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1.
j.tunis.ORL chir. cerv.-fac ; 49: 5-12, 2023. figures, tables
Article in French | AIM (Africa) | ID: biblio-1428019

ABSTRACT

Décrire les caractéristiques radiologiques de la brèche ostéo-méningée (BOM) de l'étage antérieur de la base du crâne. Méthodes: Etude rétrospective, descriptive, monocentrique, colligeant les patients hospitalisés pour prise en charge d'une brèche ostéoméningée (BOM) et explorés par le couple TDM/IRM, du 1er janvier 2012 au 31 Décembre 2021. Résultats: Ont été retenu 23 patients avec un âge moyen de 48 ans et un sex ratio de 0,3. Des antécédents de chirurgie nasosinusienne ou de neurochirurgie ont été notés chez deux patients (9 %). Treize patients (57 %) étaient victimes de traumatisme crânien. Une rhinoliquorrhée était retrouvée dans 21 cas (persistante dans trois cas (12%) et intermittente dans 18 cas (76%)). La découverte de la BOM a été faite suite à une méningite dans quatre cas. Le dosage de la bêta trace a confirmé la présence de LCR chez deux patients. La TDM a confirmé le diagnostic chez 12 patients dont deux patients qui ne présentaient pas de rhinoliquorrhée à l'examen clinique. Elle a aussi permis d'établir le bilan topographique dans ces cas. Elle a suspecté une BOM dans 9 cas et était négative dans deux cas. L'IRM a confirmé le diagnostic dans tous les cas et a permis de: localiser le siège de la fuite de LCR dans les brèches multiples, confirmer le diagnostic deméningoencéphalocèle (trois cas) et objectiver des signes indirects d'hypertension intra-crânienne (HTIC) (quatre cas). Conclusion: L'imagerie en coupes permet de localiser la BOM mais nécessite souvent, une approche par étapes et son interprétation exige une connaissance approfondie de l'anatomie normale et des variantes de la base du crâne. Mots-clés: Liquorrhée, Brèche Ostéoméningée, Chirurgie, TDM, IRM


Subject(s)
Humans , Encephalocele , Diffusion Tensor Imaging , General Surgery , Cerebrospinal Fluid Leak , Rhinorrhea , Neurosurgery
2.
J Med Vasc ; 47(3): 125-132, 2022.
Article in English | MEDLINE | ID: mdl-36055681

ABSTRACT

BACKGROUND: Over the past decades, thrombophilia testing in patients with venous thrombo-embolism has increased tremendously. However, the role of inherited thrombophilie in prediction the risk of recurrence remains controversial. Consequently, it is still unclear whether thrombophilia testing influences decisions regarding duration of anticoagulation in clinical practices. The aim of this study was to evaluate the impact if inherited thrombophilia on venous thrombosis treatment decisions and on predicting the risk of recurrence. METHODS: A retrospective longitudinal study (January 2011-Decembre 2016) including 131 patients with confirmed venous thrombo-embolism referred to the hematology laboratory from the internal medicine department for inherited thrombophilia screening was carried out. RESULTS: The mean age patients was 39.4 years and the sex ratio (M/F) was 0.61. Inherited thrombophilia was confirmed in 27.5% of patients. A long term anticoagulation was decided in 46.9% of patients with thrombophilia. There was no significant difference in the duration of anticoagulation between patients with or without thrombophilia. Thrombosis recurrence was recorded in 16 (17%) patients. The 24 years cumulative incidence of recurrence was 19% in patients with thrombophilia and 17% in those without (plog Rank= 0.6). Inherited thrombophilia was not associated with increased risk of recurrence after treatment withdrawal (Hazard ratio=1.31 IC (0.47-3.63); P=0.6). CONCLUSION: In clinical practice, inherited thrombophilia did not influence anticoagulation duration and was not associated with a higher venous thrombosis risk of recurrence. It seems to be less relevant for decision making than presumed.


Subject(s)
Venous Thromboembolism , Venous Thrombosis , Adult , Anticoagulants/therapeutic use , Humans , Longitudinal Studies , Retrospective Studies , Risk Factors , Thrombophilia , Venous Thromboembolism/diagnosis , Venous Thromboembolism/drug therapy , Venous Thromboembolism/epidemiology , Venous Thrombosis/etiology
3.
j.tunis.ORL chir. cerv.-fac ; 47: 35-40, 2022. figures, tables
Article in French | AIM (Africa) | ID: biblio-1433689

ABSTRACT

Etudier les caractéristiques cliniques, radiologiques et thérapeutiques des goitres plongeants et établir un arbre décisionnel de prise en charge Méthodes: Nous rapportons une étude rétrospective portant sur 67 cas de goitre plongeant colligés sur une période de 27 ans entre 1990 et 2016. Résultats: L'âge moyen des patients était de 53 ± 15,1 ans et le sex-ratio de 0,24. La tuméfaction basicervicale antérieure était le motif de consultation le plus fréquent, rapportée dans 82% des cas associée à des signes de compression dans 31% des cas. Une paralysie récurrentielle unilatérale a été objectivée dans quatre cas. Une radiographie de thorax a montré une opacité médiastinale dans 75% des cas et une déviation trachéale dans 85% des cas. Un scanner cervico-thoracique pratiquée dans 73% des cas a confirmé le diagnostic en objectivant cinq prolongements dépassant la crosse de l'aorte. Le traitement chirurgical était mené par voie cervicale dans 99% des patients et une sternotomie a été réalisée devant l'échec d'extraction par cervicotomie. Une paralysie récurrentielle postopératoire a été observée dans un cas et l'hypoparathyroïdie définitive a été rapportée chez six malades avec un recul moyen de trois ans. Conclusion: Les goitres plongeants sont devenus rares en Tunisie du fait de la prise en charge plus précoce des nodules thyroïdiens. Le scanner cervico-thoracique représente le gold standard pour l'étude et la confirmation du diagnostic du goitre plongeant ainsi que pour l'attitude thérapeutique. L'indication chirurgicale est toujours impérative devant le risque vital qu'il pose.


Subject(s)
Humans , Goiter, Substernal , Laryngeal Nerves , Stellate Ganglion , Tomography, X-Ray Computed , Abortion, Therapeutic , Sternotomy
4.
j.tunis.ORL chir. cerv.-fac ; 47: 13-16, 2022. figures, tables
Article in English | AIM (Africa) | ID: biblio-1433881

ABSTRACT

Purpose: To review the radio-clinical features and to evaluate the outcomes of surgery approaches for the treatment of otosclerosis in pediatric population. Methods: It's a retrospective study including 12 patients under 18 years of age, diagnosed with otosclerosis and using data over a 24 ­ year ­period (1996-2020). A clinical examination, an audiometric assessment and a computed tomography of the temporal bone (CT-Scan) prior to surgery had been performed in all cases. All patients underwent surgical treatment under general anesthesia. Follow ­up was essentially clinical and audiometric with a mean period of 4years. Results: Mean age of our patients was 15.3 with a ratio of 0.5. Only three of them had a family history of otosclerosis. Major functional sign was hearing loss; tinnitus was noted in only 5 cases. CT-Scan had shown typical radiographic evidence of otosclerosis grade Ia (Veillon classification) in 9 cases, grade Ib in 1 case and no abnormalities in 2 cases. Surgery was performed in all cases: 8 patients underwent stapedotomy and 4 had stapedectomy. The audiometric results were good, air-bone gap closure to within 10 dB was achieved in 84% of cases (10 cases) and to within 20dB in 100% of cases at last follow-up. Conclusion: Audiometric and radiological assessments are essential to guide the diagnosis and the treatment of juvenile otosclerosis. Stapes surgery is a good option for closing the air-bone gap in children with bilateral juvenile otosclerosis


Subject(s)
Humans , Otosclerosis , Arthritis, Juvenile , Therapeutics , Stapes Surgery , Critical Care , Hearing Loss
5.
j.tunis.ORL chir. cerv.-fac ; 47(3): 13-16, 2022. tales, figures
Article in English | AIM (Africa) | ID: biblio-1392510

ABSTRACT

Purpose: To review the radio-clinical features and to evaluate the outcomes of surgery approaches for the treatment of otosclerosis in pediatric population. Methods: It's a retrospective study including 12 patients under 18 years of age, diagnosed with otosclerosis and using data over a 24 ­ year ­period (1996-2020). A clinical examination, an audiometric assessment and a computed tomography of the temporal bone (CT-Scan) prior to surgery had been performed in all cases. All patients underwent surgical treatment under general anesthesia. Follow ­up was essentially clinical and audiometric with a mean period of 4years. Results: Mean age of our patients was 15.3 with a ratio of 0.5. Only three of them had a family history of otosclerosis. Major functional sign was hearing loss; tinnitus was noted in only 5 cases. CT-Scan had shown typical radiographic evidence of otosclerosis grade Ia (Veillon classification) in 9 cases, grade Ib in 1 case and no abnormalities in 2 cases. Surgery was performed in all cases: 8 patients underwent stapedotomy and 4 had stapedectomy. The audiometric results were good, air-bone gap closure to within 10 dB was achieved in 84% of cases (10 cases) and to within 20dB in 100% of cases at last follow-up. Conclusion: Audiometric and radiological assessments are essential to guide the diagnosis and the treatment of juvenile otosclerosis. Stapes surgery is a good option for closing the air-bone gap in children with bilateral juvenile otosclerosis


Subject(s)
Otosclerosis , Hearing Loss , Arthritis, Juvenile , Stapes Surgery , Therapeutics , Child Health
7.
J Mycol Med ; 28(1): 211-214, 2018 Mar.
Article in English | MEDLINE | ID: mdl-29519625

ABSTRACT

Histoplasmosis is a fungal infection caused by a dimorphic fungus, Histoplasma capsulatum. We report a first case of disseminated histoplasmosis in a 34-year-old woman, infected with human immunodeficiency virus (HIV), originating from Ivory Coast and living in Tunisia for 4 years. She was complaining from fever, chronic diarrhoea and pancytopenia. The Histoplasma capsulatum var. capsulatum was identified by direct microscopic examination of the bone marrow. She was treated by Amphotericin B, relayed by itraconazole. Even though a regression of symptoms and normalization of blood cell count (BCC), the patient died in a respiratory distress related to CMV hypoxemic pneumonia.


Subject(s)
Bone Marrow/microbiology , HIV Infections/microbiology , Histoplasma/isolation & purification , Histoplasmosis/blood , Histoplasmosis/diagnosis , AIDS-Related Opportunistic Infections/complications , AIDS-Related Opportunistic Infections/epidemiology , AIDS-Related Opportunistic Infections/microbiology , AIDS-Related Opportunistic Infections/virology , Adult , Amphotericin B/therapeutic use , Antifungal Agents/therapeutic use , Communicable Diseases, Imported/epidemiology , Communicable Diseases, Imported/microbiology , Cote d'Ivoire/epidemiology , Fatal Outcome , Female , HIV Infections/complications , HIV Infections/epidemiology , Histoplasma/ultrastructure , Histoplasmosis/epidemiology , Histoplasmosis/microbiology , Humans , Itraconazole/therapeutic use , Microscopy , Respiratory Distress Syndrome/epidemiology , Respiratory Distress Syndrome/microbiology , Tunisia/epidemiology
8.
J Clin Pharm Ther ; 43(1): 52-58, 2018 Feb.
Article in English | MEDLINE | ID: mdl-28921594

ABSTRACT

WHAT IS KNOWN AND OBJECTIVE: The international normalized ratio (INR) is widely used to monitor patients on vitamin K antagonists. This study aimed to assess the agreement of INR values obtained with different thromboplastin/instrument combinations. MATERIAL AND METHODS: International normalized ratio was determined on plasmas from 330 patients undergoing antivitamin K treatment (with acenocoumarol), using two calibration methods and four reagent/instrument combinations: Both Neoplastine CI and Neoplastine CI Plus on STA-R instrument from Diagnostica STAGO, Asnières, France; and both Thromborel S and Innovin on SYSMEX 2100i instrument from Siemens Health Care Diagnostics, Marbung, Germany. The agreement analysis was done using the Bland-Altman plot and the Cohen Kappa coefficient. RESULTS: The mean of the differences between the INR values and the limits of agreement were -0.07 [-0.51 to 0.38] for the Neoplastine CI plus and Neoplastine CI reagents, -0.08 [-1.18 to 1.03] for the Thromborel S and Innovin reagents when the INR was calculated, -0.1 [-1.15 to 0.95] for the Thromborel S and Innovin reagents when the INR was directly calibrated and -0.1 [-0.7 to 0.5] for the Neoplastine CI plus and Thromborel S. Cohen's kappa coefficients were 0.94, 0.76, 0.85 and 0.82, respectively. NEW FINDINGS AND CONCLUSION: The agreement between the four reagent/instrument combinations was high enough to classify patients as inefficaciously or efficaciously anticoagulated. The data interpretation should always be related to the clinical purpose.


Subject(s)
4-Hydroxycoumarins/therapeutic use , Indenes/therapeutic use , Indicators and Reagents/therapeutic use , International Normalized Ratio/methods , Thromboplastin/therapeutic use , Vitamin K/antagonists & inhibitors , Acenocoumarol/therapeutic use , Blood Coagulation/drug effects , Calibration , France , Germany , Healthy Volunteers , Humans , Vitamin K/metabolism , Vitamin K/therapeutic use
9.
Int J Lab Hematol ; 39(5): 502-507, 2017 Oct.
Article in English | MEDLINE | ID: mdl-28497580

ABSTRACT

INTRODUCTION: It has been recently suggested that microparticles (MP) play a role in the pathogenesis of thrombotic complications. This study aimed to assess the contribution of procoagulant activity expressed by circulating MP in thrombotic events in MPN patients. METHODS: Seventy-four MPN patients were enrolled in a trans-sectional study. The MP procoagulant activity was measured using two assays: (i) the thrombin generation (TG) assay used in different conditions with the addition of both tissue factor (TF) and phospholipids (PL) and with the addition of TF or PL alone and (ii) the PROCOAG-PPL assay. RESULTS: The mean age was 62 (26 men and 48 women). The prevalence of thrombotic events was 28%. When comparing patients with thrombosis to those without, age, sex, MPN type, cardiovascular risk factors, and history of thrombosis were not significantly associated with thrombosis. The JAK2 V617F mutation was significantly associated with thrombotic events (90% vs 67%; P=.04). Results from the TG assay and the PROCOAG-PPL assays did not demonstrate a significant association between the MP procoagulant activity and thrombotic events. CONCLUSION: The MP procoagulant activity did not predict thrombosis in MPN patients. The contribution of TG assay in the assessment of the thrombotic risk is still in debate.


Subject(s)
Blood Coagulation , Fusion Proteins, bcr-abl/genetics , Myeloproliferative Disorders/complications , Myeloproliferative Disorders/genetics , Thrombin/biosynthesis , Thrombosis/blood , Thrombosis/etiology , Adult , Aged , Aged, 80 and over , Comorbidity , Female , Humans , Janus Kinase 2/genetics , Male , Middle Aged , Mutation , Risk Assessment , Risk Factors , Thrombosis/diagnosis
10.
Transfus Clin Biol ; 24(1): 15-22, 2017 Feb.
Article in French | MEDLINE | ID: mdl-27955922

ABSTRACT

INTRODUCTION: Despite its lifesaving role, blood transfusion still has risks associated with it. Hemovigilance is a set of surveillance procedures of the transfusion chain intended to promote safe and effective use of blood components. This work aims to present a descriptive analysis of adverse reactions, which were notified over a period of 5 years (incidence and etiology); to identify malfunctions and to propose corrections. MATERIALS AND METHODS: All transfusion adverse reactions accidents reported to the blood bank of the hospital La Rabta (n=120) are explored (clinical and laboratory tests). RESULTS: The average age of patients with transfusion reaction was 51.2 years (25 days to 89 years). The transfusion accident rates ranged from 0.59 to 2.19 accidents/1000 labile blood products (LBP) distributed. The investigations were used to classify 71 % of accidents in different categories. The most prevalent reaction is the hemolytic reaction, n=24 (19.8 %), followed by allergic reactions, n=21 (17.5 %) and non-hemolytic feverish reaction, n=19 (15.8 %). Transfusion reactions of grade 1 severity were the most frequent (n=94); followed by those of grade 3 severity (n=16), accidents grade 4 (n=4) including two cases of acute pulmonary edema, one case of hyperkalemia, and the last case classified undetermined. CONCLUSION: These data are particularly rich in learning lessons. This study identified several levels failures: under-reporting of certain transfusion accidents, malfunctions at certain stages of the transfusion chain. In order to achieve an accurate statement of each transfusion reaction, it is important to plan in-service training.


Subject(s)
Blood Safety , Transfusion Reaction/epidemiology , Adolescent , Adult , Aged , Aged, 80 and over , Child , Child, Preschool , Female , Humans , Incidence , Infant , Infant, Newborn , Male , Middle Aged , Severity of Illness Index , Transfusion Reaction/classification , Tunisia/epidemiology , Young Adult
11.
Article in French | AIM (Africa) | ID: biblio-1264023

ABSTRACT

Introduction : Pendant plusieurs décennies, la pratique de septoplastie chez l'enfant était proscrite par la majorité des chirurgiens par crainte d'altération de la croissance craniofaciale. Actuellement, la septoplastie est de plus en plus indiquée chez l'enfant avec de bons résultats fonctionnels. Le but de ce travail est de révéler les particularités de la septoplastie chez l'enfant, de comparer les différentes techniques opératoires proposées etd'évaluer les résultats obtenus.Méthodes : Notre étude est rétrospective monocentrique transversale portant sur 34 enfants opérés de septoplastie sur une période de 15 ans [2000-2014].Résultats : L'âge moyen de nos patients était de 13 ans avec un sex-ratio de 5,8. Les antécédents d'adénoïdectomie ont été notés dans 9% des cas associés à une amygdalectomie dans 6% des cas. La déviation septale était posttraumatique dans 59% des cas.Elle était de siège antérieur dans 91% des cas. A côté de la déviation de la cloison nasale, nous avons noté une déviation de la pyramide nasale dans 32% des cas et un polype de Killian associé dans 3% des cas. L'indication opératoire était posée sur l'évaluation subjective de la gêne respiratoire et du degré de la déformation nasale. Une obstruction nasale sévère avec réduction importante du flux aérien a été retrouvée dans 73% des cas. Tous nos patients ont été opérés sous anesthésie générale. L'intervention était pratiquée selon la technique de Cottle dans 88% des cas et par voie endoscopique dans 12% des cas. Elle a consisté à une reposition simple de la cloison nasale dans 3% des cas, une dépose repose de la cloison dans 41% des cas, une résection sous muqueuse du cartilage dans 56% des cas. On n'a pas noté de complications majeurs post opératoires.Après un recul moyen de 22 mois, nous avons un bon résultat fonctionnel dans 88% des cas. La persistance de l'obstruction nasale a été notée dans 12% des cas et était en rapport avec la déviation septale dans 6% des cas. Une reprise chirurgicale était indiquée dans 6% des cas.Conclusion :La septoplastie de l'enfant doit être un geste conservateur limité à la zone pathologique. Le suivi post opératoire est clinique et prolongé


Subject(s)
Child , Nasal Septal Perforation/surgery , Otorhinolaryngologic Surgical Procedures , Tunisia
12.
Ann Cardiol Angeiol (Paris) ; 64(2): 113-5, 2015 Apr.
Article in French | MEDLINE | ID: mdl-25638780

ABSTRACT

The multiple exostosis is a hereditary bone tumour. Generally, its complications are benign and are related to compressing surrounding structures such as nerves and vessels. This is the case of a 52-year-old woman with a family history of multiple exostosis, which was complicated by a pseudoaneurysm of the right superficial femoral artery. The delay in diagnosis was allowed to develop this pseudoaneurysm which caused nervous and deep venous compression.


Subject(s)
Aneurysm, False/complications , Aneurysm, False/diagnosis , Exostoses, Multiple Hereditary/complications , Exostoses, Multiple Hereditary/diagnosis , Femoral Artery , Nerve Compression Syndromes/etiology , Sciatic Nerve , Venous Thrombosis/etiology , Aneurysm, False/etiology , Aneurysm, False/surgery , Exostoses, Multiple Hereditary/surgery , Female , Humans , Magnetic Resonance Angiography , Middle Aged , Nerve Compression Syndromes/therapy , Osteotomy/methods , Treatment Outcome , Vascular Surgical Procedures/methods , Venous Thrombosis/therapy
13.
Article in French | AIM (Africa) | ID: biblio-1264013

ABSTRACT

Introduction: Le kyste amygdaloide est une tumeur kystique latero-cervicale haute rare; issue de la 2eme fente branchiale. Il represente 2 des tumeurs latero-cervicales du cou; et 6;1a 85;2 des anomalies de la deuxieme fente. La forme oropharyngee est tres rare. But : Analyser les caracteristiques anatomo-cliniques et discuter les modalites de prise en charge et les indications therapeutiques de cette affection. Methode : etude retrospective menee sur une periode de 10 ans (2000- 2009) a propos de 31 cas de kystes amygdaloides colliges au service d'ORL et de chirurgie cervico-faciale de La Rabta. Resultat : L'age moyen de nos patients etait de 28 ans et 7 mois avec un sex-ratio de 0;47. La duree d'evolution etait de 14 mois. L'examen physique a trouve une masse submandibulaire dans 2 cas; retro-angulo-mandibulaire dans 2 cas et jugulo-carotidienne dans 27 cas. Vingt neuf patients ont eu une echographie cervicale ayant montre une masse kystique dans 23 cas. La TDM cervicale a ete pratiquee dans 13 cas; faisant evoquer le diagnostic dans 7 cas. Une masse liquidienne parapharyngee a ete retrouvee dans un cas motivant la pratique d'un examen radiologique complementaire par une IRM cervicale. Une ponction cytologique a ete pratiquee dans 16 cas; ayant montre un materiel kystique dans 12 cas. Trente patients ont eu un traitement chirurgical avec exerese complete du kyste. Une mise a plat de la collection paraphryngee a ete pratiquee dans un cas. L'evolution a ete favorable dans 29 cas avec un recul moyen de 2ans et 9 mois. Deux cas de recidive ont ete notes. Conclusion : Les kystes amygdaloides sont des malformations relativement rares; leur diagnostic est suspecte a l'examen physique oriente par les donnees de l'imagerie et confirmee par l'examen anatomopathologique. Le traitement est chirurgical; seule l'exerese complete du kyste permet de prevenir les recidives ulterieures


Subject(s)
Branchioma , Branchioma/diagnosis , Branchioma/surgery , Oropharynx
14.
Cancer Radiother ; 17(7): 668-70, 2013 Nov.
Article in English | MEDLINE | ID: mdl-24176664

ABSTRACT

Multiple myeloma, also known as Kahler's disease, is a cancer of plasma cells that abnormally accumulate in bone marrow. Radiation therapy may be indicated in multiple myeloma to relieve pain, alleviate spinal cord compression or reduce tumor mass. We report the case of a patient presenting a multiple myeloma, treated with radiation therapy for a spinal cord compression, who developed, eleven months later, an in-field subcutaneous relapse. We called this phenomenon myeloma appearance after local external beam radiotherapy jet (MAALEJ). A review of literature was performed trying to explain this uncommon relapse feature.


Subject(s)
Multiple Myeloma/radiotherapy , Skin Neoplasms/pathology , Female , Humans , Middle Aged , Multiple Myeloma/complications , Neoplasm Recurrence, Local/pathology , Neoplasm Recurrence, Local/radiotherapy , Skin Neoplasms/radiotherapy , Spinal Cord Compression/etiology , Spinal Cord Compression/therapy
15.
J Fr Ophtalmol ; 36(6): 488-93, 2013 Jun.
Article in French | MEDLINE | ID: mdl-23582983

ABSTRACT

PURPOSE: Orbital complications of sinusitis are rare but serious, with potential functional and even fatal consequences. The purpose of this work is to study the epidemiological, clinical and paraclinical features of the orbital complications of sinusitis, as well as the various treatment modalities and clinical course. PATIENTS AND METHODS: We report the results of a retrospective study of 34 cases of orbital complications of sinusitis, collected between 1998 and 2009. RESULTS: Mean age was 19 years 3 months, with a clear male predominance. No predisposing factors were found. All patients presented with periorbital edema. In addition to periorbital edema, ophthalmological examination also revealed: proptosis in 19 patients, decreased visual acuity in eight patients and ocular motility disturbances in 10 patients. All patients underwent emergent facial CT, two patients underwent orbital ultrasound, and one underwent magnetic resonance imaging. Initial treatment was based on empiric intravenous antibiotic therapy. Orbital drainage was performed in 20 patients. Sinus drainage was performed in 16 patients (14 cases during the acute phase) with a middle meatotomy in all cases. The initial response was favorable in 97.5% of cases. One patient had a recurrence. Mean follow-up was 11 months. CONCLUSION: Orbital complications of sinusitis are a diagnostic and therapeutic emergency. Imaging helps guide management. Directed treatment and early management can improve mortality and morbidity.


Subject(s)
Orbital Diseases/epidemiology , Orbital Diseases/etiology , Sinusitis/complications , Sinusitis/epidemiology , Abscess/complications , Abscess/diagnostic imaging , Abscess/epidemiology , Abscess/etiology , Adolescent , Adult , Cohort Studies , Exophthalmos/complications , Exophthalmos/diagnostic imaging , Exophthalmos/epidemiology , Exophthalmos/etiology , Female , Humans , Magnetic Resonance Imaging , Male , Orbital Diseases/diagnostic imaging , Retrospective Studies , Sinusitis/diagnostic imaging , Tomography, X-Ray Computed , Young Adult
16.
Mol Biol Rep ; 40(2): 851-6, 2013 Feb.
Article in English | MEDLINE | ID: mdl-23065279

ABSTRACT

Glucose-6-phosphate dehydrogenase (G6PD) deficiency is the most common human enzyme defect. In this study, we aimed to perform a molecular investigation of G6PD deficiency in Tunisia and to associate clinical manifestations and the degree of deficiency with the genotype. A total of 161 Tunisian subjects of both sexes were screened by spectrophotometric assay for enzyme activity. Out of these, 54 unrelated subjects were selected for screening of the most frequent mutations in Tunisia by PCR/RFLP, followed by size-based separation of double-stranded fragments under non-denaturing conditions on a denaturing high performance liquid chromatography system. Of the 56 altered chromosomes examined, 75 % had the GdA(-) mutation, 14.28 % showed the GdB(-) mutation and no mutations were identified in 10.72 % of cases. Hemizygous males with GdA(-) mutation were mostly of class III, while those with GdB(-) mutation were mainly of class II. The principal clinical manifestation encountered was favism. Acute hemolytic crises induced by drugs or infections and neonatal jaundice were also noted. Less severe clinical features such as low back pain were present in heterozygous females and in one homozygous female. Asymptomatic individuals were in majority heterozygote females and strangely one hemizygous male. The spectrum of mutations seems to be homogeneous and similar to that of Mediterranean countries; nevertheless 10.72 % of cases remain with undetermined mutation thus suggesting a potential heterogeneity of the deficiency at the molecular level. On the other hand, we note a better association of the molecular defects with the severity of the deficiency than with clinical manifestations.


Subject(s)
Glucosephosphate Dehydrogenase Deficiency/genetics , Glucosephosphate Dehydrogenase/genetics , Mutation, Missense , Adolescent , Adult , Amplified Fragment Length Polymorphism Analysis , Child , Child, Preschool , DNA Mutational Analysis , Female , Gene Frequency , Genetic Association Studies , Heterozygote , Humans , Male , Tunisia , Young Adult
17.
Cancer Radiother ; 16(7): 627-32, 2012 Oct.
Article in French | MEDLINE | ID: mdl-23084987

ABSTRACT

PURPOSE: To assess the epidemiological, clinical and therapeutic aspects of pediatric Hodgkin disease (HD) and to study prognosis factors. PATIENTS AND METHODS: We retrospectively reviewed the medical records of children (≤18 years) with HD treated between 1st January 1994 and 31st December 2004. Chemotherapy was undertaken in different departments of hematology and oncology in the North of the country and radiotherapy was centralized at the Salah-Azaïz National Cancer Institute. RESULTS: One hundred fourteen consecutively treated patients were collected. Median age was 12 years (4-18 years) and sex-ratio was 2.25. Peripheral lymphadenopathy was the predominant circumstance of HD detection (82.5%). The predominant histologic type was nodular sclerosing (56%). Treatment included chemotherapy and involved-field radiotherapy. With a mean follow-up of 23.5 months, relapse rate was 12.2%. Five-year overall survival (OS) and event-free survival (EFS) rates were 95% and 76%, respectively. Five-year OS was 98.2% and 90.8% for early and advanced stages respectively. In multivariate analysis, stage IV (P=0.029) and early response to initial treatment (P=0.003) retained statistical significance for EFS whereas the only prognostic factor for OS was stage IV (P=0.002). The long-term side effects were rare. No secondary tumor was noted. CONCLUSION: Combined-modality therapy using chemotherapy and involved-field radiotherapy was effective and well-tolerated in early stage pediatric HD. Stage IV patients should be referred to specialized units for intensive treatment. The short median follow-up in our study cannot allow considering long-term effects.


Subject(s)
Hodgkin Disease/epidemiology , Adolescent , Anemia, Aplastic/etiology , Antineoplastic Combined Chemotherapy Protocols/administration & dosage , Antineoplastic Combined Chemotherapy Protocols/adverse effects , Antineoplastic Combined Chemotherapy Protocols/therapeutic use , Bleomycin/administration & dosage , Bleomycin/adverse effects , Cardiomyopathy, Dilated/chemically induced , Chemoradiotherapy , Child , Child, Preschool , Cisplatin/administration & dosage , Cisplatin/adverse effects , Cyclophosphamide/administration & dosage , Cyclophosphamide/adverse effects , Doxorubicin/administration & dosage , Doxorubicin/adverse effects , Etoposide/administration & dosage , Etoposide/adverse effects , Female , Hodgkin Disease/therapy , Humans , Ifosfamide/administration & dosage , Ifosfamide/adverse effects , Male , Mechlorethamine/administration & dosage , Mechlorethamine/adverse effects , Photons/therapeutic use , Prednisone/administration & dosage , Prednisone/adverse effects , Procarbazine/administration & dosage , Procarbazine/adverse effects , Radiotherapy Dosage , Remission Induction , Retrospective Studies , Risk Factors , Treatment Outcome , Tunisia/epidemiology , Vincristine/administration & dosage , Vincristine/adverse effects
18.
Rev Stomatol Chir Maxillofac ; 113(6): 437-41, 2012 Dec.
Article in French | MEDLINE | ID: mdl-22818665

ABSTRACT

INTRODUCTION: Paranasal sinuses mucoceles are extremely rare in children and adolescents. The objective of this study was to assess their clinical presentations and the contribution of endoscopic surgery in their management. MATERIAL AND METHODS: we retrospectively reviewed 11 children with paranasal sinus mucoceles who were managed during a period of 15 years (1995-2009). RESULTS: The mean age was 11.8 years with a sex ratio of 2.66. One patient had a history of nasal polyposis surgery. The symptomatology was dominated by ophthalmological complains. The ethmoïd sinus was the main localization. Orbital extension was found in eight cases, pyomucocele in one case. The surgical management was endoscopic (nine cases), and by an external approach (two cases). Two cases of recurrence were assessed. The mean follow-up was 22.3 months. DISCUSSION: Paranasal sinuses mucocele is exceptional in children. Cystic fibrosis is the main etiologic factor. The symptoms depend on the location and size of the mucocele. The treatment is surgical and most authors prefer the endoscopic approach.


Subject(s)
Mucocele/surgery , Paranasal Sinus Diseases/surgery , Adolescent , Child , Child, Preschool , Cystic Fibrosis/complications , Endoscopy/methods , Ethmoid Sinus/pathology , Exophthalmos/diagnosis , Female , Follow-Up Studies , Humans , Magnetic Resonance Imaging , Male , Mucocele/diagnosis , Nasal Obstruction/diagnosis , Nasal Polyps/surgery , Orbital Diseases/diagnosis , Orbital Diseases/surgery , Paranasal Sinus Diseases/diagnosis , Recurrence , Retrospective Studies , Rhinitis/diagnosis , Tomography, X-Ray Computed
19.
Rev Med Interne ; 32(6): e79-80, 2011 Jun.
Article in French | MEDLINE | ID: mdl-20708307

ABSTRACT

Neurological manifestations in polycytemia vera are common. However, chorea is an exceptionally revealing feature of this disease. We report a 78-year-old man who presented with headache and an abnormal movement disorder corresponding to chorea. Laboratory findings showed increased levels of hemoglobin at 20 g/dl and hematocrit at 62.3%. An elevated erythrocyte mass to twice the normal value demonstrated the absolute erythrocytosis. A JAK2 V617F gene mutation was identified. A diagnosis of polycytemia vera-associated chorea was obtained. Clinical and biological outcomes were favorable after therapeutic phlebotomy and treatment with hydroxyurea. We recommend a complete blood cell count in elderly patient presenting with chorea to eliminate a diagnosis of polycytemia vera.


Subject(s)
Chorea/etiology , Polycythemia Vera/complications , Polycythemia Vera/diagnosis , Aged , Humans , Male
20.
Pathol Biol (Paris) ; 59(4): 240-2, 2011 Aug.
Article in English | MEDLINE | ID: mdl-20674193

ABSTRACT

The aim of this prospective study was to investigate the involvement of angiogenesis in the etiopathogenesis of the different classes of erythrocytosis (polycythemia vera PV, idiopathic erythrocytosis and secondary erythrocytosis). The angiogenic activity was evaluated by the assessment of the serum VEGF levels in 78 untreated erythrocytosis patients and 21 healthy subjects. The results indicated that VEGF was overproduced in advanced and untreated PV patients and at less degree, in early PV, a subgroup of idiopathic erythrocytosis, thus confirming an increased angiogenic activity. However, VEGF does not play an angiogenic role in secondary erythrocytosis.


Subject(s)
Polycythemia/blood , Vascular Endothelial Growth Factor A/blood , Adult , Aged , Aged, 80 and over , Female , Humans , Male , Middle Aged , Platelet Count , Polycythemia/therapy , Polycythemia Vera/blood , Prospective Studies
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