scholarly journals Anesthetic Management in Mucopolysaccharidoses

2013 ◽  
Vol 2013 ◽  
pp. 1-10 ◽  
Author(s):  
Concetta Maria Spinello ◽  
Lorena Maria Novello ◽  
Sofia Pitino ◽  
Claudia Raiti ◽  
Paolo Murabito ◽  
...  
Keyword(s):  
High Risk ◽  
General Population ◽  
Connective Tissue ◽  
Surgical Procedures ◽  
Anesthetic Management ◽  
Genetic Diseases ◽  
Elective Treatment ◽  
Risk Patients ◽  
Connective Tissue Metabolism ◽  
Experienced Personnel

Mucopolysaccharidoses (MPSs) are a group of uncommon genetic diseases of connective tissue metabolism. It is well established that the elective treatment of subjects affected by MPS is multidisciplinary and must be carried out by experienced personnel in highly specialist centers. However, there is the possibility to perform an anesthesia in a peripheral center, where anesthesiologists might not have a large experience of MPS. Various attempts to produce guidelines have been made for MPS. There has been an increasing interest in the topic as it is demonstrated by the number of scientific studies published in the last few years (peak in 2011). We want to provide a summary of anesthetic management, reviewing the current literature on the topic in a procedural algorithm for these high-risk patients, who require surgical procedures and diagnostic examinations under sedation with a higher frequency than the general population.

scholarly journals Integrated Mutational and Cytogenetic Analysis Identifies New Prognostic Subgroups in Chronic Lymphocytic Leukemia

Blood ◽  
2012 ◽  
Vol 120 (21) ◽  
pp. 712-712
Author(s):  
Davide Rossi ◽  
Silvia Rasi ◽  
Valeria Spina ◽  
Alessio Bruscaggin ◽  
Sara Monti ◽  
...  
Keyword(s):  
High Risk ◽  
General Population ◽  
Life Expectancy ◽  
Clonal Evolution ◽  
Lymphocytic Leukemia ◽  
Low Risk ◽  
Risk Category ◽  
Intermediate Risk ◽  
Newly Diagnosed ◽  
Risk Patients

Abstract Abstract 712 The identification of NOTCH1, SF3B1, MYD88 and BIRC3 genetic lesions in chronic lymphocytic leukemia (CLL) prompts a comprehensive and dynamic prognostic algorithm including gene mutations, chromosomal abnormalities, and their changes during clonal evolution. The study utilized both time-fixed (637 newly diagnosed CLL) and time-dependent (257 CLL provided with 524 sequential samples) approaches. Each sample was investigated for TP53, NOTCH1, SF3B1, MYD88, and BIRC3 mutations by Sanger sequencing and for 17p13, 11q22-q23, 13q14 and BIRC3 deletions and +12 by FISH. Del13q14 and +12 distributed in a mutually exclusive fashion (p<0.0001), and identified three main genetic subgroups: cases harboring del13q14, cases harboring +12 and cases lacking both del13q14 and +12. With the sole exception of the expected association between NOTCH1 mutations and +12 CLL (p=0.0014), the prevalence of the other genetic lesions did not differ among molecular subgroups. FISH abnormalities segregated patients in distinct prognostic groups according to Döhner (Fig 1A). Among new genetic lesions, survival analysis confirmed the independent prognostic value of NOTCH1, SF3B1 and BIRC3 lesions in this study cohort. MYD88 mutations had no prognostic effect (p=0.1728). Recursive partitioning analysis followed by random survival forest validation established the hierarchical order of relevance of the genetic lesions, and created an integrated mutational and cytogenetic (MUCY) model that classified newly diagnosed CLL into four prognostic subgroups (Fig 1B). High risk patients harbored TP53 disruption and/or BIRC3 disruption independent of co-occurring lesions (10-year survival: 29.1%). When the demographic effects of age, sex and year of diagnosis were compensated, the 10-year life expectancy of high risk patients was only 37.7% of that expected in the matched general population (p<0.0001). Intermediate risk patients harbored NOTCH1 and/or SF3B1 mutations and/or del11q22-q23 in the absence of TP53 and BIRC3 abnormalities (10-year OS: 37.1%). The 10-year life expectancy of intermediate risk patients was reduced to 48.5% compared to the matched general population (p<0.0001). The low risk category comprised both patients harboring +12 and patients wild type for all genetic lesions (i.e. normal) (10-year OS: 57.3%), with a 10-year life expectancy of 70.7% compared to the matched general population (p<0.0001). Very low risk patients harbored del13q14 as the sole genetic lesion (10-year OS: 69.3%), with a 10-year life expectancy only slightly (84.2%) and not significantly (p=0.1455) lower than that expected in the matched general population. Multivariate analysis selected the MUCY model as one of the most important independent risk factor of CLL OS (HR: 1.38; 95% CI: 1.18–1.60; p<0.0001; 99% bootstrap selection), along with age (HR: 1.06; 95% CI: 1.04–1.07; p<0.0001; 100% bootstrap selection), Rai stage (HR: 1.36; 95% CI: 1.23-1-51; p<0.0001; 100% bootstrap selection) and unmutated IGHV genes (HR: 1.63; 95% CI: 1.17–2.26; p=0.0039; 92% bootstrap selection). Overall, 21.5% (105/488) low risk patients according to the FISH model (del13q14, normal and +12) were reclassified into high risk genetic subgroups by the MUCY model because of the co-occurrence of NOTCH1 (64/488, 13.1%), SF3B1 (35/488, 7.1%), and TP53 (17/488, 3.4%) mutations or BIRC3 disruption (14/488, 2.8%). Consistently, the inclusion of NOTCH1, SF3B1 and BIRC3 lesions in addition to FISH abnormalities significantly improved the model accuracy of OS prediction (c-index: 0.617 vs c-index: 0.642 p<0.0001). At 10 years from diagnosis, 24.5% CLL of the very low and low risk genetic subgroups developed new TP53, NOTCH1, SF3B1, BIRC3 or del11q22-q23 lesions due to clonal evolution, and therefore switched to a higher risk category of the MUCY model. By time-dependent and landmark analysis, the MUCY model retained a statistically significant impact on CLL OS (HR: 1.52; 95% CI: 1.21–1.90; p=0.0003) at any time from diagnosis and independent of its dynamic changes due to clonal evolution. The MUCY model classifies CLL patients into more precise subgroups, advances our understanding of CLL biology, and improves current prognostic algorithms. These findings have relevant implications for the design of clinical trials aimed at assessing the use of mutational profiling to inform therapeutic decisions based on risk stratification. Disclosures: No relevant conflicts of interest to declare.

scholarly journals Sutureless versus Conventional Aortic Valve Replacement: Outcomes in 70 High-Risk Patients Undergoing Concomitant Cardiac Procedures

2018 ◽  
Vol 45 (1) ◽  
pp. 11-16 ◽  
Author(s):  
Muhammet Onur Hanedan ◽  
Mehmet Ali Yuruk ◽  
Ali Ihsan Parlar ◽  
Ugur Ziyrek ◽  
Ali Kemal Arslan ◽  
...  
Keyword(s):  
Cardiopulmonary Bypass ◽  
High Risk ◽  
Aortic Valve ◽  
Aortic Valve Replacement ◽  
Valve Replacement ◽  
Surgical Procedures ◽  
Cardiac Surgical Procedures ◽  
High Risk Patients ◽  
Risk Patients ◽  
Group 1

In elderly, high-risk surgical patients, sutureless aortic valve replacement (AVR) can often be an alternative to conventional AVR; shorter aortic cross-clamp and cardiopulmonary bypass times are the chief advantages. We compared the outcomes of sutureless AVR with those of conventional AVR in 70 elderly patients who underwent concomitant cardiac surgical procedures. We retrospectively analyzed the cases of 42 men and 28 women (mean age, 70.4 ± 10.3 yr; range, 34–93 yr) who underwent cardiac operations plus AVR with either a sutureless valve (group 1, n=38) or a conventional bioprosthetic or mechanical valve (group 2, n=32). Baseline patient characteristics were similar except for worse New York Heart Association functional status and the prevalence of diabetes mellitus in group 1. In group 1, the operative, cross-clamp, and cardiopulmonary bypass times were shorter (all P=0.001), postoperative drainage amounts were lower (P=0.009), hospital stays were shorter (P=0.004), and less red blood cell transfusion was needed (P=0.037). Echocardiograms before patients' discharge from the hospital showed lower peak and mean aortic gradients in group 1 (mean transvalvular gradient, 8.4 ± 2.8 vs 12.2 ± 5.2 mmHg; P=0.012). We found that elderly, high-risk patients who underwent multiple cardiac surgical procedures and sutureless AVR had better hemodynamic outcomes and shorter ischemic times than did patients who underwent conventional AVR.

scholarly journals Long-term colorectal cancer incidence after adenoma removal and the effects of surveillance on incidence: a multicentre, retrospective, cohort study

Gut ◽  
2020 ◽  
Vol 69 (9) ◽  
pp. 1645-1658 ◽  
Author(s):  
Amanda J Cross ◽  
Emma C Robbins ◽  
Kevin Pack ◽  
Iain Stenson ◽  
Paula L Kirby ◽  
...  
Keyword(s):  
Colorectal Cancer ◽  
High Risk ◽  
General Population ◽  
Cox Regression ◽  
Risk Groups ◽  
Low Risk ◽  
Intermediate Risk ◽  
Hazard Ratios ◽  
Risk Patients ◽  
Time Varying Covariates

ObjectivePostpolypectomy colonoscopy surveillance aims to prevent colorectal cancer (CRC). The 2002 UK surveillance guidelines define low-risk, intermediate-risk and high-risk groups, recommending different strategies for each. Evidence supporting the guidelines is limited. We examined CRC incidence and effects of surveillance on incidence among each risk group.DesignRetrospective study of 33 011 patients who underwent colonoscopy with adenoma removal at 17 UK hospitals, mostly (87%) from 2000 to 2010. Patients were followed up through 2016. Cox regression with time-varying covariates was used to estimate effects of surveillance on CRC incidence adjusted for patient, procedural and polyp characteristics. Standardised incidence ratios (SIRs) compared incidence with that in the general population.ResultsAfter exclusions, 28 972 patients were available for analysis; 14 401 (50%) were classed as low-risk, 11 852 (41%) as intermediate-risk and 2719 (9%) as high-risk. Median follow-up was 9.3 years. In the low-risk, intermediate-risk and high-risk groups, CRC incidence per 100 000 person-years was 140 (95% CI 122 to 162), 221 (195 to 251) and 366 (295 to 453), respectively. CRC incidence was 40%–50% lower with a single surveillance visit than with none: hazard ratios (HRs) were 0.56 (95% CI 0.39 to 0.80), 0.59 (0.43 to 0.81) and 0.49 (0.29 to 0.82) in the low-risk, intermediate-risk and high-risk groups, respectively. Compared with the general population, CRC incidence without surveillance was similar among low-risk (SIR 0.86, 95% CI 0.73 to 1.02) and intermediate-risk (1.16, 0.97 to 1.37) patients, but higher among high-risk patients (1.91, 1.39 to 2.56).ConclusionPostpolypectomy surveillance reduces CRC risk. However, even without surveillance, CRC risk in some low-risk and intermediate-risk patients is no higher than in the general population. These patients could be managed by screening rather than surveillance.

Assuring the continued safety of lactic acid bacteria used as probiotics

Biologia ◽  
2006 ◽  
Vol 61 (6) ◽  
Author(s):  
Miguel Gueimonde ◽  
Rafael Frias ◽  
Arthur Ouwehand
Keyword(s):  
Lactic Acid ◽  
Immune System ◽  
High Risk ◽  
General Population ◽  
Lactic Acid Bacteria ◽  
Rare Case ◽  
Opportunistic Pathogens ◽  
Small Risk ◽  
Risk Patients ◽  
Safety Assessments

AbstractLactic acid bacteria have long been used to improve the safety of foods through fermentation. Some fermented products were also early used for their perceived health benefits, which lead to the development of probiotics as we now know them. Probiotics mainly belong to the genera Lactobacillus and Bifidobacterium. Most members of these genera are not considered pathogens or even opportunistic pathogens. Nevertheless, rare cases of Lactobacillus and Bifidobacterium infection have been reported, possibly even associated with the consumption of probiotic products. Such cases are extremely rare and the subjects always had severe underlying conditions most often affecting the immune system. There does not seem to be any risk for the general population. Safety assessments can be performed and many possible tests exist. It is, however, not certain these tests will prevent rare case of Lactobacillus infection in certain high-risk patients. The benefits of probiotic use should be weighed against the possible small risk. Such an evaluation will, in most cases, be favourable and should therefore not discourage consumption of probiotics.

Hybrid cardiovascular therapy: interventional (and surgical) procedures in high-risk patients

2011 ◽  
Vol 3 (2) ◽  
pp. 171-189
Author(s):  
Kamran Muhammad ◽  
Thomas Helton ◽  
Gus Theodos ◽  
Samir Kapadia ◽  
E Murat Tuzcu
Keyword(s):  
High Risk ◽  
Surgical Procedures ◽  
High Risk Patients ◽  
Cardiovascular Therapy ◽  
Risk Patients

Use of NSAIDs for the Chemoprevention of Colorectal Cancer

2003 ◽  
Vol 37 (11) ◽  
pp. 1664-1674 ◽  
Author(s):  
Jill M Herendeen ◽  
Celeste Lindley
Keyword(s):  
Colorectal Cancer ◽  
High Risk ◽  
General Population ◽  
Clinical Studies ◽  
Adenomatous Polyps ◽  
Medline Search ◽  
High Risk Patients ◽  
Incidence And Mortality ◽  
Risk Patients ◽  
Cox 2

OBJECTIVE: To discuss the role of nonsteroidal antiinflammatory drugs (NSAIDs) in the chemoprevention of colorectal cancer. DATA SOURCES: A MEDLINE search (1966–May 2003) was performed to identify key literature. Search items included, but were not limited to, NSAIDs, colorectal cancer, chemoprevention, cyclooxygenase-2 (COX-2)–specific inhibitors, and familial adenomatous polyposis (FAP). STUDY SELECTION AND DATA EXTRACTION: The search included experimental (in vitro and animal models) and clinical studies evaluating the use of NSAIDs for the chemoprevention of colorectal cancer. The MEDLINE search was supplemented by references from selected articles. DATA SYNTHESIS: Numerous experimental, epidemiologic, and clinical studies suggest that NSAIDs have promise as anticancer agents. The mechanism by which NSAIDs lead to decreased colon carcinogenesis is not fully understood, but may involve restoration of apoptosis and inhibition of prostaglandin-mediated angiogenesis. Compelling evidence from many observational studies has consistently documented a 40–50% reduction in the risk of adenomatous polyps, colorectal cancer incidence, and mortality in patients using NSAIDs. Recent randomized, controlled trials have demonstrated a benefit with aspirin in reducing the rate of development of new or recurrent adenomas in high-risk patients. In addition, randomized studies using sulindac and celecoxib in patients with FAP have documented significant regression of existing adenomatous polyps. CONCLUSIONS: Inhibition of COX-2 is an example of a targeted approach to the chemoprevention of colorectal cancer. However, controversy exists about the safety, efficacy, and optimal treatment regimen of NSAIDs as long-term chemopreventive agents in the general population. Ongoing studies in high-risk patients with both selective and nonselective COX inhibitors will provide important information in the area of colorectal chemoprevention, but clinical trials' use of adenomas as surrogate markers for chemoprevention trials makes their application to the general population limited.

scholarly journals Colorectal cancer risk following polypectomy in a multicentre, retrospective, cohort study: an evaluation of the 2020 UK post-polypectomy surveillance guidelines

Gut ◽  
2021 ◽  
pp. gutjnl-2020-323411
Author(s):  
Amanda J Cross ◽  
Emma C Robbins ◽  
Kevin Pack ◽  
Iain Stenson ◽  
Bhavita Patel ◽  
...  
Keyword(s):  
Colorectal Cancer ◽  
High Risk ◽  
General Population ◽  
Cox Regression ◽  
Population Based ◽  
Low Risk ◽  
High Grade Dysplasia ◽  
High Grade ◽  
High Risk Patients ◽  
Risk Patients

ObjectiveColonoscopy surveillance aims to reduce colorectal cancer (CRC) incidence after polypectomy. The 2020 UK guidelines recommend surveillance at 3 years for ‘high-risk’ patients with ≥2 premalignant polyps (PMPs), of which ≥1 is ‘advanced’ (serrated polyp (or adenoma) ≥10 mm or with (high-grade) dysplasia); ≥5 PMPs; or ≥1 non-pedunculated polyp ≥20 mm; ‘low-risk’ patients without these findings are instead encouraged to participate in population-based CRC screening. We examined the appropriateness of these risk classification criteria and recommendations.DesignRetrospective analysis of patients who underwent colonoscopy and polypectomy mostly between 2000 and 2010 at 17 UK hospitals, followed-up through 2017. We examined CRC incidence by baseline characteristics, risk group and number of surveillance visits using Cox regression, and compared incidence with that in the general population using standardised incidence ratios (SIRs).ResultsAmong 21 318 patients, 368 CRCs occurred during follow-up (median: 10.1 years). Baseline CRC risk factors included age ≥55 years, ≥2 PMPs, adenomas with tubulovillous/villous/unknown histology or high-grade dysplasia, proximal polyps and a baseline visit spanning 2–90 days. Compared with the general population, CRC incidence without surveillance was higher among those with adenomas with high-grade dysplasia (SIR 1.74, 95% CI 1.21 to 2.42) or ≥2 PMPs, of which ≥1 was advanced (1.39, 1.09 to 1.75). For low-risk (71%) and high-risk (29%) patients, SIRs without surveillance were 0.75 (95% CI 0.63 to 0.88) and 1.30 (1.03 to 1.62), respectively; for high-risk patients after first surveillance, the SIR was 1.22 (0.91 to 1.60).ConclusionThese guidelines accurately classify post-polypectomy patients into those at high risk, for whom one surveillance colonoscopy appears appropriate, and those at low risk who can be managed by non-invasive screening.

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