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Khanfir K.,Hopital de Sion | Kallel A.,Institute Gustave Roussy | Villette S.,Center Rene Huguenin | Belkacemi Y.,Lille University Hospital Center | And 13 more authors.
International Journal of Radiation Oncology Biology Physics

Background: Mammary adenoid cystic carcinoma (ACC) is a rare breast cancer. The aim of this retrospective study was to assess prognostic factors and patterns of failure, as well as the role of radiation therapy (RT), in ACC. Methods: Between January 1980 and December 2007, 61 women with breast ACC were treated at participating centers of the Rare Cancer Network. Surgery consisted of lumpectomy in 41 patients and mastectomy in 20 patients. There were 51(84%) stage pN0 and 10 stage cN0 (16%) patients. Postoperative RT was administered to 40 patients (35 after lumpectomy, 5 after mastectomy). Results: With a median follow-up of 79 months (range, 6-285), 5-year overall and disease-free survival rates were 94% (95% confidence interval [CI], 88%-100%) and 82% (95% CI, 71%-93%), respectively. The 5-year locoregional control (LRC) rate was 95% (95% CI, 89%-100%). Axillary lymph node dissection or sentinel node biopsy was performed in 84% of cases. All patients had stage pN0 disease. In univariate analysis, survival was not influenced by the type of surgery or the use of postoperative RT. The 5-year LRC rate was 100% in the mastectomy group versus 93% (95% CI, 83%-100%) in the breast-conserving surgery group, respectively (p = 0.16). For the breast-conserving surgery group, the use of RT significantly correlated with LRC (p = 0.03); the 5-year LRC rates were 95% (95% CI, 86%-100%) for the RT group versus 83% (95% CI, 54%-100%) for the group receiving no RT. No local failures occurred in patients with positive margins, all of whom received postoperative RT. Conclusion: Breast-conserving surgery is the treatment of choice for patients with ACC breast cancer. Axillary lymph node dissection or sentinel node biopsy might not be recommended. Postoperative RT should be proposed in the case of breast-conserving surgery. © 2012 Elsevier Inc. Source

Shema-Didi L.,Western Galilee Hospital | Kristal B.,Western Galilee Hospital Nahariya | Kristal B.,Bar - Ilan University | Sela S.,Bar - Ilan University | And 4 more authors.
Nutrition Journal

Background: Atherosclerotic cardiovascular disease (CVD) is the most common cause of morbidity and mortality among hemodialysis (HD) patients. It has been attributed, among other causes, to hypertension and dyslipidemia. The aim of the present study was to investigate the effect of a year-long consumption of Pomegranate juice (PJ), on two traditional cardiovascular (CV) risk factors: hypertension and lipid profile, as well as on cardiovascular events. Methods. 101 HD patients were randomized to receive 100 cc of PJ (0.7 mM polyphenols) or matching placebo juice, three times a week for one year. The primary endpoints were traditional CV risk factors; blood pressure and lipid profile. Systolic, diastolic and pulse pressure, plasma levels of triglycerides (TG), high density lipoprotein (HDL), low density lipoprotein (LDL) and total cholesterol were monitored quarterly during the study year. Secondary endpoint was incidence of cardiovascular events. Results: PJ consumption yielded a significant time response improvement in systolic blood pressure, pulse pressure, triglycerides and HDL level; an improvement that was not observed in the placebo intake group. These beneficial outcomes were more pronounced among patients with hypertension, high level of triglycerides and low levels of HDL. Conclusion: Regular PJ consumption by HD patients reduced systolic blood pressure and improved lipid profile. These favorable changes may reduce the accelerated atherosclerosis and high incidence of CVD among HD patients. Trial registration. ClinicalTrials.gov registry, Identifier number: NCT00727519. © 2014Shema-Didi et al.; licensee BioMed Central Ltd. Source

Zivony-Elboum Y.,Institute of Human Genetics | Zivony-Elboum Y.,Technion - Israel Institute of Technology | Westbroek W.,Human Genome Research Institutes | Kfir N.,Institute of Human Genetics | And 20 more authors.
Journal of Medical Genetics

Background Members of two seemingly unrelated kindreds of Arab Moslem origin presented with pronounced early onset spastic paraparesis of upper and lower limbs, mild intellectual disability, kyphosis, pectus carinatum and hypertrichosis. Methods The authors performed neurological and developmental examinations on the affected individuals. The authors conducted whole genome linkage and haplotype analyses, followed by sequencing of candidate genes; RNA and protein expression studies; and finally proof of principle investigations on knockdown morpholino oligonucleotide injected zebrafish. Results The authors characterise a novel form of autosomal recessive complex hereditary spastic paraparesis (CHSP). MRI studies of brain and spinal cord were normal. Within a single significantly linked locus the authors ultimately identified a homozygous missense mutation c.1146A>T (p.K382N) in the vacuolar protein sorting 37A (Vps37A) gene, fully penetrant and segregating with the disease in both families. Mobility was significantly reduced in Vps37A knockdown morpholino oligonucleotide injected zebrafish, supporting the causal relationship between mutations in this gene and the phenotype described in the patients of this study. Conclusions The authors provide evidence for the involvement of Vps37A, a member of the endosomal sorting complex required for transport (ESCRT) system, in upper motor neuron disease. The ESCRT system has been shown to play a central role in intracellular trafficking, in the maturation of multivesicular bodies and the sorting of ubiquitinated membrane proteins into internal luminal vesicles. Further investigation of mechanisms by which dysfunction of this gene causes CHSP will contribute to the understanding of intracellular trafficking of vesicles by the ESCRT machinery and its relevance to CHSP. Source

Odeh M.,Western Galilee Hospital Nahariya | Odeh M.,Technion - Israel Institute of Technology | Ophir E.,Western Galilee Hospital Nahariya | Ophir E.,Technion - Israel Institute of Technology | And 4 more authors.
Ultrasound in Obstetrics and Gynecology

Objectives Absence or congenital anomalies of the parotid glands are associated with significant longterm morbidity. To date there are no published data on ultrasonographic detection of these defects in early pregnancy. We set out to demonstrate and measure the fetal parotid and submandibular salivary glands at 14-16 weeks using transvaginal ultrasound imaging. Methods During a routine fetal anomaly detection scan in 30 consecutive patients, an attempt was made to examine the fetal parotid and submandibular glands. The fetal head was scanned in transverse sections just below the fetal ears, and the area of the parotid and submandibular glands was inspected. The examination time was not prolonged for the purpose of measuring the salivary glands. The fetal biparietal diameter and the femur length were also documented. Results The median gestational age was 15.4 (range,14.4-16.5) weeks. In all 30 patients examined, at least one pair of parotid and submandibular glands was clearly visualized and measured. In seven patients the parotid and submandibular glands were visualized on both sides. The median length of the parotid gland was 7.5 (range, 5.5-11.5) mm and that of the submandibular gland was 5.4 (range, 3.7-8.5) mm. Conclusions The fetal salivary glands can be demonstrated by transvaginal ultrasound imaging at 14-16 weeks of gestation. This is the first reported study presenting the normal values of salivary gland measurements, which may be important in detecting fetuses with congenital absence or other malformations of the glands. Copyright© 2010 ISUOG. Published by John Wiley & Sons, Ltd. Source

Lee A.Y.Y.,University of British Columbia | Lee A.Y.Y.,British Columbia Cancer Agency | Kamphuisen P.W.,University of Groningen | Meyer G.,University of Paris Descartes | And 161 more authors.
JAMA - Journal of the American Medical Association

Importance: Low-molecular-weight heparin is recommended over warfarin for the treatment of acute venous thromboembolism (VTE) in patients with active cancer largely based on results of a single, large trial. Objective: To study the efficacy and safety of tinzaparin vs warfarin for treatment of acute, symptomatic VTE in patients with active cancer. Design, Settings, and Participants: A randomized, open-label study with blinded central adjudication of study outcomes enrolled patients in 164 centers in Asia, Africa, Europe, and North, Central, and South America between August 2010and November 2013. Adult patients with active cancer (defined as histologic diagnosis of cancer and receiving anticancer therapy or diagnosed with, or received such therapy, within the previous 6 months) and objectively documented proximal deep vein thrombosis (DVT) or pulmonary embolism, with a life expectancy greater than 6 months and without contraindications for anticoagulation, were followed up for 180 days and for 30days after the last study medication dose for collection of safety data. Interventions: Tinzaparin (175 IU/kg) once daily for 6 months vs conventional therapy with tinzaparin (175 IU/kg) once daily for 5 to 10 days followed bywarfarin at a dose adjusted to maintain the international normalized ratio within the therapeutic range (2.0-3.0) for 6 months. Main Outcomes and Measures: Primary efficacy outcomewas a composite of centrally adjudicated recurrent DVT, fatal or nonfatal pulmonary embolism, and incidental VTE. Safety outcomes included major bleeding, clinically relevant nonmajor bleeding, and overall mortality. Results: Nine hundred patients were randomized and included in intention-to-treat efficacy and safety analyses. Recurrent VTE occurred in 31 of 449 patients treated with tinzaparin and 45 of 451 patients treated with warfarin (6-month cumulative incidence, 7.2% for tinzaparin vs 10.5% for warfarin; hazard ratio [HR], 0.65 [95% CI, 0.41-1.03]; P = .07). There were no differences in major bleeding (12 patients for tinzaparin vs 11 patients for warfarin; HR, 0.89 [95% CI, 0.40-1.99]; P = .77) or overall mortality (150 patients for tinzaparin vs 138 patients for warfarin; HR, 1.08 [95% CI, 0.85-1.36]; P = .54). A significant reduction in clinically relevant nonmajor bleeding was observed with tinzaparin (49 of 449 patients for tinzaparin vs 69 of 451 patients for warfarin; HR, 0.58 [95% CI, 0.40-0.84]; P = .004). Conclusions and Relevance: Among patients with active cancer and acute symptomatic VTE, the use of full-dose tinzaparin (175 IU/kg) daily compared with warfarin for 6 months did not significantly reduce the composite measure of recurrent VTE and was not associated with reductions in overall mortality or major bleeding, but was associated with a lower rate of clinically relevant nonmajor bleeding. Further studies are needed to assess whether the efficacy outcomes would be different in patients at higher risk of recurrent VTE. Copyright © 2015 American Medical Association. All rights reserved. Source

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