Abstract:Osteoporosis,as a systemic bone disease characterized by reduced bone mass and destruction of bone microstructure,is prone to serious complications such as vertebral compression fractures and hip fractures.It is an important cause of disability and mortality in the elderly and seriously endangers the bone health and life safety of the elderly.The prevention and treatment of osteoporosis is a common topic and challenge in multidisciplinary clinical practice.The management of osteoporosis is developing towards standardization,individualization,and comprehensive treatment.In recent years,the concept of treatment to target for osteoporosis has milestone significance in clinical diagnosis and treatment of osteoporosis,and has important guiding significance for the development of prevention and treatment strategies for osteoporosis.
Abstract:In recent years,the incidence of osteoporosis has increased yearly and it has emerged as a global public health issue.However,public awareness of osteoporosis is significantly insufficient.Complications such as fragility fractures seriously damage patients'quality of life and increase associated healthcare expenditures.Osteoporosis is preventable and treatable.Initiating appropriate therapeutic strategies requires comprehensive assessment of bone mineral density(BMD)and individualized fracture risk.For patients with imminent risk of fracture,the administration of anabolic bone agents enables rapid and effective achievement of therapeutic goals.
Abstract:Objective To investigate the factors contributing to persistent anemia in Chinese patients with high disease-activity paroxysmal nocturnal hemoglobinuria(PNH)undergoing Eculizumab therapy.Methods A prospective registry-based analysis was conducted on 33 high disease-activity PNH patients treated with Eculizumab for at least 6 months between December 2023 and December 2024 in the hospitals of Chinese Eastern Collaboration Group of Anemia,analyze the patient's condition and causes of persistent anemia.Results Among 33 patients,9 patients maintained Hb＞100 g/L(1 patient ≥120 g/L),and 24 patients exhibited Hb＜100 g/L.The incidence of comorbid aplastic anemia was lower in patients with Hb level of 10 or more(22％vs.67％,P=0.029).Anti-C3d positivity via direct antiglobulin test(DAT)was detected in 13 patients(13/33,39％),with median onset time of 12(2-32)weeks and median anti-C3d titer of 64(16-256).7 patients demonstrated C3d titers ≥1:64.At 6-month follow-up after eculizumab treatment,the median PNH clone size in type Ⅱ+Ⅲerythrocytes was 17.7％(1.8％-99.7％),which is lower than that in neutrophil clones.Twenty patients exhibited PNH size in type Ⅱ+Ⅲ erythrocyte were 10 percentage or lower by comparing to neutrophil,while 5 patients showed 5％-9％difference.More patients with Hb levels＞100 g/L in whom with lower differences in PNH clone size between erythrocyte and neutrophil(40％vs.100％,P=0.024).Twelve patients had elevated LDH levels.All 12 patients with elevated LDH showed＞10％lower type Ⅱ+Ⅲ erythrocyte clones compared to neutrophil clones.Hb＞100 g/L was achieved in 25％(3/12)of elevated LDH patients vs.77％(10/13)of normal LDH patients(25％vs.77％,P=0.013).3 patients experienced breakthrough hemolysis(BTH),all occurring after 32(15-91)days of delayed Eculizumab administration.Conclusion Persistent anemia post-Eculizumab treatment in high disease activity statePNHcorrelates significantly with concurrent aplastic anemia,extravascular hemolysis,residual intravascular hemolysis and breakthrough hemolysis.
Abstract:Objective To explore the clinical predictive value of serum intestinal fatty acid-binding protein(Ⅰ-FABP)for the risk of rebleeding within 1 year in patients with hepatitis B cirrhosis and esophagogastric variceal bleeding(EVB).Methods We retrospectively analyzed the clinical data of patients with hepatitis B cirrhosis and EVB admitted to the Affiliated Hospital of Yanbian University between September 2020 and March 2024.Patients were divided into rebleeding and non-rebleeding groups based on whether bleeding occurred during the 1-year follow-up period.An enzyme-linked immunosorbent assay was used to measure the baseline Ⅰ-FABP level upon admission.Kaplan-Meier analysis was used to compare the risk of rebleeding between groups stratified by Ⅰ-FABP quartile,while a restricted cubic spline(RCS)was used to explore potential linear relationships between Ⅰ-FABP and rebleeding risk.Multivariate Cox proportional hazards regression models were used to assess the association between Ⅰ-FABP and rebleeding risk.Receiver operating characteristic(ROC)curves were constructed to assess the accuracy of the relevant factors in predicting rebleeding.Results A total of 137 patients were included in the study.After 1 year of follow-up,the recurrent rebleeding rate was 42.3％(58/137).The median time torebleeding was 4.2(1.8,7.5)months.Kaplan-Meier analysis indicated that patients with higher Ⅰ-FABP levels had a higher risk of rebleeding(log-rank x2=17.84,P＜0.001).Restricted cubic spline revealed that a linear relationship between Ⅰ-FABP and rebleeding(P-nonlinear=0.288,P=0.001).Multivar-iable Cox regression analysis identified Ⅰ-FABP as an independent factor contributing to rebleeding(hazard ratio=1.723,95％CI 1.340-2.216,P＜0.001).ROC curve showed that Ⅰ-FABP had a certain predictive efficacy for rebleeding.The area under the curve was 0.695(95％CI 0.611-0.771).Conclusion Ⅰ-FABP remained strongly linked to the risk of rebleeding and demonstrated good predictive value.
Abstract:Paroxysmal nocturnal hemoglobinuria(PNH)is a rare non-malignant clonal disease of blood system,and its clinical manifestations are mainly hemolysis,bone marrow failure and thrombosis.In the pre-complement inhibitor era,supportive therapy or allogeneic hematopoietic stem cell transplantation was the main treatment.In 2007,the complement C5 inhibitor came out,which significantly reduced the risk of thrombosis and prolonged the survival of patients.Then,the proximal complement inhibitor blocked the bypass pathway upstream to solve the problem of extravascular hemolysis left by C5 inhibitor.The research and development of oral preparations promotes the chronic disease model with the convenience of medication;Gene therapy is trying to cure the disease at the molecular level.The treatment path of PNH is undergoing constant iterations and breakthroughs,and it is moving towards the goal of"functional cure disease".
Abstract:The core pathogenesis of paroxysmal nocturnal hemoglobinuria(PNH)is that somatic mutation of PIG-A gene on X chromosome leads to the deletion of glycosyl phosphatidylinositol anchor proteins such as CD55 and CD59,and the vulnerability of red blood cells to complement attack increases,resulting in hemolysis.The dynamic evolution of PNH clone is closely related to immune microenvironment pressure,secondary mutation and treatment intervention,and often interacts with bone marrow failure diseases and myeloid tumors.This paper reviews the pathogenesis,clonal evolution model and clinical significance of PNH,and discusses the role of immune microenvironment,gene mutation and treatment choice in disease progression,so as to provide theoretical basis for optimizing diagnosis and treatment.
Abstract:Cognitive behavioral therapy combined with drugs or physical therapy is an effective treatment for insomnia,which can reduce patients'dependence on drugs and even stop using anti-insomnia drugs.But up to now,there is no guideline or expert consensus on the time and how to combine cognitive behavioral therapy with drug or physical therapy for insomnia.In order to standardize the clinical implementation method of cognitive behavioral therapy for insomnia and improve the prognosis of patients,the consensus was made by experts in neurology,psychiatry and related fields under the guidance of evidence-based medicine methodology,through systematic retrieval of clinical research data,evaluation of clinical evidence and judgment of quality evidence,and after several rounds of discussion,in order to provide clinical guidance for cognitive behavioral therapy for insomnia combined with drugs or physical therapy.
Abstract:Paroxysmal nocturnal hemoglobinuria(PNH)is a rare acquired clonal disease of hematopoietic stem cells,clinically characterized by intravascular hemolysis,bone marrow failure,and high-risk concurrent thrombosis.Although it is a benign intravascular hemolytic disease,severe cases can be life-threatening.PNH has diverse clinical manifestations,complex symptoms,and is prone to multiple complications,often resulting in delayed diagnosis,misdiagnosis,and missed diagnosis.The classic complement hemolysis test lacks sensitivity and specificity for the diagnosis of PNH.The application of high-sensitivity flow cytometry has made breakthrough progress in the screening and diagnosis of PNH clones.The article provides a review of the current status and prospects of PNH,clone screening,and clinical application of flow cytometry.
Abstract:Investigating the mechanisms and management strategies of extravascular hemolysis(EVH)and breakthrough hemolysis(BTH)in patients with paroxysmal nocturnal hemoglobinuria(PNH)following complement inhibitor therapy.EVH is primarily triggered by C3b deposition on erythrocyte surfaces that induces macrophage phagocytosis.Diagnostic criteria include hemoglobin ≤9.5 g/dL or a decrease ≥2 g/dL,accompanied by elevated reticulocytes(≥120×109/L)and C3 deposition on erythrocytes(C3-positive direct Coombs test or C3 fragment detection via flow cytometry).Conventional therapies show limited efficacy,while proximal complement inhibitors(e.g.,Pegcetacoplan,Iptacopan)demonstrate effective control.BTH predominantly results from breakthrough complement activation despite inhibition,diagnosed when LDH exceeds 1.5 times the upper limit of normal with acute hemoglobin decline ≥1.5 g/dL.Severity correlates with complement inhibitor targeting sites and complement-amplifying conditions(CACs).Novel complement inhibitors may optimize PNH hemolysis,though vigilance is required for potential severe BTH following proximal inhibitor therapy.
Abstract:In recent years,the research on microbiotherapy(such as fecal microbiota transplantation,probiotics,phage therapy,oncolytic bacteria,oncolytic viruses,etc.)for diseases has been increasing.However,due to the lack of a unified reporting guideline,many studies have failed to cover all the necessary reporting items required by medical practitioners,researchers,and readers,thereby hindering result reproducibility,safety evaluation,and technological implementation.In 2024,an international multidisciplinary panel of clinical experts from eight countries established the Preferred Reporting Items for Microbiotherapy(PRIM 2024)international guidelines through a Delphi consensus procedure.The PRIM 2024 guidelines included 10 statements and 18 points on diagnosis,delivery route,source,classification,preparation,dosage,state,concomitant treatment,efficacy,and safety.This article introduces and interprets the core components of the guidelines,aiming to provide methodological guidance for clinical research report and establish a foundational framework and reference for standardizing diagnosis and treatment practice in microbiota medicine.
Abstract:Recently,the development and clinical application of complement inhibitors have significantly improved the survival and quality of life of patients with paroxysmal nocturnal hemoglobinuria(PNH).The treatment goal of PNH has been upgraded from hemolysis control to transfusion independence and hemoglobin normalization.Even with the initial control of hemolysis with compliment inhibitors,there is still questions like breakthrough hemolysis,extra vascular hemolysis,thrombotic events or disease evolution due to the diversities of PNH.Because PNH clones generally do not disappear after compliment inhibitors,patients require long-term follow-up and life-long time management which is similar to the management of chronic disease.Patient reports,clinical assessments,laboratory data,efficacy assessments need to be integrated to improve the quality of life of patients.
Abstract:Objective To retrospectively analyze the treatment of patients with hematological malignancies(HM)complicated with critical coronavirus disease 2019(COVID-19)in the hematology intensive care unit(ICU).Methods The study included patients with hematological malignancy and critical COVID-19 admitted to the hematology ICU of the First Affiliated Hospital of Soochow University from December 15,2022,to March 10,2023.Demographic data,clinical manifestations,laboratory and imaging examination indicators,clinical scores and treatment methods were collected by case questionnaire.According to the prognosis,the patients were divided into death group and survival group,and the differences between the two groups were compared.Results A total of 27 patients were enrolled,including 19 patients(70.37％)aged 18-64 years and 8 patients(29.63％)aged ≥65 years.There were 13 males(48.15％)and 14 females(51.85％).Among different hematological malignancies,14 cases(51.85％)were leukemia,8 cases(29.63％)were lymphoma,3 cases(11.11％)were plasma cell diseases,and 2 cases(7.41％)were MDS.Eleven patients(40.74％)were in post-hematopoietic stem cell transplantation status,7 patients(25.93％)received endotracheal intubation mechanical ventilation support,20 patients(74.07％)received non-invasive mechanical ventilation support,4 patients received CRRT support,and 10 patients received vasopressor drug osupport.In this study,the mortality of patients with hematological malignancies and critical COVID-19 was 44.4％(12/27).The comparison between the two groups showed that the white blood cell(WBC)and neutrophil(ANC)count levels in the death group were lower,the time from the onset of COVID-19 to the hematology ICU was shorter,the sequential organ failure assessment(SOFA)score was higher,and the patients received more mechanical ventilation and vasopressor drug support.Conclusion Patients with hematological malignancies are vulnerable population in the epidemic of 2019-ncov infection.Lower WBC and ANC,and vasopressor therapy are associated with higher mortality.Hematology ICU can provide comprehensive life support for these patients.
Abstract:This article reviews the management strategies for common complications and comorbidities of paroxysmal nocturnal hemoglobinuria(PNH),with a focus on addressing thrombosis,chronic renal insufficiency,pulmonary hypertension,bone marrow failure,and infections.Key strategies include:using complement inhibitors as the cornerstone to reduce thrombosis and hemolysis risks,combining anticoagulation therapy to prevent thrombotic events,protecting renal function through supportive care,and employing targeted medications to improve pulmonary hypertension.Immunosuppressive therapy or hematopoietic stem cell transplantation is utilized to correct bone marrow failure,while enhanced vaccination and antibiotic prophylaxis are emphasized to prevent infections.The article also covers special issues such as iron metabolism disorders and pregnancy management,highlighting the importance of multidisciplinary collaboration.
Abstract:The fifth National Consensus Conference on the management ofHelicobacterpylori (H.pylori) infection organized by Chinese Medical Association,Chinese Society of Gastroenterology,H.pylori and peptic ulcer study group was held at Hangzhou,Zhejiang Province on December 15-16,2016.More than 80 members of the Study Group and experts in the field of H.pylori research and gastroenterology attended the meeting.Consensus preparatory group was established to draft the related statements.The quality of evidence and strength of recommendation was evaluated by GRADE system.The Delphi method using an anonymous electronic system was adopted to develop the consensus of relevant statements.Expert advices about the established statements were firstly consulted through the email.Undergoing two rounds of consultation,the statements initially reached a consensus were discussed in the conference item by item and were necessarily modified.A total of 21 core members voted for the final consensus.The consensus contents contained a total of 48 statements and were divided into 6 parts,induding indications for H.pylorieradication,diagnosis,treatment,H.pyloriand gastric cancer,H.pylori infection in special populations,H.pylori and gastrointestinal microbiota.