Objective To investigate the impact of red blood cell suspension infusion across various perioperative periods on patients with valvular heart disease. Methods The patients with valvular heart disease admitted to Tianjin Chest Hospital from 2018 to 2020 were selected. Based on the timing of perioperative red cell suspension infusion, patients were categorized into three groups: a group 1 receiving intraoperative red cell suspension infusion, a group 2 receiving red cell suspension infusion within 24 hours after entering the ICU, and a group 3 receiving red cell suspension infusion at both time points. The laboratory results, perioperative blood component infusion volume, and other relevant parameters were retrospectively analyzed. After propensity score matching, the differences in different variables among the three groups were compared. Results After propensity score matching, 102 patients were enrolled, including 52 males and 50 females, with an average age of (61.74±10.58) years. There were 34 patients in each group. The preoperative hemoglobin (Hb) value of the group 2 was significantly higher than that of the group 1 and the group 3, and the amount of red cell suspension and autoblood transfusion was the lowest (P<0.05). Group 1 had the highest postoperative Hb, as well as the highest Hb and hematocrit (HCT) levels within 24 hours post-surgery (P<0.05). The group 1 had the lowest plasma, platelet and cryoprecipitate infusion volumes, and the shortest cardiopulmonary bypass time, aortic occlusion time, postoperative ICU stay and hospital stay, and the least blood loss and total drainage volume (P<0.05). The difference between postoperative and preoperative Hb (△Hb1) was highest in group 1 (P<0.05). Conclusion For patients with valvular heart disease, intraoperative-only infusion of red blood cell suspension is associated with a better prognosis at discharge and during follow-up.

Tracheoesophageal fistula (TEF) is a disease characterized by an abnormal connection between the trachea and esophagus. Benign TEF often results from tracheal intubation injury, typically presenting as coughing exacerbated by swallowing, and can be potentially life-threatening. However, no unified surgical standard currently exists for TEF. The choice of surgical method mainly depends on the location and size of the fistula, as well as the condition of the surrounding tracheal tissue. For small-to-moderate fistulas, tracheal segmental resection with reconstruction is a classic approach. For large, complex, and refractory TEFs, musculocutaneous flap repair is considered an ideal option. This article reviews the application of several common musculocutaneous flaps—including the sternocleidomastoid, pectoralis major, and latissimus dorsi—in the repair of benign TEFs, with the aim of providing a clinical reference.

Objective: To investigate the ameliorative effect of vitamin D on sciatic nerve chronic compression injury(CCI) rats and its regulatory effect on nuclear factor κB(NF-κB)/cystathione-β-synthase(CBS)-hydrogen sulfide(H2S) system. Methods: Fifty rats were randomly divided into five groups: control group,model group,vitamin D group,activator group,and activator + vitamin D group,with 10 rats in each group. Except for the control group,the rats in the other groups underwent sciatic nerve ligation to establish the CCI model. The vitamin D group received intraperitoneal injection of 500 mg/kg vitamin D,the activator group received intraperitoneal injection of10 mg/kg NF-κB activator lipopolysaccharide,and the activator + vitamin D group received intraperitoneal injection of 500 mg/kg vitamin D along with 10 mg/kg lipopolysaccharide. The control and model groups received intraperitoneal injection of an equal volume of saline,once per day for 2 weeks. The following parameters were compared among the groups: paw withdraw thermal latency(PWTL),mechanical withdrawal threshold(MWT),serum 25-hydroxyvitamin D3[25(OH)D3]levels,H2S content,calcitonin gene-related peptide(CGRP),prostaglandin E2(PGE2) levels,and the expression of vitamin D receptor(VDR),NF-κBp65,and CBS proteins in the L4-L6 dorsal root ganglia of rats. Results: Compared with the control group，the model group showed decreased PWTL，MWT，25( OH) D3 levels and VDR protein expression，while H2 S content，CGRP and PGE2 levels，and NF-κBp65 and CBS protein expression significantly increased ( P＜0. 05) ．Compared with the model group，the vi- tamin D group exhibited increased PWTL，MWT，25 ( OH) D3 levels and VDR protein expression ( P ＜0. 05) ， while H2 S content，CGRP and PGE2 levels，and NF-κBp65 and CBS protein expression significantly decreased ( P < 0. 05) ．Compared with the activator group，the activator + Vitamin D group showed increased PWTL，MWT， 25( OH) D3 levels and VDR protein expression，and decreased H2 S content，CGRP and PGE2 levels and decreased NF-κBp65，CBS protein expression ( P＜0. 05) ．Compared with the vitamin D group，the activator + vitamin D group showed decreased PWTL，MWT，25( OH) D3 levels and VDR protein expression，while H2 S content，CGRP and PGE2 levels，and NF-κBp65 and CBS protein expression significantly increased ( P ＜0. 05) ． Conclusion Exogenous supplementation of vitamin D can relieve nerve pain and reduce pain sensitivity in CCI rats，possibly by inhibiting NF-κB / CBS-H2 S signaling pathway．

OBJECTIVES: To study the clinical and genetic characteristics of osteopetrosis (OPT) in children. METHODS: A retrospective analysis was performed on the clinical data of 14 children with OPT. Whole-exome sequencing was used to detect pathogenic genes, and clinical phenotypes and genotypic features were summarized. RESULTS: Among the 14 children (10 males and 4 females), the median age at diagnosis was 8 months. Clinical manifestations included systemic osteosclerosis (14 cases, 100%), anemia (12 cases, 86%), infections (10 cases, 71%), thrombocytopenia (9 cases, 64%), hepatosplenomegaly (8 cases, 57%), and developmental delay (5 cases, 36%). Malignant osteopetrosis (MOP) cases had lower platelet counts, creatine kinase isoenzyme, and serum calcium levels, but higher white blood cell counts, lactate dehydrogenase, and alkaline phosphatase levels compared to non-MOP cases (P<0.05). Genetic testing identified 15 variants in 12 patients, including 8 variants in the CLCN7 gene (53%), 6 in the TCIRG1 gene (40%), and 1 in the TNFRSF11A gene (7%). Three novel CLCN7 variants were identified: c.2351G>C, c.1215-43C>T, and c.1534G>A. All four patients with TCIRG1 variants exhibited MOP clinical phenotypes. Of the seven patients with CLCN7 variants, 4 presented with intermediate OPT, 2 with benign OPT, and 1 with MOP. CONCLUSIONS Clinical phenotypes of OPT in children are heterogeneous, predominantly involving CLCN7 and TCIRG1 gene variants, with a correlation between clinical phenotypes and genotypes.
Humans ; Osteopetrosis/genetics* ; Male ; Female ; Infant ; Child, Preschool ; Retrospective Studies ; Vacuolar Proton-Translocating ATPases/genetics* ; Child ; Chloride Channels/genetics* ; Mutation ; Receptor Activator of Nuclear Factor-kappa B

Objective To analyze the incidence and mortality of gastric cancer in countries and territories with different human development index(HDI)levels in 2022,and to understand the burden of gastric cancer globally and in China.Methods Data on gastric cancer incidence and mortality were collected from GLOBOCAN 2022 and HDI data for all countries were obtained from the Human development report 2022.Spearman correlation was applied to examine the associations between the age-standardized incidence rate(ASIR),age-standardized mortality rate(ASMR),mortality-to-incidence ratio(M/I),and HDI for gastric cancer.The Wilcoxon rank-sum test was used to assess the differences in ASIR and ASMR between males and females.Results In 2022,gastric cancer ranked the 5th in both incidence and mortality among all cancer types globally.In China,gastric cancer ranked the 5th in incidence and the 3rd in mortality among all cancer types.The ASIR and ASMR of gastric cancer showed a descending trend from high,very high,medium to low HDI countries and territories.The ASIR of gastric cancer was positively correlated with HDI(rs=0.256,P=0.001),while ASMR showed no significant correlation with HDI(rs=-0.008,P=0.918).The M/I was negatively correlated with HDI(rs=-0.831,P＜0.001).The ASIR and ASMR of gastric cancer in males were significantly higher than those in females globally,in China,and across all HDI groups(all P＜0.05).Globally,both ASIR and ASMR of gastric cancer remained relatively stable before the age of 45,but showed a consistently rising trend after the age of 45.In China,the ASIR and ASMR of gastric cancer exceeded global average level across all age groups.Conclusion The burden of gastric cancer incidence and mortality is higher in very high and high HDI countries and territories compared to medium and low HDI countries and territories.In China,the burden of gastric cancer incidence and mortality is above the global average,highlighting the need for targeted prevention and control measures.

Objective To compare the incidence and mortality of brain and central nervous system(CNS)tumors in countries and territories with different human development index(HDI)in 2022,to make a comparison with the current epidemiological situation in China,and to assess the association between HDI and the incidence and mortality of brain and CNS tumors.Methods The data on brain and CNS tumors from GLOBOCAN 2022 were collected,and HDI data were organized based on the Human development report 2022.Generalized additive model(GAM)was used to analyze the relationships between standardized incidence ratio(SIR),standardized mortality ratio(SMR),mortality-to-incidence ratio(M/I),and HDI.Results The incidence and mortality of brain and CNS tumors increased with age in 2022,with a significant increasing trend in countries and territories with very high HDI.Countries and territories with high and very high HDI had more cases and more deaths,and countries and territories with very high HDI had the highest SIR and SMR.SIR for brain and CNS tumors in China was higher than the global average,while China's SMR was lower.M/I varied among countries and territories with different HDI,with lower M/I in countries and territories with high and very high HDI.HDI had a significant nonlinear effect on SIR(edf=1.740,P＜0.000 1)and M/I(edf=1.809,P＜0.000 1),and a significant linear effect on SMR(edf=1,P＜0.000 1).As HDI increased,SIR and SMR generally showed an increasing trend,while M/I showed a decreasing trend.Conclusion There are significant global differences in incidence and mortality of brain and CNS tumors in patients with different HDI in 2022;increasing HDI can reduce the risk of brain and CNS tumors and improve treatment outcomes,and prevention and control strategies should be made for different age groups and HDI.

Objective To investigate risk factors for residual lesions after initial transurethral resection of bladder tumors(TURBT)and risk factors for tumor recurrence after second TURBT in patients with non-muscle-invasive bladder cancer(NMIBC)in order to provide reference for clinical management.Methods A case-control study design was adopted to include 120 NMIBC patients who underwent initial TURBT and then second surgery within 2～8 weeks in our department from January 2017 to January 2025.Based on the presence of residual lesions after the initial TURBT or not,the patients were divided into a residual lesion group(n=34)and a non-residual lesion group(n=86).Chi-square test and multivariate logistic regression analysis were performed to identify potential risk factors for residual lesions following the initial TURBT.Univariate and multivariate Cox regression models were used to analyze potential risk factors for tumor recurrence after the second TURBT.Results The residual lesion rate after initial TURBT was 28.33％.Chi-square test analysis revealed that tumor stage T1(Chi-square=5.756,P=0.016)and broad tumor base(Chi-square=4.331,P=0.037)were factors influencing residual lesions after initial TURBT.Multivariate logistic regression analysis identified tumor stage T1(OR=3.047,95％CI:1.128～8.226,P=0.028)as an independent risk factor for residual lesions after initial TURBT.The tumor recurrence rate after second TURBT was 17.5％.Multivariate Cox regression analysis identified tumor stage T1(OR=4.258,95％CI:1.248～14.532,P=0.021),intravesical chemotherapy instillation after second TURBT(OR=3.539,95％CI:1.284～9.752,P=0.015),history of urinary system tumors(OR=3.002,95％CI:1.145～7.873,P=0.025)and high platelet-to-lymphocyte(PLR)ratio(OR=2.798,95％CI:1.115～7.023,P=0.028)as independent risk factors for tumor recurrence after second TURBT.Conclusion Tumor stage T1 and broad tumor base are risk factors for residual lesions after initial TURBT,while tumor stage T1,intravesical chemotherapy instillation after second TURBT,history of urinary system tumors and high PLR ratio are risk factors for tumor recurrence after second TURBT.Comprehensive analysis on above 4 indicators can effectively assess the risk of tumor recurrence in NMIBC patients following second TURBT,and timely early medical intervention is beneficial for improving patient outcomes.

HDAC7, a member of class IIa HDACs, plays a pivotal regulatory role in tumor, immune, fibrosis, and angiogenesis, rendering it a potential therapeutic target. Nevertheless, due to the high similarity in the enzyme active sites of class IIa HDACs, inhibitors encounter challenges in discerning differences among them. Furthermore, the substitution of key residue in the active pocket of class IIa HDACs renders them pseudo-enzymes, leading to a limited impact of enzymatic inhibitors on their function. In this study, proteolysis targeting chimera (PROTAC) technology was employed to develop HDAC7 drugs. We developed an exceedingly selective HDAC7 PROTAC degrader B14 which showcased superior inhibitory effects on cell proliferation compared to TMP269 in various diffuse large B cell lymphoma (DLBCL) and acute myeloid leukemia (AML) cells. Subsequent investigations unveiled that B14 disrupts BCL6 forming a transcriptional inhibition complex by degrading HDAC7, thereby exerting proliferative inhibition in DLBCL. Our study broadened the understanding of the non-enzymatic functions of HDAC7 and underscored the importance of HDAC7 in the treatment of hematologic malignancies, particularly in DLBCL and AML.

Critical care emphasizes critical thinking, focuses on the triggers that lead to disease progression, and attaches great importance to early diagnosis of diseases and assessment of the compensatory capacity of vital organs. Pregnancy-associated atypical hemolytic uremic syndrome (P-aHUS) is relatively rare in the intensive care unit (ICU). Most cases occur within 10 weeks after delivery. Severe cases can be life-threatening. It characterized by microangiopathic hemolytic anemia, decreased platelet count (PLT), and acute kidney injury (AKI). Early clinical diagnosis is difficult due to its similarity to various disease manifestations. On January 28, 2024, a 26-year-old pregnant woman at 26⁺³ weeks gestation was transferred to the ICU 19 hours post-vaginal delivery due to abdominal pain, reduced urine output, decreased PLT, elevated D-dimer, tachycardia, increased respiratory rate and declined oxygenation. On the day of ICU admission, the critical care physician identified the causes that triggered the acute respiratory and circulatory events based on the "holistic and local" critical care thinking. The condition was stabilized rapidly by improving the capacity overload. In terms of etiological diagnosis, under the guidance of the "point and face" critical care thinking, starting from abnormality indicators including a decrease in hemoglobin (Hb) and PLT and elevated D-dimer and fibrin degradation product (FDP) without other abnormal coagulation indicators, the critical care physician ultimately determined the diagnosis direction of thrombotic microangiopathy (TMA) by delving deeply into the essence of the disease and formulating a laboratory examination plan in a reasonable and orderly manner. In terms of in-depth diagnosis, combining the disease development process, family history, and past history, applying the two-way falsification thinking of "forward and reverse" as well as "questioning and hypothesis", the diagnosis possibilities of preeclampsia, HELLP syndrome [including hemolysis (H), elevated liver function (EL) and low platelet count (LP)], thrombotic thrombocytopenic purpura (TTP), typical hemolytic uremic syndrome (HUS), and autoimmune inflammatory diseases inducing the condition was ruled out. The diagnosis of complement activation-induced P-aHUS was finally established for the patient, according to the positive result of the complement factor H (CFH). Active decision was made in the initial treatment. The plasma exchange was initiated early. "Small goals" were formulated in stages. The "small endpoints" were dynamically controlled in a goal-oriented manner to achieve continuous realization of the overall treatment effect through phased "small goals". On the 5th day of ICU treatment, the trend of microthrombosis in the patient was controlled, organ function damage was improved, and the patient was transferred out of the ICU. It is possible to reach a favorable clinical outcome for critically ill patients by applying a critical care mindset to quickly integrate diagnostic and therapeutic strategies, accurately identifying the triggers and causes that led to the progression of the disease, and using critical care medical techniques for early and effective intervention.
Humans ; Female ; Pregnancy ; Adult ; Atypical Hemolytic Uremic Syndrome/therapy* ; Intensive Care Units ; Pregnancy Complications, Hematologic/therapy* ; Critical Care