Objective To explore the clinical diagnosis of tetralogy of Fallot( TOF) children with concurrent DiGeorge syndrome ( DGS ) . Methods Retrospective analyses were conducted for the clinical characteristics of 715 TOF children with concurrent DGS at Henan Provincial People′s Hospital and the Third Affiliated Hospital of Zheng-zhou University from January of 2008 to October of 2014. Among them,there were 78 definite cases of thymic aplasia (DGS group),including 45 boys and 33 girls with an age range of(9. 12±4. 35) months and a body mass range of (7. 28±2. 34) kg. And the remainder was designated as non-DGS group(NDGS group),including 387 boys and 250 girls with an age range of(8. 21±5. 61) months and a body mass range of(8. 19±3. 47) kg. In DGS group,genetic screening uncovered 10 cases of chromosome 22q11. 2 gene deletion. And based upon this result,DGS group was fur-ther divided into genetic and clinical diagnosis subgroups. The genetic diagnosis group had 10 cases,including 6 boys and 4 girls with an age range of(8. 12±4. 15) months and a body mass range of(6. 28±2. 74) kg,the clinical diagnosis group had 68 cases,including 39 boys and 29 girls with an age range of(8. 19±4. 37) months and a body mass range of (7. 05±2. 39) kg. Results No statistical difference existed in age,body mass or preoperative developmental status of pulmonary vasculature between DGS group and NDGS group(P>0. 05). And the preoperative incidence of recurrent pneumonia was obviously higher in DGS group than that in NDGS group(P<0. 001). The probability of concurrent hy-pocalcemia and facial malformation was higher in DGS group than that in NDGS group. However,there was no statistical difference(P>0. 05). And an inter-group comparison of T lymphocyte immunity defect had no statistical difference(P>0. 05). The duration of on-machine and intensive care unit stay was markedly longer in DGS group than that in NDGS group(P<0. 001). And the probability of concurrent hypocalcemia and facial malformation was higher in genetic diag-nosis subgroup than that in clinical diagnosis subgroup. However,there was no statistical difference(P>0. 05). And an inter-group comparison of T lymphocyte immunity defect had no statistical difference ( P>0. 05 ) . Conclusions With diverse clinical manifestations,DGS patients may have non-specific findings of cellular immunity defect,hypocalcemia or facial malformation. TOF children with concurrent thymic aplasia should raise an alert so that effective interventions may be adopted to boost their long-term quality of life.

Objective:To study the preparation and quality control of loxoprofen sodium patches and develop an HPLC method for the determination of loxoprofen sodium. Methods:Loxoprofen sodium patches were prepared with CMC-Na and PVP as the adjuvants, and an HPLC method was used to determine the content of loxoprofen sodium. Results:The linear range of loxoprofen sodium was 4-24μg·ml-1(r=0. 999 7), the average recovery was 99. 99%(RSD=0. 99%, n=6). Conclusion:The preparation method is reason-able, simple and stable. The developed HPLC method is specific to determine the content of loxoprofen.

Objective To screen the differential proteins in the brain (neocortex and hippocampus) between the rats with cortical dysplasia (CD) and control ones,and investigate the role of their alteration in the development of epilepsy in CD.Methods Cortical dysplasia was induced in rat pups via in utero delivery of BCNU.A two-dimensional electrophoresis (2-DE)-based approach was used to construct the expression profiles of proteins in both the neocortex and hippocampus at different age groups (postnatal day 7 and 60) and to detect proteome changes between CD rats and control ones.Following gel image analysis,protein spots that differed in abundance between CD and control rats were identified by using Matrx-assisted laser desorption/ionization (MALDI) mass spectrometry (MS) and MS/MS.Results A total of 57 kinds of protein were screened out (P ＜ 0.05),in which 35 were found up-regulated and 22 were down-regulated compared with the control,35 from neonatal stage (postnatal day 7) and others from adult stage.Finally,12 among them were identified,including tubulin,alpha-lB,Beta-actin,tubulin beta-2A,GAP-43,UbCKmit,GAPDH and TMBr-3,etc.Conclusions Changed expression of specific proteins which were found in our study are involved in construction of brain 's cytoskeleton,synaptic function,mitochondrial function and so forth.Thus,they may be related to the pathogenic mechanisms of epileptogenicity of CD.

Objective To investigate the feasibility and safety of surgical device closure of doubly committed sub-arterial ventricular septal defect via left sub-axillary.Methods A total of 45 patients diagnosed as doubly committed sub-arterial ventricular septal defect (dcVSD) with transthoracic echocardiography (TTE) and transesophageal echocardiography(TEE) were enrolled from June 2014 to August 2016 in Henan Children Heart Center,Henan Provincial People's Hospital.There were 39 males and 6 females,with the mean age of (2.2 ±2.1) years old(0.5-8.0 years),the body weight (13.8 ± 7.1) kg(7.0-34.1 kg),the defect size (4.5 ± 1.0) mm (3.0-8.0 mm).After general anesthesia,the patients were in supine and evaluated by TEE which indicated whether they were fit to closure.Then,they were turned to the right lateral position while this technique was determined.A vertical incision of 2-3 cm was made between the third and the fifth intercostal space and invasion in thoracic space via fourth intercostal space.Puncture was done at the anterior surface of right ventricular outlet tract to build a delivery tract.The occluder was released and the VSD was occluded under transesophageal echocardiography guidance.Results Forty-one patients had a successful surgical dcVSD closure with asymmetric occluders sized (6.0 ± 1.5) mm(4-10 mm).Among 4 failure cases,2 cases (4.4％) were switched to open-heart surgical repair,1 case (2.2％) due to device related aortic regurgitation,the rest 1 case (2.2％) experienced a dislocation of occluder into pulmonary artery and was converted to surgical repair after retrieve of occluder.Trivial residual shunt was detected in 2 cases (4.4％) postoperatively,a spontaneous closure was observed by 1 month follow-up and 3 months follow-ups,respectively.All the patients were discharged 5 to 8 days after the operation.With a follow-up of (10.4 ±5.0) months [3-24 months],there were no complications such as pericardial effusion,displacement of device,atrioventricular block or new valvular dysfunction.Conclusions Minimally invasive device closure of doubly committed sub-arterial ventricular septal defect via left sub-axillary is a feasible and safe treatment for closure of dcVSD.This technique has advantages of minor wound,less exudation,covert incision,however,long term follow-up is necessary.

Objective To investigate the frequency of JAK2 V617F mutation and JAK2 V617F mutation allele burden in patients with essential thrombocythemia (ET), and explore the relationship between mutation and hematological parameters and coagulation function. Methods The clinical and laboratory parameters of 90 ET patients were analyzed. JAK2 V617F mutation was detected by AS-PCR and the mutation allele burden of JAK2 V617F was detected by qPCR. The correlation between mutation frequency and mutation burden of JAK2 V617F and blood laboratory parameters were investigated in ET. Results JAK2 V617F mutation was found in 50 patients (55.6 %). RBC [(4.67±0.89)×109/L vs (4.04±0.99)×109/L, P =0.003], WBC (11.64±5.20)×109/L vs (9.11±4.11)×109/L, P = 0.014], HCT (0.41±0.07) vs (0.36±0.07), P =0.005) in the JAK2 V617F mutated group were higher than those in the wild-type group. PT in mutated patients was longer than that in wild-type group [(13.18±1.63) s vs (12.02±1.24) s, P = 0.000]. The JAK2 V617F mutation allele burden was (29.91 ±18.63) %. No significant correlation was found between JAK2 V617F mutation allele burden and hematological parameters such as WBC, RBC and Plt (all P>0.05), but the JAK2 V617F mutation allele burden had a significant correlation with FDP (r = 0.456, P = 0.001). Conclusions JAK2 V617F mutation occurs in significant percentage patients with ET. Detection of JAK2 V617F mutation allele burden at diagnosis may play an important role in the early prevention of vascular events.

Objective To assess the diet quality of pregnant women during the second trimester using the dietary inflammatory index ( DII) and to explore the correlation between the DII in second trimester of preg-nancy and preterm delivery.Methods A total of 253 women with singleton pregnancy in 16-20 gestational weeks who had received routine prenatal care between August 2014 and April 2015 at the First Affiliated Hospi-tal of Anhui Medical University were enrolled with cluster random sampling.The included women were asked to recall daily dietary intake in the 3 days prior to the survey.All dietary data were analyzed for energy and nutri-ents intake with a nutritional analysis software, followed by calculation of DII according to previous reports. Based on quartiles of the DII scores, the participants were divided into three groups, namely the anti-inflam-matory group (DII<-5.10), intermediate group (DII -5.10--2.55), and the pro-inflammatory group (DII>-2.55).The participants were followed up until delivery and the pregnancy outcomes were recorded. The relationship between the DII in second trimester of pregnancy and preterm delivery were analyzed. Results DII scores of the 253 pregnant women during the second trimester of pregnancy ranged from -7.913 to 3.872.The risks of preterm birth in the anti-inflammatory diet group, the intermediate group, and the pro-inflammatory diet group were 0, 1.6%, and 6.3%, respectively, with statistically significant differences among the groups (P=0.034).The higher DII scores (pro-inflammatory) were associated with higher inci-dence of preterm birth ( P<0.05 ) .Conclusion DII may be used to assess diet quality of pregnant women during the second trimester and to predict the risk of preterm birth.

Objective: To analyze the association between each regional fat mass and total body bone mineral density (BMD) in children and adolescents aged 7-17 years in Beijing, so as to provide theoretical basis and practical guidance for implementing interventions. Methods: From September to December 2020, a stratified cluster random sampling method was used to select 1 423 children and adolescents aged 7-17 years in Tongzhou District, Beijing. Dual energy X-ray absorptiometry (DXA) was employed to measure regional body composition and total body BMD. Multiple linear regression was used to analyze the association between regional fat mass and total body BMD. Results: The median (interquartile range) fat mass values for total body, upper limbs, abdomen, hips, and thighs were 13.51(8.84, 19.21), 1.59(1.08, 2.23), 0.73(0.39, 1.29), 2.32(1.46, 3.26), 5.29(3.59, 7.21)kg, respectively. After adjusting for covariates, the results of multiple linear regression analysis showed that total body fat mass (β=0.010), abdominal fat mass (β=-0.100), and hip fat mass (β=0.104) were significant associations with total body BMD (all P<0.01). Sexstratified analysis revealed that in boys, total body fat mass (β=0.008) and hip fat mass (β=0.058) were positively associated with BMD, while thigh fat mass (β=-0.038) showed a negative association with total body BMD (all P<0.05). In girls, total body fat mass (β=0.013), hip fat mass (β=0.163), and thigh fat mass (β=0.023) were positively associated with total body BMD, whereas abdominal fat mass (β=-0.196) showed a negative association with total body BMD (all P<0.05). Among children and adolescents with body fat percentage below the standard range, within the standard range and above the standard range, total body fat masses were positively associated with total body BMD (β=0.021, 0.016, 0.015); among children and adolescents with body fat percentage within the standard range while upper limb (β=-0.042), abdominal (β=-0.067), and thigh fat mass (β=-0.018) showed negative associations with total body BMD, and hip fat mass demonstrated a positive association with total body BMD (β=0.082) (all P<0.05). Conclusion Regional fat distribution is associated with total body BMD in children and adolescents, with the nature of these associations varying by sex and body fat percentage.

Objective:To investigate the efficacy and safety of Treprostinil in the treatment of children with early decompensation after the Fontan procedure.Methods:A retrospectively analysis was performed on the clinical data of 16 children with early decompensation after the Fontan procedure treated with Treprostinil injection from December 2017 to June 2020 at Fuwai Central China Cardiovascular Hospital.A total of 16 patients were included, including 5 boys (31.2%) and 11 girls (68.8%). The age was (4.6 ±1.2) years, the weight was (16.0±2.1) kg.The changes of central venous pressure (CVP), heart rate (HR), systolic blood pressure (SBP), central venous oxygen saturation (ScvO 2), lactic acid (Lac), oxygenation index and B-type natriuretic peptide (BNP) were recorded at the infusion of Treprostinil and 3 hours, 24 hours, 48 hours and 72 hours after the infusion.The short-term efficacy of Treprostinil was observed[mortality, mechanical ventilation time, and length of intensive care unit (ICU) stay]; paired t-test was used to analyze the above indexes at different time points.The adverse reactions during the administration were also recorded. Results:Of the 16 children, the median mechanical ventilation time was 9 (5, 22) h, and the median ICU stay time was 2 (1, 12)days.After 72 hours of drug administration, CVP, Lac, BNP and HR decreased: CVP decreased from(16±5) mmHg (1 mmHg=0.133 kPa) to (11±2) mmHg ( P<0.001), Lac decreased from(6.8±3.2) mmol/L to (3.2±1.2) mmol/L ( P=0.002), BNP decreased from(980±223) ng/L to (250±120) ng/L( P<0.001), HR decreased from(150±20) times/min to (125±16) times/min( P=0.002); SBP, ScvO 2 and oxygenation index increased: SBP increased from(83±10) mmHg to (98±12) mmHg( P<0.001), ScvO 2 increased from 0.53±0.13 to 0.65±0.11 ( P=0.003), oxygenation index increased from (200±72) mmHg to (298±13) mmHg ( P<0.001), and the differences were statistically significant(all P< 0.05). One case died (6.3%), 2 cases (12.5%) had transient blood pressure drop and 1 case (6.3%) had nausea and vomiting.Besides, no other treatment-related complications were observed. Conclusions:As for children with early decompensation after the Fontan procedure, the intravenous application of Treprostinil can reduce pulmonary artery pressure rapidly, effectively improve circulatory status and oxygenation and ultimately improve the prognosis.

Objective:To analyze the short and mid-term efficacy of aortic valvuloplasty with autopericardium on children with aortic valve diseases.Methods:A total of 26 children with aortic valve diseases (stenosis or regurgitation) who underwent aortic valvuloplasty with autopericardium in Fuwai Central China Cardiovascular Hospital from September 2017 to June 2021 were retrospectively analyzed.The short-term and mid-term follow-up data were collected.The maximum aortic valve pressure gradient, subaortic regurgitation area, left ventricular end-diastolic volume (LVEDV) and left ventricular ejection fraction (LVEF) were compared before and after operation.Paired t test was used to analyze the short-term and mid-term efficacy of aortic valvuloplasty with autopericardium on children with aortic valve diseases. Results:All 26 cases were successfully operated, and there were no deaths and serious complications during the follow-up period of (22.96±6.45) months.There was a significant difference between the preoperative and postoperative maximum aortic valve pressure gradient at 1 month ( t=7.85, P<0.05), 6 months ( t=6.43, P<0.05), 1 year ( t=6.16, P<0.05) and 2 years postoperatively ( t=4.22, P<0.05) in children with aortic stenosis or that combined with mild-to-moderate closure.The follow-up data of 9 children with simple aortic stenosis showed that there was a significant difference between the preoperative (8.87±3.57) cm 2 and postoperative aortic regurgitation area at 1 month ( t=6.85, P<0.05), 6 months ( t=5.13, P<0.05), 1 year ( t=6.62, P<0.05) and 2 years postoperatively ( t=5.41, P<0.05). The LVEDV of 26 children was significantly lower at 6 months[(63.54±27.61) mL], 1 year [(53.61±20.20) mL] and 2 years postoperatively [(64.39±17.78) mL] compared with that of preoperative level[(89.42±45.89) mL]( t=3.89, 4.67, 3.58, all P<0.05). The left ventricular pressure and volume decreased, the enlarged heart was narrowed down, and the geometry of the heart was restored.The LVEF of 26 patients also from (61.65±9.67)% before surgery increased to (67.88±4.69)% 6 months after surgery( t=3.68, P<0.05), and increased to (68.62±4.46)% 1 year after surgery( t=4.01, P<0.05), and increased to (67.55±3.09)% 2 years after operation( t=3.01, P<0.05), and the heart function was improved. Conclusions:Aortic valvuloplasty with autopericardium presents an effective short and mid-term efficacy on children with aortic valve diseases, which prevents or delays the aortic valve replacement.

Objective:To explore the relationship between the triglyceride glucose (TyG) index and the risk of developing hypertension among rural Chinese adults.Methods:A prospective cohort study was conducted from 2007 to 2008, involving 20 194 adults selected through random cluster sampling from a rural community in Luoyang City, Henan Province. Follow-ups were carried out in 2013-2014 and 2018-2020. After excluding participants with hypertension at baseline, those with missing TyG index data, individuals who passed away during follow-up, and those with incomplete hypertension status at the second visit, 9 802 participants were included in the analysis. Baseline and follow-up assessments included questionnaire interviews, physical measurements (including blood pressure), and blood sample collection for fasting lipid and glucose levels. Participants were divided into four groups according to TyG index quartiles, and a modified Poisson regression model was utilized to assess the association between TyG index quartiles and hypertension risk.Results:The study cohort comprised 9 802 participants with a median age of 48 (39, 57) years, including 3 803 males (38.80%). Participants were distributed across TyG index quartiles as follows: TyG<8.2 group (2 224 individuals), TyG 8.2-8.5 group (2 653 individuals), TyG 8.6-8.9 (2 441 individuals), and TyG≥9.0 (2 484 individuals). Over a follow-up period of (11.1±1.3) years, 3 378 subjects developed hypertension, resulting in a cumulative incidence of 34.46% (3 378/9 802). The risk of hypertension increased with higher TyG index quartiles ( Ptrend<0.05). Compared to the TyG<8.2, the TyG 8.2-8.5 ( RR=1.11, 95% CI 1.01-1.22, P=0.023), TyG 8.6-8.9 ( RR=1.16, 95%CI 1.06-1.27, P=0.023), and TyG≥9.0 ( RR=1.20, 95%CI 1.10-1.31, P=0.023) exhibited increased hypertension risk after adjusting for age, gender, educational level, and other potential confounders. Subgroup analyses based on gender and age at baseline yielded results consistent with the main analysis. Conclusions:The TyG index is positively correlated with the risk of developing hypertension in the rural adult population.