Objective:To evaluate the safety and efficacy of accelerator-based boron neutron capture therapy (AB-BNCT) in the treatment of recurrent and refractory malignant tumors.Methods:The data of 14 patients admitted to Xiamen Humanity Hospital from September 2022 to April 2023 were prospectively collected, including 7 patients with primary brain malignancies and 7 patients with locally recurrent inoperable head and neck malignancies. All patients received intravenous infusion of boron drug (NBB-001, p-dihydroxyborylphe nylalanine, a patented freeze-dried formulation) at a total nominal dosage of 500 mg/kg (11 patients) or 750 mg/kg (3 patients), and were irradiated with neutrons (operating with NeuPex system). Adverse events after treatment were recorded and assessed. The primary efficacy endpoint was the 90 d objective response rate (ORR), while the secondary endpoints included progression-free survival (PFS) and complete response rate (CRR). Data were compiled and analyzed by SAS 9.4 software. The rate and 95% CI were calculated using Clopper-Pearson method. Results:The median dose delivered to 80% of the target volume (D 80%) was 16.80 GyE (range: 8.93-23.79 GyE). The most common adverse reactions were hyperamylasemia, alopecia, and hyperprolactinemia. Five patients experienced 8 cases of grade 3 or above adverse events, including 1 case of grade 4 acute kidney injury and 7 cases of grade 3 adverse events. All adverse events were recovered after observation or treatment. At 90 d after treatment, the ORR of all patients was 9/14 (64%, 95% CI: 35%-87%), disease control rate (DCR) was 10/14 (71%, 95% CI: 42%-92%), CRR was 2/14 (14%, 95% CI: 2%-42%); and the best overall response during the entire course included an ORR of 10/14 (71% ,95% CI: 42%-92%), DCR of 13/14 (93%, 95% CI: 66%-100%), and CRR of 3/14 (21% ,95% CI: 5%-51%). The 1-year survival rate for head and neck malignancies was 71.4%, and the 2-year survival rate was 42.8%. The 1-year survival rate for recurrent brain malignancies was 42.8%. Conclusion:AB-BNCT demonstrates favorable safety and promising efficacy in treating primary brain malignancies and recurrent/refractory head and neck malignancies, representing a potential therapeutic option.

Objective To investigate the correlation between serum monoamine oxidase(MAO),soluble growth stimulation expressed gene 2(sST2)and coronary slow flow(CSF),and to evaluate their value in pre-dicting CSF risk and guiding clinical decision-making.Methods A total of 118 patients with CSF confirmed by coronary angiography from January 2022 to September Dazhu Hospital Affiliated to North Sichuan Medical College 2023 were selected as the observation group,while 120 patients with normal coronary blood flow were selected as the control group.The coronary flow velocity was assessed using the TIMI frame count(TFC).Baseline characteristics,as well as serum MAO and sST2 levels,were compared between the two groups.Multi model calibration Logistic regression analysis was used to examine the relationship between the two markers and CSF.Pearson correlation analysis was conducted to evaluate the association between the markers and TFC.The predictive efficacy of MAO and sST2 for CSF and their benefit in clinical decision-making were as-sessed using receiver operating characteristic(ROC)curve and decision curve analysis(DCA).Results(1)The levels of body mass index(BMI),smoking proportion,C-reactive protein(CRP),uric acid(UA),creati-nine(Cr),MAO,and sST2 in the observation group were all higher than those in the control group(P＜0.05).(2)The TFC and mean TFC of LAD,LCX,and RCA in the observation group were higher than those in the control group(P＜0.05).(3)Multivariate Logistic regression analysis suggested that serum MAO and sST2 were independent risk factors for the occurrence of CSF(OR＞1,P＜0.05).(4)Pearson correlation a-nalysis showed that serum MAO and sST2 levels were positively correlated with the TFC of the LAD,LCX,and RCA,as well as the mean TFC(r=0.735,0.710,0.728,0.703,r=0.727,0.669,0.684,0.705,P＜0.05).(5)ROC curve revealed that the area under the curve(AUC)for predicting CSF was 0.761 of MAO,0.750 of sST2,and 0.807 of their combination.(6)DCA curve showed that the net benefit of serum MAO was greater than 0 when the threshold probability was between 0.05 and 0.70,the net benefit of serum sST2 was greater than 0 when the threshold probability was between 0.05 and 0.85,and their combined prediction yielded a greater net benefit within the threshold range of 0.40 to 0.90.Conclusion Elevated serum MAO and sST2 levels are independent risk factors for CSF.Both markers have good predictive value for CSF,and their combined detec-tion further improves predictive performance and clinical net benefit.

Objective:To evaluate the safety and efficacy of accelerator-based boron neutron capture therapy (AB-BNCT) in the treatment of recurrent and refractory malignant tumors.Methods:The data of 14 patients admitted to Xiamen Humanity Hospital from September 2022 to April 2023 were prospectively collected, including 7 patients with primary brain malignancies and 7 patients with locally recurrent inoperable head and neck malignancies. All patients received intravenous infusion of boron drug (NBB-001, p-dihydroxyborylphe nylalanine, a patented freeze-dried formulation) at a total nominal dosage of 500 mg/kg (11 patients) or 750 mg/kg (3 patients), and were irradiated with neutrons (operating with NeuPex system). Adverse events after treatment were recorded and assessed. The primary efficacy endpoint was the 90 d objective response rate (ORR), while the secondary endpoints included progression-free survival (PFS) and complete response rate (CRR). Data were compiled and analyzed by SAS 9.4 software. The rate and 95% CI were calculated using Clopper-Pearson method. Results:The median dose delivered to 80% of the target volume (D 80%) was 16.80 GyE (range: 8.93-23.79 GyE). The most common adverse reactions were hyperamylasemia, alopecia, and hyperprolactinemia. Five patients experienced 8 cases of grade 3 or above adverse events, including 1 case of grade 4 acute kidney injury and 7 cases of grade 3 adverse events. All adverse events were recovered after observation or treatment. At 90 d after treatment, the ORR of all patients was 9/14 (64%, 95% CI: 35%-87%), disease control rate (DCR) was 10/14 (71%, 95% CI: 42%-92%), CRR was 2/14 (14%, 95% CI: 2%-42%); and the best overall response during the entire course included an ORR of 10/14 (71% ,95% CI: 42%-92%), DCR of 13/14 (93%, 95% CI: 66%-100%), and CRR of 3/14 (21% ,95% CI: 5%-51%). The 1-year survival rate for head and neck malignancies was 71.4%, and the 2-year survival rate was 42.8%. The 1-year survival rate for recurrent brain malignancies was 42.8%. Conclusion:AB-BNCT demonstrates favorable safety and promising efficacy in treating primary brain malignancies and recurrent/refractory head and neck malignancies, representing a potential therapeutic option.

Objective::Patients with untreated severe aortic regurgitation (AR) have a high risk of mortality. Transfemoral transcatheter aortic valve replacement (TF-TAVR) is a treatment option for AR; however, the safety and efficacy of this technique have not been sufficiently established. This study aimed to evaluate the clinical and anatomical variables correlating with device success of TF-TAVR using a self-expanding valve system for pure AR.Methods::Patients with pure native severe AR who underwent TF-TAVR using a self-expanding valve system were registered at 5 Chinese centers. The primary endpoint was device success at 1 month after TAVR. The secondary endpoint was the composite of major adverse cardiovascular events (MACE) at 6 months, including all-cause death, ischemic stroke, emergency conversion to cardiac surgery, and permanent pacemaker implantation. Echocardiography was used to analyze the left ventricular function before the TAVR procedure and during follow-up. Multivariable logistic regression and Cox regression analyses were performed to find relevant independent risk factors.Results::Between September 2019 and February 2022, 79 patients with AR were enrolled in the study. At 1 month, device success was achieved in 60 (75.9%) patients. By 6 months, 29 (36.7%) patients had MACE. Echocardiography revealed improved left ventricular function after TAVR. Multivariate regression analysis demonstrated that the Society of Thoracic Surgeons risk score (odds ratio 0.760, 95% confidence interval (CI): 0.584-0.989; P = 0.041) and annulus perimeter (odds ratio 0.888, 95% CI: 0.796-0.992; P = 0.035) were 2 predictors of device success. Moreover, annulus perimeter (<80.2 mm), but not Society of Thoracic Surgeons risk score, was associated with a significant reduction in MACE at 6 months (hazard ratio 2.223, 95% CI: 1.060-4.659; P = 0.028). Conclusions::TF-TAVR using a self-expanding valve system appears to be a safe and feasible treatment for patients with pure native severe AR, particularly those with a less enlarged annulus.

Objective:To explore the long-term effects of intravascular ultrasound (IVUS) guidance on patients with acute coronary syndrome (ACS) undergoing drug-eluting stents (DES) implantation.Methods:Data used in this study derived from ULTIMATE trial, which was a prospective, multicenter, randomized study. A total of 1 448 all-comer patients were enrolled between 2014 August and 2017 May. Primary endpoint of this study was target vessel failure (TVF) at 3 years, including cardiac death, target-vessel-related myocardial infarction, and clinically-driven target vessel revascularization.Results:ACS was present in 1 136 (78.5%) patients, and 3-year clinical follow-up was available in 1 423 patients (98.3%). TVF in the ACS group was 9.6% (109/1 136), which was significantly higher than 4.5% (14/312) in the non-ACS group (log-rank P=0.005). There were 109 TVFs in the ACS patients, with 7.6% (43/569) TVFs in the IVUS group and 11.6% (66/567) TVFs in the angiography group (log-rank P=0.019). Moreover, patients with optimal IVUS guidance were associated with a lower risk of 3-year TVF compared to those with suboptimal IVUS results (5.4% (16/296) vs. 9.9% (27/273),log-rank P=0.041). Conclusions:This ULTIMATE-ACS subgroup analysis showed that ACS patients undergoing DES implantation were associated with a higher risk of 3-year TVF. More importantly, the risk of TVF could be significantly decreased through IVUS guidance in patients with ACS, especially in those who had an IVUS-defined optimal procedure.

Objective::Patients with untreated severe aortic regurgitation (AR) have a high risk of mortality. Transfemoral transcatheter aortic valve replacement (TF-TAVR) is a treatment option for AR; however, the safety and efficacy of this technique have not been sufficiently established. This study aimed to evaluate the clinical and anatomical variables correlating with device success of TF-TAVR using a self-expanding valve system for pure AR.Methods::Patients with pure native severe AR who underwent TF-TAVR using a self-expanding valve system were registered at 5 Chinese centers. The primary endpoint was device success at 1 month after TAVR. The secondary endpoint was the composite of major adverse cardiovascular events (MACE) at 6 months, including all-cause death, ischemic stroke, emergency conversion to cardiac surgery, and permanent pacemaker implantation. Echocardiography was used to analyze the left ventricular function before the TAVR procedure and during follow-up. Multivariable logistic regression and Cox regression analyses were performed to find relevant independent risk factors.Results::Between September 2019 and February 2022, 79 patients with AR were enrolled in the study. At 1 month, device success was achieved in 60 (75.9%) patients. By 6 months, 29 (36.7%) patients had MACE. Echocardiography revealed improved left ventricular function after TAVR. Multivariate regression analysis demonstrated that the Society of Thoracic Surgeons risk score (odds ratio 0.760, 95% confidence interval (CI): 0.584-0.989; P = 0.041) and annulus perimeter (odds ratio 0.888, 95% CI: 0.796-0.992; P = 0.035) were 2 predictors of device success. Moreover, annulus perimeter (<80.2 mm), but not Society of Thoracic Surgeons risk score, was associated with a significant reduction in MACE at 6 months (hazard ratio 2.223, 95% CI: 1.060-4.659; P = 0.028). Conclusions::TF-TAVR using a self-expanding valve system appears to be a safe and feasible treatment for patients with pure native severe AR, particularly those with a less enlarged annulus.

Objective:To investigate the diagnostic value and cutoff value of the basal luteinizing hormone (LH) level for central precocious puberty (CPP) in girls with different body mass index (BMI) values.Methods:A total of 1 087 girls aged 6 to 8 years old with breast development and visiting the Department of Endocrine Genetics and Metabolism, Fuzhou Children′s Hospital of Fujian Medical University from January 2014 to December 2018 were enrolled.The girls had the breast development of Tanner stage B2 or B3.They were divided into groups of normal weight (840 cases), overweight (133 cases) and obesity (114 cases) according to BMI percentiles corresponding to their age and sex.All individuals underwent gonadotropin-releasing hormone (GnRH) agonist test.Statistical analysis was performed using SPSS 18.0 software, and the receiver operating characteristic (ROC) curve was drawn.Results:In the normal weight group, 393 girls were positive and 447 girls were negative for GnRH test.In the overweight group, 72 girls were positive and 61 girls were negative for GnRH test.In the obesity group, 40 girls were positive and 74 girls were negative for GnRH test.The areas under the ROC curve (AUC) of the basal LH level for the diagnosis of CPP in the normal weight, overweight, and obese girls were 0.826, 0.774, and 0.801, respectively.When the Youden index was maximum, the cutoff values of the basal LH level were 0.245 IU/L, 0.225 IU/L and 0.145 IU/L, respectively; the sensitivities were 63.9%, 65.3%, and 80.0%, respectively; the specificities were 87.9%, 82.0%, and 71.6%, respectively.When the cutoff value of the basal LH level was 0.455 IU/L in the normal weight group, 0.480 IU/L in the overweight group, and 0.335 IU/L in the obesity group, the specificities of the 3 groups were all above 96.6%.Conclusions:It is reliable to use the basal LH level for CPP diagnosis for girls with different BMI values.The cutoff value of the basal LH level for CPP diagnosis in obese girls is the lowest.

Objective To analyze the clinical characteristic s of an infant with congenital lipoid adrenal hyperplasia (CLAH),and to sequence the acute regulatory protein (steroid acute regulatory,StAR) gene of the infant patient and her pedigree.Methods Physical examination,laboratory tests,and imaging examination of the 11-month-old patient with CLAH were collected.DNA was extracted from blood samples of the patient and her parents.The 7 exons of StAR gene were amplified by PCR and then sequenced.Results Dark skin,girl vulva,and one each 1.0 cm×1.0 cm palpable lump in inguinal area bilaterally were observed.The adrenocorticotropic hormone (ACTH) was 253 pg/ml,cortisol was 27.6 nmol/L at 8 am,17-hydroxyprogesterone was 3 nmol/L.Uhrasound showed that sign of testicular ultrasonography existed in bilateral inguinal regions.Karyotype analysis showed 46,XY.Sequencing of PCR amplified fragments showed that there were two heterozygous mutations c.229C ＞ T,p (Gln77X) and C.659A＞G,p (His220Arg) of StAR gene in this patient.By rectifying the disturbance of electrolyte,and treating with hydrocortisone and 9α fludrocortisone,etc,the patient has been stable so far.Conclusion The patient presents typical clinical manifestations.Two heterozygous mutations including c.229C ＞T,p (Gln77X) from maternal and C.659A＞G,p (His220Arg) from paternal of StAR gene were detected.Wherein the c.659A＞G,p.(His220Arg) as a novel point mutation of StAR gene,has not been reported so far.

Objective To explore the efficacy of modified uvula palatopharyngeal plasty in the treatment of obstructive sleep ap-nea syndrome(OSAHS) .Methods 145 cases of OSAHS patients were treated from June 2006 to June 2010 in our hospital ,of which 54 were treated by traditional UPPP surgery ,and the remaining 91 cases were treated by h-UPPP surgery .Compared its effi-cacy by the preoperative and postoperative polysomnography map (PSG) analysis .Results In 145 cases of patients with OSAHS , the PSG monitoring indicators of 1 year after surgery were compared with preoperative ,and there were significant difference in the apnea total time(AI) ,the breathing hypopnea index(AHI) ,the lowest oxygen saturation(SaO2 )(P<0 .05) .The subjective symp-toms in postoperative recovery process of modified UPPP ,such as dry throat ,open nasal ,nasopharyngeal reflux symptoms were sig-nificantly reduced than that of traditional UPPP surgery .Conclusion h-UPPP surgery have a good effect .

Objectives To investigate the causes of short stature in Turner syndrome (TS). Methods 86 patients were di-agnosed with TS by karyotypes from 2004 to 2013. According to the deletion types of the X chromosome short arm, growth hor-mone (GH), insulin-like growth factor-1 (IGF-1) and thyroid function, the TS patients were divided into different groups and com-parison was made among groups. Results Ht SDS in three groups with different extent of the deletion of the X chromosome short arm were (-4.39±1.08), (-3.26±1.25) and (-2.84±0.15) (P<0.05). The proportion of growth hormone deifciency (GHD) in the three groups were 62.5%, 38.9%and 0%(P<0.05). Ht SDS in groups with different degree of IGF-1 level were (-4.37±1.10), (-3.82±1.07) and (-3.25±0.91) (P<0.05). There was no signiifcant difference of Ht SDS between hypothyroidism patients with and without GHD (P>0.05). Conclusions The deletion of X chromosome short arm may cause the short stature in TS. The GH-IGF-1 axis in TS is impaired, but GHD is not related to short stature in TS.