Pulmonary hypertension has a high mortality rate,and although targeted therapy is available,it is still incurable,and the long-term prognosis for patients is poor.As a tyrosine kinase inhibitor,imatinib was approved for marketing in China in 2002 for the treatment of chronic myelogenous leukemia and other tumor diseases.In addition to the antitumor effects,imatinib was found to improve hemodynamics and exercise tolerance in patients with severe pulmonary arterial hypertension,but the safety was suboptimal.With the emergence of new formulations of imatinib targeted at the lungs,it is expected to become a new targeted drug for pulmonary arterial hypertension.

School-age children are in a specific development stage corresponding to juvenility, when the white matter of the brain experiences ongoing maturation. Diffusion-weighted magnetic resonance imaging (DWI), especially diffusion tensor imaging (DTI), is extensively used to characterize the maturation by assessing white matter properties in vivo. In the analysis of DWI data, spatial normalization is crucial for conducting inter-subject analyses or linking the individual space with the reference space. Using tensor-based registration with an appropriate diffusion tensor template presents high accuracy regarding spatial normalization. However, there is a lack of a standardized diffusion tensor template dedicated to school-age children with ongoing brain development. Here, we established the school-age children diffusion tensor (SACT) template by optimizing tensor reorientation on high-quality DTI data from a large sample of cognitively normal participants aged 6-12 years. With an age-balanced design, the SACT template represented the entire age range well by showing high similarity to the age-specific templates. Compared with the tensor template of adults, the SACT template revealed significantly higher spatial normalization accuracy and inter-subject coherence upon evaluation of subjects in two different datasets of school-age children. A practical application regarding the age associations with the normalized DTI-derived data was conducted to further compare the SACT template and the adult template. Although similar spatial patterns were found, the SACT template showed significant effects on the distributions of the statistical results, which may be related to the performance of spatial normalization. Looking forward, the SACT template could contribute to future studies of white matter development in both healthy and clinical populations. The SACT template is publicly available now ( https://figshare.com/articles/dataset/SACT_template/14071283 ).

ObjectiveTo investigate the value of remote consultation of heart sound acquisition in screening and referral of neonates with congenital heart diseases （CHD） in primary hospitals. MethodsA total of 4 030 neonates with non-critical diseases were selected. They were born in Shanghai Pudong New Area Maternal and Child Health Hospital from November 5， 2019 to March 31， 2021. After birth， routine cardiac auscultation was performed and remote consultation of heart sound collection were performed at the same time in combination with percutaneous oxygen saturation measurement to screen CHD. The children with any positive screening index were advised to verify the diagnosis by cardiac ultrasound examination in Shanghai Children's Medical Center. The diagnostic value of different screening methods was compared. ResultsA total of 110 cases were detected positive by routine screening. Among them， 16 cases were lost to follow-up， and 46 cases were confirmed by cardiac ultrasound examination， with a positive diagnosis rate of 48.94% （46/94）. A total of 51 cases were detected positive by routine screening and remote consultation of heart sound collection simultaneously. Among them， 42 cases were confirmed by cardiac ultrasound examination， with a positive diagnosis rate of 82.35% （42/51）. The difference between the two positive diagnosis rates was statistically significant （P<0.001）. ConclusionRemote consultation of heart sound acquisition on the basis of routine neonatal CHD screening can effectively improve the positive diagnosis rate of CHD screening in primary hospitals， and reduce unnecessary referrals. This method is simple and feasible. It has practical value in primary hospitals that lack professional technicians for the diagnosis and treatment of CHD.

Objective To understand the level of quality control in medical linear accelerator in Guangxi, and to provide technical support for the health administrative department to make related standards. Methods To select medical linear accelerators in Guangxi by random sampling method. Results A total of 118 accelerators were checked in 2017—2019, the initial inspection pass rates of medical linear accelerators were 56.8% and re-inspect pass rate were 96.6%. Conclusion The quality control level of the radiology institutions needs to be improved. The health administrative department should strengthen routine management，it is necessary to implement quality control in medical linear accelerator.

Objective To evaluate the fatty infiltration of lower limbs (pelvis, thighs and calf) by using iterative decomposition of water and fat with asymmetry and least squares estimation‐quantitative fat imaging (IDEAL‐IQ) in Duchenne muscular dystrophy (DMD) patients, and correlate muscle fat fraction(FF) with clinical assessments. Methods The prospective study method was applied, twenty‐one patients with DMD were diagnosed by genetic analysis and/or muscle biopsy and twenty‐one age‐matched healthy control subjects, were evaluated using IDEAL‐IQ, calculating fat fraction (FF) of muscles in the lower limbs. The clinical data of patients, such as age, height, weight, course of disease, time to walk 10 m, Medical Research Council Scale score, were collected. The age and height between DMD group and control group were compared by independent sample t test. The weight, BMI and FF values between two groups were compared by independent samples nonparametric tests (Mann‐Whitney U tests). K related samples nonparametric test (Friedman test) was used for comparison of FF values between pelvic muscles, thighs muscles and calf muscles. The relationships between FF values and clinical assessments were investigated using Spearman correlation test. Results The mean FF of pelvic muscles [13.1(6.9, 33.8)] was significantly higher than that of the thighs muscles [8.3 (23.4, 4.5)] and calf muscles [8.3 (23.4, 4.5)] (all P<0.05) in DMD group. The FF of all the pelvic muscles in DMD were significantly higher than that of the control group muscles (all P<0.05). The gluteus maximus had the greatest mean FF values [34.7 (26.4, 63.7)] in pelvic muscles. The FF of all the thigh muscles in DMD were significantly higher than that of the control group muscles, except for the sartorius, adductor longus and gracilis (Z=0.69, 1.67 and 1.22, respectively, P=0.489, 0.094 and 0.222, respectively). The adductor magnus had the greatest mean FF values [27.2 (12.4, 58.5)] in thigh muscles. The FF of all the calf muscles in DMD were significantly higher than that of the control group muscles, except for the tibialis anterior and tibialis posterior (Z value was1.77 and 1.85, respectively, P value was 0.076 and 0.064, respectively). The gastrocnemius lateral had the greatest mean FF values [7.4 (4.6, 20.9)] in calf muscles. The mean FF value of pelvic muscles (r=0.679, 0.704,?0.761, 0.755, respectively, all P<0.001), thighs muscles (r=0.801, 0.813,?0.738, 0.706, respectively, all P<0.001) and calf muscles (r=0.502, 0.682,?0.637, 0.664, respectively, all P<0.05) were significantly correlate with age, course of disease, %MRC and time to walk 10 m. Conclusions The fat infiltration of lower limbs in DMD patients showes a distinct involvement pattern. IDEAL‐IQ technique is able to quantitatively and objectively evaluate the degree of fat infiltration of lower limbs in DMD, and it has great value in clinical application.

Objective To compare the efficacy of CT-guided radiofrequency thermocoagulation (RFTC) treatment through the inside and outside of the foramen rotundum for primary trigeminal maxillary neuralgia.Methods Forty-eight patients of both sexes,aged 58-75 yr,suffering from primary trigeminal neuralgia the 2nd trigeminal branch pain,scheduled for elective CT-guided trigeminal RFTC,were divided into 2 groups (n =24 each) using a random number table:inside of foramen rotundum group and outside of foramen rotundum group.The needle was inserted until the lateral 1/3 of the foramen rotundum was reached under CT guidance in inside of foramen rotundum group or until the outside of the foramen rotundum was reached in outside of foramen rotundum group.High-temperature RFTC 95 ℃ was performed for 120 s,repeating for 2-3 cycles.The current sensory threshold at different frequencies was measured at 1 day before and after operation.Numeric rating scale score was performed at 1 day,30 days,1 yr and 2 yr after operation,effective RFTC treatment was defined as numeric rating scale score≤ 1,and the condition of postoperative effective RFTC treatment was recorded.The development of adverse reactions such as facial hematoma,corneal ulcer,injuring the other branch and cerebrovascular accidents was recorded.Results Compared with outside of foramen rotundum group,the current sensory threshold on the affected side at frequency of 2 000 Hz was significantly increased at 1 day after operation,the rate of effective RFTC treatment was increased 2 yr after operation (P＜0.05),and no significant change was found in the incidence of postoperative facial hematoma in inside of foramen rotundum group (P＞0.05).Conclusion The long-term curative effect of CT-guided RFTC treatment for primary trigeminal maxillary neuralgia through the inside of the foramen rotundum is better than that through the outside of foramen rotundum.

Objective To explore the application effects of quality control circle (QCC) activity in reducing the peripherally inserted central venous catheters (PICC) maintenance missing rate of discharged tumor patients. Methods Firstly, QCC method was applied to set up the activity topic and carry out investigation of the status quo. Secondly, the targets were set up by analyzing the reasons for the missing of PICC maintenance of the discharged tumor patients. The appropriate measures were established and implemented by strengthening the maintenance point nurse PICC training, expanding the province's PICC maintenance network, enhancing patients' awareness for the importance of PICC maintenance, and the effects were evaluated in the end. Results The missing rate of the discharged tumor patients decreased from 40.00% to 10.92%. The target yield rate was 112.19% and the progressive rate was 78.70%. The number of the PICC maintenance network stations in the whole province increased from 21 to 96, and was constantly increasing. The pass rate of the maintenance increased from 76.02% to 96.00%. Patients and their families recognized these advances. Conclusions The QCC activity not only reduces the missing rate of PICC maintenance of the discharged tumor patients, enhances the percentage of the pass rate the maintenance, but also improves the QCC members' problem solving ability, sense of responsibility, cohesion and other comprehensive qualities.

OBJECTIVETo explore the correlation between cytogenetic findings and clinical manifestations of Turner syndrome.

METHODS607 cases of cytogenetically diagnosed Turner syndrome, including those with a major manifestation of Turner syndrome, were analyzed with conventional G-banding. Correlation between the karyotypes and clinical features were analyzed.

RESULTSAmong the 607 cases, there were 154 cases with monosomy X (25.37%). Mosaicism monosomy X was found in 240 patients (39.54%), which included 194 (80.83%) with a low proportion of 45,X (3 ≤ the number of 45, X ≤5, while the normal cells ≥ 30). Structural X chromosome abnormalities were found in 173 patients (28.50%). A supernumerary marker chromosome was found in 40 cases (6.59%). Most patients with typical manifestations of Turner syndrome were under 11 years of age and whose karyotypes were mainly 45,X. The karyotype of patients between 11 and 18 years old was mainly 45,X, 46,X,i(X)(q10) and mos45,X/46,X,i(X)(q10), which all had primary amenorrhea in addition to the typical clinical manifestations. The karyotype of patients over 18 years of age were mainly mosaicism with a low proportion of 45,X, whom all had primary infertility. 53 patients had a history of pregnancy, which included 48 with non-structural abnormalities of X chromosome and 5 with abnormal structure of X chromosome.

CONCLUSIONGenerally, the higher proportion of cells with an abnormal karyotype, the more severe were the clinical symptoms and the earlier clinical recognition. Karyotyping analysis can provide guidance for the early diagnosis of Turner syndrome, especially those with a low proportion of 45,X.

Abortion, Spontaneous ; genetics ; Adolescent ; Adult ; Amenorrhea ; genetics ; Child ; Child, Preschool ; Chromosomes, Human, X ; genetics ; Cytogenetic Analysis ; methods ; Female ; Humans ; Infant ; Infant, Newborn ; Karyotyping ; Middle Aged ; Mosaicism ; Pregnancy ; Sex Chromosome Aberrations ; Turner Syndrome ; genetics ; pathology ; Young Adult

Objective To evaluate the effects of oral motor interventions on feeding performance of preterm infants with feeding and swallowing disorders. Methods According to the bed number, the premature infants were divided into two groups in equal number: the control group received non-nutritive sucking for 30 minutes before feeding , while the experiment group infants received oral motor interventions. The proficiency and volume transfer were observed and recorded. Result The proficiency and volume transfered in the experiment group were significantly better than those of the control group (P<0.05). Conclusion The oral motor interventions can effectively improve the feeding performance in preterm infants with feeding and swallowing disorders.

Objective To investigate radioactivity levels in the main agriculture products around a uranium mine in Northern Guangxi.Methods The agriculture products and soil samples were collected and analyzed by using HPGe gamma ray spectrometer.Results The specific activity of 226Ra in radish (including leaf),radish leaves and radish,collected in one place,were 45.0,66.7 and 32.3 Bq/kg,respectively.Those of 226Ra and 23SU in the radish soil collected in the same place were 19 672 and 85 917 Bq/kg,respectively.The transfer coefficients of soil-to-radish and soil-to-leaves were 1.61 × 10-3 and 3.40 × 10-3,consistent with those reported in relevant literature.Radioactivity levels in agricultural products in another survey was in consistence with those in the national survey for food products.Radioactivity levels in soil elsewhere near the radish site was consistent with the results of the national soil radioactivive background survey.Conclusions The soil in this place has been contaminated by the nearby uranium mine.It is important to investigate this place further and take the necessary measures.