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ABSTRACT: Behçet's disease (BD) is a multisystemic inflammatory disease characterized, on the one hand, by the frequency of benign mucocutaneous and articular manifestations, and on the other hand by the severity of ocular, neurological, vascular and digestive manifestations which may threaten functional or vital prognosis. The male BD predominance is obvious in many retrospective studies and a few studies only have focused on BD in women.
In this study, we report the epidemiological, clinical, outcome and therapeutic data of a series of 71 female BD compared to a series of 111 male BD. This retrospective study was conducted in the internal medicine department of Monastir university hospital over a 19-year period (1990 to 2008), including all patients followed-up and treated for BD.
The comparison of clinical manifestations of BD between men and women showed the predominance of erythema nodosum (P=0.016), necrotic pseudofolliculitis (P=0.001), and joint involvement (P=0.009) in women, while genital ulcers (P=0.039), ocular involvement (P=0.003) and vascular (P=0.002) involvement were more common in men.
Our results confirmed the results of previous studies regarding the benign nature of BD in women compared to men.
La Revue de Médecine Interne 07/2012; 33(10):552-5. · 0.61 Impact Factor
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ABSTRACT: Parathyroid carcinoma (PTC) is a rare parathyroid tumor. We report a case of PTC revealed by erythema nodosum (EN).
A 53-year-old woman was admitted for EN exploration. Biology found hypercalcemia (3 mmol/L) and 184.89 ng/L parathormonemia. Histology following cervicotomy diagnosed PTC. Postoperative course was free of complications. At eight months' follow-up, there was no recurrence of EN.
PCT is rare and life threatening. Paraneoplastic EN is rare, and has not previously been reported in association with PCT. Solid neoplasia and hemopathy should be systematically explored for in case of recurrent EN or resistance to conventional treatment.
European Annals of Otorhinolaryngology, Head and Neck Diseases 04/2011; 128(5):272-4.
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The Journal of Clinical Pharmacology 01/2011; · 2.91 Impact Factor
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ABSTRACT: Gougerot Sjögren syndrome is rare during childhood. Diagnosis in adult patients is usually based on sets of criteria combining clinical, serological, and salivary gland histopathological findings. In the pediatric age group, clinical manifestations might be different from the adult form. We report on 3 cases of childhood Gougerot Sjögren syndrome.
Archives de Pédiatrie 11/2010; 17(11):1531-4. · 0.30 Impact Factor
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ABSTRACT: Periodic fever or hereditary inflammatory fevers are characterized by intermittent inflammatory attacks. Many entities are well recognized today such as familial mediterranean fever (FMF) and hyperimmunoglobulinemia D syndrome (HIDS). We report on the case of a 6-year-old boy referred for evaluation of a recurrent fever associated with chest pain, pneumonitis, or pleuritis since the age of 5 years. Laboratory data showed leukocytosis, a high erythrocyte sedimentation rate, and C-reactive protein; however, a permanent high serum level IgD was noted. Stereotypical episodes of fever appeared every 4-6 weeks, while infectious, malignant, and auto-immune causes were eliminated. A search for the most common mutations of the FMF gene in Tunisian patients (M694V, M680I, V726A, E148Q, M694I, and A744S) were negative. Likewise, urinary leukotriene E(4), which may be increased in HIDS, was normal in this patient. Mevalonate kinase activity in lymphocytes was not assayed. Ethnic origin and clinical presentation suggest FMF with an increased IgD rather than authentic HIDS, in spite of the lack of improvement under colchicine treatment and the negativity of the main mutations involved in FMF.
Archives de Pédiatrie 09/2010; 17(9):1313-6. · 0.30 Impact Factor
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ABSTRACT: We describe a case of a 55-year-old man who presented with pulmonary embolism and who was found to have nephrotic syndrome due to idiopathic membranous nephropathy. There are no other signs of nephrotic syndrome such as edema.
Case Reports in Medicine 01/2010; 2010.
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Revue de Chirurgie Orthopédique et Traumatologique 12/2009; 95(8):772-775.
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ABSTRACT: Vascular endothelial growth factor (VEGF) is important for angiogenesis and inflammation, both of which are codependent and contribute to the pathophysiology of Behçet's disease (BD). In this report, we sought to investigate whether the selected VEGF polymorphisms [-634 C/G, +936 C/T and an 18 bp insertion/deletion (I/D) at -2549 of the VEGF promoter region] are associated with susceptibility and severity of BD in the Tunisian population. One hundred and thirty-five Tunisian BD patients and 157 healthy controls were recruited. The VEGF gene was genotyped by polymerase chain reaction followed by digestion with restriction endonucleases. VEGF serum levels of BD patients and healthy controls were measured by enzyme-linked immunosorbent assay. We found no association between the VEGF polymorphisms and the susceptibility to BD. However, when data were analysed according to the presence of each symptom, we found a positive association between VEGF 18 bp I/D polymorphism and ocular involvement as well as BD severity. Indeed, among the BD patients, the frequency of the 18 bp I/I genotype was less in patients with ocular inflammation (6.1% vs 24.6%, P = 0.007) and in patients with severe BD (9.2% vs 21.4%, P = 0.0014). Moreover, the mean serum VEGF level was considerably higher in BD patients (P < 0.01) than in healthy controls. We suggest that VEGF gene polymorphisms may be involved in the development of the ocular BD as well as the severity of the disease.
Tissue Antigens 01/2009; 72(6):581-7. · 2.59 Impact Factor
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ABSTRACT: Asthma is a complex genetic disorder characterized by chronic airway inflammation. We hypothesized that genetic polymorphisms in chemokines and their receptors alter leukocyte mobilization and may thus influence the risk and severity of childhood asthma. Distributions of the chemokine CCL2-2578G, CCL2-927C, CCR2-V64I, CX3CR1-V249I and CX3CR1-T280M receptor polymorphisms were examined in a case-control study of 121 children with asthma and 226 age-matched healthy controls and then replicated in a family study of 99 simplex families (297 individuals). The case-control study revealed that the CCL2-2578G allele was less frequent in children with than in those without asthma (P=0.0012). No association with asthma was found for the CCL2-927, CCR2 or CX3CR1 polymorphisms. The finding in the family study that the CCL2-2578G allele was transmitted less often by heterozygous parents to their children with asthma (P=0.0016) confirms the association of CCL2-2578G with asthma risk. Biochemical studies indicated that plasma CCL2 concentrations were higher in both patients (P=0.0214) and controls (P=0.001) carrying the G allele than in subjects with other polymorphisms. Both case-control and family-based studies suggest a protective effect of allele CCL2-2578G in Tunisian asthmatic children.
Genes and immunity 08/2008; 9(7):575-81. · 4.22 Impact Factor
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ABSTRACT: Many studies have shown the implication of CD14 and toll-like receptors (TLRs) 2, 4 and 9 in the pathogenesis of asthma or atopy. To evaluate the association of CD14 and TLRs gene polymorphisms with asthma or atopy, 210 asthmatic children, 224 controls and 80 families were enrolled in this study. Six single nucleotide polymorphisms TLR2 (+2408 G-->A), TLR4 (+1196 C-->T), TLR4 (+896 A-->G), TLR9 (-1237 T-->C), TLR9 (-1486 T-->C) and CD14 (-159 C-->T) were genotyped using polymerase chain reaction followed by restriction fragment length polymorphism in the case-control and family study. The -1237C allele in TLR9 gene polymorphisms was associated with increased risk of asthma [odds ratio 1.53, 95% confidence interval (1.03-2.27)], although no statistically significant differences in allele or genotype frequencies of four other TLRs polymorphisms were evident between the asthmatic and control groups. The CD14 -159 C allele was found to be significantly higher in the asthmatic group when compared with controls (P=0.0006<0.05). Transmission disequilibrium test of 80 asthmatic families showed significant transmission of the -159 C allele in the CD14 gene to asthma-affected offspring. It was concluded that TLR9 and CD14 gene polymorphisms may contribute to an inherited predisposition to asthma in Tunisian children.
Tissue Antigens 05/2008; 71(5):417-25. · 2.59 Impact Factor
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ABSTRACT: Several lines of evidence point to a relevant role of IL-18 in the process of asthma. Some studies suggest that the polymorphism in the gene of IL-18 can be involved in many inflammatory and atopic diseases such as asthma. The aim of our study is to estimate the frequency of the IL-18-607 C/A (rs 1946518) promoter polymorphism in Tunisian children with asthma. We investigated whether the presence of this polymorphism -607 C/A was associated with asthma or atopy and whether this polymorphism influenced the severity of asthma in affected children. We examined also the relationship between the IL-18 gene polymorphism and the serum total IgE level. The IL-18/-607 C/A polymorphism was analysed by polymerase chain reaction and restriction fragment-length polymorphism (PCR-RFLP) analysis. A total of 105 asthma patients and 112 controls as part of the whole children population were studied in a case-control study. Among the 105 children with asthma, 40 were also studied for linkage analyses with their respective parents. We noted that the A allele was associated with statistically significant increases in the risk of asthma in the case-control study (odd ratio (OR) = 1.55, 95% confidence interval (CI) 1.03-2.33. Moreover, the A allele was also associated with atopic asthma (P = 0.008), but not with asthma severity. The transmission disequilibrium test (TDT) analysis in this family study did not suggest a preferential transmission of the IL-18/ -607 C/A polymorphism to affected children. There is no correlation between the IgE level and the IL-18 -607 C/A promoter polymorphism. Our data indicate that IL-18 -607 C/A promoter polymorphism is associated with susceptibility to developing asthma in Tunisian population.
International Journal of Immunogenetics 03/2008; 35(1):63-8. · 1.29 Impact Factor
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ABSTRACT: Broncholithiasis is an exceptional condition characterized by the presence of stony formations in the bronchial lumen. We report six cases. Mean age was 41 years. Revealing signs were hemoptysis (n=5), cough (n=5), fever (n=1) and recurrent lower respiratory tract infections (n=1). Physical examination found sonorous rales in two patients and was normal in four. The chest x-ray showed a parenchymal opacity suggestive of calcification in one patient, atelectasia in two, and alveolar images in three. Bronchial endoscopy demonstrated broncholithiasis in one patient, an endobronchial blood clot in one patient with abundant hemoptysis, an endoluminal bud simulating a tumor in two, an inflammatory aspect in one, and was normal in one. Thoracic computed tomography demonstrated broncholithiasis in three patients. Treatment consisted in lobectomy in five patients. The pathology specimen confirmed broncholithiasis in all five and in one revealed caseofollicular lesions of the hillar nodes. Anti-tuberculosis treatment was prescribed for this patient. Therapeutic abstention with regular surveillance was chosen for one patient with an uncomplicated broncholithiasis. Broncholithiasis is an exceptional condition with potentially serious consequences. Certain diagnosis is based on high-resolution computed tomography and endoscopic findings but can nevertheless be a surgical discovery.
Revue de Pneumologie Clinique 05/2007; 63(2):94-9. · 0.24 Impact Factor
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ABSTRACT: Mucoepidermoid cancer is exceptional in the respiratory tract, accounting for only 0.2% of primary lung cancers.
We report three cases of mucoepidermoid carcinoma. The inaugural signs were hemoptysia in a 10-year-old child, recurrent lower respiratory tract infections in a 13-year-old child, and dyspnea with chest pain in a 32-year-old adult. Bronchial fibroscopy disclosed a proximal endobronchial tumor in all three patients. Pathology study of the operative specimen identified low-grade malignant mucoepidermoid carcinoma in the two children and high-grade malignant mucoepidermoid carcinoma in the adult. Surgical resection was performed for the pediatric cases. Outcome was favorable with recurrence-free survival at eight years in the first child. The surgical resection was less radical in the second child due to locoregional extension. This child was lost to follow-up. The clinical course was rapidly fatal in the third patient who presented metastatic spread at diagnosis and died one month later.
The prognosis of mucoepidermoid tumors of the bronchi is closely related to tumor grade and extension at diagnosis. Unlike high-grade mucoepidermoid carcinoma, the progression of low-grade tumors, which predominate in children, is generally slow, enabling good prognosis if diagnosis is established early. Early search for these tumors in patients presenting chronic or recurrent respiratory manifestations would avoid late diagnosis and improve prognosis.
Revue de Pneumologie Clinique 03/2007; 63(1):29-34. · 0.24 Impact Factor
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ABSTRACT: Interleukin-15 (IL-15) is a novel proinflammatory cytokine, involved in the pathogenesis of inflammatory/autoimmune disease. The objective of our study was to measure serum and cerebrospinal fluid (CSF) IL-15 levels in patients with Behçet's disease (BD). CSF/serum IL-15 ratio was introduced to assess the origin of elevated IL-15 levels. We measured serum and CSF-IL-15 levels in 40 patients with BD (20 patients in active stage). Inflammatory and non-inflammatory neurological disease patients acted as controls. Active BD patients have significantly higher serum IL-15 levels (median 10.4 pg/ml; range 5.3-17.4) compared with BD in remission (6.05 pg/ml; 4-10.4) and healthy controls (4.65 pg/ml; 3.9-6.2). Similar serum IL-15 levels were found in active neuro-BD and inflammatory neurological disease (9.5 pg/ml; 5-13). Elevated levels of IL-15 were observed in CSF samples from neuro-BD patients (11 pg/ml; 8.5-15) and inflammatory neurological disease patients (10 pg/ml; 6.5-14) compared with patients with non-inflammatory neurological disease (4 pg/ml; 4-5.5; P < 0.001). Vascular cerebral BD lesions were associated with high CSF/serum IL-15 ratio. Our findings suggest that IL-15 is involved in BD inflammatory process, particularly in vasculitis foci, as an elevated CSF/serum IL-15 ratio characterizes vascular cerebral lesions.
Scandinavian Journal of Immunology 12/2006; 64(6):655-60. · 2.23 Impact Factor
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ABSTRACT: Pulmonary embolism is an unusual complication of Behçet's disease. Our aim study is to analyse epidemiological, physiopathological and evolutive aspects of this condition. Among 153 patients with Behçet's disease according to the criteria of the International Study Group for Behçet's disease, seven (5 men and 2 women mean aged at 26.6 +/- 6 years) were diagnosed as having pulmonary embolism. This was inaugural in 3 cases; for 2 females, pulmonary embolism complicated pregnancy. Cardiac thrombosis was presented in 2 cases and pulmonary aneurysm in 2 patients. Pulmonary infarction has been noted in 4 cases. Protein C, protein S and antithrombin III levels were normal in all cases. One patient was positive for IgG anticardiolipin antibody. The hyperhomocysteinemia has been noted (17 to 30 micromol/) in 5 cases. All our patients were treated successfully by anticoagulation therapy combined with high dose prednisone, colchicine and intravenous cyclophosphamide in 6 patients. Pulmonary embolism is one of the severe and worst prognostic manifestations of the disease. Furthermore, the hyperhomocysteinemia may play an important role in the pathogenesis of such complication. Immunomodulation therapy associated to folate may be beneficial to attenuate this hyperhomocysteinemia especially, when introduced in the early stages.
Archives des maladies du coeur et des vaisseaux 10/2006; 99(9):786-90. · 0.40 Impact Factor
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ABSTRACT: Hemothorax is an exceptional, serious and misleading manifestation of extralobular sequestration. We report the case of a 15-year-old patient who consulted for chest pain and sudden onset dyspnea. The chest x-ray revealed fluid pleural effusion. Pleural puncture produced a bloody fluid and the angioscan showed a hyperdense formation without a systemic artery. Thoracotomy was performed. A left intrapleural pyramidal formation vascularized with a pedicle from the thoracic aorta was identified and sequestrectomy performed. At two years, the patient has remained symptom free. Extralobular sequestration is an exceptional cause of hemothorax. The absence of an aberrant systemic vessel on the angioscan should not rule out the diagnosis of sequestration. Precautions must be taken at surgery to avoid vessel damage.
Revue de Pneumologie Clinique 03/2006; 62(1):27-9. · 0.24 Impact Factor
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ABSTRACT: Behçet's disease and Takayasu's arteritis are both forms of large vessel vasculitis involving autoreactive T cells active against HSP 60.
Behçet's disease is more common than Takayasu's arteritis. Pulmonary aneurysms represent the major complication of pulmonary Behçet's disease and have a poor prognosis, being associated with massive haemoptysis. In situ pulmonary artery thrombus can lead to pulmonary infarction. Superior vena cava thrombosis progresses slowly, allowing the development of a prominent collateral circulation. Vascular inflammation can spread to the mediastinum, the pleura and the lungs with diffuse pulmonary haemorrhages, bronchiolitis and organising pneumonia. Electron beam tomodensitometry and MRI are the best diagnostic techniques for assessing pulmonary vascular lesions. In Takayasu's arteritis the pulmonary arteries are less frequently involved than the aorta. Pulmonary hypertension (PHT) and lung infarcts can complicate pulmonary arterial involvement and the association of malaise, fever and weight loss with PHT is characteristic of the disease. The diagnosis is confirmed by imaging means, particularly MRI, demonstrating parietal vascular inflammation.
Treatment of both conditions is based upon steroids and immunosuppression. Endovascular treatment may be used in a complementary way for aneurysms, arterial occlusion, and vena cava obstruction. However, patients often respond poorly to treatment and clinical trials using TNFalpha blockade, interferon alpha and vasodilators are in progress.
Revue des Maladies Respiratoires 01/2006; 22(6 Pt 1):999-1019. · 0.59 Impact Factor
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ABSTRACT: Introduction : L’asthme est une maladie multifactorielle. Une prédisposition génétique, des anomalies immunogénétiques et une implication des facteurs environnementaux seraient responsables de sa pathogénie. Les polymorphismes des gènes de la glutathion S-transférase (GST) sont des facteurs de risque pour certaines maladies liées à l’environnement. Dans cette étude nous avons examiné l’hypothèse : les polymorphismes au niveau des gènes GSTT1, GSTM1 et GSTP1 sont associés avec l’asthme atopique et non atopique.
Patients et méthodes : la population d’étude comporte 112 individus sains et 115 enfants asthmatiques (62 atopiques et 50 non atopiques, obtenus après diagnostic clinique dans le service du Pr. A Hamzaoui Pav. B Ariana). Les Polymorphismes au niveau des gènes GSTTM1 et GSTT1 ont été détectés par PCR multiplexe. Le polymorphisme Il105Val au niveau du gène GSTP1 a été détecté par la technique (PCR)-RFLP.
Résultat : Les malades avec asthme atopique présentent une forte prévalence du génotype GSTT1*0/*0 comparativement aux malades asthmatiques non atopiques (OR= 0,17 95 % CL 0,06-0,48 ; p = 0,000119). Nous avons trouvé que Les malades présentent une forte prévalence des génotypes GSTM1*0/*0 que le groupe de contrôle (OR= 2,09 95 % CL 1,15-3,78 ; p=0.009). La proportion du génotype homozygote Val105 du gène GSTP1 est inférieure chez les malades asthmatiques par rapport à celle au niveau des témoins (7 % et 12 %). Il n’a y a pas de différence significative dans la répartition du génotype GSTM1*0/*0 ou le génotype homozygote Val105 du gène GSTP1, entre les malades à asthme atopique et non atopique.
Conclusion. Ces résultats suggèrent que le génotype GSTM1*0/*0 et GSTT1*0/*0 pourraient être impliqués dans la pathogénie de l’asthme. Et que le gène GSTP1 pourrait être protecteur contre le développement de cette maladie.
Revue des Maladies Respiratoires. 01/2006; 23(N° 4-C2):P. 123.
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ABSTRACT: We report a case of a 39-year-old woman who presented a right basicervical tumefaction. The chest X rays show a right cervicomediastinal opacity. Ultrasonography and computed tomography failed to describe the nature of the opacity. Because of the strong suspicion of neoplasm or a tuberculosis abscess, a needle aspiration of the cervical mass was performed and surprisingly revealed the presence of a cyst membrane allowing surgical resection. CONCLUSION: Hydatid disease is a likely diagnosis in endemic areas in patients presenting a cystic mediastinal opacity of unknown orign.
Revue de Pneumologie Clinique 11/2005; 61(5 Pt 1):312-4. · 0.24 Impact Factor
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Médecine et Maladies Infectieuses 05/2005; 35(4):228-9. · 0.72 Impact Factor
