Congenital orofacial cleft, the most common birth defect in the maxillofacial region, exhibits a wide range of prognosis depending on the severity of deformity and underlying etiology. Non-syndromic congenital orofacial clefts typically present with milder deformities and more favorable treatment outcomes, whereas syndromic congenital orofacial clefts often manifest with concomitant organ abnormalities, which pose greater challenges for treatment and result in poorer prognosis. This consensus provides an elaborate classification system for varying degrees of orofacial clefts along with corresponding diagnostic and therapeutic guidelines. Results serve as a crucial resource for families to navigate prenatal screening results or make informed decisions regarding treatment options while also contributing significantly to preventing serious birth defects within the development of population.
Humans ; Cleft Lip/diagnosis* ; Cleft Palate/diagnosis* ; Consensus ; Prenatal Diagnosis ; Female

Non-ischemic cardiomyopathy(NICM)encompasses heterogeneous subtypes and complex etiology,and sudden cardiac death(SCD)is a particularly severe adverse outcome of NICM.Cardiac magnetic resonance(CMR)has capability of multiparametric micro-tissue characterization,multi-sequence macroscopic morpho-structural imaging and multi-dimensional quantitative analysis of cardiac function,which plays a central role in diagnosis and sub-typing,evaluating risk stratification and assessment of treatment response of NICM.The clinical application status and cutting-edge progresses of CMR in NICM were systematically described in this article.

Objective: To explore the association between physical activity and sleep quality among middle school students, so as to provide reference for adolescent sleep improvement. Methods: From September to December 2023, 5 713 middle school students aged 13-18 years were selected from Shanghai, Suzhou, Taiyuan, Wuyuan, Xingyi and Urumqi by stratified cluster random sampling method. Pittsburgh Sleep Quality Index (PSQI) and Evaluation of Physical Activity Levels of Children and Adolescents Aged 7-18 Years were used to investigate and evaluate sleep quality and physical activity. Comparisons between groups were made using the t-test, Mann-Whitney U-test, and associations between physical activity and sleep quality of middle school students were analyzed using Spearman correlation and linear regression methods. Results: The total PSQI scores were 4.0(2.0,6.0) and 5.0 (3.0,6.0) for boys and girls, respectively, with significant sex difference ( Z =-10.90, P <0.01); light physical activity(LPA) and moderate to vigorous physical activity(MVPA) of boys were 18.57 (2.86, 42.86) and 68.57 (35.71, 119.18)min, and girls were 14.29 (0.00, 30.00) and 55.71 (31.43, 92.86)min respectively, and the differences were statistically significant ( Z =3.65, -8.65 , P <0.01). The results of Spearman correlation regression showed that adolescents MVPA was negatively correlated with the total PSQI score ( r =-0.04, P <0.01). After controlling for variables such as mental health, nutritional status and maximum oxygen uptake, the results of linear regression analysis showed that PSQI total score negatively predicted MVPA among middle school students ( B =-4.76, 95% CI =-7.16 to -2.36, P <0.05). Conclusion The longer the duration of physical activity among middle school students, the better the quality of sleep.

Non-ischemic cardiomyopathy(NICM)encompasses heterogeneous subtypes and complex etiology,and sudden cardiac death(SCD)is a particularly severe adverse outcome of NICM.Cardiac magnetic resonance(CMR)has capability of multiparametric micro-tissue characterization,multi-sequence macroscopic morpho-structural imaging and multi-dimensional quantitative analysis of cardiac function,which plays a central role in diagnosis and sub-typing,evaluating risk stratification and assessment of treatment response of NICM.The clinical application status and cutting-edge progresses of CMR in NICM were systematically described in this article.

Objective To evaluate the safety and efficacy of the Neuroform EZ self expanding stent for severe symptomatic intracranial atherosclerotic stenosis(sICAS).Methods Retrospectively enrolled consecutive patients with severe sICAS who underwent percutaneous transluminal angioplasty and stenting(PTAS)with a Neuroform EZ stent in the Department of Cerebrovascular Disease,Henan Provincial People's Hospital,from March 2020 to December 2022.Baseline demographic and clinical data were collected,including age,sex,hypertension,diabetes mellitus,coronary artery disease,dyslipidemia,hyperhomocysteinemia,transient ischemic attack(TIA)and ischemic stroke,smoking history,modified Rankin scale(mRS)score at admission,and National Institutes of Health stroke scale(NIHSS)score.Preoperative imaging data included target vessel(basilar artery,intracranial segment of the internal carotid artery,middle cerebral artery,and intracranial vertebral artery),lesion length,degree of stenosis,and vascular morphology according to the Mori classification(type A,lesion length＜5 mm with concentric or moderately eccentric stenosis;type B,lesion length＜10 mm with severely eccentric stenosis;type C,lesion length＞10 mm or arterial angulation＞90°).Technical success was defined as accurate delivery and deployment of the stent with complete coverage of the target lesion and immediate post deployment residual stenosis＜50%.Postoperative head CT was performed to detect intracranial hemorrhage.Periprocedural complications were recorded,including intracranial hemorrhage,arterial dissection,in stent thrombosis,and perforator occlusion occurring intraoperatively within 72 hours after the procedure.At one-month post-operation,patients were seen through outpatient follow-up for TIA,hemorrhagic or ischemic stroke,and all cause death.At 6 months after surgery,DSA or CT angiography(CTA)was performed to assess in stent restenosis(ISR,defined as＞50%stenosis within the stent or within5mm of its edges,or＞20%luminal loss).At 1 and 2 years postoperatively,ipsilateral ischemic stroke or TIA recurrence was assessed by outpatient visit or telephone follow up.Results A total of 76 patients with severe sICAS underwent PTAS with a Neuroform EZ stent(56 males,20 females,age 47-80 years,with a mean age of[61±10]years).(1)Within all patients enrolled,40 had middle cerebral artery,16 with basilar artery,6 with intracranial vertebral artery and 14 with intracranial internal carotid artery.The preprocedural lesion length was 2-15 mm,with a mean length of(6.2±2.5)mm,and stenosis severity was70%-99%,the mean severity was(83.2±6.9)%,with Mori type B being the most common type(57.9%[44/76]).(2)PTAS was successfully completed on all patients(technical success 100%).Pre dilation with a conventional balloon was performed in all cases(using balloon with diameter of 1.5-3.5mm,and stent with diameter of 2.5-4.5 mm and length of 15-30 mm).Immediate post procedural residual stenosis was(17.4±9.0)%,significantly lower than baseline(t=52.9,P＜0.05),with a mean difference of 65.8%(95%CI63.3%-68.3%).(3)Among all 76patients,one patient developed a flow limiting dissection post balloon angioplasty,which recovered after stent deployment.One patient with basilar artery stenosis experienced recurrent ischemic stroke at 5-day postoperatively,presenting with right sided weakness and coughing on liquids.Imaging showed an acute infarct in the left pons,considered perforator occlusion.The overall periprocedural complication rate was 2.6%(2/76).(4)No deaths occurred within 30 days after surgery.Sixty nine patients(90.8%)underwent 6 month imaging follow up with DSA(52 cases)or CTA(17 cases).ISR occurred in 12 patients(17.4%),including 6 asymptomatic and 6symptomatic cases.The ipsilateral ischemic stroke recurrence rate was 6.6%(5/76)at1 year and13.2%(10/76)at2years.Conclusions Neuroform EZstent assisted PTASappears safe and feasible for the treatment of severe sICAS.The long term effectiveness requires confirmation in large,multicenter,prospective studies.

Objective To evaluate the safety and efficacy of the Neuroform EZ self expanding stent for severe symptomatic intracranial atherosclerotic stenosis(sICAS).Methods Retrospectively enrolled consecutive patients with severe sICAS who underwent percutaneous transluminal angioplasty and stenting(PTAS)with a Neuroform EZ stent in the Department of Cerebrovascular Disease,Henan Provincial People's Hospital,from March 2020 to December 2022.Baseline demographic and clinical data were collected,including age,sex,hypertension,diabetes mellitus,coronary artery disease,dyslipidemia,hyperhomocysteinemia,transient ischemic attack(TIA)and ischemic stroke,smoking history,modified Rankin scale(mRS)score at admission,and National Institutes of Health stroke scale(NIHSS)score.Preoperative imaging data included target vessel(basilar artery,intracranial segment of the internal carotid artery,middle cerebral artery,and intracranial vertebral artery),lesion length,degree of stenosis,and vascular morphology according to the Mori classification(type A,lesion length＜5 mm with concentric or moderately eccentric stenosis;type B,lesion length＜10 mm with severely eccentric stenosis;type C,lesion length＞10 mm or arterial angulation＞90°).Technical success was defined as accurate delivery and deployment of the stent with complete coverage of the target lesion and immediate post deployment residual stenosis＜50%.Postoperative head CT was performed to detect intracranial hemorrhage.Periprocedural complications were recorded,including intracranial hemorrhage,arterial dissection,in stent thrombosis,and perforator occlusion occurring intraoperatively within 72 hours after the procedure.At one-month post-operation,patients were seen through outpatient follow-up for TIA,hemorrhagic or ischemic stroke,and all cause death.At 6 months after surgery,DSA or CT angiography(CTA)was performed to assess in stent restenosis(ISR,defined as＞50%stenosis within the stent or within5mm of its edges,or＞20%luminal loss).At 1 and 2 years postoperatively,ipsilateral ischemic stroke or TIA recurrence was assessed by outpatient visit or telephone follow up.Results A total of 76 patients with severe sICAS underwent PTAS with a Neuroform EZ stent(56 males,20 females,age 47-80 years,with a mean age of[61±10]years).(1)Within all patients enrolled,40 had middle cerebral artery,16 with basilar artery,6 with intracranial vertebral artery and 14 with intracranial internal carotid artery.The preprocedural lesion length was 2-15 mm,with a mean length of(6.2±2.5)mm,and stenosis severity was70%-99%,the mean severity was(83.2±6.9)%,with Mori type B being the most common type(57.9%[44/76]).(2)PTAS was successfully completed on all patients(technical success 100%).Pre dilation with a conventional balloon was performed in all cases(using balloon with diameter of 1.5-3.5mm,and stent with diameter of 2.5-4.5 mm and length of 15-30 mm).Immediate post procedural residual stenosis was(17.4±9.0)%,significantly lower than baseline(t=52.9,P＜0.05),with a mean difference of 65.8%(95%CI63.3%-68.3%).(3)Among all 76patients,one patient developed a flow limiting dissection post balloon angioplasty,which recovered after stent deployment.One patient with basilar artery stenosis experienced recurrent ischemic stroke at 5-day postoperatively,presenting with right sided weakness and coughing on liquids.Imaging showed an acute infarct in the left pons,considered perforator occlusion.The overall periprocedural complication rate was 2.6%(2/76).(4)No deaths occurred within 30 days after surgery.Sixty nine patients(90.8%)underwent 6 month imaging follow up with DSA(52 cases)or CTA(17 cases).ISR occurred in 12 patients(17.4%),including 6 asymptomatic and 6symptomatic cases.The ipsilateral ischemic stroke recurrence rate was 6.6%(5/76)at1 year and13.2%(10/76)at2years.Conclusions Neuroform EZstent assisted PTASappears safe and feasible for the treatment of severe sICAS.The long term effectiveness requires confirmation in large,multicenter,prospective studies.

Objective The aim of this study is to explore the practical value of prenatal magnetic resonance imaging(MRI)in the assessment of congenital cystic lung disease in fetuses,to evaluate the relative size of the lesion and the status of lung development,and to make an attempt at utilizing the strength of MRI in post-processing to obtain assessment indicators of the size of the lesion and the status of lung development,with which predictions can be made for the prognosis that these fetuses may face after birth.We retrospectively collected and analyzed the data of fetuses diagnosed with congenital cystic lung disease.Prenatal ultrasound examination of these fetuses led to the diagnosis that they were suspected of having congenital cystic lung disease and the diagnosis was confirmed by subsequent prenatal MRI.The fetuses were followed up to track their condition at birth(postnatal respiratory distress,mechanical ventilation,etc.),whether the fetuses underwent surgical treatment,and the recovery of the fetuses after surgical treatment.The recovery of the fetuses was followed up to explore the feasibility of prenatal MRI examination to assess fetal congenital pulmonary cystic disease,and to preliminarily explore the predictive value of prenatal MRI for the prognosis of fetuses with congenital pulmonary cystic disease.Methods MRI fetal images were collected from pregnant women who attended the West China Second University Hospital of Sichuan University between May 2018 and March 2023 and who were diagnosed with fetal congenital pulmonary cystic disease by prenatal ultrasound and subsequent MRI.Fetal MRI images of congenital cystic lung disease were post-processed to obtain the fetal lung lesion volume,the fetal affected lung volume,the healthy lung volume,and the fetal head circumference measurements.The signal intensity of both lungs and livers,the lesion volume/the affected lung volume,the lesion volume/total lung volume,the cystic volume ratio(CVR),and the bilateral lung-liver signal intensity ratio were measured.The feasibility and value of MRI post-processing acquisition indexes for evaluating the prognosis of fetuses with congenital cystic lung disease were further analyzed by combining the follow-up results obtained 6 months after the birth of the fetus.Logistic regression models were used to quantify the differences in maternal age,gestational week at the time of MRI,CVR,and bilateral lung-to-liver signal intensity ratio,and to assess whether these metrics correlate with poor prognosis.Receiver operating characteristic(ROC)curves were used to assess the value of the parameters obtained by MRI calculations alone and in combination with multiple metrics for predicting poor prognosis after birth.Results We collected a total of 67 cases of fetuses diagnosed with congenital cystic lung disease by fetal MRI between May 2018 and March 2023,and excluded 6 cases with no normal lung tissue in the affected lungs,11 cases of fetal induction,and 3 cases of loss of pregnancy.In the end,47 cases of fetuses with congenital cystic lung disease were included,of which 30 cases had a good prognosis and 17 cases had a poor prognosis.The difference in the difference between the signal intensity ratios of the affected and healthy sides of the lungs and livers of the fetuses in the good prognosis group and that in the poor prognosis group was statistically significant(P<0.05),and the signal intensity ratio of the healthy side of the lungs and livers was higher than the signal intensity ratio of the affected side of the lungs and livers.Further analysis showed that CVR(odds ratio[OR]=1.058,95% confidence interval[CI]:1.014-1.104),and the difference between the lung-to-liver signal intensity ratios of the affected and healthy sides(OR=0.814,95% CI:0.700-0.947)were correlated with poor prognosis of birth in fetuses with congenital cystic lung disease.In addition,ROC curve analysis showed that the combined application of lesion volume/affected lung volume and the observed difference in the signal intensity ratio between the affected and healthy lungs and liver predicted the prognosis of children with congenital cystic lung disease more accurately than the single-parameter judgment did,with the area under the curve being 0.988,and the cut-off value being 0.33,which corresponded to a sensitivity of 100%,a specificity of 93.3%,and a 95% CI of 0.966-1.000.Conclusions Based on the MRI of fetuses with congenital cystic lung disease,we obtained information on lesion volume,lesion volume/affected lung volume,lesion volume/total lung volume,CVR,and bilateral lung-to-liver signal intensity ratio difference,all of which showing some clinical value in predicting the poor prognosis in fetuses with congenital cystic lung disease.Furthermore,among the combined indexes,the lesion volume/affected lung volume and bilateral lung-to-liver signal intensity ratio difference are more effective predictors for the poor prognosis of fetuses with congenital cystic lung disease,and show better efficacy in predicting the poor prognosis of fetuses with congenital cystic lung disease.This provides a new and effective predictive method for further assessment of pulmonary lung development in fetuses with congenital cystic lung disease,and helps improve the assessment and prediction of the prognosis of fetuses with congenital cystic lung disease.

Hypertrophic cardiomyopathy(HCM)is the most common type of primary cardiomyopathy that causes sudden cardiac death in adolescents and athletes.With over 1 million HCM patients,China has the largest population of HCM patients in the world,and the total number of cases is increasing year on year.Myocardial fibrosis is the most important histopathological characterization in HCM and is regarded as the primary cause of malignant ventricular arrhythmia,cardiac remodeling,and heart failure.At present,cardiac magnetic resonance imaging(MRI)serves as the gold-standard imaging modality for noninvasive evaluation of myocardial fibrosis.Several techniques,such as late gadolinium enhancement and T1 mapping,are showing considerable promise for potential applications.These techniques have emerged as viable imaging approaches to the elucidation of HCM tissue characterization.They are also helpful in predicting the long-term prognosis of patients.Herein,we summarized recent advances in using cardiac MRI to assess myocardial fibrosis in HCM from four perspectives,including late gadolinium enhancement,T1 mapping,T1p mapping,and MRI-based radiomics and machine learning models.

Objective: To explore the relationship between cardiorespiratory fitness and executive function in Chinese adolescents, so as to provide a reference for promoting the overall development of Chinese adolescents physical and mental health. Methods: From September to December 2022, a total of 5 018 adolescents aged 13 to 18 years from Shanghai, Suzhou, Taiyuan, Wuyuan, Xingyi, and Urumqi were selected by stratified cluster sampling method to assess cardiorespiratory fitness and executive function. Pearson s correlation and linear hierarchical regression were performed to analyze the relationship between cardiorespiratory fitness and executive functions. Results: Among the sample of adolescents, maximal oxygen consumption (VO 2max ) was negatively correlated with the refreshing(1-back, 2-back) and conversion executive function responses ( r=-0.07, -0.12, -0.12, P <0.01). Linear regression analysis showed that VO 2max was negatively correlated with the reaction times of the refreshing(1-back, 2-back) and conversion functions ( B=-2.99, -6.44, -1.69, P <0.01). Conclusions Higher cardiorespiratory fitness among adolescents is associated with better performance in executive function. Teenagers should strengthen high intensity cardiopulmonary endurance exercise to promote the improvement of executive function.

Objective:To explore the characteristics and changes of cardiac injury with age in Duchenne muscular dystrophy (DMD) and its clinical significance.Methods:A prospective cohort study was conducted. The 215 patients diagnosed with DMD in West China Second Hospital from January 2019 to November 2022 and aged from 6 to 18 years were enrolled. Their clinical data, myocardial injury markers, routine electrocardiogram, cardiac magnetic resonance (CMR) and echocardiography were collected. The patients were divided into five age groups: 6-<8, 8-<10, 10-<12, 12-<14 and 14-18 years of age, and matched with healthy boys respectively. Independent sample t test or Mann-Whitney U test was used to compare the clinical data and CMR indexes between DMD patients and controls in all age subgroups, and to compare the value of left ventricular ejection fraction (LVEF) measured by echocardiography and CMR in each subgroup of DMD patitents. Pearson correlation analysis or Spearman correlation analysis was used to explore the relation between the CMR indexes and age in DMD patients. Results:A total of 215 patients with DMD (all male) and 122 healthy boys were included in the study. There were 75 DMD patients and 23 controls in 6-<8 years of age group, 77 DMD and 28 controls in 8-<10 years of age group, 39 DMD and 23 controls in 10-<12 years of age group, 10 DMD and 31 controls in the 12-<14 years of age group, and 14 DMD and 17 controls in 14-18 years of age group. In the DMD patients, the older the age, the lower the levels of creatine kinase (CK) and creatine kinase isoenzyme (CK-MB). In the 6-<8 years of age group, the CK level was 10 760 (7 800, 15 757) U/L, while in the group of 14-18 years of age, it was 2 369 (1 480, 6 944) U/L. As for CK-MB, it was (189±17) μg/L in the 6-<8 years of age group and (62±16) μg/L in the 14-18 years of age group. Cardiac troponin I remained unchanged in <12 years of age groups, but significantly increased in 12-<14 years of age group, reaching the highest value of 0.112 (0.006, 0.085) μg/L. In the DMD patients, the older the age, the higher the proportion of abnormal electrocardiogram (ECG). In the 6-<8 years of age group, the proportion is 29.3% (22/75), while in the 14-18 years of age group, it was 10/14. Correlation analysis showed that the left ventricular end-diastolic volume index was positively related with age ( r=0.18, P=0.015), and the left ventricular stroke volume index and cardiac output index were negatively related with age ( r=-0.34 and -0.31, respectively, both P<0.001). In the DMD patients, the older the age, the lower LVEF, with the LVEF decreasing to (49.3±3.1)% in the 14-18 years of age group. The LVEF of DMD cases was significantly lower than that of controls in the age subgroups of 8-<10, 10-<12, 12-<14 and 14-18 years of age groups ((57.9±5.2) % vs. (63.6±0.8)%, 60.7% (55.9%, 61.9%) vs. 63.7% (60.2%, 66.0%), 57.1% (51.8%, 63.4%) vs. 62.1 % (59.5%, 64.5)%, (49.3±3.1) % vs. (61.6±1.3)%, respectively; all P<0.01). In the DMD patients, the older the age, the higher the proportion of positive late gadolinium enhancement (LGE). In the 6-<8 years of age group, it was 22% (11/51), in the 12-<14 years of age group, it was 13/14, and in the 14-18 years of age group, all DMD showed positive LGE. The value of LVEF of DMD cases measured by echocardiography was significantly higher than that measured by CMR in 6-<8 years of age group and 8-<10 years of age group (63.2% (60.1%, 66.4%) vs. 59.1 % (55.4%, 62.9%), and (62.8±5.2) % vs. (57.9±5.2)%, all P<0.001). Conclusion:DMD patients develop cardiac injury in the early stage of the disease, and the incidence of cardiac damage gradually increases with both age and the progression of disease.