Silvia Mahjoub

Oxidative stress: Correlation with Behçet's disease duration, activity and severity

OBJECTIVE To study the oxidant/antioxidant status in Behçets disease (BD) patients and the potential link between antioxidant enzymatic defences impairment and the disease duration, activity and severity. METHODS 40 BD patients (27 males, 13 females; mean age: 38.8 years) were prospectively enrolled in the study and compared to a sex and age matched control group. Plasma malondialdehyde (MDA), the reduced glutathione (GSH)/oxidised glutathione (GSSG) ratio, erythrocyte superoxide dismutase, catalase and glutathione peroxidase (GSH-PX) were analysed in both groups. A correlation analysis was performed between these parameters and disease duration, activity and severity. Activity and severity of BD were assessed meaning two respective clinical scores. RESULTS When compared to controls MDA was increased and GSH/GSSG reduced in BD patients (respectively p<0.005). Superoxide dismutase (SOD) was significantly lower while catalase was significantly higher in BD than in controls. Correlation analysis showed that SOD activity was significantly and negatively correlated to disease duration and activity but not to severity. Other oxidant/antioxidant markers were not significantly linked to neither disease duration nor activity and severity. CONCLUSION Our study confirm the existence of an oxidative stress (OS) state in BD as shown by the increase of MDA and the diminution of GSH/GSSG ratio which can be used as another index of OS. Despite this OS state, the activity of antioxidant enzymes and especially of SOD is impaired and negatively correlated to the disease duration and activity. We think that a rational strengthening of antioxidant defences should be part of an optimal treatment strategy.

Co-existing sarcoidosis and Takayasu arteritis: report of a case

Introduction Takayasu arteritis (TA) is a chronic vasculitis of unknown origin, affecting mainly the aorta and its main branches. As a result of the inflammation, stenosis, occlusion or dilatation of the involved vessels may occur and cause a wide range of symptoms. It has been described in association with various auto-immune disorders (mainly inflammatory digestive tract diseases). However, only few cases of TA associated with sarcoidosis have been reported, raising the question of an association by chance. Case report We report a case of a 34 year-old woman, with one year history of sarcoidosis, who presented with asymmetric high hypertension revealing inflammatory humeral, axillary and subclavian arteritis related to TA, successfully treated by steroid and immunosuppressive therapy(MethotrexateR). Conclusion TA and sarcoidosis may be related, rising the hypothesis that TA or Takayasu arteritis-like granulomatous vasculitis may be, in fact, a complication of sarcoidosis.

Gastrointestinal involvement revealing Henoch Schonlein purpura in adults: Report of three cases and review of the literature

The diagnosis of Henoch-Schönlein purpura (HSP) is difficult, especially when abdominal symptoms precede cutaneous lesions. We report three cases of adult HSP revealed by gastrointestinal (GI) involvement.

Managing the risk of cancer in Cowden syndrome: a case report

IntroductionCowden syndrome is a rare cancer predisposition syndrome inherited in an autosomal-dominant fashion. The syndrome is characterized by hamartomatous polyps that affect multiple organs: skin, mucous membranes, thyroid, breast, gastrointestinal tract, endometrium and brain. It is also associated with an increased risk of developing malignancy in many tissues but especially breast, thyroid and endometrium.Case presentationWe present the case of a 30-year-old Tunisian woman with mental retardation who presented to our facility with rectal hamartomatous polyps. Her medical history included fibrocystic disease of the breast over the last three years. A physical examination revealed macrocephaly, hyperkeratotic papules on the mid-facial skin, palmoplantar keratosis and oral mucosal papillomatosis. A breast examination revealed nodular breast tissue bilaterally and a diffuse thyroid goiter. Our patient was clinically euthyroid. A total thyroidectomy was performed. A histopathologic examination revealed thyroid papillary carcinoma. A gastrointestinal evaluation revealed esophageal and gastric polyps. Biopsies showed hyperplastic and adenomatous lesions associated with Helicobacter pylori. A final diagnosis of Cowden syndrome was made according to the syndrome testing criteria adapted by the US National Comprehensive Cancer Network. A prophylactic bilateral mastectomy was proposed but refused by our patient. Our patient was kept under surveillance for breast and colorectal malignancies.ConclusionsEarly and accurate diagnosis of Cowden syndrome is essential because it is a cancer predisposition syndrome that carries an increased risk for developing malignancy in many tissues, especially breast and thyroid. For this reason, education regarding the signs and symptoms of cancer is important. All patients must be screened for malignancies and options for prophylactic mastectomy should be discussed. Guidelines for cancer screening including surveillance and management plans for these patients should be distinguished from those of the general population, and may lead to a more timely diagnosis and treatment of cancers associated with this syndrome.

Pericarditis as an initial symptom in Takayasu arteritis

Takayasu arteritis (TA) is a chronic inflammatory vasculitis with unknown etiology, affecting the large and medium sized arteries with a striking predilection for aorta and its major branches. Because of the non specific initial clinical presentation, the disease remains undiagnosed for a long period of time. Cardiac manifestations in TA are rarely reported in the literature. They determine the disease prognosis. Pericardial effusion was rarely reported in TA, and is exceptionally the first manifestation. We report a case, of TA presented initially with acute pericardial effusion. A 18yearold female was admitted to the hospital with chest pain and dyspnea. The 2-dimensional echocardiography showed moderate pericardial effusion measuring 1.1 cm anteriorly and 1.2 cm posteriorly. Laboratory finding showed elevated erythrocyte sedimentation rate (ESR; 120 mm/hr) and C-reactive protein (CRP; 6 mg/dl), hemoglobin 11.2 g/dl and platelet count 413.000/ml. She received non steroidal antiinflammatory drugs (NSAID) for 2 months under the impression of acute viral pericarditis. Her symptoms did not improve and the echocardiography revealed the persistence of pericardial effusion. The nature of pericardial fluid was an exudate. Protein counts were 5600 mg/dl. Lactic dehydrogenase was 538 UI/L and WBC counts was 204/μl, with 15% lymphocytes, 10% neutrophil and 75% others. Gram and AFB stains of pericardial fluid showed no organisms and the cytology was negative for malignancy. Pericardial biopsy revealed neither evidence of tuberculosis nor signs of malignancy. Viral and bacterial serology was negatives (B and C hepatitis, Epstein Barr Virus, Herpes Simplex Virus, tuberculosis). On physical examination, there were pulse differences and carotid tenderness, with asymmetric blood pressure (110/60 mm Hg in the left arm and 135/80 mm Hg in the right arm) and bilateral systolic subclavian and carotid murmurs. The electrocardiogram showed low voltage in the precordial leads.

Hyperhomocysteinaemia in Behçet's Disease

Objectives. The aim of this study was to investigate if hyperhomocysteinaemia is a contributive risk factor for the pathogenesis and the activity of Behçets disease (BD). Design and Methods. Fifty four patients fullfiling the criteria of the International Study Group for BD were enrolled. Fifty healthy volunteers matched for age and sex with the BD group were included as a negative control group. Patients, with any condition that might affect plasma homocysteine concentration, were excluded. Results. Mean serum homocysteine concentration was significantly higher in patients with BD than in the healthy controls (P < .001), in patients with active disease (P = .04), and in masculine gender (P = .05). There was no significant difference between homocysteine level and clinical involvement. Conclusions. We demonstrated that plasma total homocysteine level (tHcy) is increased in BD and correlated with disease activity. No association was found between homocysteine levels and clinical involvement.

The international normalized ratio (INR): What reagent, what instrument? The assessment of the agreement between INR values according to different reagent/instrument combinations

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.

The evaluation of the relevance of thrombin generation and procoagulant activity in thrombotic risk assessment in BCR-ABL-negative myeloproliferative neoplasm patients

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.