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Christoffersen Gammelgaard opublikował 1 rok, 3 miesiące temu
Hence, the first step acts as the rate limiting step. The present calculations also show that the Lys258 residue undergoes a conformational change after the first step of transamination reaction and becomes proximal to C4A atom of the Asp-PLP Schiff base to favor the second step. The active site residues Tyr70* and Gly38 anchor the Lys258 in proper position and orientation during the first step of the reaction and stabilize the positive charge over Lys258 generated at the intermediate step.Clinical audits should underpin everything we do as clinicians – to constantly evaluate and improve our day-to-day clinical practice. Errors in practice, suboptimal practice or inefficiencies can occur in any part of our health-care system, despite the training and best intentions of health-care professionals. Audits examine how clinical care is being provided and whether benchmarks are being met, and identify opportunities for improvement. Detection of problems is greatly improved when audits of practice are undertaken, ideally regularly, and as part of a continuous process of quality improvement. Audits also make ideal entry-level research projects for students and trainees through to senior clinicians. Despite a willingness to undertake audits, and improvements in both undergraduate and postgraduate training, not all clinicians have had formal teaching in audit methodology, and a refresher can also be helpful. This short overview covers basic clinical audit methods, discusses key facilitators for embedding audit into every day practice, and references additional resources to guide clinicians wanting to take up the challenge of regularly and efficiently undertaking audits.The characterization of platelet concentrates (PCs) in transfusion medicine has been performed with different analytical methods and platelet lesions (from biochemistry to cell biology) have been documented. In routine quality assessment and validation of manufacturing processes of PCs for transfusion purposes, only basic parameters are monitored and the platelet functions are not included. However, PCs undergo several manipulations during the processing and the basic parameters do not provide sensitive analyses to properly picture out the impact of the blood component preparation and storage on platelets. To improve the transfusion supply chain and the platelet functionalities, additional parameters should be used. The present short review will focus on the different techniques to monitor ex vivo platelet lesions from phenotype characterization to advanced omic analyses. Then, the opportunities to use these methods in quality control, process validation, development, and research will be discussed. Functional markers should be considered because they would be an advantage for the future developments in transfusion medicine.Platelets play a major role in primary hemostasis, where activated platelets form plugs to stop hemorrhaging in response to vessel injuries. Defects in any step of the platelet activation process can cause a variety of platelet dysfunction conditions associated with bleeding. To make an accurate diagnosis, constitutional platelet dysfunction (CPDF) should be considered once von Willebrand disease and drug intake are ruled out. CPDF may be associated with thrombocytopenia or a genetic syndrome. CPDF diagnosis is complex, as no single test enables the analysis of all aspects of platelet function. Furthermore, the available tests lack standardization, and repeat tests must be performed in specialized laboratories especially for mild and moderate forms of the disease. In this review, we provide an overview of the laboratory tests used to diagnose CPDF, with a focus on light transmission platelet aggregation (LTA), flow cytometry (FC), and granules assessment. Global tests, mainly represented by LTA, are often initially performed to investigate the consequences of platelet activation on platelet aggregation in a single step. Global test results should be confirmed by additional analytical tests. FC represents an accurate, simple, and reliable test to analyze abnormalities in platelet receptors, and granule content and release. This technique may also be used to investigate platelet function by comparing resting- and activated-state platelet populations. Assessment of granule content and release also requires additional specialized analytical tests. High-throughput sequencing has become increasingly useful to diagnose CPDF. Advanced tests or external research laboratory techniques may also be beneficial in some cases.
Antiphospholipid antibodies (APAs) are found quite frequently in patients with non-Hodgkin’s lymphoma (NHL). However, the clinical significance of these antibodies is largely unknown. This study aims to delineate the clinical and prognostic role of APAs in NHL patients.
Consecutive patients of NHL were screened for lupus anticoagulant (LA), IgG/IgM anticardiolipin antibody, and IgG/IgM anti-β2-glycoprotein I at the time of diagnosis. Baseline investigations, staging, and treatment were done as per institutional protocol. Patients were followed up until the last known outpatient visit or death. All were screened at each visit for any thromboembolic event. The association of APA status with baseline NHL characteristics and treatment response was evaluated by univariate analysis. Kaplan-Meier survival analysis was used to compare the final outcome in patients with or without APAs. Patients who were initially APA positive were retested for the corresponding antibody at the end of chemotherapy.
Twenty-four out of 105 patients (22.8%) were APA positive at diagnosis. The presence of APA was not significantly associated with NHL stage, histology, International Prognostic Index score, activated partial thromboplastin time, or treatment response. The median duration of follow-up was 15 months. Only four patients developed venous thrombosis; none was APA positive. There was no statistically significant difference in overall survival between the two groups (
= 0.471). Patients, who were APA positive initially, tested negative at the end of treatment, irrespective of treatment response.
APAs are encountered more frequently in NHL patients than in the general population. However, APAs do not correlate with disease severity, thrombosis risk, treatment outcome, or overall survival.
APAs are encountered more frequently in NHL patients than in the general population. However, APAs do not correlate with disease severity, thrombosis risk, treatment outcome, or overall survival.
The relationship between von Willebrand factor antigen (VWFAg), VWF propeptide (VWFpp), VWFpp/VWFAg ratio, ADAMTS13 activity, and microembolic signal (MES) status in carotid stenosis is unknown.
This prospective, multicenter study simultaneously assessed plasma VWFAg levels, VWFpp levels and ADAMTS13 activity, and their relationship with MES in asymptomatic versus symptomatic moderate-to-severe (≥50-99%) carotid stenosis patients. One-hour transcranial Doppler ultrasound of the middle cerebral arteries classified patients as MES+ve or MES-ve.
Data from 34 asymptomatic patients were compared with 43 symptomatic patients in the „early phase” (≤4 weeks) and 37 patients in the „late phase” (≥3 months) after transient ischemic attack (TIA)/ischemic stroke. VWFAg levels were higher (
= 0.049) and VWFpp/VWFAg ratios lower (
= 0.006) in early symptomatic than in asymptomatic patients overall, and in early symptomatic versus asymptomatic MES-ve subgroups (
≤0.02). There were no intergroup differences in with ADAMTS13 activity over time following atherosclerotic TIA/ischemic stroke.Viscoelastic point-of-care (VET POC) tests provide a global assessment of hemostasis and have an increasing role in the management of bleeding and blood component delivery across several clinical settings. VET POC tests have a rapid turnaround time, provide a better overall picture of hemostasis, predict bleeding more accurately than conventional coagulation tests, and reduce blood component usage and health care costs. Despite commonly having abnormal conventional coagulation tests, most patients with chronic liver disease have a „rebalanced” hemostasis. However, this hemostatic balance is delicate and these patients are predisposed to both bleeding and thromboembolic events. Over recent years, VET POC tests have been increasingly studied for their potential as better functional tests of hemostasis in liver disease patients. This review provides a background on the most common VET POC tests (thromboelastography and rotational thromboelastometry) and discusses the current evidence for these tests in the prediction and management of bleeding and thrombosis in patients with chronic liver disease, and in liver resection and transplant. With the recent publication of several randomized controlled trials, there is growing evidence that VET POC tests may be used to improve bleeding risk assessment and reduce blood product use in liver disease patients outside of the transplant setting. However, consensus is still lacking regarding the VET POC tests’ thresholds that should be used to trigger blood product transfusion. VET POC tests also show promise in predicting thrombosis in patients with liver disease, but further research is needed before they can be used to guide anticoagulant therapy.
The prognosis of stage 4S/MS neuroblastoma has traditionally been reported as excellent, yet conflicting treatment protocols exist for this enigmatic disease. To critically address this question, we have undertaken a systematic review of published studies to accurately determine outcomes for infants with stage 4S/MS neuroblastoma.
Studies were identified using MEDLINE, Embase, and Cochrane databases using the relevant search terms. Literature reviews, case reports, and adult studies were excluded. Data were extracted independently following article selection by three authors and reviewed by the senior author.
The original search retrieved 2,325 articles. Following application of exclusion criteria and removing duplicate data, 37 studies (1,105 patients) were included for final review. Overall patient survival was 84%. Twelve studies (544 patients) recorded MYCN status. Mortality in MYCN amplified tumors was 56%. Chromosome 1p/11q status was reported in four studies and 1p/11q deletion carried a 40% fnagement or surgery to excise the primary tumor carry the best prognosis.
Esophageal atresia (EA) is a congenital anomaly, presenting multifactorial etiology. Swallowing problems and gastroesophageal reflux disease may accompany EA, which have adverse effects on oral health.
In this descriptive study, intraoral examination of the children with repaired EA and of the dental patients without systemic/chronic disease was performed. Dental caries, dental erosion, and halitosis status were evaluated using the International Caries Detection and Evaluation System II, and the Basic Erosive Wear Examination indices as well as the Halimeter, respectively.
There were 19 (
= 12 male;
= 7 female) case subjects and 16 (
= 10 male;
= 6 female) control subjects whose age ranged between 14 and 72 months. Among cases, 15 children had dental caries (78.9%; initial caries
= 4, moderate caries
= 4, and extensive caries
= 7). Of the controls, 13 had dental caries (81.2%; initial caries
= 5, moderate caries
= 5, and extensive caries
= 3). Although the median scores of decayed, missing, filled teeth (dmft) and decayed, missing, filled surfaces (dmfs)-for primary dentition-were not statistically significantly different between two groups, both dmft and dmfs were found to be higher among the case subjects (
= 0.


