BACKGROUND Survival in patients with autoimmune liver disease overlap syndromes(AILDOS)compared to those with single autoimmune liver disease is unclear.AIM To investigate the survival of patients with AILDOS and asse...BACKGROUND Survival in patients with autoimmune liver disease overlap syndromes(AILDOS)compared to those with single autoimmune liver disease is unclear.AIM To investigate the survival of patients with AILDOS and assess the accuracy of non-invasive serum models for predicting liver-related death.METHODS Patients with AILDOS were defined as either autoimmune hepatitis and primary biliary cholangitis overlap(AIH-PBC)or autoimmune hepatitis and primary sclerosing cholangitis overlap(AIH-PSC)and were identified from three tertiary centres for this cohort study.Liver-related death or transplantation(liver-related mortality)was determined using a population-based data linkage system.Prognostic scores for liver-related death were compared for accuracy[including liver outcome score(LOS),Hepascore,Mayo Score,model for end-stage liver disease(MELD)score and MELD incorporated with serum sodium(MELD-Na)score].RESULTS Twenty-two AILDOS patients were followed for a median of 3.1 years(range,0.35-7.7).Fourteen were female,the median age was 46.7 years(range,17.8 to 82.1)and median Hepascore was 1(range,0.07-1).At five years post enrolment,57%of patients remained free from liver-related mortality(74%AIH-PBC,27%AIH-PSC).There was no significant difference in survival between AIH-PBC and AIH-PSC.LOS was a significant predictor of liver-related mortality(P<0.05)in patients with AIH-PBC(n=14)but not AIH-PSC(n=8).A LOS cut-point of 6 discriminated liver-related mortality in AIH-PBC patients(P=0.012,log-rank test,100%sensitivity,77.8%specificity)(Harrell's C-statistic 0.867).The MELD score,MELD-Na score and Mayo Score were not predictive of liver-related mortality in any group.CONCLUSION Survival in the rare,AILDOS is unclear.The current study supports the LOS as a predictor of liver-related mortality in AIH-PBC patients.Further trials investigating predictors of survival in AILDOS are required.展开更多
Introduction: Acute coronary syndromes (ACS) are a diagnostic and therapeutic emergency. Objective: Studying the feasibility, difficulties and results of coronary angioplasty in acute coronary syndromes at the Luxembo...Introduction: Acute coronary syndromes (ACS) are a diagnostic and therapeutic emergency. Objective: Studying the feasibility, difficulties and results of coronary angioplasty in acute coronary syndromes at the Luxembourg Mother-Child University Hospital in Bamako. Patients and Methods: Cross-sectional, descriptive study with prospective recruitment over 18 months from September 2020 to February 2022. All patients aged at least 18 years old admitted for SCA and having undergone PCI during the study period were included. Result: We collected 249 patients for SCA, of whom 160 underwent angioplasty, either an angioplasty/SCA ratio of 0.64. The average age of the patients was 59.54 ± 11.62 with extremes of 32 and 92 years. The age group of 45 to 65 years was the most representative. The predominance was male, sex ratio of 2.4. The main cardiovascular risk factors were high blood pressure (58.23%) and diabetes (45.78%). Persistent ST-segment elevation on the electrocardiogram was present in 71.48%. The treatment time was more than 12 hours after the first medical contact in 95.5%. The approach was radial in 96.5% of cases. Coronarography was pathological in 91.16% of our patients. The lesions were tri-truncular in 34.13% with the anterior inter ventricular as culprit artery in 72% of cases. The majority of patients (64%) had undergone angioplasty with implantation of an active stent. Angioplasty was performed successfully in 98% and per procedural mortality was 1.87%. Only 6.45% of ACS with ST elevation benefited from primary angioplasty. Conclusion: Percutaneous coronary intervention is performed routinely in our center with satisfactory results. Difficulties exist, related to the diagnostic delay of ACS and the high cost of angioplasty.展开更多
Background: Myocardial ischemia in addition to other several cardiac syndromes represent a pathological proinflammatory state alongside a complex cellular microenvironment that can be modified by using cannabinoids. C...Background: Myocardial ischemia in addition to other several cardiac syndromes represent a pathological proinflammatory state alongside a complex cellular microenvironment that can be modified by using cannabinoids. Cannabidiol (CBD), a non-psychoactive compound of cannabis has been recently proposed as an immudomodulatory and cardioprotective drug. Objectives: In this systematic review we sought to clarify and summarize the clinical and preclinical evidence of potential benefit of the use of CBD in coronary syndromes. Methods: We conducted a systematic search and review according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) and Review of Animal Data from Experimental Studies (CAMARADES) guidelines, in the electronic database from PubMed, Web of Science and Scopus up to April 2022 using predefined search terms. Pre-specified exclusion and inclusion criteria were considered, finally 11 articles were chosen to be included for this peer review. Results: Currently there are no good-quality clinical trials with the use of CBD in acute or chronic coronary syndromes. A total of 11 preclinical studies where prescreened and 5 demonstrated reproducible positive cardiovascular outcomes on in-vivo models treated with CBD. Mechanisms of CBD cardioprotection observed: 1) reduction in oxidative stress and inflammation, 2) activation of adenosine receptors and 3) increased expression of angiotensin type 2-receptor. Experimental models included ischemia/reperfusion injury, myocardial infarction, arrhythmias, and metabolic syndrome-like conditions. Conclusion: No clinical recommendation can be issued with the current evidence, on the use of CBD in acute and chronic coronary syndromes. Based on preclinical evidence, we considered there is enough evidence to propose the development of well-designed clinical trials that include CBD in the management of coronary syndromes.展开更多
Obesity and related metabolic syndromes have been recognized as important disease risks,in which the role of adipokines cannot be ignored.Adiponectin(ADP)is one of the key adipokines with various beneficial effects,in...Obesity and related metabolic syndromes have been recognized as important disease risks,in which the role of adipokines cannot be ignored.Adiponectin(ADP)is one of the key adipokines with various beneficial effects,including improving glucose and lipid metabolism,enhancing insulin sensitivity,reducing oxidative stress and inflammation,promoting ceramides degradation,and stimulating adipose tissue vascularity.Based on those,it can serve as a positive regulator in many metabolic syndromes,such as type 2 diabetes(T2D),cardiovascular diseases,non-alcoholic fatty liver disease(NAFLD),sarcopenia,neurodegenerative diseases,and certain cancers.Therefore,a promising therapeutic approach for treating various metabolic diseases may involve elevating ADP levels or activating ADP receptors.The modulation of ADP genes,multimerization,and secretion covers the main processes of ADP generation,providing a comprehensive orientation for the development of more appropriate therapeutic strategies.In order to have a deeper understanding of ADP,this paper will provide an all-encompassing review of ADP.展开更多
Objective: Hyperlipidemia is a representative nutritional metabolic disease in clinic, which is easy to induce atherosclerotic cardiovascular and cerebrovascular diseases, with complex classification. Traditional Chin...Objective: Hyperlipidemia is a representative nutritional metabolic disease in clinic, which is easy to induce atherosclerotic cardiovascular and cerebrovascular diseases, with complex classification. Traditional Chinese Medicine (TCM) syndrome aims to reflect the characteristics of diseases and is the basic principle of TCM treatment of diseases. This study aimed to summarize the distribution pattern of TCM syndromes in patients with hyperlipidemia. Methods: The frequency, characteristics and distribution of all TCM syndromes of 1012 patients with hyperlipidemia were analyzed. Results: The main disease types determined by frequency of 1012 patients included hypertriglyceridemia combined with hypo high-density lipoprotein cholesterolemia (19.76%), hypo high-density lipoprotein cholesterolemia (18.58%), hypercholesterolemia (16.50%), mixed hyperlipidemia (16.40%), and hypertriglyceridemia (15.12%). The distribution of TCM syndromes, in order of frequency, was as follows: Qi-deficiency and blood stasis syndrome (23.52%), liver depression and spleen deficiency (9.88%), syndrome of qi stagnation and blood stasis (9.29%), phlegm stasis syndrome (7.41%), and syndrome of yang deficiency of spleen and kidney (6.92%). Conclusion: Qi-deficiency and blood stasis syndrome and liver depression and spleen deficiency are the most common TCM syndromes in patients with hyperlipidemia.展开更多
The association between genetic syndromes and odontogenic tumors encompasses several entities,reflecting the intricate interplay between genetic factors and the development of these lesions.The present study aimed to ...The association between genetic syndromes and odontogenic tumors encompasses several entities,reflecting the intricate interplay between genetic factors and the development of these lesions.The present study aimed to comprehensively investigate the associations between genetic syndromes and odontogenic tumors.We delineated the diverse spectrum of syndromic connections,including key syndromes such as Gardner syndrome,Gorlin syndrome,Schimmelpenning syndrome,and others.Our findings underscore the clinical significance of recognizing odontogenic tumors associated with genetic syndromes as diagnostic indicators for early intervention.We advocate for multidisciplinary collaboration among clinicians,geneticists,and researchers to deepen our understanding of the underlying mechanisms driving these syndromic associations.In light of this,our study contributes to the growing body of knowledge in dentistry and medical genetics,offering insights that may inform clinical practice and enhance patient care for individuals affected by genetic syndromes and odontogenic tumors.展开更多
Background: In response to the escalating burden of cardiovascular diseases (CVDs) worldwide, exacerbated by lifestyle changes and socioeconomic shifts, acute coronary syndromes (ACS) stand out as a leading cause of m...Background: In response to the escalating burden of cardiovascular diseases (CVDs) worldwide, exacerbated by lifestyle changes and socioeconomic shifts, acute coronary syndromes (ACS) stand out as a leading cause of morbidity and mortality. The pivotal role of insulin resistance in the pathogenesis of atherosclerosis, independent of traditional risk factors, has garnered significant interest. Objective: This review aims to synthesize the recent advancements in the utilization of the triglyceride glucose index (TyG index) as a biomarker for assessing the severity and predicting the prognosis of ACS lesions. Methods: A systematic search was conducted across PubMed, Embase, and Scopus databases, incorporating keywords such as “triglyceride glucose index”, “TyG index”, “acute coronary syndrome”, “cardiovascular disease”, “insulin resistance”, “coronary artery calcification”, “SYNTAX score”, “Gensini score”, and “major adverse cardiac events”. Studies were included from the inception of each database up to July 2024. Selection criteria encompassed observational studies, case-control studies, and randomized controlled trials, with a particular emphasis on evaluating the diagnostic and prognostic value of the TyG index in patients with acute coronary syndromes. Ultimately, 46 publications met the inclusion criteria. Data extraction and quality assessment were performed in accordance with established guidelines. Results: Evidence suggests that the TyG index, reflecting insulin resistance, blood glucose, and lipid levels, is significantly associated with lesion severity in ACS, including coronary artery calcification, SYNTAX score, and Gensini score. Moreover, it demonstrates predictive power for major adverse cardiovascular events, underscoring its potential as a valuable tool in clinical decision-making. Conclusion: The review highlights the emerging role of the TyG index in the assessment and prognosis of ACS, advocating for its incorporation into clinical practice as a complement to existing diagnostic modalities. However, the establishment of standardized reference ranges and further validation across diverse populations are warranted to refine its applicability in personalized medicine. The interdisciplinary approach is essential to advance our understanding of the complex interplay between insulin resistance and cardiovascular disease, paving the way for the development of more effective prevention and treatment strategies.展开更多
Paraneoplastic neurological syndrome refers to certain malignant tumors that have affected the distant nervous system and caused corresponding dysfunction in the absence of tumor metastasis.Patients with this syndrome...Paraneoplastic neurological syndrome refers to certain malignant tumors that have affected the distant nervous system and caused corresponding dysfunction in the absence of tumor metastasis.Patients with this syndrome produce multiple antibodies,each targeting a different antigen and causing different symptoms and signs.The CV2/collapsin response mediator protein 5(CRMP5)antibody is a major antibody of this type.It damages the nervous system,which often manifests as limbic encephalitis,chorea,ocular manifestation,cerebellar ataxia,myelopathy,and peripheral neuropathy.Detecting CV2/CRMP5 antibody is crucial for the clinical diagnosis of paraneoplastic neurological syndrome,and anti-tumor and immunological therapies can help to alleviate symptoms and improve prognosis.However,because of the low incidence of this disease,few repo rts and no reviews have been published about it so far.This article intends to review the research on CV2/CRMP5antibody-associated paraneoplastic neurological syndrome and summarize its clinical features to help clinicians comprehensively understand the disease.Additionally,this review discusses the curre nt challenges that this disease poses,and the application prospects of new detection and diagnostic techniques in the field of paraneoplastic neurological syndrom e,including CV2/CRMP5-associated paraneoplastic neurological syndrome,in recent years.展开更多
The present article tests the following general assumption:plant taxa with different specializations towards mycorrhizal interactions should have different root syndromes.Roots of 61 species common in boreal zone were...The present article tests the following general assumption:plant taxa with different specializations towards mycorrhizal interactions should have different root syndromes.Roots of 61 species common in boreal zone were studied:16 species of Poaceae,24 species of Cyperaceae,14 species of Orchidaceae,and 7 species of Iridaceae.Using a fixed material of 5 individuals of each species,the following was determined:number of orders of branching roots;transverse dimensions of root,stele and cortex;number of primary xylem vessels and exodermis layers;length of root hairs;abundance of mycorrhiza.Species of each family had well-defined syndromes.Roots of Orchidaceae and Iridaceae were thick with a large stele and developed exodermis.Orchidaceae had no branching roots and had long root hairs.In Iridaceae,roots were branched,and root hairs were short.Roots of Poaceae and Cyperaceae were thin with a relatively thin stele.Root hairs were short in Poaceae and long in Cyperaceae.Our finding that root syndromes of four families of monocots differed is a new and unexpected discovery.The high specificity of root syndromes in Cyperaceae,Iridaceae,Poaceae,and Orchidaceae indicates that species of these families use different strategies to obtain water and soil nutrients.展开更多
Background:Traditional Chinese medicine(TCM)syndrome is the basic unit of TCM treatment,which help clinicians assess the disease progression and treatment preoperative of tumor patients.However,the prognostic signific...Background:Traditional Chinese medicine(TCM)syndrome is the basic unit of TCM treatment,which help clinicians assess the disease progression and treatment preoperative of tumor patients.However,the prognostic significance of TCM syndrome is still unclear.This study aims to detect the differences in overall survival between different TCM syndrome and further develop a new nomogram with TCM syndrome for predicting overall survival in colorectal cancer.Methods:A total of 324 patients with colorectal cancer were enrolled and categorized into three groups based on TCM syndrome:deficiency,excess,and deficiency-excess.The prognosis of colorectal cancer patients with different TCM syndromes was evaluated using Kaplan-Meier analysis and Cox regression analysis.Results:The proportion of advanced stage and lymph metastasis in the patients with deficiency syndrome was higher,and the overall survival was shorter than other syndromes.Meanwhile,the TCM syndrome(P<0.001),tumor invasion depth(P<0.001),lymph metastasis(P=0.018),organic metastasis(P=0.005)and tumor node metastasis(TNM)stage(P=0.029)were the independent prognostic factor.Then,a new nomogram with TCM syndrome was established and assessed.324 colorectal cancer patients were randomly divided into training(n=215)and validation cohorts(n=109).A nomogram incorporating preoperative TCM syndrome,gender,age,T,N,and M status was developed,which had good discrimination and calibration.Conclusion:Taken together,our results indicated that TCM syndrome could assess the prognosis of colorectal cancer.The nomogram incorporating TCM syndromes and tumor information is helpful for risk stratification and prognostic predictions in colorectal cancer preoperatively.展开更多
Hereditary cancer syndromes(HCSs)are arguably the most frequent category of Mendelian genetic diseases,as at least 2%of presumably healthy subjects carry highly-penetrant tumor-predisposing pathogenic variants(PVs).He...Hereditary cancer syndromes(HCSs)are arguably the most frequent category of Mendelian genetic diseases,as at least 2%of presumably healthy subjects carry highly-penetrant tumor-predisposing pathogenic variants(PVs).Hereditary breast-ovarian cancer and Lynch syndrome make the highest contribution to cancer morbidity;in addition,there are several dozen less frequent types of familial tumors.The development of the majority albeit not all hereditary malignancies involves two-hit mechanism,i.e.the somatic inactivation of the remaining copy of the affected gene.Earlier studies on cancer families suggested nearly fatal penetrance for the majority of HCS genes;however,population-based investigations and especially large-scale next-generation sequencing data sets demonstrate that the presence of some highly-penetrant PVs is often compatible with healthy status.Hereditary cancer research initially focused mainly on cancer detection and prevention.Recent studies identified multiple HCS-specific drug vulnerabilities,which translated into the development of highly efficient therapeutic options.展开更多
In this editorial we present an overview and insights of the management of hereditary polyposis syndromes.The primary focus was on familial adenomatous polyposis,juvenile polyposis syndrome and Peutz-Jegher syndrome.G...In this editorial we present an overview and insights of the management of hereditary polyposis syndromes.The primary focus was on familial adenomatous polyposis,juvenile polyposis syndrome and Peutz-Jegher syndrome.Genetic testing has become increasingly available and is easier than ever to integrate into clinical practice.Furthermore,several genes have been added to the expanding list of genes associated with hereditary polyposis syndromes,allowing for precise diagnostics and tailored follow-up.Endoscopic evaluation of patients with hereditary polyposis syndromes is paramount in the surveillance strategies.Current endoscopic procedures include both diagnostic procedures and surveillance as well as therapeutic interventions.Recommendations for endoscopic procedures in the upper and lower gastrointestinal canal were described.Surgery is still a key component in the management of patients with hereditary polyposis syndromes.The increased cancer risk in these patients often render prophylactic procedures or intended curative procedures in the case of cancer development.Surgical interventions in the upper and lower gastrointestinal canal were described with relevant considerations.Development of chemopreventive medications is ongoing.Few drugs have been investigated,including nonsteroidal anti-inflammatory drugs.It has been demonstrated that cyclooxygenase-2 inhibitors may lower the number of polyps.Other medications are currently under investigation,but none have,to date,consistently been able to prevent development of disease.展开更多
Background: Ischaemic heart disease is the cause of 7.4 million deaths per year. Their prevention is based on the management of cardiovascular risk factors, but also on the early detection and management of chronic co...Background: Ischaemic heart disease is the cause of 7.4 million deaths per year. Their prevention is based on the management of cardiovascular risk factors, but also on the early detection and management of chronic coronary syndromes (CCS), for which few data are available in Africa. The main objective of our study was to determine the factors related to significative coronary artery disease in patients undergoing coronarography for suspected chronic coronary syndrome (CCS). Methodology: We conducted a retrospective descriptive and analytical study over 2 years (from January, 1<sup>st</sup>, 2018 to December 31<sup>st</sup>, 2019) in the Cardiology Department of the University Hospital Aristide Le DANTEC in Dakar. All patients admitted for coronary angiography for suspected chronic coronary syndrome were included. Results: One hundred and fifty-two patients were included with a mean age of 60.79 ± 9.73 years, the most represented age group was 60 - 69 years. Advanced age was the most frequent risk factor (77.63%) followed by sedentary lifestyle (56.58%) and hypertension (41.45%). Diabetes was present in 17.1% of cases. A history of angioplasty was found in 1.97% of patients. Typical pain was found in 71.05% of cases, atypical pain in 19.74% and exertional dyspnoea in 2.63%. The pre-test probability was intermediate in 67.1% of cases, low in 25% and high in 7.9%. Significative coronary lesion was found in 52.63% of the patients, while coronary angiography was normal in the remaining cases. Tritroncular status was observed in 37.50%, it was bitroncular in 26.25% and monotroncular in 36.25% of cases. Factors associated with significative coronary artery disease were age (p = 0.0001), diabetes (p = 0.006), previous angioplasty (p = 0.023), previous myocardial infarction (p = 0.018), typical angina (p = 0.001), intermediate pretest probability (p = 0.001). Low pretest probability was significantly correlated with the absence of a coronary lesion with a p = 0.001. Conclusion: Our study shows that screening for chronic coronary disease should be done especially in diabetics, elderly subjects and those with previous angioplasty taking into account symptoms and pretest probability to avoid unnecessary invasive procedures.展开更多
BACKGROUND During the second wave of the coronavirus disease 2019(COVID-19)pandemic,a subset of critically ill patients developed delayed respiratory deterioration in the absence of new infection,fluid overload or ext...BACKGROUND During the second wave of the coronavirus disease 2019(COVID-19)pandemic,a subset of critically ill patients developed delayed respiratory deterioration in the absence of new infection,fluid overload or extra-pulmonary organ dysfunction.AIM To describe the clinical and laboratory characteristics,outcomes,and management of these patients,and to contrast this entity with other post COVID-19 immune dysregulation related inflammatory disorders.METHODS This was a retrospective observational study of adult patients admitted to the medical intensive care unit of a 2200-bed university affiliated teaching hospital,between May and August 2021,who fulfilled clearly defined inclusion and exclusion criteria.Outcome was assessed by a change in PaO_(2)/FiO_(2) ratio and levels of inflammatory markers before and after immunomodulation,duration of mechanical ventilation after starting treatment,and survival to discharge.RESULTS Five patients developed delayed respiratory deterioration in the absence of new infection,fluid overload or extrapulmonary organ dysfunction at a median interquartile range(IQR)duration of 32(23-35)d after the onset of symptoms.These patients had elevated inflammatory markers,required mechanical ventilation for 13(IQR 10-23)d,and responded to glucocorticoids and/or intravenous immunoglobulin.One patient died(20%).CONCLUSION This delayed respiratory worsening with elevated inflammatory markers and clinical response to immunomodulation appears to contrast the well described Multisystem Inflammatory Syndrome–Adults by the paucity of extrapulmonary organ involvement.The diagnosis can be considered in patients presenting with delayed respiratory worsening,that is not attributable to cardiac dysfunction,fluid overload or ongoing infections,and associated with an increase in systemic inflammatory markers like C-reactive protein,inteleukin-6 and ferritin.A good response to immunomodulation can be expected.This delayed inflammatory pulmonary syndrome may represent a distinct clinical entity in the spectrum of inflammatory syndromes in COVID-19 infection.展开更多
Objective To investigate the relationship among serum vitamin D levels, physical performance impairment, and geriatric syndromes in elders with hypertension. Methods According to the concentration of vitamin D levels,...Objective To investigate the relationship among serum vitamin D levels, physical performance impairment, and geriatric syndromes in elders with hypertension. Methods According to the concentration of vitamin D levels, a total of 143 elderly patients with hypertension were classified into vitamin D deficient group （vitamin D 〈 20 ng/mL, n = 94） and vitamin D appropriate group （vitamin D 〉 20 ng/rnL, n = 49）. Geriatric syndromes and physical performance were assessed by using comprehensive geriatric assessment （CGA）. Correlation among vitamin D levels, geriatric syndromes and physical performance was analyzed. Results No statistical differences were found in various aspects of geriatric syndromes between the two groups （P 〉 0.05）. While correlation analysis indicated that vitamin D levels had a positive association with ADL score （r = 0.235, P 〈 0.01） and a negative association with Morse fall scale score （r = 0.238, P 〈 0.01）. Patients with deficient vitamin D level had longer time both in the Five Time Sit to Stand Test （5tSTS）, （15.765 ± 5.593） and the four-meter walk test [7.440 （5.620, 9.200）], a weaker hand-grip in the grip strength test （28.049 ± 9.522）, and a lower Tinetti performance-oriented mobility assessment （Tinetti POMA） [26 （22, 27）] and Balance subscale of the Tinetti performance-oriented mobility assessment （B-POMA） score [ 14 （12, 16）], compared with appropriate vitamin D level [（13.275 ± 3.692）; 5.810 （4.728, 7.325）]; （31.989 ± 10.217）; [26.5 （25, 28）]; [15 （14, 16）, respectively, all P 〈 0.05]. Furthermore, results of logistic regression indicated that vitamin D was significantly associated with 5tSTS （OR = 1.2, 95% CI = 1.050-1.331, P 〈 0.01）, Tinetti POMA （OR = 3.7, 95% CI：1.284-10.830, P 〈 0.05） and B-POMA （OR = 0.8, 95% CI：0.643-0.973, P 〈 0.05）. Conclusions In elderly hypertensive patients, serum vitamin D deficient level is associated with physical performance impairment. However, no statistical significance was found between vitamin D and geriatric syndromes. Further study is required to investigate possible mechanisms for the association between vitamin D and physical performance.展开更多
The myelodysplastic/myeloproliferative neoplasms(MDS/MPNs) are a unique group of hematologic malignancies characterized by concomitant myelodysplastic and myeloproliferative features. According to the 2008 WHO classif...The myelodysplastic/myeloproliferative neoplasms(MDS/MPNs) are a unique group of hematologic malignancies characterized by concomitant myelodysplastic and myeloproliferative features. According to the 2008 WHO classification, the category includes atypical chronic myeloid leukemia(a CML), chronic myelomonocytic leukemia(CMML), juvenile myelomonocytic leukemia(JMML), MDS/MPN-unclassifiable(MDS/MPN-U), and the provisional entity refractory anemia with ring sideroblasts and thrombocytosis(RARS-T). Although diagnosis currently remains based on clinicopathologic features, the incorporation of nextgeneration platforms has allowed for the recent molecular characterization of these diseases which has revealed unique and complex mutational profiles that support their distinct biology and is anticipated to soon play an integral role in diagnosis,prognostication, and treatment. Future goals of research should include the development of disease-modifying therapies, and further genetic understanding of the category will likely form the foundation of these efforts.展开更多
Because of the rarity of familial gastrointestinal cancerpredisposing syndromes,their exploration in literature is not extensive.In this review,an update of the clinicopathological and molecular criteria of gastrointe...Because of the rarity of familial gastrointestinal cancerpredisposing syndromes,their exploration in literature is not extensive.In this review,an update of the clinicopathological and molecular criteria of gastrointestinal familial polyposis syndromes with potential malignant transformation is performed.In addition,a guide for screening and surveillance was synthesized and a distribution of gene mutations according to the specific syndromes and geographic distribution was included.The following inherited polyposes syndromes were analyzed: familial adenomatous polyposis,the hamartomatous familial polyposes(Juvenile polyposis,Peutz-Jeghers syndrome,Cowden syndrome,BannayanRiley-Ruvalcaba syndrome,hereditary mixed polyposis syndrome,Gorlin syndrome,Birt-Hogg-Dube syndrome,neurofibromatosis type Ⅰand multiple endocrine neoplasia syndrome 2B),Li-Fraumeni syndrome,and MUTYHassociated adenomatous polyposis.For proper medical care,subspecialization of gastroenterologists,pathologists,and genticists in the field of familial diseases should be introduced in the medical curriculum.展开更多
Background Elderly patients with non-ST-segment elevation acute coronary syndromes(NSTE-ACS)may present delirium but its clinical relevance is unknown.This study aimed at detennining the clinical associated factors,an...Background Elderly patients with non-ST-segment elevation acute coronary syndromes(NSTE-ACS)may present delirium but its clinical relevance is unknown.This study aimed at detennining the clinical associated factors,and prognostic implications of delirium in old-aged patients admitted for NSTE-ACS.Methods LONGEVO-SCA is a prospective multicenter registry including unselected patients with NSTE-ACS aged>80 years.Clinical variables and a complete geriatric evaluation were assessed during hospitalization.The association between delirium and 6-month mortality was assessed by a Cox regression model weighted for a propensity score including the potential confounding variables.We also analysed its association with 6-month bleeding and cognitive or functional decline.Results Among 527 patients included,thirty-seven(7%)patients presented delirium during the hospitalization.Delirium was more frequent in patients with dementia or depression and in those from nursing homes(27.0%vs.3.1%,24.3%vs.11.6%,and 11.1%V5.2.2%,respectively;all P<0.05).Delirium was significantly associated with in-hospital infections(27.0%vs.5.3%,P<0.001)and usage of diuretics(70.3%vs.49.8%,P=0.02).Patients with delirium had longer hospitalizations[median 8.5(5.5-14)vs.6.0(4.0-10)days,P=0.02]and higher incidence of 6-month bleeding and mortality(32.3%vs.10.0%and 24.3%vs.10.8%,respectively;both P<0.05)but similar cognitive or functional decline.Delirium was independently associated with 6-month mortality(HR=1.47,95%CI:1.02-2.13,P=0.04)and 6-month bleeding events(OR=2.87;95%CI:1.98-4」6,P<0.01).Conclusions In-hospital delirium in elderly patients with NSTE-ACS is associated with some preventable risk factors and it is an independent predictor of 6-month mortality.展开更多
BACKGROUND:The complications of systemic inflammatory response syndrome(SIRS)include acute lung injury,acute kidney injury,shock,and multiple organ dysfunction syndrome(MODS).In recent years,how to clear inflammatory ...BACKGROUND:The complications of systemic inflammatory response syndrome(SIRS)include acute lung injury,acute kidney injury,shock,and multiple organ dysfunction syndrome(MODS).In recent years,how to clear inflammatory mediators has become a hot topic in critical care medicine.Researchers hypothesize that continuous blood purification(CBP) can effectively eliminate a variety of inflammatory mediators which participate in the occurrence of MODS and adjust the immune imbalance.This study aimed to observe the effects of CBP in MODS patients.METHODS:In this retrospective clinical study,a total of 38 MODS patients,18 males and 20 females,were enrolled.After conventional therapy,all the patients received CBP.Biochemistry,blood gas analysis,oxygenation index,mean arterial blood pressure(MAP),acute physiology and chronic health evaluation(APACHE) II scores were monitored.RESULTS:After CBP,the vital signs of patients were rapidly stable,and electrolyte disorders and acid-base imbalance were corrected.Renal function,blood gas,oxygenation index were all improved.MAP was increased,and APACHE II score was significantly decreased.All patients had good tolerance,stable hemodynamics,and no obvious adverse reaction on CBP compared with pre-CBP.CONCLUSION:CBP can effectively clean toxins,correct electrolyte acid-base balance,and improve systemic inflammatory response syndrome and the organ function of MODS patients.展开更多
文摘BACKGROUND Survival in patients with autoimmune liver disease overlap syndromes(AILDOS)compared to those with single autoimmune liver disease is unclear.AIM To investigate the survival of patients with AILDOS and assess the accuracy of non-invasive serum models for predicting liver-related death.METHODS Patients with AILDOS were defined as either autoimmune hepatitis and primary biliary cholangitis overlap(AIH-PBC)or autoimmune hepatitis and primary sclerosing cholangitis overlap(AIH-PSC)and were identified from three tertiary centres for this cohort study.Liver-related death or transplantation(liver-related mortality)was determined using a population-based data linkage system.Prognostic scores for liver-related death were compared for accuracy[including liver outcome score(LOS),Hepascore,Mayo Score,model for end-stage liver disease(MELD)score and MELD incorporated with serum sodium(MELD-Na)score].RESULTS Twenty-two AILDOS patients were followed for a median of 3.1 years(range,0.35-7.7).Fourteen were female,the median age was 46.7 years(range,17.8 to 82.1)and median Hepascore was 1(range,0.07-1).At five years post enrolment,57%of patients remained free from liver-related mortality(74%AIH-PBC,27%AIH-PSC).There was no significant difference in survival between AIH-PBC and AIH-PSC.LOS was a significant predictor of liver-related mortality(P<0.05)in patients with AIH-PBC(n=14)but not AIH-PSC(n=8).A LOS cut-point of 6 discriminated liver-related mortality in AIH-PBC patients(P=0.012,log-rank test,100%sensitivity,77.8%specificity)(Harrell's C-statistic 0.867).The MELD score,MELD-Na score and Mayo Score were not predictive of liver-related mortality in any group.CONCLUSION Survival in the rare,AILDOS is unclear.The current study supports the LOS as a predictor of liver-related mortality in AIH-PBC patients.Further trials investigating predictors of survival in AILDOS are required.
文摘Introduction: Acute coronary syndromes (ACS) are a diagnostic and therapeutic emergency. Objective: Studying the feasibility, difficulties and results of coronary angioplasty in acute coronary syndromes at the Luxembourg Mother-Child University Hospital in Bamako. Patients and Methods: Cross-sectional, descriptive study with prospective recruitment over 18 months from September 2020 to February 2022. All patients aged at least 18 years old admitted for SCA and having undergone PCI during the study period were included. Result: We collected 249 patients for SCA, of whom 160 underwent angioplasty, either an angioplasty/SCA ratio of 0.64. The average age of the patients was 59.54 ± 11.62 with extremes of 32 and 92 years. The age group of 45 to 65 years was the most representative. The predominance was male, sex ratio of 2.4. The main cardiovascular risk factors were high blood pressure (58.23%) and diabetes (45.78%). Persistent ST-segment elevation on the electrocardiogram was present in 71.48%. The treatment time was more than 12 hours after the first medical contact in 95.5%. The approach was radial in 96.5% of cases. Coronarography was pathological in 91.16% of our patients. The lesions were tri-truncular in 34.13% with the anterior inter ventricular as culprit artery in 72% of cases. The majority of patients (64%) had undergone angioplasty with implantation of an active stent. Angioplasty was performed successfully in 98% and per procedural mortality was 1.87%. Only 6.45% of ACS with ST elevation benefited from primary angioplasty. Conclusion: Percutaneous coronary intervention is performed routinely in our center with satisfactory results. Difficulties exist, related to the diagnostic delay of ACS and the high cost of angioplasty.
文摘Background: Myocardial ischemia in addition to other several cardiac syndromes represent a pathological proinflammatory state alongside a complex cellular microenvironment that can be modified by using cannabinoids. Cannabidiol (CBD), a non-psychoactive compound of cannabis has been recently proposed as an immudomodulatory and cardioprotective drug. Objectives: In this systematic review we sought to clarify and summarize the clinical and preclinical evidence of potential benefit of the use of CBD in coronary syndromes. Methods: We conducted a systematic search and review according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) and Review of Animal Data from Experimental Studies (CAMARADES) guidelines, in the electronic database from PubMed, Web of Science and Scopus up to April 2022 using predefined search terms. Pre-specified exclusion and inclusion criteria were considered, finally 11 articles were chosen to be included for this peer review. Results: Currently there are no good-quality clinical trials with the use of CBD in acute or chronic coronary syndromes. A total of 11 preclinical studies where prescreened and 5 demonstrated reproducible positive cardiovascular outcomes on in-vivo models treated with CBD. Mechanisms of CBD cardioprotection observed: 1) reduction in oxidative stress and inflammation, 2) activation of adenosine receptors and 3) increased expression of angiotensin type 2-receptor. Experimental models included ischemia/reperfusion injury, myocardial infarction, arrhythmias, and metabolic syndrome-like conditions. Conclusion: No clinical recommendation can be issued with the current evidence, on the use of CBD in acute and chronic coronary syndromes. Based on preclinical evidence, we considered there is enough evidence to propose the development of well-designed clinical trials that include CBD in the management of coronary syndromes.
基金supported by the grants from the CAMS Innovation Fund for Medical Sciences(CIFMS)(Grant No.:2021-I2M-1-026)the Beijing Natural Science Foundation of China(Grant Nos.:7212155 and 7162135).
文摘Obesity and related metabolic syndromes have been recognized as important disease risks,in which the role of adipokines cannot be ignored.Adiponectin(ADP)is one of the key adipokines with various beneficial effects,including improving glucose and lipid metabolism,enhancing insulin sensitivity,reducing oxidative stress and inflammation,promoting ceramides degradation,and stimulating adipose tissue vascularity.Based on those,it can serve as a positive regulator in many metabolic syndromes,such as type 2 diabetes(T2D),cardiovascular diseases,non-alcoholic fatty liver disease(NAFLD),sarcopenia,neurodegenerative diseases,and certain cancers.Therefore,a promising therapeutic approach for treating various metabolic diseases may involve elevating ADP levels or activating ADP receptors.The modulation of ADP genes,multimerization,and secretion covers the main processes of ADP generation,providing a comprehensive orientation for the development of more appropriate therapeutic strategies.In order to have a deeper understanding of ADP,this paper will provide an all-encompassing review of ADP.
文摘Objective: Hyperlipidemia is a representative nutritional metabolic disease in clinic, which is easy to induce atherosclerotic cardiovascular and cerebrovascular diseases, with complex classification. Traditional Chinese Medicine (TCM) syndrome aims to reflect the characteristics of diseases and is the basic principle of TCM treatment of diseases. This study aimed to summarize the distribution pattern of TCM syndromes in patients with hyperlipidemia. Methods: The frequency, characteristics and distribution of all TCM syndromes of 1012 patients with hyperlipidemia were analyzed. Results: The main disease types determined by frequency of 1012 patients included hypertriglyceridemia combined with hypo high-density lipoprotein cholesterolemia (19.76%), hypo high-density lipoprotein cholesterolemia (18.58%), hypercholesterolemia (16.50%), mixed hyperlipidemia (16.40%), and hypertriglyceridemia (15.12%). The distribution of TCM syndromes, in order of frequency, was as follows: Qi-deficiency and blood stasis syndrome (23.52%), liver depression and spleen deficiency (9.88%), syndrome of qi stagnation and blood stasis (9.29%), phlegm stasis syndrome (7.41%), and syndrome of yang deficiency of spleen and kidney (6.92%). Conclusion: Qi-deficiency and blood stasis syndrome and liver depression and spleen deficiency are the most common TCM syndromes in patients with hyperlipidemia.
文摘The association between genetic syndromes and odontogenic tumors encompasses several entities,reflecting the intricate interplay between genetic factors and the development of these lesions.The present study aimed to comprehensively investigate the associations between genetic syndromes and odontogenic tumors.We delineated the diverse spectrum of syndromic connections,including key syndromes such as Gardner syndrome,Gorlin syndrome,Schimmelpenning syndrome,and others.Our findings underscore the clinical significance of recognizing odontogenic tumors associated with genetic syndromes as diagnostic indicators for early intervention.We advocate for multidisciplinary collaboration among clinicians,geneticists,and researchers to deepen our understanding of the underlying mechanisms driving these syndromic associations.In light of this,our study contributes to the growing body of knowledge in dentistry and medical genetics,offering insights that may inform clinical practice and enhance patient care for individuals affected by genetic syndromes and odontogenic tumors.
文摘Background: In response to the escalating burden of cardiovascular diseases (CVDs) worldwide, exacerbated by lifestyle changes and socioeconomic shifts, acute coronary syndromes (ACS) stand out as a leading cause of morbidity and mortality. The pivotal role of insulin resistance in the pathogenesis of atherosclerosis, independent of traditional risk factors, has garnered significant interest. Objective: This review aims to synthesize the recent advancements in the utilization of the triglyceride glucose index (TyG index) as a biomarker for assessing the severity and predicting the prognosis of ACS lesions. Methods: A systematic search was conducted across PubMed, Embase, and Scopus databases, incorporating keywords such as “triglyceride glucose index”, “TyG index”, “acute coronary syndrome”, “cardiovascular disease”, “insulin resistance”, “coronary artery calcification”, “SYNTAX score”, “Gensini score”, and “major adverse cardiac events”. Studies were included from the inception of each database up to July 2024. Selection criteria encompassed observational studies, case-control studies, and randomized controlled trials, with a particular emphasis on evaluating the diagnostic and prognostic value of the TyG index in patients with acute coronary syndromes. Ultimately, 46 publications met the inclusion criteria. Data extraction and quality assessment were performed in accordance with established guidelines. Results: Evidence suggests that the TyG index, reflecting insulin resistance, blood glucose, and lipid levels, is significantly associated with lesion severity in ACS, including coronary artery calcification, SYNTAX score, and Gensini score. Moreover, it demonstrates predictive power for major adverse cardiovascular events, underscoring its potential as a valuable tool in clinical decision-making. Conclusion: The review highlights the emerging role of the TyG index in the assessment and prognosis of ACS, advocating for its incorporation into clinical practice as a complement to existing diagnostic modalities. However, the establishment of standardized reference ranges and further validation across diverse populations are warranted to refine its applicability in personalized medicine. The interdisciplinary approach is essential to advance our understanding of the complex interplay between insulin resistance and cardiovascular disease, paving the way for the development of more effective prevention and treatment strategies.
基金National Natural Science Foundation of China,No.U1604181Henan Province Key R&D and Promotion Special Project (Science and Technology Tackle),No.212102310834+1 种基金Henan Medical Education Research Project,No.Wjlx2020531the Joint project of Medical Science and Technology Research Program of Henan Province,No.LHGJ20190078 (all to JW)。
文摘Paraneoplastic neurological syndrome refers to certain malignant tumors that have affected the distant nervous system and caused corresponding dysfunction in the absence of tumor metastasis.Patients with this syndrome produce multiple antibodies,each targeting a different antigen and causing different symptoms and signs.The CV2/collapsin response mediator protein 5(CRMP5)antibody is a major antibody of this type.It damages the nervous system,which often manifests as limbic encephalitis,chorea,ocular manifestation,cerebellar ataxia,myelopathy,and peripheral neuropathy.Detecting CV2/CRMP5 antibody is crucial for the clinical diagnosis of paraneoplastic neurological syndrome,and anti-tumor and immunological therapies can help to alleviate symptoms and improve prognosis.However,because of the low incidence of this disease,few repo rts and no reviews have been published about it so far.This article intends to review the research on CV2/CRMP5antibody-associated paraneoplastic neurological syndrome and summarize its clinical features to help clinicians comprehensively understand the disease.Additionally,this review discusses the curre nt challenges that this disease poses,and the application prospects of new detection and diagnostic techniques in the field of paraneoplastic neurological syndrom e,including CV2/CRMP5-associated paraneoplastic neurological syndrome,in recent years.
基金a part of the research project of the Institute of Plant and Animal Ecology,Ural Branch,Russian Academy of Sciences (№122021000092-9)
文摘The present article tests the following general assumption:plant taxa with different specializations towards mycorrhizal interactions should have different root syndromes.Roots of 61 species common in boreal zone were studied:16 species of Poaceae,24 species of Cyperaceae,14 species of Orchidaceae,and 7 species of Iridaceae.Using a fixed material of 5 individuals of each species,the following was determined:number of orders of branching roots;transverse dimensions of root,stele and cortex;number of primary xylem vessels and exodermis layers;length of root hairs;abundance of mycorrhiza.Species of each family had well-defined syndromes.Roots of Orchidaceae and Iridaceae were thick with a large stele and developed exodermis.Orchidaceae had no branching roots and had long root hairs.In Iridaceae,roots were branched,and root hairs were short.Roots of Poaceae and Cyperaceae were thin with a relatively thin stele.Root hairs were short in Poaceae and long in Cyperaceae.Our finding that root syndromes of four families of monocots differed is a new and unexpected discovery.The high specificity of root syndromes in Cyperaceae,Iridaceae,Poaceae,and Orchidaceae indicates that species of these families use different strategies to obtain water and soil nutrients.
基金the support from science and technology innovation special topic of Maoming City(No.2021KJZXZJYX011 and No.2022S014).
文摘Background:Traditional Chinese medicine(TCM)syndrome is the basic unit of TCM treatment,which help clinicians assess the disease progression and treatment preoperative of tumor patients.However,the prognostic significance of TCM syndrome is still unclear.This study aims to detect the differences in overall survival between different TCM syndrome and further develop a new nomogram with TCM syndrome for predicting overall survival in colorectal cancer.Methods:A total of 324 patients with colorectal cancer were enrolled and categorized into three groups based on TCM syndrome:deficiency,excess,and deficiency-excess.The prognosis of colorectal cancer patients with different TCM syndromes was evaluated using Kaplan-Meier analysis and Cox regression analysis.Results:The proportion of advanced stage and lymph metastasis in the patients with deficiency syndrome was higher,and the overall survival was shorter than other syndromes.Meanwhile,the TCM syndrome(P<0.001),tumor invasion depth(P<0.001),lymph metastasis(P=0.018),organic metastasis(P=0.005)and tumor node metastasis(TNM)stage(P=0.029)were the independent prognostic factor.Then,a new nomogram with TCM syndrome was established and assessed.324 colorectal cancer patients were randomly divided into training(n=215)and validation cohorts(n=109).A nomogram incorporating preoperative TCM syndrome,gender,age,T,N,and M status was developed,which had good discrimination and calibration.Conclusion:Taken together,our results indicated that TCM syndrome could assess the prognosis of colorectal cancer.The nomogram incorporating TCM syndromes and tumor information is helpful for risk stratification and prognostic predictions in colorectal cancer preoperatively.
基金Supported by The Russian Science Foundation,No.17-75-30027。
文摘Hereditary cancer syndromes(HCSs)are arguably the most frequent category of Mendelian genetic diseases,as at least 2%of presumably healthy subjects carry highly-penetrant tumor-predisposing pathogenic variants(PVs).Hereditary breast-ovarian cancer and Lynch syndrome make the highest contribution to cancer morbidity;in addition,there are several dozen less frequent types of familial tumors.The development of the majority albeit not all hereditary malignancies involves two-hit mechanism,i.e.the somatic inactivation of the remaining copy of the affected gene.Earlier studies on cancer families suggested nearly fatal penetrance for the majority of HCS genes;however,population-based investigations and especially large-scale next-generation sequencing data sets demonstrate that the presence of some highly-penetrant PVs is often compatible with healthy status.Hereditary cancer research initially focused mainly on cancer detection and prevention.Recent studies identified multiple HCS-specific drug vulnerabilities,which translated into the development of highly efficient therapeutic options.
文摘In this editorial we present an overview and insights of the management of hereditary polyposis syndromes.The primary focus was on familial adenomatous polyposis,juvenile polyposis syndrome and Peutz-Jegher syndrome.Genetic testing has become increasingly available and is easier than ever to integrate into clinical practice.Furthermore,several genes have been added to the expanding list of genes associated with hereditary polyposis syndromes,allowing for precise diagnostics and tailored follow-up.Endoscopic evaluation of patients with hereditary polyposis syndromes is paramount in the surveillance strategies.Current endoscopic procedures include both diagnostic procedures and surveillance as well as therapeutic interventions.Recommendations for endoscopic procedures in the upper and lower gastrointestinal canal were described.Surgery is still a key component in the management of patients with hereditary polyposis syndromes.The increased cancer risk in these patients often render prophylactic procedures or intended curative procedures in the case of cancer development.Surgical interventions in the upper and lower gastrointestinal canal were described with relevant considerations.Development of chemopreventive medications is ongoing.Few drugs have been investigated,including nonsteroidal anti-inflammatory drugs.It has been demonstrated that cyclooxygenase-2 inhibitors may lower the number of polyps.Other medications are currently under investigation,but none have,to date,consistently been able to prevent development of disease.
文摘Background: Ischaemic heart disease is the cause of 7.4 million deaths per year. Their prevention is based on the management of cardiovascular risk factors, but also on the early detection and management of chronic coronary syndromes (CCS), for which few data are available in Africa. The main objective of our study was to determine the factors related to significative coronary artery disease in patients undergoing coronarography for suspected chronic coronary syndrome (CCS). Methodology: We conducted a retrospective descriptive and analytical study over 2 years (from January, 1<sup>st</sup>, 2018 to December 31<sup>st</sup>, 2019) in the Cardiology Department of the University Hospital Aristide Le DANTEC in Dakar. All patients admitted for coronary angiography for suspected chronic coronary syndrome were included. Results: One hundred and fifty-two patients were included with a mean age of 60.79 ± 9.73 years, the most represented age group was 60 - 69 years. Advanced age was the most frequent risk factor (77.63%) followed by sedentary lifestyle (56.58%) and hypertension (41.45%). Diabetes was present in 17.1% of cases. A history of angioplasty was found in 1.97% of patients. Typical pain was found in 71.05% of cases, atypical pain in 19.74% and exertional dyspnoea in 2.63%. The pre-test probability was intermediate in 67.1% of cases, low in 25% and high in 7.9%. Significative coronary lesion was found in 52.63% of the patients, while coronary angiography was normal in the remaining cases. Tritroncular status was observed in 37.50%, it was bitroncular in 26.25% and monotroncular in 36.25% of cases. Factors associated with significative coronary artery disease were age (p = 0.0001), diabetes (p = 0.006), previous angioplasty (p = 0.023), previous myocardial infarction (p = 0.018), typical angina (p = 0.001), intermediate pretest probability (p = 0.001). Low pretest probability was significantly correlated with the absence of a coronary lesion with a p = 0.001. Conclusion: Our study shows that screening for chronic coronary disease should be done especially in diabetics, elderly subjects and those with previous angioplasty taking into account symptoms and pretest probability to avoid unnecessary invasive procedures.
文摘BACKGROUND During the second wave of the coronavirus disease 2019(COVID-19)pandemic,a subset of critically ill patients developed delayed respiratory deterioration in the absence of new infection,fluid overload or extra-pulmonary organ dysfunction.AIM To describe the clinical and laboratory characteristics,outcomes,and management of these patients,and to contrast this entity with other post COVID-19 immune dysregulation related inflammatory disorders.METHODS This was a retrospective observational study of adult patients admitted to the medical intensive care unit of a 2200-bed university affiliated teaching hospital,between May and August 2021,who fulfilled clearly defined inclusion and exclusion criteria.Outcome was assessed by a change in PaO_(2)/FiO_(2) ratio and levels of inflammatory markers before and after immunomodulation,duration of mechanical ventilation after starting treatment,and survival to discharge.RESULTS Five patients developed delayed respiratory deterioration in the absence of new infection,fluid overload or extrapulmonary organ dysfunction at a median interquartile range(IQR)duration of 32(23-35)d after the onset of symptoms.These patients had elevated inflammatory markers,required mechanical ventilation for 13(IQR 10-23)d,and responded to glucocorticoids and/or intravenous immunoglobulin.One patient died(20%).CONCLUSION This delayed respiratory worsening with elevated inflammatory markers and clinical response to immunomodulation appears to contrast the well described Multisystem Inflammatory Syndrome–Adults by the paucity of extrapulmonary organ involvement.The diagnosis can be considered in patients presenting with delayed respiratory worsening,that is not attributable to cardiac dysfunction,fluid overload or ongoing infections,and associated with an increase in systemic inflammatory markers like C-reactive protein,inteleukin-6 and ferritin.A good response to immunomodulation can be expected.This delayed inflammatory pulmonary syndrome may represent a distinct clinical entity in the spectrum of inflammatory syndromes in COVID-19 infection.
文摘Objective To investigate the relationship among serum vitamin D levels, physical performance impairment, and geriatric syndromes in elders with hypertension. Methods According to the concentration of vitamin D levels, a total of 143 elderly patients with hypertension were classified into vitamin D deficient group （vitamin D 〈 20 ng/mL, n = 94） and vitamin D appropriate group （vitamin D 〉 20 ng/rnL, n = 49）. Geriatric syndromes and physical performance were assessed by using comprehensive geriatric assessment （CGA）. Correlation among vitamin D levels, geriatric syndromes and physical performance was analyzed. Results No statistical differences were found in various aspects of geriatric syndromes between the two groups （P 〉 0.05）. While correlation analysis indicated that vitamin D levels had a positive association with ADL score （r = 0.235, P 〈 0.01） and a negative association with Morse fall scale score （r = 0.238, P 〈 0.01）. Patients with deficient vitamin D level had longer time both in the Five Time Sit to Stand Test （5tSTS）, （15.765 ± 5.593） and the four-meter walk test [7.440 （5.620, 9.200）], a weaker hand-grip in the grip strength test （28.049 ± 9.522）, and a lower Tinetti performance-oriented mobility assessment （Tinetti POMA） [26 （22, 27）] and Balance subscale of the Tinetti performance-oriented mobility assessment （B-POMA） score [ 14 （12, 16）], compared with appropriate vitamin D level [（13.275 ± 3.692）; 5.810 （4.728, 7.325）]; （31.989 ± 10.217）; [26.5 （25, 28）]; [15 （14, 16）, respectively, all P 〈 0.05]. Furthermore, results of logistic regression indicated that vitamin D was significantly associated with 5tSTS （OR = 1.2, 95% CI = 1.050-1.331, P 〈 0.01）, Tinetti POMA （OR = 3.7, 95% CI：1.284-10.830, P 〈 0.05） and B-POMA （OR = 0.8, 95% CI：0.643-0.973, P 〈 0.05）. Conclusions In elderly hypertensive patients, serum vitamin D deficient level is associated with physical performance impairment. However, no statistical significance was found between vitamin D and geriatric syndromes. Further study is required to investigate possible mechanisms for the association between vitamin D and physical performance.
文摘The myelodysplastic/myeloproliferative neoplasms(MDS/MPNs) are a unique group of hematologic malignancies characterized by concomitant myelodysplastic and myeloproliferative features. According to the 2008 WHO classification, the category includes atypical chronic myeloid leukemia(a CML), chronic myelomonocytic leukemia(CMML), juvenile myelomonocytic leukemia(JMML), MDS/MPN-unclassifiable(MDS/MPN-U), and the provisional entity refractory anemia with ring sideroblasts and thrombocytosis(RARS-T). Although diagnosis currently remains based on clinicopathologic features, the incorporation of nextgeneration platforms has allowed for the recent molecular characterization of these diseases which has revealed unique and complex mutational profiles that support their distinct biology and is anticipated to soon play an integral role in diagnosis,prognostication, and treatment. Future goals of research should include the development of disease-modifying therapies, and further genetic understanding of the category will likely form the foundation of these efforts.
基金Supported by The University of Medicine and Pharmacy of Tirgu-Mures,Romania,team research projects frame:UMFTGMPO-CC-02-F01,No.19/2014
文摘Because of the rarity of familial gastrointestinal cancerpredisposing syndromes,their exploration in literature is not extensive.In this review,an update of the clinicopathological and molecular criteria of gastrointestinal familial polyposis syndromes with potential malignant transformation is performed.In addition,a guide for screening and surveillance was synthesized and a distribution of gene mutations according to the specific syndromes and geographic distribution was included.The following inherited polyposes syndromes were analyzed: familial adenomatous polyposis,the hamartomatous familial polyposes(Juvenile polyposis,Peutz-Jeghers syndrome,Cowden syndrome,BannayanRiley-Ruvalcaba syndrome,hereditary mixed polyposis syndrome,Gorlin syndrome,Birt-Hogg-Dube syndrome,neurofibromatosis type Ⅰand multiple endocrine neoplasia syndrome 2B),Li-Fraumeni syndrome,and MUTYHassociated adenomatous polyposis.For proper medical care,subspecialization of gastroenterologists,pathologists,and genticists in the field of familial diseases should be introduced in the medical curriculum.
基金supported by the funding from the Spanish Society of Cardiology
文摘Background Elderly patients with non-ST-segment elevation acute coronary syndromes(NSTE-ACS)may present delirium but its clinical relevance is unknown.This study aimed at detennining the clinical associated factors,and prognostic implications of delirium in old-aged patients admitted for NSTE-ACS.Methods LONGEVO-SCA is a prospective multicenter registry including unselected patients with NSTE-ACS aged>80 years.Clinical variables and a complete geriatric evaluation were assessed during hospitalization.The association between delirium and 6-month mortality was assessed by a Cox regression model weighted for a propensity score including the potential confounding variables.We also analysed its association with 6-month bleeding and cognitive or functional decline.Results Among 527 patients included,thirty-seven(7%)patients presented delirium during the hospitalization.Delirium was more frequent in patients with dementia or depression and in those from nursing homes(27.0%vs.3.1%,24.3%vs.11.6%,and 11.1%V5.2.2%,respectively;all P<0.05).Delirium was significantly associated with in-hospital infections(27.0%vs.5.3%,P<0.001)and usage of diuretics(70.3%vs.49.8%,P=0.02).Patients with delirium had longer hospitalizations[median 8.5(5.5-14)vs.6.0(4.0-10)days,P=0.02]and higher incidence of 6-month bleeding and mortality(32.3%vs.10.0%and 24.3%vs.10.8%,respectively;both P<0.05)but similar cognitive or functional decline.Delirium was independently associated with 6-month mortality(HR=1.47,95%CI:1.02-2.13,P=0.04)and 6-month bleeding events(OR=2.87;95%CI:1.98-4」6,P<0.01).Conclusions In-hospital delirium in elderly patients with NSTE-ACS is associated with some preventable risk factors and it is an independent predictor of 6-month mortality.
文摘BACKGROUND:The complications of systemic inflammatory response syndrome(SIRS)include acute lung injury,acute kidney injury,shock,and multiple organ dysfunction syndrome(MODS).In recent years,how to clear inflammatory mediators has become a hot topic in critical care medicine.Researchers hypothesize that continuous blood purification(CBP) can effectively eliminate a variety of inflammatory mediators which participate in the occurrence of MODS and adjust the immune imbalance.This study aimed to observe the effects of CBP in MODS patients.METHODS:In this retrospective clinical study,a total of 38 MODS patients,18 males and 20 females,were enrolled.After conventional therapy,all the patients received CBP.Biochemistry,blood gas analysis,oxygenation index,mean arterial blood pressure(MAP),acute physiology and chronic health evaluation(APACHE) II scores were monitored.RESULTS:After CBP,the vital signs of patients were rapidly stable,and electrolyte disorders and acid-base imbalance were corrected.Renal function,blood gas,oxygenation index were all improved.MAP was increased,and APACHE II score was significantly decreased.All patients had good tolerance,stable hemodynamics,and no obvious adverse reaction on CBP compared with pre-CBP.CONCLUSION:CBP can effectively clean toxins,correct electrolyte acid-base balance,and improve systemic inflammatory response syndrome and the organ function of MODS patients.
基金Supported by A grant(in part)from the Municipal Key Discipline of Beijing,China,No.HK100230446the National Natural Science Foundation of China,No.81372578+1 种基金International Science and Technology Cooperation Projects,No.2010DFB33720Program for New Century Excellent Talents in University,No.NCET-11-0288
文摘AIM: To evaluate the risk factors for ampullary adenoma and ampullary cancer.
