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111.
Monika Koziel Dan Corcoran Isabelle O'Callaghan Roy D. Sleator Brigid Lucey 《Diagnostic microbiology and infectious disease》2013
A total of 717 faeces samples were tested prospectively using the EntericBio Panel II® detection system (Serosep, Limerick, Ireland), in parallel with routine laboratory testing, which combines the EntericBio® system with retrospective culture of each specimen where a target is detected. Discrepancy analysis was conducted using molecular methods. The EntericBio Panel II® assay produced 585 negative and 132 positive results, namely, Campylobacter spp. (n = 66); SLT 1 and/or SLT 2 (n = 64); Salmonella spp. (n = 5); and Shigella spp. (n = 0). Three samples were positive for more than 1 target. Of these results, 4 Campylobacter spp. detections and 4 SLT 1/ SLT 2 detections remained unconfirmed, and the system failed to detect 2 Campylobacter spp. targets detected by routine laboratory detection. The sensitivity, specificity, positive predictive value, negative predictive value, and efficiency were calculated to be 98.4%, 98.7%, 93.9%, 99.7%, and 98.6%, respectively. 相似文献
112.
Julien Guy Ilana Antony‐Debr Emmanuel Benayoun Isabelle Arnoux Chantal Fossat Magali Le Garff‐Tavernier Anna Raimbault Michle Imbert Marc Maynadi Francis Lacombe Marie C Bn Orianne Wagner‐Ballon 《British journal of haematology》2013,161(4):551-555
The World Health Organization 2008 Classification emphasizes myeloperoxidase (MPO) detection as sufficient for assigning a blast population to the myeloid lineage. Published MPO positivity thresholds are 10% for flow cytometry (FCM) but 3% for cytochemistry. Here we re‐evaluated the FCM‐MPO threshold by comparing retrospectively 128 acute lymphoblastic leukaemias and 75 acute myeloid leukaemias without maturation, all assessed by benzidine‐based cytochemistry. A 13% threshold was found to be relevant using an isotype control as background‐reference (sensitivity 95·1%, specificity 91·7%). Residual normal lymphocytes proved to be an advantageous alternative reference, a threshold of 28% yielding improved 97·4% sensitivity and 96·1% specificity. 相似文献
113.
Molecular analyses of patients with hyperferritinemia and normal serum iron values reveal both L ferritin IRE and 3 new ferroportin (slc11A3) mutations 总被引:9,自引:1,他引:9 下载免费PDF全文
Unexplained hyperferritinemia is a common clinical finding, even in asymptomatic persons. When early onset bilateral cataracts are also present, the hereditary hyperferritinemia-cataract syndrome (HHCS), because of heterozygous point mutation in the L ferritin iron-responsive element (IRE) sequence, can be suspected. We sequenced the L ferritin exon 1 in 52 DNA samples from patients referred to us for molecular diagnosis of HHCS. We identified 24 samples with a point mutation/deletion in the IRE. For the 28 samples in which no IRE mutation was present, we also genotyped HFE mutations and sequenced both H ferritin and ferroportin genes. We found an increased frequency of His63Asp heterozygotes (12 of 28) but no H ferritin mutations. We identified 3 new ferroportin mutations, producing, respectively, Asp157Gly, Gln182His, and Gly323Val amino acid replacements, suggesting that these patients have dominant type 4 hemochromatosis. This study demonstrates that both L ferritin IRE and ferroportin mutations can account for isolated hyperferritinemia. The presence of cataract does not permit the unambiguous identification of patients with HHCS, although the existence of a family history of cataract was only encountered in these patients. This raises the intriguing possibility that lens ferritin accumulation might be a factor contributing to age-related cataract in the general population. Additional causes of isolated hyperferritinemia remain to be identified. 相似文献
114.
The role of von Willebrand factor (VWF) in thrombosis involves its binding to a number of ligands. To investigate the relative importance of these particular interactions in the thrombosis process, we have introduced mutations into murine VWF (mVWF) cDNA inhibiting VWF binding to glycoprotein (Gp) Ib, GPIIbIIIa, or to fibrillar collagen. These VWF mutants were expressed in VWF-deficient mice (VWF–/–) by using an hydrodynamic injection approach, and the mice were studied in the ferric chloride–induced injury model. Expression of the collagen and the GPIIbIIIa VWF-binding mutants in VWF–/– mice resulted in delayed thrombus growth and significantly increased vessel occlusion times compared with mice expressing wild-type (WT) mVWF (30 ± 3 minutes and 38 ± 4 minutes for the collagen and GPIIbIIIa mutants, respectively, vs 19 ± 3 minutes for WT mVWF). Interestingly, these mutants were able to correct bleeding time as efficiently as WT mVWF. In contrast, VWF–/– mice expressing the GPIb binding mutant failed to restore thrombus formation and were bleeding for as long as they were observed, confirming the critical importance of the VWF-GPIb interaction. Our observations suggest that targeting the VWF-collagen or VWF-GPIIbIIIa interactions could be an interesting alternative for new antithrombotic strategies. 相似文献
115.
Yoav Michowitz Anat Milman Antoine Andorin Georgia Sarquella-Brugada M. Cecilia Gonzalez Corcia Jean-Baptiste Gourraud Giulio Conte Frederic Sacher Jimmy J.M. Juang Sung-Hwan Kim Eran Leshem Philippe Mabo Pieter G. Postema Aviram Hochstadt Yanushi D. Wijeyeratne Isabelle Denjoy Carla Giustetto Yuka Mizusawa Bernard Belhassen 《Journal of the American College of Cardiology》2019,73(14):1756-1765
Background
Information on young patients with Brugada syndrome (BrS) and arrhythmic events (AEs) is limited.Objectives
The purpose of this study was to describe their characteristics and management as well as risk factors for AE recurrence.Methods
A total of 57 patients (age ≤20 years), all with BrS and AEs, were divided into pediatric (age ≤12 years; n = 26) and adolescents (age 13 to 20 years; n = 31).Results
Patients’ median age at time of first AE was 14 years, with a majority of males (74%), Caucasians (70%), and probands (79%) who presented as aborted cardiac arrest (84%). A significant proportion of patients (28%) exhibited fever-related AE. Family history of sudden cardiac death (SCD), prior syncope, spontaneous type 1 Brugada electrocardiogram (ECG), inducible ventricular fibrillation at electrophysiological study, and SCN5A mutations were present in 26%, 49%, 65%, 28%, and 58% of patients, respectively. The pediatric group differed from the adolescents, with a greater proportion of females, Caucasians, fever-related AEs, and spontaneous type-1 ECG. During follow-up, 68% of pediatric and 64% of adolescents had recurrent AE, with median time of 9.9 and 27.0 months, respectively. Approximately one-third of recurrent AEs occurred on quinidine therapy, and among the pediatric group, 60% of recurrent AEs were fever-related. Risk factors for recurrent AE included sinus node dysfunction, atrial arrhythmias, intraventricular conduction delay, or large S-wave on ECG lead I in the pediatric group and the presence of SCN5A mutation among adolescents.Conclusions
Young BrS patients with AE represent a very arrhythmogenic group. Current management after first arrhythmia episode is associated with high recurrence rate. Alternative therapies, besides defibrillator implantation, should be considered. 相似文献116.
Antonin Levy Cécile Le Péchoux Hitesh Mistry Isabelle Martel-Lafay Andrea Bezjak Delphine Lerouge Laetitia Padovani Paul Taylor Corinne Faivre-Finn 《Journal of thoracic oncology》2019,14(2):294-297
Introduction
The impact of the dose and fractionation of thoracic radiotherapy on the risk of developing brain metastasis (BM) has not been evaluated prospectively in limited stage SCLC patients receiving prophylactic cerebral irradiation (PCI).Methods
Data from patients treated with PCI from the CONVERT trial were analyzed.Results
Four hundred forty-nine of 547 patients (82%) received PCI after completion of chemoradiotherapy. Baseline brain imaging consisted of computed tomographic scans in 356 of 449 patients (79%) and magnetic resonance imaging in 83 of 449 (18%) patients. PCI was delivered to 220 of 273 participants (81%) in the twice-daily (BD) group and 229 of 270 in the once-daily (OD) group (85%; p = 0.49). Total median PCI dose was 25 Gy in both the BD and OD groups (p = 0.74). In patients who received PCI, 75 (17%) developed BM (35 [8%] in OD and 40 [9%] in BD) and 173 (39%) other extracranial progression. In the univariate analysis, gross tumor volume (GTV) was associated with an increased risk of BM (p = 0.007) or other radiological progression events (p = 0.006), whereas in a multivariate analysis both thoracic GTV (tGTV) and ECOG performance score were associated with either progression type. The median overall survival (OS) of patients treated with PCI was 29 months. In the univariate analysis of OS, PCI timing from end of chemotherapy, weight loss of more than 10%, and tGTV were prognostic factors associated with OS. In the multivariate analysis, only tGTV was associated with OS. Delay between end of chemotherapy and PCI was not associated with OS.Conclusions
Patients receiving OD or BD thoracic radiotherapy have the same risk of developing BM. Larger tumors are associated with a higher risk of BM. 相似文献117.
Patricia Blackburn Isabelle Lemieux Jean Bergeron Angelo Tremblay Jean-Pierre Després 《Metabolism: clinical and experimental》2009,58(8):1123-1130
The hypertriglyceridemic waist phenotype, the National Cholesterol Education Program-Adult Treatment Panel III (NCEP-ATP III) criteria, and the International Diabetes Federation (IDF) criteria have been proposed as screening tools to identify subjects with features of the metabolic syndrome and therefore at increased cardiometabolic risk. The aim of the present study was to compare the ability of these 3 clinical approaches to identify individuals at increased cardiometabolic risk as suggested by the presence of deteriorated markers such as hyperinsulinemia, elevated apolipoprotein B levels, small low-density lipoprotein particles, high C-reactive protein concentrations, and low adiponectin levels. For that purpose, physical and cardiometabolic characteristics of a sample of 272 white men recruited for various metabolic investigations were studied. The hypertriglyceridemic waist phenotype was defined as having both a high waist circumference (≥90 cm) and increased fasting triglyceride levels (≥2.0 mmol/L). Having at least 3 of the 5 NCEP-ATP III criteria or waist circumference of at least 94 cm plus any 2 of the 4 additional IDF criteria was also used to identify individuals at increased cardiometabolic risk. A large proportion of men with the hypertriglyceridemic waist phenotype also met the NCEP-ATP III (82.7%) or IDF (89.2%) criteria. Men with the hypertriglyceridemic waist phenotype were characterized by alterations in their lipoprotein-lipid profile that included small low-density lipoprotein particles, increased apolipoprotein B and insulin levels, as well as reduced adiponectin concentrations, which were similar to individuals meeting the NCEP-ATP III or the IDF criteria. Moreover, the Framingham risk score of men meeting any of the 3 screening tools criteria was higher and was similar across the 3 approaches (4.2, 3.8, and 3.7, respectively). These results suggest that hypertriglyceridemic waist may be as discriminant as the NCEP-ATP III or the IDF criteria and could be used as an initial screening approach to identify individuals with deteriorated cardiometabolic risk markers. 相似文献
118.
We described the case of an infant with compound heterozygozity for a b0-thalassemic mutation and Hemoglobin (Hb) Genova, an unstable Hb variant. He has required regular transfusions as early as the second month of life and since then, behaves like a thalassemia major patient. This association leads to the most severe clinical course involving an unstable variant, reported so far. 相似文献
119.
Marc Leopold Hautekeete Isabelle Dodeman Véronique Azais-Braesco Kit Van den Berg Carine Seynaeve Albert Geerts 《Alcoholism, clinical and experimental research》1998,22(2):494-500
Body retinoids are stored in the lipid droplets of hepatic stellate (Ito) cells. In chronic liver disease, the stellate cells differentiate into myo-fibroblast-like cells, a process whereby they lose their retinoid-con-taining lipid droplets. We studied the relation between liver retinoid content, the number of lipid droplets per stellate cell, and the number of stellate cells per mm2 in human alcoholic liver disease. Semithin sections of liver biopsies from normal subjects and patients with early (steatosis, inflammation, and mild fibrosis) and late (cirrhosis and cirrhosis with acute alcoholic hepatitis) alcoholic liver disease were morphometrically evaluated. Liver retinoid content was determined by HPLC. In normal patients, liver retinoid content was 901 ± 213 lU/g of liver (mean ± SEM). There was a decrease in liver retinoid content in early alcoholic liver disease (409 ± 50 IU/g) and a further reduction in cirrhosis (153 ± 50 IU/g). In patients with acute alcoholic hepatitis, retinoid content was strikingly low (5.2 ± 1.8 IU/g). There was a progressive decrease in the number of stellate cells per mm2 associated with progressive liver damage. We found a fair correlation between the number of stellate cells per mm2 and liver retinoid content in all patient groups (overall correlation: 0.71). In normal subjects, the mean number of lipid droplets per stellate cell was 7.4 ± 0.7. In patients with early alcoholic liver disease and in patients with alcoholic cirrhosis, this value was increased to 13.6 ± 0.8 and 10.4 ± 2.0, respectively. In patients with acute alcoholic hepatitis, only a few lipid droplets were present (4.2 ± 0.5). There was a good correlation between liver retinoid content and mean number of lipid droplets in normal patients (r= 0.58). In alcoholic cirrhosis, however, correlation was poor (r= 0.34). In early alcoholic liver disease, the correlation was absent (r= 0.004). In conclusion, the major finding of our study is that the correlation between the mean number of lipid droplets per stellate cell and liver retinoid content varies according to the hepatic pathology considered. Marked lipid droplet accumulation occurs in stellate cells in early alcoholic liver disease and, to a lesser extent, in alcoholic cirrhosis, but there is no correlation between the mean number of lipid droplets per stellate cell and liver retinoid content. Therefore, not retinoids but probably lipids are responsible for the accumulation of lipid droplets. We also find that there is a fair correlation between the number of stellate cells per mm2 and liver retinoid content in all patient groups. Finally, we confirm the decrease in hepatic retinoid content that occurs in alcoholic liver disease in humans, even at the early stages of the disease. 相似文献
120.
Paillaud E Bories PN Merlier I Richardet JP Jeanfaivre V Campillo B 《Gastroentérologie clinique et biologique》2002,26(5):443-447
OBJECTIVE: Percutaneous endoscopic gastrostomy (PEG) is the procedure of choice to achieve long-term enteral nutrition. The risks and benefits of PEG in elderly hospitalized patients have been poorly documented. The objective of this study was to describe the outcome of elderly patients one-year after insertion of a PEG tube.PATIENTS AND METHODS: Hospital records of 73 patients who underwent PEG for enteral nutrition were reviewed retrospectively. Data on patient age and sex, preexisting medical conditions such as dementia or pressure sores, indication for PEG, concomitant infection, complications of PEG and death were obtained from the hospital charts.RESULTS: The main indication for PEG was anorexia (49%). Before insertion of the gastrostomy tube, 44% of the patients had pressure scores, 30% had concomitant infection, 45% had dementia. PEG complications were observed in 51 patients. The survival rate at 1, 6 and 12 months was 0.68 [95% confidence interval - CI 95%: 0.56-0.78], 0.48 [CI 95%: 0.36-0.59] and 0.37 [CI 95%: 0.26-0.48] respectively. The presence of an infectious disease or of pressure sores at the time of PEG tube insertion were independently associated with mortality. Median survival of patients with these two factors was 32 days [CI 95%: 11-98].CONCLUSION: According to these results, the PEG tubes should be inserted with a delay from infectious diseases and before the occurrence of pressure sores. 相似文献