labidi, A., Lalaoui, A., Masrar, A., Mchachi, A., Nakhli, A., Ouakaa, A., Siati, A., Toumi, A., Zaouali, A., Condé, A. Y., Haggui, A., Belaguid, A., abdelkader jalil el hangouche, Gharbi, A., Mahfoudh, A., Bouzouita, A., Aissaoui, A., Ben Hamouda, A., Hedhli, A., Ammous, A., Bahlous, A., Ben Halima, A., Belhadj, A., Blel, A., Brahem, A., Banasr, A., Meherzi, A., Saadi, A., Sellami, A., Turki, A., Ben Miled, A., Ben Slama, A., Daib, A., Zommiti, A., Chadly, A., Jmaa, A., Mtiraoui, A., Ksentini, A., Methnani, A., Zehani, A., Kessantini, A., Farah, A., Mankai, A., Mellouli, A., Touil, A., Hssine, A., Ben Safta, A., Derouiche, A., Jmal, A., Ferjani, A., Djobbi, A., Dridi, A., Aridhi, A., Bahdoudi, A., Ben Amara, A., Benzarti, A., Ben Slama, A. Y., Oueslati, A., Soltani, A., Chadli, A., Aloui, A., Belghuith Sriha, A., Bouden, A., Laabidi, A., Mensi, A., Sabbek, A., Zribi, A., Green, A., Ben Nasr, A., Azaiez, A., Yeades, A., Belhaj, A., Mediouni, A., Sammoud, A., Slim, A., Amine, B., Chelly, B., Jatik, B., Lmimouni, B., Daouahi, B., Ben Khelifa, B., Louzir, B., Dorra, A., Dhahri, B., Ben Nasrallah, C., Chefchaouni, C., Konzi, C., Loussaief, C., Makni, C., Dziri, C., Bouguerra, C., Kays, C., Zedini, C., Dhouha, C., Mohamed, C., Aichaouia, C., Dhieb, C., Fofana, D., Gargouri, D., Chebil, D., Issaoui, D., Gouiaa, D., Brahim, D., Essid, D., Jarraya, D., Trad, D., Ben Hmida, E., Sboui, E., Ben Brahim, E., Baati, E., Talbi, E., Chaari, E., Hammami, E., Ghazouani, E., Ayari, F., Ben Hariz, F., Bennaoui, F., Chebbi, F., Chigr, F., Guemira, F., Harrar, F., Benmoula, F. Z., Ouali, F. Z., Maoulainine, F. M. R., Bouden, F., Fdhila, F., Améziani, F., Bouhaouala, F., Charfi, F., Chermiti Ben Abdallah, F., Hammemi, F., Jarraya, F., Khanchel, F., Ourda, F., Sellami, F., Trabelsi, F., Yangui, F., Fekih Romdhane, F., Mellouli, F., Nacef Jomli, F., Mghaieth, F., Draiss, G., Elamine, G., Kablouti, G., Touzani, G., Manzeki, G. B., Garali, G., Drissi, G., Besbes, G., Abaza, H., Azzouz, H., Said Latiri, H., Rejeb, H., Ben Ammar, H., Ben Brahim, H., Ben Jeddi, H., Ben Mahjouba, H., Besbes, H., Dabbebi, H., Douik, H., El Haoury, H., Elannaz, H., Elloumi, H., Hachim, H., Iraqi, H., Kalboussi, H., Khadhraoui, H., Khouni, H., Mamad, H., Metjaouel, H., Naoui, H., Zargouni, H., Elmalki, H. O., Feki, H., Haouala, H., Jaafoura, H., Drissa, H., Mizouni, H., Kamoun, H., Ouerda, H., Zaibi, H., Chiha, H., Saibi, H., Skhiri, H., Boussaffa, H., Majed, H., Blibech, H., Daami, H., Harzallah, H., Rkain, H., Ben Massoud, H., Jaziri, H., Ben Said, H., Ayed, H., Harrabi, H., Chaabouni, H., Ladida Debbache, H., Harbi, H., Yacoub, H., Abroug, H., Ghali, H., Kchir, H., Msaad, H., Manai, H., Riahi, H., Bousselmi, H., Limem, H., Aouina, H., Jerraya, H., Ben Ayed, H., Chahed, H., Snéne, H., Lahlou Amine, I., Nouiser, I., Ait Sab, I., Chelly, I., Elboukhani, I., Ghanmi, I., Kallala, I., Kooli, I., Bouasker, I., Fetni, I., Bachouch, I., Bouguecha, I., Chaabani, I., Gazzeh, I., Samaali, I., Youssef, I., Zemni, I., Bachouche, I., Bouannene, I., Kasraoui, I., Laouini, I., Mahjoubi, I., Maoudoud, I., Riahi, I., Selmi, I., Tka, I., Hadj Khalifa, I., Mejri, I., Béjia, I., Bellagha, J., Boubaker, J., Daghfous, J., Dammak, J., Hleli, J., Ben Amar, J., Jedidi, J., Marrakchi, J., Kaoutar, K., Arjouni, K., Ben Helel, K., Benouhoud, K., Rjeb, K., Imene, K., Samoud, K., El Jeri, K., Abid, K., Chaker, K., Bouzghaîa, K., Kamoun, K., Zitouna, K., Oughlani, K., Lassoued, K., Letaif, K., Hakim, K., Cherif Alami, L., Benhmidoune, L., Boumhil, L., Bouzgarrou, L., Dhidah, L., Ifrine, L., Kallel, L., Merzougui, L., Errguig, L., Mouelhi, L., Sahli, L., Maoua, M., Rejeb, M., Ben Rejeb, M., Bouchrik, M., Bouhoula, M., Bourrous, M., Bouskraoui, M., El Belhadji, M., Essakhi, M., Essid, M., Gharbaoui, M., Haboub, M., Iken, M., Krifa, M., Lagrine, M., Leboyer, M., Najimi, M., Rahoui, M., Sabbah, M., Sbihi, M., Zouine, M., Chefchaouni, M. C., Gharbi, M. H., El Fakiri, M. M., Tagajdid, M. R., Shimi, M., Touaibia, M., Jguirim, M., Barsaoui, M., Belghith, M., Ben Jmaa, M., Koubaa, M., Tbini, M., Boughdir, M., Ben Salah, M., Ben Fraj, M., Ben Halima, M., Ben Khalifa, M., Bousleh, M., Limam, M., Mabrouk, M., Mallouli, M., Rebeii, M., Ayari, M., Belhadj, M., Ben Hmida, M., Boughattas, M., Drissa, M., El Ghardallou, M., Fejjeri, M., Hamza, M., Jaidane, M., Jrad, M., Kacem, M., Mersni, M., Mjid, M., Serghini, M., Triki, M., Ben Abbes, M., Boussaid, M., Gharbi, M., Hafi, M., Slama, M., Trigui, M., Taoueb, M., Chakroun, M., Ben Cheikh, M., Chebbi, M., Hadj Taieb, M., Ben Khelil, M., Hammami, M., Khalfallah, M., Ksiaa, M., Mechri, M., Mrad, M., Sboui, M., Bani, M., Hajri, M., Mellouli, M., Allouche, M., Mesrati, M. A., Mseddi, M. A., Amri, M., Bejaoui, M., Bellali, M., Ben Amor, M., Ben Dhieb, M., Ben Moussa, M., Chebil, M., Cherif, M., Fourati, M., Kahloul, M., Khaled, M., Machghoul, M., Mansour, M., Abdesslem, M. M., Ben Chehida, M. A., Chaouch, M. A., Essid, M. A., Meddeb, M. A., Gharbi, M. C., Elleuch, M. H., Loueslati, M. H., Sboui, M. M., Mhiri, M. N., Kilani, M. O., Ben Slama, M. R., Charfi, M. R., Nakhli, M. S., Mourali, M. S., El Asli, M. S., Lamouchi, M. T., Cherti, M., Khadhraoui, M., Bibi, M., Hamdoun, M., Kassis, M., Touzi, M., Ben Khaled, M., Fekih, M., Khemiri, M., Ouederni, M., Hchicha, M., Ben Attia, M., Yahyaoui, M., Ben Azaiez, M., Bousnina, M., Ben Jemaa, M., Ben Yahia, M., Daghfous, M., Haj Slimen, M., Assidi, M., Belhadj, N., Ben Mustapha, N., El Idrissislitine, N., Hikki, N., Kchir, N., Mars, N., Meddeb, N., Ouni, N., Rada, N., Rezg, N., Trabelsi, N., Bouafia, N., Haloui, N., Benfenatki, N., Bergaoui, N., Yomn, N., Maamouri, N., Mehiri, N., Siala, N., Beltaief, N., Aridhi, N., Sidaoui, N., Walid, N., Mechergui, N., Mnif, N., Ben Chekaya, N., Bellil, N., Dhouib, N., Achour, N., Kaabar, N., Mrizak, N., Chaouech, N., Hasni, N., Issaoui, N., Ati, N., Balloumi, N., Haj Salem, N., Ladhari, N., Akif, N., Liani, N., Hajji, N., Trad, N., Elleuch, N., Marzouki, N. E. H., Larbi, N., M Barek, N., Rebai, N., Bibani, N., Ben Salah, N., Belmaachi, O., Elmaalel, O., Jlassi, O., Mihoub, O., Ben Zaid, O., Bouallègue, O., Bousnina, O., Bouyahia, O., El Maalel, O., Fendri, O., Azzabi, O., Borgi, O., Ghdes, O., Ben Rejeb, O., Rachid, R., Abi, R., Bahiri, R., Boulma, R., Elkhayat, R., Habbal, R., Tamouza, R., Jomli, R., Ben Abdallah, R., Smaoui, R., Debbeche, R., Fakhfakh, R., El Kamel, R., Gargouri, R., Jouini, R., Nouira, R., Fessi, R., Bannour, R., Ben Rabeh, R., Kacem, R., Khmakhem, R., Ben Younes, R., Karray, R., Cheikh, R., Ben Malek, R., Ben Slama, R., Kouki, R., Baati, R., Bechraoui, R., Fradi, R., Lahiani, R., Ridha, R., Zainine, R., Kallel, R., Rostom, S., Ben Abdallah, S., Ben Hammamia, S., Benchérifa, S., Benkirane, S., Chatti, S., El Guedri, S., El Oussaoui, S., Elkochri, S., Elmoussaoui, S., Enbili, S., Gara, S., Haouet, S., Khammeri, S., Khefecha, S., Khtrouche, S., Macheghoul, S., Mallouli, S., Rharrit, S., Skouri, S., Helali, S., Boulehmi, S., Abid, S., Naouar, S., Zelfani, S., Ben Amar, S., Ajmi, S., Braiek, S., Yahiaoui, S., Ghezaiel, S., Ben Toumia, S., Thabeti, S., Daboussi, S., Ben Abderahman, S., Rhaiem, S., Ben Rhouma, S., Rekaya, S., Haddad, S., Kammoun, S., Merai, S., Mhamdi, S., Ben Ali, R., Gaaloul, S., Ouali, S., Taleb, S., Zrour, S., Hamdi, S., Zaghdoudi, S., Ammari, S., Ben Abderrahim, S., Karaa, S., Maazaoui, S., Saidani, S., Stambouli, S., Mokadem, S., Boudiche, S., Zaghbib, S., Ayedi, S., Jardek, S., Bouselmi, S., Chtourou, S., Manoubi, S., Bahri, S., Halioui, S., Jrad, S., Mazigh, S., Ouerghi, S., Toujani, S., Fenniche, S., Aboudrar, S., Meriem Amari, S., Karouia, S., Bourgou, S., Halayem, S., Rammeh, S., Yaïch, S., Ben Nasrallah, S., Chouchane, S., Ftini, S., Makni, S., Miri, S., Saadi, S., Manoubi, S. A., Khalfallah, T., Mechergui, T., Dakka, T., Barhoumi, T., M Rad, T. E. B., Ajmi, T., Dorra, T., Ouali, U., Hannachi, W., Ferjaoui, W., Aissi, W., Dahmani, W., Dhouib, W., Koubaa, W., Zhir, W., Gheriani, W., Arfa, W., Dougaz, W., Sahnoun, W., Naija, W., Sami, Y., Bouteraa, Y., Elhamdaoui, Y., Hama, Y., Ouahchi, Y., Guebsi, Y., Nouira, Y., Daly, Y., Mahjoubi, Y., Mejdoub, Y., Mosbahi, Y., Said, Y., Zaimi, Y., Zgueb, Y., Dridi, Y., Mesbahi, Y., Gharbi, Y., Hellal, Y., Hechmi, Z., Zid, Z., Elmouatassim, Z., Ghorbel, Z., Habbadi, Z., Marrakchi, Z., Hidouri, Z., Abbes, Z., Ouhachi, Z., Khessairi, Z., Khlayfia, Z., Mahjoubi, Z., and Moatemri, Z.
27. Pancytopenia revealing phenylketonuria: Coincidence or first case report
- Author
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Ben Abdelaziz, R., Mellouli, F., Lamouchi, M. T., Ben Messaoud, S., Ben Khaled, M., Doghri, R., Boudabous, H., Amel Ben Chehida, Azzouz, H., Bejaoui, M., and Tebib, N.
28. Incidence and risk factors for osteonecrosis of the femoral head in five hundred and ten sickle cell disease paediatric patients.
- Author
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Ouederni M, Rouag H, Ben Fraj I, Rekaya S, Kouki R, Lamouchi T, Zaiter I, Mellouli F, Bejaoui M, and Ben Khaled M
- Subjects
- Humans, Child, Retrospective Studies, Incidence, Femur Head, Risk Factors, Volatile Organic Compounds, Anemia, Sickle Cell complications, Anemia, Sickle Cell epidemiology, Femur Head Necrosis etiology, Femur Head Necrosis complications
- Abstract
Purpose: Osteonecrosis of the femoral head (ONFH) is a degenerative and progressive disorder that mainly affects people with sickle cell disease (SCD). Herein, we aimed to search for a better understanding of markers that can act as risk factors for ONFH in patients with SCD., Methods: We conducted a retrospective study including 510 SCD patients followed over 23 years. Patients were divided into the ONFH group and the no-ONHF control group. Univariate and multivariate logistic regression analyses were performed to identify risk factors., Results: Among 510 SCD patients, 41(8%) were diagnosed with ONFH at a mean age of 167 months ± 64 (72-288). The cumulative incidence of ONHF increased from 2.3% at ten years to 18.3% at 20 years of age. The radiological grade 3 ONHF was predominant. No significant differences in sex, age at diagnosis of SCD, and Hb genotype were found between groups. The patient age and the time since diagnosis of SCD were statistically higher in patients with ONHF in univariate and multivariate analysis. ONHF was also associated with higher creatinine level (p = 0.001) lower LDH level (p = 0.006), and higher number of vaso-occlusive crisis (VOC)/patient/year (p < 0.001). The cumulative incidence of ONHF in patients having more than 3 VOC/year was significantly higher (43% versus 18.9% at 20 years, p < 0.001). In addition, infections, gallstones, growth delay, delayed initiation of hydroxyurea, and a higher transfusion rate were significantly associated with ONFH., Conclusion: These findings confirm that ONFH is closely related to the age, severity, and duration of SCD. Better management of this disease prevents acute and chronic complications, and early screening of the ONFH as soon as the first signs of the severity of the disease are detected provides a better functional prognosis., (© 2023. The Author(s) under exclusive licence to SICOT aisbl.)
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- 2023
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29. Clinical features and predictors of osteoarticular manifestations in common variable immunodeficiency.
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Ben Khaled M, Merdassi A, Rekaya S, Fraj IB, Lamouchi T, Zaiter I, Kouki R, Bejaoui M, Mellouli F, and Ouederni M
- Subjects
- Humans, Child, Retrospective Studies, Hepatomegaly, Diarrhea, Body Weight, Autoimmunity, Common Variable Immunodeficiency complications, Common Variable Immunodeficiency diagnosis, Common Variable Immunodeficiency epidemiology, Iron Deficiencies
- Abstract
Introduction: This study aimed to assess osteoarticular manifestations in patients with common variable immunodeficiency (CIVD) and to identify the predictive factors., Methods: This was a retrospective and prognostic study conducted in the pediatrics: immuno-hematology and stem cell transplantation department, including patients who fit the definition of CVID. A Cox model analysis was used to identify predictive factors., Results: A total of 36 patients were enrolled. Osteoarticular involvement was noted in 15 patients (42%) with a cumulative incidence of 90% after a median follow-up of 25 years. Non-infectious manifestations were reported in 14 patients (39%). The cumulative risk of inflammatory or autoimmune osteoarticular etiology was 74%. Well-characterized rheumatic diseases were retained in six patients and unlabeled autoimmune or inflammatory mechanism in five cases. Bone mineral density revealed osteoporosis in six cases leading to a cumulative risk of degenerative complications of 72%. The cumulative incidence of infectious complications was 17%. In multivariate analysis, predictors of osteoarticular complications were low body weight (HR = 8.67, CI: 1.496-50.278, p = 0.01) and hepatomegaly at diagnosis (HR = 6.2, CI: 1.537-25.075, p = 0.01). Reduced CD4 cells rate < 600 cells/mm
3 and hepatomegaly were predictors of autoimmune or inflammatory complications, while chronic diarrhea and iron deficiency were associated with degenerative manifestations., Conclusions: Osteoarticular manifestations have emerged as a real health problem for CVID patients. Risk increases with low body weight, hepatomegaly, chronic diarrhea, iron deficiency, and CD4 cells rate under 600 cell/mm3 . Elucidating the mechanisms of these complications in CVID is important for developing preventive strategies. Key Points • This retrospective and prognostic study described the clinical characteristics of osteoarticular manifestations in 36 patients with CVID to ensure better recognition and understanding of this association by clinicians. • Identification of predictive factors of osteoarticular complications according to its etiology is crucial to establish appropriate, optimal and early management of patients at risk., (© 2023. The Author(s), under exclusive licence to International League of Associations for Rheumatology (ILAR).)- Published
- 2023
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30. Long-Term Observational Study of Chronic Granulomatous Disease About 41 Patients From Tunisia and Comparison to Other Long-Term Follow-Up Studies.
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Mellouli F, Ksouri H, Lajhouri M, Ben Khaled M, Rekaya S, Ben Fraj E, Ouederni M, Barbouche MR, and Bejaoui M
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- Child, Preschool, Follow-Up Studies, Humans, Leukocytes, Mononuclear, Quality of Life, Tunisia epidemiology, Anti-Infective Agents, Granulomatous Disease, Chronic complications, Granulomatous Disease, Chronic diagnosis
- Abstract
Chronic granulomatous disease (CGD) is an inherited autosomal recessive or X-Linked primitive immunodeficiency (PID), due to a defective nicotinamide adenine dinucleotide phosphate (NADPH) oxidase complex impairing anti-infectious and anti-inflammatory role of peripheral blood mononuclear cells. It is characterized by severe bacterial and fungal infections and by excessive inflammation leading to granulomatous complications. This work was made over a period of 34 years on 41 Tunisian patients suffering from CGD. Cumulative follow-up of patients was 2768.5 months, median 31 months. Survival was studied by survival curves according to Kaplan-Meier method. Lymphatic nodes, pulmonary and cutaneous infections predominate as revealing manifestations and as infectious events during patients' monitoring. At study end 12 patients died mainly of invasive pulmonary aspergillosis and septicemia. Median age of death was 30 months. CGD remains compatible with a decent quality of life. Early diagnosis, anti-infectious prophylaxis, and initiation of adequate management, as soon as complication is perceived, promote pretty good evolution.
- Published
- 2022
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31. First Observation of HbM-Saskatoon at the Origin of Neonatal Cyanosis in a Tunisian Baby.
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Bouatrous E, Nouira S, Ben Khaled M, Ouederni M, Abbes S, Menif S, and Ouragini H
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- Cyanosis etiology, Cyanosis metabolism, Humans, Infant, Male, Prognosis, Tunisia, Cyanosis pathology, Hemoglobin M genetics, Hemoglobins, Abnormal genetics, Mutation
- Abstract
Several causes are known to be at the origin of neonatal cyanosis among them methemoglobinemia is by inheritance of an hemoglobin (Hb) M variant. This is a rare condition never been reported in Tunisia so far. Here, we report a Tunisian newborn with refractory cyanosis since birth. As cardiac and respiratory diseases were ruled out, methemoglobinemia was suspected. Hematological parameters, concentration of methemoglobin, capillary electrophoresis, and amplification sequencing of the HBB gene were performed. Computational analysis was achieved by different in silico tools to investigate the mutation effect. The diagnosis was established by a raised MetHb, confirmed by the presence HbM-Saskatoon [Beta63 (E7) His>Tyr] by capillary electrophoresis and molecular analysis. The identified mutation occurred as a de novo mutation. In silico analysis confirmed the pathogenicity of the mutation. To our knowledge, this is the first time that this mutation has been reported in the Tunisian population. In view of its low incidence rate, clinicians might misdiagnose cyanosis caused by HbM, which can lead to inappropriate treatment and clinical complications. An up-to-date literature review of HbM disease is presented in this study., Competing Interests: The authors declare no conflict of interest., (Copyright © 2021 Wolters Kluwer Health, Inc. All rights reserved.)
- Published
- 2021
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32. Containment of Local COVID-19 Outbreak Among Hematopoietic Stem Cell Transplant Recipients and Healthcare Workers in a Pediatric Stem Cell Unit.
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Ouederni M, Rekaya S, Bouabdallah O, Ben Fradj I, Kouki R, Chebbi Y, Ammar SB, Lamouchi T, Lachiheb A, Zekri N, Laajili S, Zaiter I, Hamzaoui A, Bejaoui M, Mellouli F, Achour W, and Ben Khaled M
- Subjects
- COVID-19 therapy, Disease Outbreaks, Hematopoietic Stem Cell Transplantation, Hospitals, Pediatric, Humans, Transplant Recipients, Tunisia epidemiology, COVID-19 epidemiology, Health Personnel, SARS-CoV-2 physiology
- Published
- 2021
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33. Case Report: FOXP3 Mutation in a Patient Presenting With ALPS.
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Rais A, Mekki N, Fedhila F, Alosaimi MF, Ben Khaled M, Zameli A, Agrebi N, Sellami MK, Geha R, Ben-Mustapha I, and Barbouche MR
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- Autoimmune Lymphoproliferative Syndrome diagnosis, Autoimmune Lymphoproliferative Syndrome immunology, Child, Humans, Male, Mutation, Autoimmune Lymphoproliferative Syndrome genetics, Forkhead Transcription Factors genetics
- Abstract
ALPS and IPEX are two well-characterized inborn errors of immunity with immune dysregulation, considered as two master models of monogenic auto-immune diseases. Thus, with autoimmunity as their primary clinical manifestation, these two entities may show clinical overlap. Traditionally, immunological biomarkers are used to establish an accurate differential diagnosis. Herein, we describe a patient who presented with clinical features and biomarkers fulfilling the diagnostic criteria of ALPS. Severe apoptotic defect was also shown in the patient's cell lines and PHA-activated peripheral blood lymphocytes. Sanger sequencing of the FAS gene did not reveal any causal mutation. NGS screening revealed a novel deleterious variant located in the N terminal repressor domain of FOXP3 but no mutations in the FAS pathway-related genes. TEMRA cells (terminally differentiated effector memory cells re-expressing CD45RA) and PD1 expression were increased arguing in favor of T-cell exhaustion, which could be induced by unrestrained activation of T effector cells because of Treg deficiency. Moreover, defective FOXP3 observed in the patient could intrinsically induce increased proliferation and resistance to apoptosis in T effector cells. This observation expands the spectrum of FOXP3 deficiency and underscores the role of NGS in detecting mutations that induce overlapping phenotypes among inborn errors of immunity with immune dysregulation. In addition, these findings suggest a potential link between FOXP3 and FAS pathways., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2021 Rais, Mekki, Fedhila, Alosaimi, Ben Khaled, Zameli, Agrebi, Sellami, Geha, Ben-Mustapha and Barbouche.)
- Published
- 2021
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34. Prevalence and predictive factors of splenic sequestration crisis among 423 pediatric patients with sickle cell disease in Tunisia.
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Ben Khaled M, Ouederni M, Mankai Y, Rekaya S, Ben Fraj I, Dhouib N, Kouki R, Mellouli F, and Bejaoui M
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- Acute Disease, Anemia, Sickle Cell diagnosis, Biomarkers, Child, Child, Preschool, Female, Humans, Incidence, Infant, Male, Phenotype, Prevalence, Prognosis, Public Health Surveillance, Risk, Splenic Diseases diagnosis, Splenomegaly, Tunisia epidemiology, Anemia, Sickle Cell complications, Anemia, Sickle Cell epidemiology, Splenic Diseases epidemiology, Splenic Diseases etiology
- Abstract
This study was aimed to identify the predictors of splenic sequestration crisis (SSC) among pediatric patients with sickle cell disease (SCD). This prognosis study was carried out in the pediatric immuno-hematology unit, over 20 years (1998 to 2017), enrolling patients with SCD. The cox model was used in multivariate analysis. Among 423 patients with SCD (240 S/S phenotype, 128 S/B0, 30 S/B+, 14 S/O arab and 11 S/C), 150(35.4%) had at least one episode of SSC. The average age of patients at the first episode was 48.3 months ± 32.4(2-168). Recurrence of SSC was observed in 117 patients (78%). Spleen size ≥3 cm at baseline was the strongest predictor of SSC occurrence (HR = 7.27, CI: 4.01-13.20, p = 0.05) and recurrence (HR = 6.37, CI: 1,46-27.83, p = 0.01). Pallor revealing the disease, age at onset of symptoms <24 months and reticulocytosis ≥300,000/mm
3 increased the risk of SSC. Pain crisis revealing the disease as well as neutrophilia was associated with a lower risk of SSC. In conclusion, this study confirmed the high prevalence of SSC in SCD and the high frequency of recurrence after a first episode. The SSC occurrence and recurrence were intimately linked to the presence of splenomegaly, chronic pallor revealing the disease as well as reticulocytosis., (Copyright © 2019 Elsevier Inc. All rights reserved.)- Published
- 2020
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35. 46th Medical Maghrebian Congress. November 9-10, 2018. Tunis.
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Alami Aroussi A, Fouad A, Omrane A, Razzak A, Aissa A, Akkad A, Amraoui A, Aouam A, Arfaoui A, Belkouchi A, Ben Chaaben A, Ben Cheikh A, Ben Khélifa A, Ben Mabrouk A, Benhima A, Bezza A, Bezzine A, Bourrahouat A, Chaieb A, Chakib A, Chetoui A, Daoudi A, Ech-Chenbouli A, Gaaliche A, Hassani A, Kassimi A, Khachane A, Labidi A, Lalaoui A, Masrar A, McHachi A, Nakhli A, Ouakaa A, Siati A, Toumi A, Zaouali A, Condé AY, Haggui A, Belaguid A, El Hangouche AJ, Gharbi A, Mahfoudh A, Bouzouita A, Aissaoui A, Ben Hamouda A, Hedhli A, Ammous A, Bahlous A, Ben Halima A, Belhadj A, Bezzine A, Blel A, Brahem A, Banasr A, Meherzi A, Saadi A, Sellami A, Turki A, Ben Miled A, Ben Slama A, Daib A, Zommiti A, Chadly A, Jmaa A, Mtiraoui A, Ksentini A, Methnani A, Zehani A, Kessantini A, Farah A, Mankai A, Mellouli A, Zaouali A, Touil A, Hssine A, Ben Safta A, Derouiche A, Jmal A, Ferjani A, Djobbi A, Dridi A, Aridhi A, Bahdoudi A, Ben Amara A, Benzarti A, Ben Slama AY, Oueslati A, Soltani A, Chadli A, Aloui A, Belghuith Sriha A, Bouden A, Laabidi A, Mensi A, Ouakaa A, Sabbek A, Zribi A, Green A, Ben Nasr A, Azaiez A, Yeades A, Belhaj A, Mediouni A, Sammoud A, Slim A, Amine B, Chelly B, Jatik B, Lmimouni B, Daouahi B, Ben Khelifa B, Louzir B, Dorra A, Dhahri B, Ben Nasrallah C, Chefchaouni C, Konzi C, Loussaief C, Makni C, Dziri C, Bouguerra C, Kays C, Zedini C, Dhouha C, Mohamed C, Aichaouia C, Dhieb C, Fofana D, Gargouri D, Chebil D, Issaoui D, Gouiaa D, Brahim D, Essid D, Jarraya D, Trad D, Ben Hmida E, Sboui E, Ben Brahim E, Baati E, Talbi E, Chaari E, Hammami E, Ghazouani E, Ayari F, Ben Hariz F, Bennaoui F, Chebbi F, Chigr F, Guemira F, Harrar F, Benmoula FZ, Ouali FZ, Maoulainine FMR, Bouden F, Fdhila F, Améziani F, Bouhaouala F, Charfi F, Chermiti Ben Abdallah F, Hammemi F, Jarraya F, Khanchel F, Ourda F, Sellami F, Trabelsi F, Yangui F, Fekih Romdhane F, Mellouli F, Nacef Jomli F, Mghaieth F, Draiss G, Elamine G, Kablouti G, Touzani G, Manzeki GB, Garali G, Drissi G, Besbes G, Abaza H, Azzouz H, Said Latiri H, Rejeb H, Ben Ammar H, Ben Brahim H, Ben Jeddi H, Ben Mahjouba H, Besbes H, Dabbebi H, Douik H, El Haoury H, Elannaz H, Elloumi H, Hachim H, Iraqi H, Kalboussi H, Khadhraoui H, Khouni H, Mamad H, Metjaouel H, Naoui H, Zargouni H, Elmalki HO, Feki H, Haouala H, Jaafoura H, Drissa H, Mizouni H, Kamoun H, Ouerda H, Zaibi H, Chiha H, Kamoun H, Saibi H, Skhiri H, Boussaffa H, Majed H, Blibech H, Daami H, Harzallah H, Rkain H, Ben Massoud H, Jaziri H, Ben Said H, Ayed H, Harrabi H, Chaabouni H, Ladida Debbache H, Harbi H, Yacoub H, Abroug H, Ghali H, Kchir H, Msaad H, Ghali H, Manai H, Riahi H, Bousselmi H, Limem H, Aouina H, Jerraya H, Ben Ayed H, Chahed H, Snéne H, Lahlou Amine I, Nouiser I, Ait Sab I, Chelly I, Elboukhani I, Ghanmi I, Kallala I, Kooli I, Bouasker I, Fetni I, Bachouch I, Bouguecha I, Chaabani I, Gazzeh I, Samaali I, Youssef I, Zemni I, Bachouche I, Youssef I, Bouannene I, Kasraoui I, Laouini I, Mahjoubi I, Maoudoud I, Riahi I, Selmi I, Tka I, Hadj Khalifa I, Mejri I, Béjia I, Bellagha J, Boubaker J, Daghfous J, Dammak J, Hleli J, Ben Amar J, Jedidi J, Marrakchi J, Kaoutar K, Arjouni K, Ben Helel K, Benouhoud K, Rjeb K, Imene K, Samoud K, El Jeri K, Abid K, Chaker K, Abid K, Bouzghaîa K, Kamoun K, Zitouna K, Oughlani K, Lassoued K, Letaif K, Hakim K, Cherif Alami L, Benhmidoune L, Boumhil L, Bouzgarrou L, Dhidah L, Ifrine L, Kallel L, Merzougui L, Errguig L, Mouelhi L, Sahli L, Maoua M, Rejeb M, Ben Rejeb M, Bouchrik M, Bouhoula M, Bourrous M, Bouskraoui M, El Belhadji M, El Belhadji M, Essakhi M, Essid M, Gharbaoui M, Haboub M, Iken M, Krifa M, Lagrine M, Leboyer M, Najimi M, Rahoui M, Sabbah M, Sbihi M, Zouine M, Chefchaouni MC, Gharbi MH, El Fakiri MM, Tagajdid MR, Shimi M, Touaibia M, Jguirim M, Barsaoui M, Belghith M, Ben Jmaa M, Koubaa M, Tbini M, Boughdir M, Ben Salah M, Ben Fraj M, Ben Halima M, Ben Khalifa M, Bousleh M, Limam M, Mabrouk M, Mallouli M, Rebeii M, Ayari M, Belhadj M, Ben Hmida M, Boughattas M, Drissa M, El Ghardallou M, Fejjeri M, Hamza M, Jaidane M, Jrad M, Kacem M, Mersni M, Mjid M, Sabbah M, Serghini M, Triki M, Ben Abbes M, Boussaid M, Gharbi M, Hafi M, Slama M, Trigui M, Taoueb M, Chakroun M, Ben Cheikh M, Chebbi M, Hadj Taieb M, Kacem M, Ben Khelil M, Hammami M, Khalfallah M, Ksiaa M, Mechri M, Mrad M, Sboui M, Bani M, Hajri M, Mellouli M, Allouche M, Mesrati MA, Mseddi MA, Amri M, Bejaoui M, Bellali M, Ben Amor M, Ben Dhieb M, Ben Moussa M, Chebil M, Cherif M, Fourati M, Kahloul M, Khaled M, Machghoul M, Mansour M, Abdesslem MM, Ben Chehida MA, Chaouch MA, Essid MA, Meddeb MA, Gharbi MC, Elleuch MH, Loueslati MH, Sboui MM, Mhiri MN, Kilani MO, Ben Slama MR, Charfi MR, Nakhli MS, Mourali MS, El Asli MS, Lamouchi MT, Cherti M, Khadhraoui M, Bibi M, Hamdoun M, Kassis M, Touzi M, Ben Khaled M, Fekih M, Khemiri M, Ouederni M, Hchicha M, Kassis M, Ben Attia M, Yahyaoui M, Ben Azaiez M, Bousnina M, Ben Jemaa M, Ben Yahia M, Daghfous M, Haj Slimen M, Assidi M, Belhadj N, Ben Mustapha N, El Idrissislitine N, Hikki N, Kchir N, Mars N, Meddeb N, Ouni N, Rada N, Rezg N, Trabelsi N, Bouafia N, Haloui N, Benfenatki N, Bergaoui N, Yomn N, Ben Mustapha N, Maamouri N, Mehiri N, Siala N, Beltaief N, Aridhi N, Sidaoui N, Walid N, Mechergui N, Mnif N, Ben Chekaya N, Bellil N, Dhouib N, Achour N, Kaabar N, Mrizak N, Mnif N, Chaouech N, Hasni N, Issaoui N, Ati N, Balloumi N, Haj Salem N, Ladhari N, Akif N, Liani N, Hajji N, Trad N, Elleuch N, Marzouki NEH, Larbi N, M'barek N, Rebai N, Bibani N, Ben Salah N, Belmaachi O, Elmaalel O, Jlassi O, Mihoub O, Ben Zaid O, Bouallègue O, Bousnina O, Bouyahia O, El Maalel O, Fendri O, Azzabi O, Borgi O, Ghdes O, Ben Rejeb O, Rachid R, Abi R, Bahiri R, Boulma R, Elkhayat R, Habbal R, Rachid R, Tamouza R, Jomli R, Ben Abdallah R, Smaoui R, Debbeche R, Fakhfakh R, El Kamel R, Gargouri R, Jouini R, Nouira R, Fessi R, Bannour R, Ben Rabeh R, Kacem R, Khmakhem R, Ben Younes R, Karray R, Cheikh R, Ben Malek R, Ben Slama R, Kouki R, Baati R, Bechraoui R, Fakhfakh R, Fradi R, Lahiani R, Ridha R, Zainine R, Kallel R, Rostom S, Ben Abdallah S, Ben Hammamia S, Benchérifa S, Benkirane S, Chatti S, El Guedri S, El Oussaoui S, Elkochri S, Elmoussaoui S, Enbili S, Gara S, Haouet S, Khammeri S, Khefecha S, Khtrouche S, Macheghoul S, Mallouli S, Rharrit S, Skouri S, Helali S, Boulehmi S, Abid S, Naouar S, Zelfani S, Ben Amar S, Ajmi S, Braiek S, Yahiaoui S, Ghezaiel S, Ben Toumia S, Thabeti S, Daboussi S, Ben Abderahman S, Rhaiem S, Ben Rhouma S, Rekaya S, Haddad S, Kammoun S, Merai S, Mhamdi S, Ben Ali R, Gaaloul S, Ouali S, Taleb S, Zrour S, Hamdi S, Zaghdoudi S, Ammari S, Ben Abderrahim S, Karaa S, Maazaoui S, Saidani S, Stambouli S, Mokadem S, Boudiche S, Zaghbib S, Ayedi S, Jardek S, Bouselmi S, Chtourou S, Manoubi S, Bahri S, Halioui S, Jrad S, Mazigh S, Ouerghi S, Toujani S, Fenniche S, Aboudrar S, Meriem Amari S, Karouia S, Bourgou S, Halayem S, Rammeh S, Yaïch S, Ben Nasrallah S, Chouchane S, Ftini S, Makni S, Manoubi S, Miri S, Saadi S, Manoubi SA, Khalfallah T, Mechergui T, Dakka T, Barhoumi T, M'rad TEB, Ajmi T, Dorra T, Ouali U, Hannachi W, Ferjaoui W, Aissi W, Dahmani W, Dhouib W, Koubaa W, Zhir W, Gheriani W, Arfa W, Dougaz W, Sahnoun W, Naija W, Sami Y, Bouteraa Y, Elhamdaoui Y, Hama Y, Ouahchi Y, Guebsi Y, Nouira Y, Daly Y, Mahjoubi Y, Mejdoub Y, Mosbahi Y, Said Y, Zaimi Y, Zgueb Y, Dridi Y, Mesbahi Y, Gharbi Y, Hellal Y, Hechmi Z, Zid Z, Elmouatassim Z, Ghorbel Z, Habbadi Z, Marrakchi Z, Hidouri Z, Abbes Z, Ouhachi Z, Khessairi Z, Khlayfia Z, Mahjoubi Z, and Moatemri Z
- Subjects
- Africa, Northern epidemiology, Anatomy education, Education, Medical history, Education, Medical methods, Education, Medical organization & administration, History, 21st Century, Humans, Internship and Residency standards, Internship and Residency trends, Job Satisfaction, Pathology, Clinical education, Tunisia epidemiology, Education, Medical trends, Medicine methods, Medicine organization & administration, Medicine trends
- Published
- 2019
36. Cutaneous Manifestations of Primary Immunodeficiency Diseases in Tunisian Children.
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Dhouib NG, Ben Khaled M, Ouederni M, Ben-Mustapha I, Kouki R, Besbes H, Barbouche MR, Mellouli F, and Bejaoui M
- Abstract
Skin manifestations are frequent among patients with primary immunodeficiency diseases (PIDs). Their prevalence varies according to the type of immunodeficiency. This review provides the reader with an up-to-date summary of the common dermatologic manifestations of PIDs among Tunisian children. We conducted a prospective study on two hundred and ninety children with immune deficiency. Demographic details (including age, sex, and consanguinity) with personal and family history were recorded. Special attention was paid to cutaneous manifestations. Dermatological involvements were grouped according to the etiology of their most prominent sign. Cutaneous manifestations were found in 164 patients (56.5%). They revealed the diagnosis of PIDs in 71 patients (24.5 %). The mean age at presentation was 21 months. Overall the most prominent cutaneous alterations were infectious. They accounted for 106 cases (36.55%). The most prevalent causes of cutaneous infections were bacterial: 93 cases (32.06%). Immuno-allergic skin diseases were among the common findings in our study. These include eczematous dermatitis found in 62 cases (21.38%). Malignancy related PIDs was seen in a boy with Wiskott Aldrich syndrome. He developed Kaposi's sarcoma at the age of 14 months. Cutaneous changes are common among children with PIDs. In pediatric patients with failure to thrive, chronic refractory systemic manifestations often present in other family members, recurrent cutaneous infections unresponsive to adequate therapy, atypical forms of eczematous dermatitis or unusual features should arouse the suspicion of PIDs and prompt specialized immunologic consultation should be made., Competing Interests: Competing interests: The authors have declared that no competing interests exist.
- Published
- 2018
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37. Increasing medium chain fatty acids production in Yarrowia lipolytica by metabolic engineering.
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Rigouin C, Croux C, Borsenberger V, Ben Khaled M, Chardot T, Marty A, and Bordes F
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- Fatty Acids metabolism, Metabolic Engineering methods, Yarrowia metabolism
- Abstract
Background: Oleaginous yeast Yarrowia lipolytica is an organism of choice for the development of biofuel and oleochemicals. It has become a chassis for metabolic engineering in order to produce targeted lipids. Understanding the function of key-enzymes involved in lipid metabolism is essential to design better routes for enhanced lipid production and for strains producing lipids of interest. Because medium chain fatty acids (MCFA) are valuable compounds for biokerosene production, we previously generated strains capable of producing MCFA up to 12% of total lipid content (Rigouin et al. in ACS Synth Biol 6:1870-1879, 2017). In order to improve accumulation and content of C14 fatty acid (FA), the elongation, degradation and accumulation of these MCFA in Yarrowia lipolytica were studied., Results: We brought evidence of the role of YALI0F0654 (YlELO1) protein in the elongation of exogenous or de novo synthesized C14 FA into C16 FA and C18 FA. YlELO1 deletion into a αFAS_I
1220 W expressing strain leads to the sole production of C14 FA. However, because this strain does not provide the FA essential for its growth, it requires being cultivated with essential fatty acids and C14 FA yield is limited. To promote MCFA accumulation in Y. lipolytica without compromising the growth, we overexpressed a plant diglyceride acyltransferase specific for MCFA and reached an accumulation of MCFA up to 45% of total lipid content., Conclusion: We characterized the role of YlELO1 in Y. lipolytica by proving its involvement in Medium chain fatty acids elongation. We showed that MCFA content can be increased in Yarrowia lipolytica by promoting their accumulation into a stable storage form (triacylglycerides) to limit their elongation and their degradation.- Published
- 2018
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38. Growth and Endocrine Function in Tunisian Thalassemia Major Patients.
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Dhouib NG, Ben Khaled M, Ouederni M, Besbes H, Kouki R, Mellouli F, and Bejaoui M
- Abstract
β-thalassemia major (β-TM) is among the most common hereditary disorders imposing high expenses on health-care system worldwide. The patient's survival is dependent on lifetime blood transfusion which leads to iron overload and its toxicity in various organs including endocrine glands. This article provides an overview of endocrine disorders in beta-TM patients. This single center investigation enrolled 28 β-TM patients (16 males, 12 females) regularly transfused with packed red cell since early years of life. For each patient were determined: age, sex, number of transfusions received, history of splenectomy and anthropometric parameters. All patients underwent an evaluation of hormonal status including growth, gonadal, thyroid, adrenal cortex, and parathyroid glands. Dual-energy X-ray absorptiometry was used to diagnose low bone mass. Assessment of iron overload status was performed by measuring the serum ferritin concentration and the results of magnetic resonance imaging T
2 *. Growth retardation was found in 16 of the 28 studied patients (57 %). Thirteen among them had delayed puberty. Spontaneous puberty was achieved in 16 cases. Growth hormone (GH) deficiency was found in 10 cases (35 %). Seventeen among the studied patients (60 %) developed disorders of glucose homeostasis. Subclinical hypothyroidism was found in six patients (21 %). Intensive chelation therapy had allowed the reversibility of this complication in five cases. Adrenal Insufficiency was observed in 9 cases (32%). Hypoparathyroidism has occurred in one case. Ten of the 28 studied patients had low bone mass (35%). Twenty-three of the 28 studied patients (82%) had at least one endocrine complication., Competing Interests: Competing interests: The authors have declared that no competing interests exist.- Published
- 2018
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39. A nine-month-old-boy with Atypical Hemophagocytic Lymphohistiocytosis.
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Ouederni M, Ben Khaled M, Rekaya S, Ben Fraj I, Mellouli F, and Bejaoui M
- Abstract
Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening hyperinflammation caused by uncontrolled proliferation of activated lymphocytes and histiocytes. Often, HLH is an acquired syndrome. We report a case of a nine month-old-boy presented with hepatosplenomegaly, severe anemia, thrombocytopenia, hypertriglyceridemia and high hyperferritinemia. These clinical features of HLH prompted a wide range of infectious and auto-immune tests to be performed. After an extensive diagnostic workup, he was referred to the immune-hematologic unit for HLH suspicion with an unknown cause. Primary HLH due to familial lymphohistiocytosis (FLH) was first evoked in front of consanguinity, probable HLH in the family, early onset, and in the absence of a causative pathology like infection or cancer. However, functional tests were normal. Atypical features like the: absence of fever, hypotonia, recurrent diarrhea since diversification, hematuria, and proteinuria suggested an inborn metabolism error with gastrointestinal involvement. Specific tests were performed to reach a final diagnosis., Competing Interests: Competing interests: The authors have declared that no competing interests exist.
- Published
- 2017
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40. Myocardial and liver iron overload, assessed using T2* magnetic resonance imaging with an excel spreadsheet for post processing in Tunisian thalassemia major patients.
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Ouederni M, Ben Khaled M, Mellouli F, Ben Fraj E, Dhouib N, Yakoub IB, Abbes S, Mnif N, and Bejaoui M
- Subjects
- Adolescent, Adult, Child, Cohort Studies, Female, Follow-Up Studies, Humans, Iron Overload diagnostic imaging, Iron Overload epidemiology, Liver diagnostic imaging, Male, Transfusion Reaction, Tunisia epidemiology, Young Adult, beta-Thalassemia diagnostic imaging, beta-Thalassemia epidemiology, Iron Overload blood, Liver metabolism, Magnetic Resonance Imaging statistics & numerical data, Myocardium metabolism, Software, beta-Thalassemia blood
- Abstract
Thalassemia is a common genetic disorder in Tunisia. Early iron concentration assessment is a crucial and challenging issue. Most of annual deaths due to iron overload occurred in underdeveloped regions of the world. Limited access to liver and heart MRI monitoring might partially explain these poor prognostic results. Standard software programs are not available in Tunisia. This study is the first to evaluate iron overload in heart and liver using the MRI T2* with excel spreadsheet for post processing. Association of this MRI tool results to serum ferritin level, and echocardiography was also investigated. One hundred Tunisian-transfused thalassemia patients older than 10 years (16.1 ± 5.2) were enrolled in the study. The mean myocardial iron concentration (MIC) was 1.26 ± 1.65 mg/g dw (0.06-8.32). Cardiac T2* (CT2*) was under 20 ms in 30 % of patients and under 10 ms in 21 % of patients. Left ventricular ejection function was significantly lower in patients with CT2* <10 ms. Abnormal liver iron concentration (LIC >3 mg/g dw) was found in 95 % of patients. LIC was over 15 mg/g dw in 25 % of patients. MIC was more correlated than CT2* to LIC and serum ferritin. Among patients with SF <1000 μg/l, 13 % had CT2* <20 ms. Our data showed that 30 % of the Tunisian thalassemia major patients enrolled in this cohort had myocardial iron overload despite being treated by iron chelators. SF could not reliably predict iron overload in all thalassemia patients. MRI T2* using excel spreadsheet for routine follow-up of iron overload might improve the prognosis of thalassemia major patients in developing countries, such as Tunisia, where standard MRI tools are not available or expensive.
- Published
- 2017
- Full Text
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41. [Effectiveness and acceptance of hydroxyurea in the treatment of severe forms of sickle cell disease: a prospective study of 65 cases].
- Author
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Mellouli F, Chouaibi S, Dhouib N, Ouederni M, Ben Khaled M, Abbes S, and Bejaoui M
- Subjects
- Adolescent, Adult, Anemia, Sickle Cell epidemiology, Child, Child, Preschool, Female, Humans, Male, Severity of Illness Index, Treatment Outcome, Young Adult, Anemia, Sickle Cell drug therapy, Antisickling Agents therapeutic use, Hydroxyurea therapeutic use, Patient Acceptance of Health Care
- Abstract
Background: Sickle cell disease is a serious illness by its complications. For the severe forms, three therapeutic options are actually allowed: transfusion therapy, hydroxyurea and bone marrow transplantation. aim: To evaluate the contribution of hydroxyurea in the management of severe forms of sickle cell disease. methods: It is a prospective study carried out a period of 11 years in "Centre National de Greffe de Moelle Osseuse" of Tunis. They were 65 patients divided into 38 homozygote forms and 27 double heterozygous composite S/β thalassemics. The mean age was 130 months. The failure criterion of the treatment was hospitalization duration more than 15 days/ patient / year or the occurrence of a severe complication of the disease. results: The main indications of hydroxyurea were the prevention of the recidivism of an acute chest syndrome in 8 cases, iterative painful crises, more than 3 events per year, in 53 cases and anemic forms of the disease in 4 cases. We have observed, a rapid and durable improvement in the clinical manifestations and a significant fall of the number of hospitalization days / patient/year from 25.2 days to 2.6 days (p<0.001). The treatment was well tolerated. The rates of foetal hemoglobin have significantly increased from 6.4 to 27.45 % (p<0.001), of hemoglobin from 7.6 to 9.4 g/dl(p<0.001), of the mean corpuscular volume from 80.3 to 99.1 fl (p<0.001), and a significant fall of the white blood cell rate from 15077 to 8170/mm3 (p<0.001), of polynuclear neutrophils from 8015 to 3509/mm3 (p<0.001), and reticulocytes from 693736 to 209837 /mm3(p<0.001) was observed.Ten patients were considered as treatment failure with a failure rate of 15.3%. The main failure etiology was represented with bad observance., Conclusion: Hydroxyurea has a favored place in management of severe forms of sickle cell diseases of the child. Carefully used, with frequent monitoring does not have problems in short range but acceptance studies on the long term mast be undertaken.
- Published
- 2013
42. Mycoplasma pneumoniae associated with severe autoimmune hemolytic anemia in a child with homozygous beta-thalassemia.
- Author
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Dhouib N, Guedhami H, Mellouli F, Ben Khaled M, Kaabi H, Belhassen E, Hmida S, and Bejaoui M
- Subjects
- Child, Preschool, Homozygote, Humans, Male, Severity of Illness Index, beta-Thalassemia genetics, Anemia, Hemolytic, Autoimmune etiology, Pneumonia, Mycoplasma complications, beta-Thalassemia complications
- Published
- 2011
43. [Immune recovery after allogeneic stem cell transplantation: study of 19 patients].
- Author
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Mellouli E, Ben Khaled M, Regaya Z, Dhouib N, Ouederni M, Kouki R, Jenhani F, and Bejaoui M
- Subjects
- Child, Child, Preschool, Female, Humans, Infant, Male, Prospective Studies, Immune System, Lymphocytes, Myeloid Cells, Stem Cell Transplantation
- Abstract
The aim of this study was to access average delays for novogeneration of myeloid and lymphoid cells after allogeneic bone marrow transplantation (BMT) outcome and factors affecting this organization. A prospective analysis over 2 years (01/01/07 to 31/12/08) enrolling 19 children treated with allogeneic intrafamilial bone marrow transplantation. Indications for bone marrow transplantation were: aplastic anemia (3 cases), bemoglobinopathies (9 cases), myelodysplastic syndrome (1 case) and primary immunodeficiency (6 cases). Different conditioning regiments were used according to the indication. The study of immune reconstitution was based on the quantitative determination of immunoglobulin and lymphocyte subpopulation. These tests were routinely requested to 1 month, 2 months, 3 months, 6 months, 9 months and 12 months. The average time of engraftment was 18 days (12-24). A rate of CD4+T lymphocytes>200/mm3 was provided within an average of 2,5 months (1-7). The average time to obtain CD8+T lymphocytes>200/mm3 was 2 months (1-5). The humoral immune reconstitution was made within an average of 2 months (1-4). A report of CD4+/CD8+T lymphocytes>I was obtained within 10 months and a half (1-24). Univaried analysis showed a correlation between the bone marrow sex matched and the faster reorganization of CD8+T cells (p=0.042). A quantity of CD34+>6 10(6)/kg was significantly associated with the recapture of a formula lymphocyte CD4+/CD8+T>1 (p=0.03) Immune recovery post bone marrow transplantation in children begins with myeloid lineage then lymphoid B then lymphoid T The inversion of the report CD4+/CD8+T lymphocytes, seems to be influenced by the high contain of CD34+cells in the graft as well as the type of conditioning.
- Published
- 2010
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