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6. 65 First francophone e-learning of pediatric rheumatology in Africa: the trainers’ opinion

Author

Cherif Ines, Ferjani Hanene, Khamessi Ichrak, Maatallah Kaouther, Guedri Rahma, Miladi Saoussen, Ben Khaled Monia, Fazaa Alia, Jallouli Manel, Ben Abdelghani Kaouther, Khemiri Monia, Fitouri Zohra, and Hamdi Wafa

Subjects
Rheumatology, Pharmacology (medical)
Abstract

Background Pediatric Rheumatology is currently gaining importance across Africa. Initiatives such as the creation of the paediatric society of the African league against rheumatism (PAFLAR) proved the efforts made to bridge the insufficiency. In the same line, training in pediatric rheumatology remains the main challenge of this orphan subspecialty. For this purpose, a post-graduate certification in Pediatric Rheumatology was created in 2021 in the Faculty of Medicine of Tunis—Tunis-El Manar University. It is the first online certification dedicated to French speakers in Africa. The certification is entitled “musculoskeletal diseases of children and adolescents”. Objective To assess the learners' perceptions and views of this certification. Methods We established an online survey targeting the participants. The questionnaire was divided into three main sections: the first section examined the participant characteristics: their specialty, the use of the Moodle platform of the Virtual University of Tunis (UVT), their previous participation in an online post-graduation course, etc.). The second section focused on platform acceptability. The third and final section evaluated the content of the course. The Likert scale was used in most questions. Results Fifteen participants responded to the survey. Among them, one is specialized in family medicine, 1 in orthopedics, 5 in pediatrics, and 8 in rheumatology. Fifty-three percent have never used the platform of the virtual university of Tunis (UVT) before, and 26.7% have never attended an online course. Most participants found the platform easy to access and easy to use with a mean Likert scale of 2.2 and 2.13, respectively. In the third section of the questionnaire, the course met the expectations of the candidates with a mean Likert scale of 2.1. Most of them found the post-test useful to their learning experience, and only two found it to be the opposite. This can be explained by the pressure that it may generate. Eighty-six percent of the candidates had an interactive experience with the educator and enjoyed the interactions and the discussions between the participants. Most participants were pleased with the seminar organization and with the number of h devoted to each seminar (mean Likert Scale = 2.1). Eighty-six percent of the participants have found that the course improved their medical practice and 93.3% confessed that they were satisfied with the online nature of the certification and did not wish it to be physical. Conclusion Overall, the post-graduate “musculoskeletal diseases of children and adolescents” course was beneficial and enriching/rewarding for the participants. Developing this training program helps to promote pediatric rheumatology in Africa and to provide children with rheumatic diseases access to healthcare.

Published
2022
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8. Case Report: FOXP3 Mutation in a Patient Presenting With ALPS

Author

Rais, Afef, primary, Mekki, Najla, additional, Fedhila, Faten, additional, Alosaimi, Mohammed Faraj, additional, Ben Khaled, Monia, additional, Zameli, Amal, additional, Agrebi, Nourhen, additional, Sellami, Maryam Kallel, additional, Geha, Raif, additional, Ben-Mustapha, Imen, additional, and Barbouche, Mohamed-Ridha, additional

Published
2021
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10. Myocardial and liver iron overload, assessed using T2* magnetic resonance imaging with an excel spreadsheet for post processing in Tunisian thalassemia major patients

Author

Ouederni, Monia, Ben Khaled, Monia, Mellouli, Fethi, Ben Fraj, Elhem, Dhouib, Nawel, Abbes, Selem, Ben Yakoub , Ismehen, Mnif, Najla, Bejaoui, Mohamed, Yakoub, Ismehen Ben, Faculty of Medicine of Tunis, Université de Tunis El Manar (UTM), Department of Peadiatric Immuno-Haematology, National Bone Marrow Transplantation, Laboratoire d'hématologie moléculaire et cellulaire (LR11IPT07), Institut Pasteur de Tunis, Réseau International des Instituts Pasteur (RIIP)-Réseau International des Instituts Pasteur (RIIP), Department of Radiology, and Hôpital Charles Nicolle [Tunis]

Subjects
Adult, Male, medicine.medical_specialty, Liver Iron Concentration, Iron Overload, Tunisia, Adolescent, [SDV]Life Sciences [q-bio], Thalassemia, Gastroenterology, 030218 nuclear medicine & medical imaging, Cohort Studies, Young Adult, 03 medical and health sciences, 0302 clinical medicine, Internal medicine, medicine, Humans, Liver iron, Child, Serum ferritin, ComputingMilieux_MISCELLANEOUS, Hematology, medicine.diagnostic_test, business.industry, Myocardium, beta-Thalassemia, Serum ferritin level, Transfusion Reaction, Magnetic resonance imaging, General Medicine, medicine.disease, Magnetic Resonance Imaging, 3. Good health, Surgery, Liver, Cohort, Female, business, Software, Follow-Up Studies, 030215 immunology
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 (LIC3 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 SF1000 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
2016
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11. diagnostic et prise en charge des syndromes drépanocytaires majeurs

Author

BEN KHALED Monia

Subjects
anémie hémolytique, pédiatrie, syndrome drépanocytaire majeur
Abstract

Il s'agit d'une présentation power point sur "les syndromesdrépanocytaires majeurs" qui s'adresse aux internes, résidents en pédiatrie, étudiants de 3 ème et 4 ème année Médecine.Elle a pour objectifs: •Expliquer la physiopathologie des syndromes drépanocytaires majeurs •Annoncer le diagnostic de syndrome drépanocytaire majeur aux parent •Procéder à une éducation des parents d’un enfant ayant un syndrome drépanocytaire majeur •Traiter et prévenir les complications infectieuses

Published
2018
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14. GROWTH AND ENDOCRINE FUNCTION IN TUNISIAN THALASSEMIA MAJOR PATIENTS

Author

Dhouib, Naouel Guirat, Ben Khaled, Monia, Ouederni, Monia, Besbes, Habib, Kouki, Ridha, Mellouli, Fethi, and Bejaoui, Mohamed

Subjects
Delayed puberty, medicine.medical_specialty, Thalassemic Syndromes , endocrine disorders, Thalassemia, Gastroenterology, 03 medical and health sciences, 0302 clinical medicine, Internal medicine, medicine, Adrenal insufficiency, Endocrine system, Glucose homeostasis, Thalassemic syndromes, lcsh:RC633-647.5, business.industry, 05 social sciences, 050301 education, lcsh:Diseases of the blood and blood-forming organs, Hematology, medicine.disease, Infectious Diseases, Hypoparathyroidism, Endocrine disorders, Original Article, medicine.symptom, business, 0503 education, 030215 immunology, Endocrine gland, Hormone
Abstract

Beta-thalassemia major (TM) is among prevalent 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 on various organs including endocrine glands. This article provides an overview of endocrine disorders in beta-TM patients. This single center investigation enrolled 28 beta-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. Evaluation for hormonal status including growth, gonadal, thyroid, adrenal cortex, and parathyroid glands was done for all patients. Dual energy X-ray absorptiometry was used to diagnose osteoporosis. Assessment of iron overload status was performed by measuring the serum ferritin concentration and the results of magnetic resonance imaging T2*. 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 found in 9 cases (32%). Hypoparathyroidism has occurred in one case. Ten of the 28 studied patients had osteoporosis (35%). Twenty-three of the 28 studied patients (82%) had at least one endocrine complication.

Published
2018
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17. Comparative resource analyses for ethanol produced from corn and sugarcane in different climatic zones.

Author

Garoma, T., Ben-Khaled, Monia, and Beyene, A.

Subjects
*ETHANOL, *CHEMICAL alcohol synthesis, *UTILIZATION of corn, *CROP yields, *SUGARCANE, *RESOURCE allocation, *CLIMATIC zones, *CROPS, *NITROGEN
Abstract

SUMMARY Comparative analysis of resource input for ethanol produced from corn and sugarcane in temperate, dry, and tropical climate zones was conducted. Parameters such as the Net Energy Value (NEV), water requirement, land requirement, carbon dioxide emission with and without impact of changes in land use, and fertilizer released to the environment-as surface runoff for nitrogen and phosphate, were compared for corn and sugarcane ethanol production. The estimates of NEV for corn ethanol varied from −462 to 1757 kJ l−1, while those of sugarcane ethanol were between 16 057 and 17 092 kJ l−1 for the three climatic zones considered in this study. The results of the study also indicate that ethanol produced from sugarcane uses less or comparable amount of resources in contrast to ethanol produced from corn. Copyright © 2011 John Wiley & Sons, Ltd. [ABSTRACT FROM AUTHOR]

Published
2012
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18. 46th Medical Maghrebian Congress. November 9-10, 2018. Tunis.

Author

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

19. Cutaneous Manifestations of Primary Immunodeficiency Diseases in Tunisian Children.

Author

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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20. A nine-month-old-boy with Atypical Hemophagocytic Lymphohistiocytosis.

Author

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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21. [Effectiveness and acceptance of hydroxyurea in the treatment of severe forms of sickle cell disease: a prospective study of 65 cases].

Author

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

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