Objective:To predict Chinese materia medica that may prevent and treat coronary microvascular dysfunction (CMD) by identifying disease core targets.Methods:CMD- related targets were obtained through GeneCards and OMIM databases. Subnetworks were extracted by using MCODE plugin in Cytoscape 3.9.1. Core targets of subnetworks were obtained by using cytoNCA plugin. GO function and KEGG pathway enrichment analysis for core targets were performed by using Metascape. Coremine Medical database was used to match targets with Chinese materia medica. Obtained Chinese materia medica was screened, and their properties and tastes, meridians and efficacy categories were under statistics.Results:Totally 3 859 disease-related targets were screened and five subnetworks were obtained. An in-depth study of MCODE1 yielded ten core targets, including IL-1β, IL6, TNF, STAT3, AKT1, ACTB, VEGFA, GAPDH, TP53, and ALB. GO functional enrichment analysis showed that these core targets were mainly involved in biological processes, such as positive regulation of gene expression, positive regulation of transcription, DNA template, and negative regulation of gene expression. KEGG pathway enrichment analysis identified 67 signaling pathways, including the AGEs-RAGE signaling pathway, HIF-1 signaling pathway, adipocytokine signaling pathway, fluid shear stress, and atherosclerosis. The researchers identified 36 kinds of Chinese materia medica associated with the ten core targets, including Salviea Miltiorrhizae Radix et Rhizoma, Chuanxiaong Rhizoma, Carthami Flos, Paeoniae Radix Rubra, Coptidis Rhizoma, Ginseng Radix et Rhizoma, Ophiopogonis Radix, Schisandrae Chinensis Fructus, Cinnamomi Cortex, Nelumbinis Semen, and Valerianae Jatamansi Rhizoma et Radix among 880 herbs.Conclusion:This study predicts 36 kinds of Chinese materia medica that have the effect of preventing and treating CMD, which can provide research ideas for the development of new drugs.

Objective:To investigate the clinical characteristics and management status of children with Turner syndrome (TS) in China.Methods:As a cross-sectional study, 1 089 TS patients were included in the database of the National Collaborative Alliance for the Diagnosis and Treatment of Turner Syndrome from August 2019 to November 2023. Clinical characteristics (growth development, sexual development, organ anomalies, etc.), karyotypes, auxiliary examinations, and treatments were collected and analyzed.Results:Among the 1 089 TS cases, 809 were recorded karyotypes. The karyotype distribution was as follows: 45, X in 317 cases (39.2%), X chromosome structural variants (including partial deletions of p or q arm, ring chromosome, and marker chromosome) in 89 cases (11.0%), 45, X/46, XX mosaicism in 158 cases (19.5%), mosaicism with X chromosome structural variants in 209 cases (25.8%), and presence of Y chromosome material in 36 cases (4.4%). Among the 824 TS cases, the age of diagnosis was 9.7(6.4, 12.2) years, with a height standard deviation score (HtSDS) of -3.1±1.2. Five hundred and fifty three cases underwent growth hormone (GH) stimulation test, and 352 cases (63.7%) had GH peak values <10 μg/L and 75.9% (577/760) had low IGF1 levels, with IGF1 SDS ≤-2 accounting for 38.2% (290 cases). Among 471 cases aged ≥8 years, 132 cases (28.0%) showed spontaneous sexual development (mean bone age (11.0±1.7) years), 10 cases had spontaneous menarche (mean bone age (12.0±2.2) years), and 2 cases had regular menstrual cycles. Common physical features included cubitus valgus (311 cases (28.5%)), neck webbing (188 cases (17.2%)), low posterior hairline (185 cases (17.0%)), shield chest (153 cases (14.0%)), high arched palate (127 cases (11.6%)), short fourth metacarpal (43 cases (3.9%)), and spinal abnormalities (38 cases (3.5%)). Congenital cardiovascular and urogenital anomalies occurred in 91 cases (19.4%) and 66 cases (12.0%)respectively. Abdominal ultrasound in 33 cases (7.2%) indicated fatty liver, hepatomegaly, intrahepatic bile duct stones, and splenomegaly. Among 23 cases undergoing oral glucose tolerance test (OGTT) test, 2 were diagnosed with diabetes mellitus and 4 with impaired glucose tolerance. Following diagnosis, 669 cases (80.7%) received rhGH treatment at a chronological age of (9±4) years and bone age of (8.3±3.2) years. Additionally, 112 cases (19.4%) received sex hormone replacement therapy starting at the age of (14±4) years and bone age of (12.6±1.2) years.Conclusions:The karyotypes of 45, X and mosaicism were most common in Chinese children with TS. The clinical manifestations were mainly short stature and gonadal dysplasia. However, a few TS children could be in the normal range of height, and some cases among those aged of ≥8 years old had spontaneous sexual development. Some exhibited physical features, congenital cardiovascular and urogenital anomalies, and dysfunction of the hypothalamic-pituitary-IGF1 axis. Moreover, a few of them developed impaired glucose tolerance and diabetes mellitus. Following diagnosis, most of the patients received rhGH treatment, and a few of them received sex hormone replacement therapy.

Objective:To investigate the diagnostic value of serum Aspergillus fumigatus immunoglobulin G (IgG), bronchoalveolar lavage fluid (BALF) galactomannan antigen test (GM test) and combined detection of the two methods in chronic pulmonary aspergillosis (CPA). Methods:A total of 310 patients with suspected CPA who were hospitalized in Wuhan Pulmonary Hospital from January 2020 to December 2022 were screened. BALF-GM test and serum Aspergillus fumigatus IgG were detected. According to the inclusion and exclusion criteria, 151 confirmed or clinically diagnosed CPA patients were enrolled and 60 non-CPA cases were included as controls. The underlying diseases, clinical symptoms and imaging findings of the two groups were analyzed using case-control study. Statistical comparison was performed by chi-square test. The efficacies of BALF-GM test, serum Aspergillus fumigatus IgG test, and their combined detection for CPA were analyzed by receiver operating characteristic curve. Results:There were 130 confirmed cases and 21 clinically diagnosed cases among 151 patients with CPA, including 106 males (70.2%) with age of (54.6±15.5) years. The age of 60 non-CPA patients was (53.6±17.8) years, including 42 males (70.0%). The proportions of pulmonary tuberculosis, bronchiectasis and chronic obstructive pulmonary disease, and the incidence of hemoptysis in CPA group were 79.47%(120/151), 86.09%(130/151), 26.49%(40/151) and 43.71%(66/151), respectively, which were all higher than those in non-CPA group (10.00%(6/60), 25.00%(15/60), 8.33%(5/60) and 11.67%(7/60), respectively), and the differences were statistically significant ( χ2=86.14, 74.56, 8.44 and 19.48, respectively, all P<0.05). The proportion of interstitial lung disease in non-CPA group was 28.33%(17/60), which was higher than that in CPA group (2.65%, 4/151), and the difference was statistically significant ( χ2=31.61, P<0.001). The common imaging findings of CPA patients were pulmonary cavity (70.86%, 107/151), cavity inclusions (41.72%, 63/151) and lung damage (33.77%, 51/151). The specificity and sensitivity of serum Aspergillus fumigatus IgG for CPA diagnosis were 81.7% and 68.9%, respectively, and the area under the curve (AUC) was 0.753 (95% confidence interval (95% CI) 0.681 to 0.825, P< 0.001). The specificity and sensitivity of BALF-GM test were 76.7% and 67.5%, respectively, and the AUC was 0.724 (95% CI 0.649 to 0.800, P<0.001). The specificity and sensitivity of the two methods in series combined detection were 95.0% and 44.4%, respectively, and those of the two methods in parallel detection were 60.3% and 94.5%, respectively. The AUC of the combined diagnosis of CPA by the two methods was 0.843 (95% CI 0.783 to 0.903, P<0.001). Conclusions:Serum Aspergillus fumigatus IgG combined with BALF-GM test has a good diagnostic efficacy for CPA. The detection of serum Aspergillus fumigatus IgG is non-invasive and highly specific, which is beneficial to the early diagnosis and treatment of CPA.

In November 2023, the seventh National Nucleic Acid Vaccine Conference was held to deeply discuss the immune mechanism, safety risks, advantages, and disadvantages of nucleic acid vaccines, and review the safety and effectiveness of COVID-19 vaccines developed by nucleic acid vaccine technology. Some prospectives were formed in the meeting that in the post-pandemic era, nucleic acid vaccine technology will play a role in the following areas: dealing with pathogens that are difficult to be prevented by traditional vaccines, promoting the upgrading of traditional live attenuated vaccines, contributing to the development of multivalent and combined vaccines, and rapid response to emerging and re-emerging infectious diseases. These views point out the direction for the future development of nucleic acid vaccine technology.

The paper reports two cases of lipoprotein glomerulopathy (LPG) in children. The Sanger sequencing results in 2 cases indicated apolipoprotein E gene mutation[c.127 (exon3) C>T, p.R43C (p.Arg43Cys); c.494 (exon4) G>C, p.R165P (p.Arg165Pro),respectively]. Renal pathological presentation of two children showed that a large number of lipoprotein emboli were formed in the glomerular capillary loop, and the diagnosis of LPG was confirmed. The onset of LPG has no specific clinical manifestation, which is easy to be undiagnosed or misdiagnosed. Renal biopsy is a diagnostic means, glucocorticoid treatment is ineffective, and long-term lipid-lowering treatment may be required for LPG.

Objective:To analyze the long-term prognosis of IgA nephropathy (IgAN) with focal segmental glomerulosclerosis (FSGS) and the risk factors related to renal prognosis in children with IgAN-FSGS.Methods:A retrospective study was concluded in IgAN-FSGS children who were followed up for more than 5 years and diagnosed by renal biopsy for the first time in the Eastern Theater General Hospital from January, 2004 to December, 2018. The end-point events of the study were entering end-stage kidney disease (ESKD) or estimated glomerular filtration rate (eGFR) decreased by ≥50% from baseline, which were defined as poor renal prognosis. Baseline clinicopathologic data of IgAN-FSGS children were compared between the end-point event group and the non-end-point event group. The cumulative renal survival rate of IgAN-FSGS children was calculated by Kaplan-Meier survival analysis. The influencing factors of poor renal prognosis in IgAN-FSGS children were analyzed by Cox proportional hazards model, and the diagnostic value was evaluated by the receiver operating characteristic curve (ROC curve) and area under the curve (AUC). The diagnostic value was verified by time dependent-ROC and time dependent-AUC.Results:A total of 204 IgAN-FSGS children were enrolled in this study, of whom 132 cases were males (64.7%). The median age of renal biopsy was 16 (14, 17) years old. During a median follow-up time of 90.7 (71.7, 114.8) months, 57 cases (27.9%) reached the end-point events. Compared with the non-end-point event group ( n=147), the end-point event group ( n=57) had higher proportions of males and hypertension, higher levels of 24-hour urinary protein, serum creatinine, serum uric acid, urinary N-acetyl-β- D-glucosaminidase, urinary retinol binding protein, higher proportions of glomerular segmental sclerosis (S1) ≥25% and tubular atrophy/interstitial fibrosis (T1/T2), and lower levels of serum albumin, serum IgA, and serum IgG (all P<0.05). There was no statistical difference between the two groups in treatment (all P>0.05). Kaplan-Meier survival analysis showed that with entry of ESKD or eGFR decreased by ≥50% from baseline as the end-point events, the 5-year, 10-year, and 15-year cumulative renal survival rates in IgAN-FSGS children were 88.7%, 67.6%, and 50.7%, respectively. Multivariate Cox regression analysis showed that proteinuria >1 g/24 h ( HR=3.702, 95% CI 1.657-8.272, P=0.001), hyperuricemia ( HR=3.066, 95% CI 1.793-5.245, P<0.001), S1≥25% ( HR=2.017, 95% CI 1.050-3.874, P=0.035), T1/T2 ( HR=1.863, 95% CI 1.021-3.158, P=0.016) were the independent related factors for poor renal prognosis. ROC curve analysis showed that S1≥25% ( AUC=0.605, P=0.021, sensitivity 26.3%, specificity 94.6%), T1/T2 ( AUC=0.624, P=0.006, sensitivity 43.9%, specificity 81.0%), hyperuricemia ( AUC=0.658, P<0.001, sensitivity 52.6%, specificity 78.9%), proteinuria>1 g/24 h ( AUC=0.670, P<0.001, sensitivity 87.7%, specificity 46.3%) could accurately predict the renal outcome of IgAN-FSGS. Time dependent-ROC curve validation showed that the combined diagnosis of S1≥25%, T1/T2, hyperuricemia and proteinuria>1 g/24 h had a good predictive value for renal prognosis (3-year AUC=0.846 and 5-year AUC=0.777, respectively). Conclusions:During a median follow-up of 90.7 months, 27.9% of IgAN-FSGS children have poor renal prognosis, and the 5-year, 10-year, and 15-year cumulative renal survival rates are 88.7%, 67.6%, and 50.7%, respectively. Urinary protein >1 g/24 h, hyperuricemia, T1/T2, and S1 ≥25% are the risk factors for renal prognosis in IgAN-FSGS children.

Objective     To investigate short-term outcomes of robot-assisted Heller-Dor myotomy (RAHM-Dor) for achalasia of cardia and our learning curve experience. Methods     The clinical data and recent follow-up results of 42 patients who received RAHM-Dor from November 2015 to January 2020 in the Department of Thoracic Surgery of the First Affiliated Hospital of Nanchang University were retrospectively analyzed, including 20 males and 22 females with a mean age of 40.8±18.4 years. Results    Dysphagia was the most common symptom, followed by heartburn and regurgitation. The mean operation time was 122.8±23.9 min. The mean blood loss was 47.5±32.7 mL. Two patients suffered mucosal injury, and successfully repaired by suturing during surgery. There was no esophageal fistula, conversion to an open operation or perioperative death in this series. The median length of hospital stay was 8 (6, 9) d. In all patients, the Stooler and Eckardt scores of postoperative 1, 6 and 12 months decreased compared to those of pre-operation (P<0.001). Conclusion     RAHM-Dor is a safe and feasible avenue for the treatment of achalasia of cardia, and can achieve a satisfying short-term results. The learning curve shows a transition to the standard stage from the learning stage after 16-18 operations.

Liver transplantation is one of the main treatments of early hepatocellular carcinoma (HCC). The recurrence of HCC after liver transplantation severely affects the long-term survival rate of the recipients. Targeted therapy and immunotherapy play a critical role in HCC downstaging, preventing disease progression, reducing recurrence rate, prolonging the survival and improving the quality of life. However, no consensus has been reached on the application of targeted therapy and immunotherapy in recipients undergoing liver transplantation for HCC, including indications, timing and dosage. In this article, clinical research progresses on the indications and timing of targeted therapy and immunotherapy before and after liver transplantation for HCC were reviewed, aiming to provide reference for prolonging the survival of recipients after liver transplantation for HCC.

Cleft lip and/or palate (CLP) are the most common craniofacial malformations in humans. Speech problems often persist even after cleft repair, such that follow-up articulation training is usually required. However, the neural mechanism behind effective articulation training remains largely unknown. We used fMRI to investigate the differences in brain activation, functional connectivity, and effective connectivity across CLP patients with and without articulation training and matched normal participants. We found that training promoted task-related brain activation among the articulation-related brain networks, as well as the global attributes and nodal efficiency in the functional-connectivity-based graph of the network. Our results reveal the neural correlates of effective articulation training in CLP patients, and this could contribute to the future improvement of the post-repair articulation training program.
Brain/diagnostic imaging* ; Cleft Lip/surgery* ; Cleft Palate/surgery* ; Humans ; Magnetic Resonance Imaging ; Reading

The adjacent anatomy of the pelvis is complicated, with digestive, urinary, reproductive and other organs as well as important blood vessels and nerves. Therefore, accurate resection of pelvic tumors and precise reconstruction of defects after resection are extremely difficult. The development of medical 3D printing technology provides new ideas for precise resection and personalized reconstruction of pelvic tumors. The “triune” application of 3D printing personalized lesion model, osteotomy guide plate and reconstruction prosthesis in pelvic tumor limb salvage reconstruction treatment has achieved good clinical results. However, the current lack of normative guidance standards such as preparation and application of 3D printing personalized lesion model, osteotomy guide plate and reconstruction prosthesis restricts its promotion and application. The formulation of this consensus provides normative guidance for 3D printing personalized pelvic tumor limb salvage reconstruction treatment.