Tunis, Tunisia
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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.

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.

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.

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.

the erythrodermic psoriasis (EP) is a rare but severe form of psoriasis that may be potentially life-threatening. To study the characteristics of this severe form of psoriasis. We present a retrospective study, including all cases of EP followed in the dermatology department of the La Rabta hospital of Tunis over a 31-year-period from January, 1980 to June, 2010. sixty patients were included, concerning 46 men and 14 women, with an average age of 53.7. A history of psoriasis was reported in 78 % of the cases. A triggering factor was found in 53 % of the cases. Systemic treatments were required in 55 % of cases. An improvement was noted in 69.4 % of the cases. A recurrence of the EP was observed in 15% of the cases. Three cases of sepicemia and one of stroke were noted. Erythrodermic psoriasis is the most common etiology of erythroderma. It represents more than half of severe psoriasis. As shown in our study it affects mainly adults' males. It complicates usually a common psoriasis. Septic and thromboembolic complications ones justify a close follow up.

The hepatic artery is prone to numerous anatomic variations dictated by certain variables of the organogenesis. Aim : To research study the anatomic variations of the hepatic artery as well as their implications within surgical practice. Study of a post-mortem series of 33 cases of dissection of the hepatic pedicle with fresh corpses (deaths amounting no longer than 24 hours), carried out in conditions that are quite close to those met with the living ones. The common hepatic artery was divided at the foot of the hepatic pedicle into a gastro duodenal artery and into a proper hepatic artery in 94% of the cases. In 6% of the cases, we noticed a three level change, a branching of the average hepatic artery into a gastro duodenal artery, a right hepatic artery and a left hepatic artery. The proper hepatic artery represented the pedicle segment of the hepatic artery in 94% of the cases. In 36% of the cases, the liver was irrigated by two hepatic arteries. In 12% of the cases, there was the matter of the right hepatic artery, branch of the mesenteric artery. In 24% of the cases, there was the matter of the left hepatic artery branch of the left gastric artery. The perfect knowledge in preoperative and preoperative stages of the anatomic variations of the hepatic pedicle is quite essential. Actually, the lack of knowledge of these variations exposes to the risk of certain complications which are sometimes deadly during hepatic transplants, pancreatic surgery, cholecystectomies, through laroscopic way as well as in the treatment of gastro-esophagus surging.

Small-bowel videocapsule endoscopy (VCE) is a new technique in evaluation of intestinal involvement in several pathologies. Crohn's disease affects principally terminal ileum. Small bowel involvement in Crohn's disease is not well estimated by endoscopic and radiologic conventional techniques. To evaluate the performances of VCE in detection of asymptomatic proximal small bowel lesions in consecutive patients with Crohn's disease with terminal ileal involvement, to compare the results of VCE to small bowel radiography and CT-enteroclysis and to determine the therapeutic impact of VCE in these patients. A prospective study which included Crohn's disease patients with distal ileal involvement, based on radiological or endoscopic findings. We performed in all patients small bowel radiography, CT enteroclysis and VCE. Proximal involvement was characterized by presence of aphtoid, superficial or deep ulcerations in the jejunum or the proximal ileum. We studied 20 patients (12 men, mean age 31.6 years). VCE confirmed the distal ileal involvement in all patients. Significative proximal lesions was observed in nine patients (jejunum only: one case, jejunum and ileum: six cases and proximal ileum: two cases), in most cases aphtoid or superficial lesions. Deep ulcerations were observed in two patients. Small bowel radiography showed proximal ileal lesions in only two patients, and CT-enteroclysis in only one patient. Treatment by azathioprine was prescribed in two patients with severe and extended small bowel lesions in VCE examination. VCE is more accurate than radiologic techniques in detection of small bowel lesions in Crohn's disease. In cases of severe and extended small bowel involvement, VCE can conduct to changes of therapeutic approach.

PubMed | Reference Center for Inherited Metabolic Diseases, Helmholtz Center Munich, Hopital La Rabta, French Institute of Health and Medical Research and University Pierre and Marie Curie
Type: Journal Article | Journal: Journal of hepatology | Year: 2016

Next generation sequencing approaches have tremendously improved the diagnosis of rare genetic diseases. It may however be faced with difficult clinical interpretation of variants. Inherited enzymatic diseases provide an invaluable possibility to evaluate the function of the defective enzyme in human cell biology. This is the case for respiratory complex III, which has 11 structural subunits and requires several assembly factors. An important role of complex III in liver function is suggested by its frequent impairment in human cases of genetic complex III defects.We report the case of a child with complex III defect and acute liver dysfunction with lactic acidosis, hypoglycemia, and hyperammonemia. Mitochondrial activities were assessed in liver and fibroblasts using spectrophotometric assays. Genetic analysis was done by exome followed by Sanger sequencing. Functional complementation of defective fibroblasts was performed using lentiviral transduction followed by enzymatic analyses and expression assays.Homozygous, truncating, mutations in LYRM7 and MTO1, two genes encoding essential mitochondrial proteins were found. Functional complementation of the complex III defect in fibroblasts demonstrated the causal role of LYRM7 mutations. Comparison of the patients clinical history to previously reported patients with complex III defect due to nuclear DNA mutations, some actually followed by us, showed striking similarities allowing us to propose common pathophysiology.Profound complex III defect in liver does not induce actual liver failure but impedes liver adaptation to prolonged fasting leading to severe lactic acidosis, hypoglycemia, and hyperammonemia, potentially leading to irreversible brain damage.The diagnosis of rare genetic disease has been tremendously accelerated by the development of high throughput sequencing technology. In this paper we report the investigations that have led to identify LYRM7 mutations causing severe hepatic defect of respiratory complex III. Based on the comparison of the patients phenotype with other cases of complex III defect, we propose that profound complex III defect in liver does not induce actual liver failure but impedes liver adaptation to prolonged fasting.

PubMed | Tunis el Manar University, Hopital La Rabta and Institute National Of Cancerologie Salah Azaiez Of Tunis
Type: | Journal: Biomedicine & pharmacotherapy = Biomedecine & pharmacotherapie | Year: 2016

to investigate the anti-inflammatory effect of the Pistacia lentiscus oil in experimental colitis model.Colitis was induced in male rats by instillation of 2,4,6-trinitrobenzenesulfonic acid (TNBS) in all groups. The experimental groups consisted of: 5 rats received Lentisc oil 2months before colitis induction (preventive group), 5 rats received the oil on the day of colitis induction (curative group) and 5 control rats. Lentisc oil was extracted from the ripe fruit of the plant by the cold press method and was analyzed by spectro-chromatography. Lentisc oil has been inserted with a standard diet at the dose of 30mg oil/100g of food/rat.The lentisc oil sample is composed mainly by Oleic acid (47.96%), Palmitic acid (27.94%) and Linoleic acid (20.22%).There was a significant difference between control rats and treated rats with lentisc oil concerned body mass (p=0.009), bleeding index (p=0.005 and p=0.018) and diarrhea (p=0.012). Histological examination revealed a clear difference between the control and preventive groups with disappearance of erosion, decreased of cryptitis, irregular crypts and crypt loss in the preventive group. Curative group showed a significant decrease of ulceration, hyperplasia, cryptitis, irregular crypts and crypt loss compared to the control group. There was an attenuation of inflammation in the preventive group compared to the curative group without statistically significant.Lentisc oil administration could provide a protective effect on intestinal inflammation in colitis rats induced by TNBS mainly when it is administered at a young age in preventive mode. This beneficial effect would involve a modification of arachidonic acid metabolism.

PubMed | Center Hospitalier University Of Cocody, Hopital Militaire dInstruction Mohammed V, Hopital La Rabta, Hopital Lariboisiere de Paris and Hopital Ait Idir
Type: | Journal: The Pan African medical journal | Year: 2016

Migraine is a primary headache disorder (according to the latest International Headache Society criteria) affecting approximately 8% of African population. Women are more often affected than men and attacks usually occur before the age of 40 years Although some treatments, hygienic-dietary measures and other non-pharmacological methods can reduce the intensity and frequency of attacks, medicinal treatment of migraine attack is often necessary. Availability of treatments and access to care differ in Africa and led to the implementation of the first expert consensus recommendations for the management of the migraine in african adult patients. This multinational collaborative study is intended for health practitioners. It aims to provide 16 simple, evidence-based recommendations and is adapted to african medical practice.

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