Objective: To evaluate the diagnostic performance of magnetic resonance spectroscopy (MRS) in discriminating fumarate hydratase-deficient (FH-d) uterine leiomyomas (ULs) from FH-preserved ULs. Materials and Methods: This study consisted of three stages, with independent cohorts recruited for each stage: 1) sample-size estimation was retrospectively performed on UL specimens (diameter ≥3 cm; age, 20–40 years) from our database with immunohistochemistry (IHC) for 2-succinocysteine (2-SC) as the reference, without genetic testing, 2) MRS sequence optimization in confirmed FH germline mutation participants with ultrasound-detected ULs (diameter ≥3 cm), without IHC analysis, and 3) prospective diagnostic test accuracy was evaluated in consecutive participants with ultrasound-detected ULs (diameter ≥3 cm;age, 20–40 years), using IHC for 2-SC for determining the FH status and subsequent genetic testing in those with positive 2-SC results to identify whether FH mutations were germline or somatic in origin. The choline and fumarate peaks in MRS were classified as positive, negative, or technical failure (TF). TFs were analyzed separately and excluded from the primary diagnostic accuracy calculations. T1-, T2-, and diffusion-weighted images were interpreted as hyperintense or hypointense. The enhancement rate and apparent diffusion coefficient were also acquired. Diagnostic performance was compared between MRS and various magnetic resonance imaging (MRI) features. Results: The optimal MRS parameters for the fumarate peak were echo time (TE) = 140 ms and an average of 256. Among the 360 prospective participants, 37 were confirmed to have FH-dULs. MRS showed positive fumarate peaks in 35 of 37 FH-dULs.After excluding six TFs, the positive fumarate peak on MRS showed 94.6% (35/37) sensitivity, 99.7% (316/317) specificity, and 99.2% (351/354) accuracy, all of which were significantly superior to those of other MRI features (P ≤ 0.002). Conclusion A positive fumarate peak on MRS may be a useful imaging biomarker for diagnosing FH-dULs.

BACKGROUND: Delayed platelet engraftment is a common complication after umbilical cord blood transplantation (UCBT), and there is no standard therapy. Avatrombopag (AVA) is a second-generation thrombopoietin (TPO) receptor agonist (TPO-RA) that has shown efficacy in immune thrombocytopenia (ITP). However, few reports have focused on its efficacy in patients diagnosed with thrombocytopenia after allogeneic hematopoietic stem cell transplantation (allo-HSCT). METHODS: We conducted a retrospective study at the First Affiliated Hospital of the University of Science and Technology of China to evaluate the efficacy of AVA as a first-line TPO-RA in 65 patients after UCBT; these patients were compared with 118 historical controls. Response rates, platelet counts, megakaryocyte counts in bone marrow, bleeding events, adverse events and survival rates were evaluated in this study. Platelet reconstitution differences were compared between different medication groups. Multivariable analysis was used to explore the independent beneficial factors for platelet implantation. RESULTS: Fifty-two patients were given AVA within 30 days post-UCBT, and the treatment was continued for more than 7 days to promote platelet engraftment (AVA group); the other 13 patients were given AVA for secondary failure of platelet recovery (SFPR group). The median time to platelet engraftment was shorter in the AVA group than in the historical control group (32.5 days vs . 38.0 days, Z  = 2.095, P  = 0.036). Among the 52 patients in the AVA group, 46 achieved an overall response (OR) (88.5%), and the cumulative incidence of OR was 91.9%. Patients treated with AVA only had a greater 60-day cumulative incidence of platelet engraftment than patients treated with recombinant human thrombopoietin (rhTPO) only or rhTPO combined with AVA (95.2% vs . 84.5% vs . 80.6%, P  <0.001). Patients suffering from SFPR had a slightly better cumulative incidence of OR (100%, P  = 0.104). Patients who initiated AVA treatment within 14 days post-UCBT had a better 60-day cumulative incidence of platelet engraftment than did those who received AVA after 14 days post-UCBT (96.6% vs . 73.9%, P  = 0.003). CONCLUSION Compared with those in the historical control group, our results indicate that AVA could effectively promote platelet engraftment and recovery after UCBT, especially when used in the early period (≤14 days post-UCBT).
Humans ; Female ; Male ; Thrombocytopenia/etiology* ; Adult ; Retrospective Studies ; Cord Blood Stem Cell Transplantation/adverse effects* ; Middle Aged ; Adolescent ; Young Adult ; Thiazoles/adverse effects* ; Platelet Count ; Receptors, Thrombopoietin/agonists* ; Child ; Thiophenes

Objective:To study the distribution characteristics of current patients with Kashin-Beck disease (KBD) in Molidawa Daur Autonomous Banner (referred to as Morin Banner), and provide suggestions for service management.Methods:Information of KBD current patients in Morin Banner was collected from January 1, 2018 to June 30, 2024 using the "KBD Current Patient Survey System" provided by the Center for Endemic Disease Control, Chinese Center for Disease Control and Prevention. A descriptive study method was used to analyze the basic information and clinical data of current patients.Results:As of June 30, 2024, a total of 6 223 KBD current patients were reported in Morin Banner, and the patients were distributed in 15 townships (towns). There was a statistically significant difference in the prevalence rate of KBD among different townships (towns, χ 2 = 3 069.01, P < 0.001). The minimum age of the KBD current patients was 27 years old, and the maximum was 98 years old, mainly concentrated in the age range of 45 - 74 years old, accounting for 95.7% (5 954/6 223). There was a significant difference in the prevalence rate of KBD among different age groups (χ 2 = 5 912.76, P < 0.001). The male to female ratio was 1.00∶1.14 (2 910 ∶ 3 313), and there was a statistically significant difference in prevalence rate of KBD between genders(χ 2 = 44.38, P < 0.001). The KBD current patients mainly had a primary school education, married, and farmers, accounting for 59.2% (3 685/6 223), 89.8% (5 590/6 223), 93.2% (5 802/6 223), respectively; and the clinical grading of patients is mainly degree Ⅰ. There was a statistically significant difference in the rate of limb disability among patients with different clinical grades (χ 2 = 64.26, P < 0.001). The rate of limb disability in males was higher than that in females (χ 2 = 10.36, P = 0.001). Conclusions:The KBD current patients in Morin Banner are distributed in various township (town), with middle-aged and elderly famers being the main ones. It is necessary to strengthen monitoring of KBD, and pay attention to personalized treatment and management of KBD current patients.

Isolated axillary recurrence(AR)after breast cancer surgery is one of the critical factors influencing patients'prognosis.With advancements in diagnostic and therapeutic techniques,the clinical understanding of AR has progressively deepened.However,due to the low incidence of AR,systematic studies on its clinical features remain limited.High-quality clinical trials,such as ACOSOG Z0011 and AMAROS,have demonstrated that in patients with varying statuses of sentinel lymph node,axillary lymph node dissection(ALND)and sentinel lymph node dissection(SLND)provide comparable control of AR.In recent years,the trend towards de-escalation axillary surgery has advanced the development of less invasive techniques such as targeted axillary lymph node dissection.The SOUND trial further confirmed the safety and feasibility of omitting axillary surgery in patients with tumors≤2 cm.In addition,a series of clinical studies have identified a variety of potential high-risk factors,including patient age,number of positive lymph nodes,high Ki-67 proliferation,extranodal extension,and axillary soft tissue infiltration.However,there is no broad consensus regarding the association of these factors with AR.This review comprehensively summarized the clinical characteristics,risk factors and personalized management strategies of AR,with an emphasis on the impact of different axillary surgical approaches,radiotherapy and systemic therapy on the AR risk.In addition,more high-quality clinical studies are urgently needed to further clarify prognostic factors and optimize individualized treatment strategies,so as to provide more precise management for patients.

Objective:To study the distribution characteristics of current patients with Kashin-Beck disease (KBD) in Molidawa Daur Autonomous Banner (referred to as Morin Banner), and provide suggestions for service management.Methods:Information of KBD current patients in Morin Banner was collected from January 1, 2018 to June 30, 2024 using the "KBD Current Patient Survey System" provided by the Center for Endemic Disease Control, Chinese Center for Disease Control and Prevention. A descriptive study method was used to analyze the basic information and clinical data of current patients.Results:As of June 30, 2024, a total of 6 223 KBD current patients were reported in Morin Banner, and the patients were distributed in 15 townships (towns). There was a statistically significant difference in the prevalence rate of KBD among different townships (towns, χ 2 = 3 069.01, P < 0.001). The minimum age of the KBD current patients was 27 years old, and the maximum was 98 years old, mainly concentrated in the age range of 45 - 74 years old, accounting for 95.7% (5 954/6 223). There was a significant difference in the prevalence rate of KBD among different age groups (χ 2 = 5 912.76, P < 0.001). The male to female ratio was 1.00∶1.14 (2 910 ∶ 3 313), and there was a statistically significant difference in prevalence rate of KBD between genders(χ 2 = 44.38, P < 0.001). The KBD current patients mainly had a primary school education, married, and farmers, accounting for 59.2% (3 685/6 223), 89.8% (5 590/6 223), 93.2% (5 802/6 223), respectively; and the clinical grading of patients is mainly degree Ⅰ. There was a statistically significant difference in the rate of limb disability among patients with different clinical grades (χ 2 = 64.26, P < 0.001). The rate of limb disability in males was higher than that in females (χ 2 = 10.36, P = 0.001). Conclusions:The KBD current patients in Morin Banner are distributed in various township (town), with middle-aged and elderly famers being the main ones. It is necessary to strengthen monitoring of KBD, and pay attention to personalized treatment and management of KBD current patients.

Isolated axillary recurrence(AR)after breast cancer surgery is one of the critical factors influencing patients'prognosis.With advancements in diagnostic and therapeutic techniques,the clinical understanding of AR has progressively deepened.However,due to the low incidence of AR,systematic studies on its clinical features remain limited.High-quality clinical trials,such as ACOSOG Z0011 and AMAROS,have demonstrated that in patients with varying statuses of sentinel lymph node,axillary lymph node dissection(ALND)and sentinel lymph node dissection(SLND)provide comparable control of AR.In recent years,the trend towards de-escalation axillary surgery has advanced the development of less invasive techniques such as targeted axillary lymph node dissection.The SOUND trial further confirmed the safety and feasibility of omitting axillary surgery in patients with tumors≤2 cm.In addition,a series of clinical studies have identified a variety of potential high-risk factors,including patient age,number of positive lymph nodes,high Ki-67 proliferation,extranodal extension,and axillary soft tissue infiltration.However,there is no broad consensus regarding the association of these factors with AR.This review comprehensively summarized the clinical characteristics,risk factors and personalized management strategies of AR,with an emphasis on the impact of different axillary surgical approaches,radiotherapy and systemic therapy on the AR risk.In addition,more high-quality clinical studies are urgently needed to further clarify prognostic factors and optimize individualized treatment strategies,so as to provide more precise management for patients.

BACKGROUND:A large number of previous studies have confirmed that a high concentration of metabolites is significantly correlated with embryo quality and clinical outcome,and the theory of silencing embryo development indicates that normally developed embryos maintain a low level of material exchange with the outside world during in vitro culture,while embryos often show abnormal metabolic activity due to stress repair mechanism when DNA damage occurs. OBJECTIVE:To establish and verify an embryo quality prediction model based on the third-day 340 nm absorbance embryo cultures to provide the basis for a more objective and accurate embryo quality assessment. METHODS:269 patients at the Nanxishan Hospital of Guangxi Zhuang Autonomous Region for in vitro fertilization and embryo transplantation from November 2019 to December 2021 were retrospectively analyzed.Among them,on day 3,162 cases who had 873 optimal embryos and 214 high-quality blastocysts were included in the high-quality embryo group.On day 3,107 cases who had 859 non-optimal embryos and 214 non-high-quality blastocysts were included in the non-high-quality embryo group.Lambert-beer law was used to screen out the characteristic wavelength with distinguishing degree between superior and non-superior embryos,analyze its correlation and influence trend with high-quality embryos,and establish the clinical prediction model and validation of absorbance for high-quality and non-high-quality embryos at this wavelength. RESULTS AND CONCLUSION:(1)There was a significant difference in absorbance between high-quality and non-high-quality embryos at 340 nm on day 3(P<0.001),and a negative correlation was found with the formation of high-quality embryos on day 3(r=-0.486,P<0.001).The absorbance of high-quality and non-high-quality blastocyst at 340 nm was significantly different(P<0.05),and was negatively correlated with the formation of high-quality blastocyst(r=-0.642,P<0.001).(2)The optimal cut-off value of absorbance at 340 nm between high-quality and non-high-quality embryos on day 3 was 0.235.The area under the curve was 0.799.Sensitivity was 62.9%.Specificity was 78.0%.Accuracy was 70.5%.The optimum cutoff value of high-quality and non-high-quality blastocysts of absorbance at 340 nm was 0.175.The area under the curve was 0.871.Sensitivity was 74.3%.Specificity was 89.1%.Accuracy was 82.2%.(3)Restricted cubic spline curve analysis showed that when the absorbance of the culture medium at 340 nm was greater than 0.221,there was a significant positive trend on the formation of non-high-quality embryos at day 3,and when the absorbance of the culture medium at 340 nm was greater than 0.160,there was a significant positive trend on the formation of non-high-quality blastocysts.(4)The clinical decision curve and clinical influence curve showed that the absorbance of the culture medium at 340 nm had the maximum clinical net benefit for the prediction models of high-quality embryos and high-quality blastocysts on the third day when the valve probability was 0.18-0.95 and 0.16-1.00,respectively,and the ratio of loss to gain within the valve probability range was always less than 1.It is proven that the prediction model has good efficacy in clinical applications.The results of embryo transfer showed that the absorbance of embryo culture medium at 340 nm in non-pregnant patients was significantly higher than that in clinical pregnancy,biochemical pregnancy and early abortion patients(P<0.05).(5)The high-quality and non-high-quality embryo culture in 340 nm absorbance has a significant difference with correlation.The embryo quality prediction model has a certain clinical value and application effectiveness.The joint embryo morphology evaluation to a certain extent improves the objectivity and accuracy of embryo quality evaluation.

Tirzepatide,the first and currently the only approved GIP/GLP-1 receptor agonist worldwide,has demonstrated favorable effects in glycemic control and body weight reduction in several studies.In recent years,the effects of Tirzepatide on improving cardiovascular risk factors have gained increasing attentions.This article reviews the research progress of the effects of Tirzepatide on the cardiovascular system.

Objective:To evaluated the clinical efficacy of a reduced-intensity preconditioning regimen for single non-blood-related umbilical cord blood transplantation (sUCBT) in the treatment of severe aplastic anemia (SAA) .Methods:The clinical data of 63 patients with SAA who underwent sUCBT from January 2021 to July 2023 at the Department of Hematology of the First Affiliated Hospital of USTC were retrospectively analyzed. Fifty-two patients received total body irradiation/total bone marrow irradiation (TMI) combined with fludarabine or a cyclophosphamide- conditioning regimen (non-rATG group) , while 11 patients received rabbit anti-human thymocyte immunoglobulin (rATG) combined with TMI, fludarabine, or the cyclophosphamide-conditioning regimen (rATG group) . All patients received cyclosporine A and mycophenolate mofetil for graft-versus-host disease (GVHD) prophylaxis. Complications post-transplantation and long-term survival were compared between the two groups.Results:The baseline parameters were balanced between the two groups ( P>0.05) . In the rATG group, all patients achieved stem cell engraftment, and in the non-rATG group, five patients had primary graft failure. There was no significant difference in the cumulative incidence of neutrophil engraftment at 42 days after transplantation or platelet engraftment at 60 days between the two groups. The incidence of grade Ⅱ-Ⅳ acute GVHD in the rATG group was significantly lower than in the non-rATG group (10.0% vs. 46.2% , P=0.032) , and the differences in the cumulative incidences of grade Ⅲ/Ⅳ acute GVHD and 1-year chronic GVHD were not statistically significant ( P=0.367 and P=0.053, respectively) . There were no significant differences in the incidences of pre-engraftment syndrome, bacterial bloodstream infections, cytomegalovirus viremia, or hemorrhagic cystitis between the two groups ( P>0.05 for all) . The median follow-up time for surviving patients was 536 (61-993) days, and the 1-year transplantation related mortality (TRM) of all patients after transplantation was 13.0% (95% CI 6.7% -24.3% ) . Among the patients in the non-rATG and rATG groups, 15.5% (95% CI 8.1% -28.6% ) and 0% ( P=0.189) , respectively, had mutations. The 1-year overall survival (OS) rate of all patients after transplantation was 87.0% (95% CI 75.7% -93.3% ) . The 1-year OS rates in the rATG group and non-rATG group after transplantation were 100% and 84.5% , respectively (95% CI 71.4% -91.9% ) ( P=0.198) . Conclusion:The preliminary results of sUCBT with a low-dose irradiation-based reduced-intensity conditioning regimen with fludarabine/cyclophosphamide for the treatment of patients with SAA showed good efficacy. Early application of low-dose rATG can reduce the incidence of acute GVHD after transplantation without increasing the risk of implantation failure or infection.

Background::Previous studies have reported associations of specific maternal and paternal lifestyle factors with offspring’s cognitive development during early childhood. This study aimed to investigate the prospective associations between overall parental lifestyle and offspring’s cognitive performance during adolescence and young adulthood in China.Methods::We included 2531 adolescents aged 10-15 years at baseline in 2010 from the China Family Panel Studies. A healthy parental lifestyle score (ranged 0-5) was constructed based on the following five modifiable lifestyle factors: Smoking, drinking, exercise, sleep, and diet. Generalized estimating equation models were used to examine the association between baseline parental healthy lifestyle scores and offspring’s fluid and crystallized intelligence in subsequent years (2012, 2014, 2016, and 2018).Results::Offspring in the top tertile of parental healthy lifestyle scores performed better in overall fluid intelligence (multivariable-adjusted β = 0.53, 95% confidence interval [CI]: 0.29-0.77) and overall crystallized intelligence (multivariable-adjusted β = 0.35, 95% CI: 0.16-0.54) than those in the bottom tertile of parental healthy lifestyle scores. The results were similar after further adjustment for the offspring’s healthy lifestyle scores and persisted across the subgroups of parental socioeconomic status. Additionally, maternal and paternal healthy lifestyle scores were independently associated with better offspring’s cognitive performance, with significant contribution observed for paternal never-smoking, weekly exercise, and diversified diet. When both parents and offspring adhered to a healthier lifestyle, we observed the highest level of the offspring’s overall crystallized intelligence. Conclusions::Our study indicates that parental adherence to a healthier lifestyle is associated with significantly better offspring’s cognitive performance during adolescence and early adulthood, regardless of socioeconomic status. These findings highlight the potential cognitive benefits of promoting healthy lifestyles among parents of adolescents.