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Tunis, Tunisia

Mistry P.K.,Yale University | Lukina E.,Hematology Research Center | Turkia H.B.,Hopital La Rabta | Amato D.,Mount Sinai Hospital | And 17 more authors.
JAMA - Journal of the American Medical Association | Year: 2015

Importance: Gaucher disease type 1 is characterized by hepatosplenomegaly, anemia, thrombocytopenia, and skeletal disease. A safe, effective oral therapy is needed. Objective: To determine whether eliglustat, a novel oral substrate reduction therapy, safely reverses clinical manifestations in untreated adults with Gaucher disease type 1. Design, Setting, and Participants: Phase 3, randomized, double-blind, placebo-controlled trial conducted at 18 sites in 12 countries from November 2009 to July 2012 among eligible patients with splenomegaly plus thrombocytopenia and/or anemia. Of 72 patients screened, 40 were enrolled. Interventions: Patients were stratified by spleen volume and randomized 1:1 to receive eliglustat (50 or 100mg twice daily; n = 20) or placebo (n = 20) for 9 months. Main Outcomes and Measures: The primary efficacy end pointwas percentage change in spleen volume in multiples of normal from baseline to 9 months; secondary efficacy end points were change in hemoglobin level and percentage changes in liver volume and platelet count. Results: All patients had baseline splenomegaly and thrombocytopenia (mostly moderate or severe), most had mild or moderate hepatomegaly, and 20% had mild anemia. Least-square mean spleen volume decreased by 27.77% (95% CI, -32.57% to -22.97%) in the eliglustat group (from 13.89 to 10.17 multiples of normal) vs an increase of 2.26% (95% CI, -2.54% to 7.06%) in the placebo group (from 12.50 to 12.84 multiples of normal) for an absolute treatment difference of -30.03% (95% CI, -36.82% to -23.24%; P < .001). For the secondary end points, the least-square mean absolute differences between groups all favored eliglustat, with a 1.22-g/dL increase in hemoglobin level (95% CI, 0.57-1.88 g/dL; P < .001), 6.64% decrease in liver volume (95% CI, -11.37% to -1.91%; P = .007), and 41.06% increase in platelet count (95% CI, 23.95%-58.17%; P < .001). No serious adverse events occurred. One patient in the eliglustat group withdrew (non-treatment related); 39 of the 40 patients transitioned to an open-label extension study. Conclusions and Relevance: Among previously untreated adults with Gaucher disease type 1, treatment with eliglustat compared with placebo for 9 months resulted in significant improvements in spleen volume, hemoglobin level, liver volume, and platelet count. The clinical significance of these findings is uncertain, and more definitive conclusions about clinical efficacy and utility will require comparison with the standard treatment of enzyme replacement therapy as well as longer-term follow-up. Trial Registration: clinicaltrials.gov Identifier: NCT00891202. Copyright © 2015 American Medical Association. All rights reserved. Source

Inhaled corticosteroids are widely used in the treatment of asthma but their safety on bone density is controversial. Aim: To study in a population of adults with asthma receiving inhaled corticosteroids for long term, the effect of this therapy on bone mineral density. Prospective study including 20 asthmatics, aged from 20 to 45 years, treated with inhaled corticosteroids for 12 months or more, with no risk factors for osteoporosis. We compared osteodensitometric parameters in our patients, bone mass and T-score at vertebral and femoral witness to a population matched by age and sex. The average age of our patients was 35.45 ± 5.43 years and the average BMI was 30.03 ± 5.77 kg/m2. The average daily dose of corticosteroid was 925 ± 133.27g of equivalent of beclometasone with an average take of 33.5 ± 21.3 months. The vertebral site bone mass average was 1216 ± 0106 g/cm2 in the studied group and 1201 ± 0099 g/cm2 in the control group without significant difference (p = 0380). At femur, the bone mass average was 1037 ± 0143 g/cm2 in the patient group and 1056 ± 0107 g/cm2 in the control group (p = 0380). We did not find a significant link between bone mineral density and cumulative dose or the duration of taking inhaled corticosteroids, BMI and tobacco. Our work confirms like other previous studies the safety of inhaled corticosteroids on bone mineral density. Source

Zainine R.,Hopital La Rabta
La Tunisie médicale | Year: 2011

The plunging goiter consists in a goiter whose lower limit is not palpable in surgical position. To study the epidemiologic characteristics, the circumstances of discovery, the clinical signs, and the management of this disease. A retrospective study about 43 cases of plunging goiters operated during a period of 14 years in the ENT department of the hospital The Rabta Tunis. The average age of our patients was 59.3 years. On physical examination, goiter was palpable in 41 patients (95.3%). The plunging character of the goiter was noted, at echography, in 26 patients. A cervico-thoracic scanner was performed in 41 patients (95.3%). It helped to confirm the plunging goiter in all patients. These goiters were most frequently pre vascular (73.2%) compared to the innomined venous trunk. The lower limits of the intrathoracic extensions were on the level of the superior vena cava in 4 cases (9.7%), on the level of the left brachio-cephalic venous trunk in 16 cases (39%) and on the level of the aortic arch in 15 cases (36,5%). The cervical incision was sufficient in 39 cases (97.5%) and we used a combined sternotomy in one patient (2.5%). The plunging goiter is a thyroid tumour cervical originally descended, then developed, in the mediastinum. A good clinical examination and paraclinical can reach a definite diagnosis and to achieve better surgical. Source

Ploux S.,Bordeaux University Hospital Center | Swerdlow C.D.,Cedars Sinai Medical Center | Eschalier R.,Interventional Imaging | Monteil B.,Bordeaux University Hospital Center | And 3 more authors.
PACE - Pacing and Clinical Electrophysiology | Year: 2016

Diaphragmatic myopotential oversensing (DMO) causes inhibition of pacing and inappropriate detection of ventricular fibrillation in implantable cardioverter defibrillators (ICDs). It occurs almost exclusively with integrated bipolar leads and is extremely rare with dedicated bipolar leads. If DMO cannot be corrected by reducing programmed sensitivity, ventricular lead revision is often required. The new Low Frequency Attenuation (LFA) filter in St. Jude Medical ICDs (St. Jude Medical, Sylmar, CA, USA) alters the sensing bandpass to reduce T-wave oversensing. This paper aims to present the LFA filter as a reversible cause of DMO. Unnecessary lead revision can be avoided by the simple programming solution of deactivating this LFA filter. ©2016 Wiley Periodicals, Inc. Source

Makni A.,Hopital La Rabta
La Tunisie médicale | Year: 2013

The sigmoid diverticulitis is the most common complication of diverticulosis of the colon. The remaining questions concern the current risk factors for recurrence after a first acute episode, radiological asking immediately upon suspicion of diverticulitis and therapeutic management including the indications of surgical treatment, what to conduct and its principles. Literature review. Risk factors of recurrence are: persistent or recurrent diverticulitis, abscess, stenosis and / or fistula. Abdominal CT is recommended in all patients clinically suspected diverticulitis of the colon. Treatment of acute diverticulitis is medical. The emergency surgical treatment depends on the stage of Hinchey. Stage I: In case of failure of medical treatment, resection-anastomosis in an emergency time delay to be proposed. Stage II: a percutaneous drainage followed by resection-anastomosis in 1 time. Stage III: surgery in emergency sigmoid colectomy based on. Stage IV: Hartmann procedure is the procedure of reference. Prophylactic colectomy is proposed in the case of presence of risk factors of recurrence. Comparison with other literature review were allowed to find that ultrasound made by an experienced radiologist could replace abdominal CT, and for stage III and IV Hinchey, laparoscopic resection can be performed with an immediate restoration of digestive continuity in well selected patients. Source

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