Two pediatric heart failure patients were treated with pulmonary artery banding(PAB)at Fuwai Hospital,from December 2021 to January 2022.In the first case,an 8-month-old patient presented with left ventricular non-compaction cardiomyopathy(LVNC),left ventricular systolic dysfunction,ventricular septal defect,and atrial septal defect.The second case was a 4-month-old patient with LVNC,left ventricular systolic dysfunction,and coarctation of the aorta.After PAB,the left ventricular function and shape of both patients were significantly improved,without serious surgery-related complications.In these individual cases of pediatric heart failure,pulmonary artery banding exhibited a more satisfactory efficacy and safety compared to pharmacological treatment,especially for those with unsatisfactory medication results.Future clinical data are needed to promote the rational and broader application of this therapeutic option for indicated patients.

OBJECTIVE To mine the focus and emotional attitude of the public on rare diseases， and to provide decision- making reference for relevant departments to formulate and implement relevant policies， systems and strategies for medical security of rare diseases. METHODS Latent Dirichlet allocation （LDA） topic model and sentiment analysis method were used to analyze the comment text of short videos related to the “nosinasine” medical insurance admission event on the Bilibili video website， mine the theme and sentiment tendency of the text， and put forward relevant strategy suggestions. RESULTS A total of 8 videos with tens of thousands of playback and 7 109 text data were obtained. According to the LDA analysis， online public paid attention to 9 topics related to rare diseases （the price of rare disease drugs， the inclusion of rare disease drugs in medical insurance， the status quo of research and development of rare disease drugs， the scope of medical insurance and the medical security system， the plight of rare disease patients， the prevention and screening of rare diseases， the value of rare disease drugs in medical insurance， the Chinese and western medical methods of rare diseases， and the supply and demand status of rare disease drugs）， which could be summarized into 4 categories according to the objects of concern （rare disease drugs， rare disease medical insurance， rare disease medical and health services and rare disease patient groups）. On the whole， the emotional tendency of the Internet public towards each topic showed a low positive and high negative tendency. CONCLUSIONS The public paid more attention to the price， research and development， supply and demand of rare disease drugs， and was deeply worried about the current medical status of rare diseases in China. To strengthen and improve the medical security for patients with rare diseases， such as actively carrying out public health services for rare diseases， strengthening cooperation in the diagnosis and treatment of rare diseases， and researching rare disease drugs， etc. Chinese medical and health departments can make concerted efforts in medical and health services， drug supply security， medical security and other social security.

Objective:To investigate the influential factors of adverse pregnancy outcomes in women with subchorionic hematoma.Methods:A total of 101 women with subchorionic hematoma who received treatment in the People's Hospital of Xinjiang Uygur Autonomous Region from January 2017 to June 2020 were included in this study. They were divided into a control group and an adverse pregnancy outcome group according to whether there was an adverse pregnancy outcome. The epidemiological characteristics, hematoma characteristics observed on ultrasound images, and pregnancy outcomes were compared between the two groups.Results:There was no significant difference in the number of women who used assisted reproductive technology between the control and adverse pregnancy outcome groups [6 (8.0%) vs. 8 (30.7%), χ2 = 8.38, P = 0.004]. There was a significant difference in hematoma volume between adverse pregnancy outcome and control groups [(4.12 ± 0.61) mL vs. (6.36 ± 0.87) mL, t = 6.73, P = 0.009]. There was a significant difference in the number of patients who had obstetric complications between control and adverse pregnancy outcome groups [11 (14.7%) vs. 16 (61.5%), χ2 = 21.66, P = 0.001]. There was a significant difference in the number of patients who had hematomas located at the edge of the placenta between the control and adverse pregnancy outcome groups [15 (20.0%) vs. 12 (46.2%), χ2 = -4.81, P = 0.001]. Conclusion:Women who use assisted reproductive technology for pregnancy, have obstetric complications, or have a subchorionic hematoma with hematoma at the edge of the placenta are more likely to experience a miscarriage. Therefore, women of childbearing age should actively treat the primary disease and be alert to the occurrence of placental abruption.

ObjectiveTo explore the features and assessment for far space neglect in left spatial neglect patients after right brain stroke. MethodsFrom January to October, 2021, 30 left unilateral spatial neglect (USN) patients after right stroke (patients, n = 30) from Beijing Bo'ai Hospital and healthy volunteers matching with gender, age and level of education (controls, n = 30) were evaluated with line cancelation (LC), star cancelation (SC) and line bisection (LB) tests, nearly and far away. The 25 controls were evaluated with LB on the second day. ResultsNo line or star was omissed in the controls. Both the deviation and percentage were more in the patients than in the controls (|t| > 4.319, P < 0.001). Both the deviation and percentage were less different for all the test (|Z| < 1.638, t = -1.282, P > 0.05) between nearly and far away, except the deviation of LB (t = -4.994, P < 0.001). The ICC of test-retest was above 0.462 (P < 0.01). ConclusionRight brain stroke patients with USN may present far spatial neglect, which can be assessed with LB.

OBJECTIVE: To investigate the clinical features of fetuses with Wolf-Hirschhorn syndrome(WHS) and explore the diagnostic methods and prenatal ultrasound characteristics and provide evidence for prenatal genetic counseling. METHODS: We retrospectively analyzed 5 cases of WHS fetuses diagnosed from March 2016 to February 2020, and analyzed the results of chromosomal karyotype analysis and chromosomal microarray analysis (CMA) of the fetuses. RESULTS: Five cases of WHS were detected by CMA, four cases were detected by karyotype analysis. Prenatal ultrasound revealed 4 abnormalities, of which 3 had intrauterine growth restriction, and only 1 had abnormalities of the maxillofacial region. The sequence of the fragments was 4p16.3p16.1 with a loss of 6.5 Mb, 4p16.3p15.32 with a loss of 15.6 Mb combined with 2p25.3 increased by 906kb, 4p16.3p15.31 with a loss of 20.4 Mb, 4p16.p15.1 with a loss of 35 Mb and 4p16.3p14 with a loss of 37 Mb. CONCLUSION Fetal growth restriction may be one of the early manifestations of WHS. Absence of fetal facial abnormality by prenatal ultrasound screening cannot exclude WHS. Karyotype analysis may miss the diagnosis of WHS, while combined CMA techniques can improve the diagnostic accuracy.
Chromosomes, Human, Pair 4/genetics* ; Female ; Fetal Growth Retardation/genetics* ; Humans ; Karyotyping ; Pregnancy ; Prenatal Diagnosis ; Retrospective Studies ; Wolf-Hirschhorn Syndrome/genetics*

Objective:To explore the factors influencing executive functioning after a brain injury and analyze the relationship between executive functioning and other cognitive functions.Methods:Forty-six brain injury survivors were given the Executive Function Performance Task (EFPT) assessment and the Loewenstein Occupational Therapy Cognitive Assessment (LOTCA). The scores and time of the EFPT tasks, and the orientation, perception, visual motor organization, thinking, and attention and concentration results in the LOTCA were observed. Regression analysis compared the males and females, those younger and older than 40, those with more or less than 12 years of education, trauma and stroke survivors, as well as those with left, right and bilateral lesion to isolate the factors most influencing executive functioning.Results:The average EFPT score on the cooking task, the medication score and the total score of the young group were all significantly higher than those of the older group. Their telephone time, medication time and bill payment time were all significantly shorter. Those with more than 12 years of education had average scores on the cooking, bill payment and EFPT significantly higher than those with less than 12 years of education. The average bill paying and total EFPT scores of the brain trauma group were significantly higher than the stroke group′s averages. No significant differences were observed between the different genders or those with different injury sites. Age was the strongest predictor of total EFPT scores. Except for the medication scores, the average scores of the other three tasks and the total score of EFPT were moderately correlated with the visual motor organization, thinking, attention and concentration, and total LOTCA scores, with correlation coefficients ranging from 0.31 to 0.64.Conclusions:Older persons and those will less education tend to have worse executive functioning. Traumatic damage to executive functioning is more serious than that caused by stroke. Executive functioning is closely related to visual-motor organization, and to the ability to think, attend and concentrate.

The original version of this article unfortunately contained some mistakes.

Objective:To test the reliability and validity of the Chinese version of the executive function performance test (EFPT) when it is used with persons with a brain injury.Methods:Forty-six brain injury patients comprised the patient group, and forty-nine healthy counterparts formed the normal group. All completed the EFPT, the Flanker task, a color shape task, the speech and visual space 2-back and the Wisconsin Card Sorting Test (WCST). Thirty normal subjects were asked to complete the EFPT again 4 weeks after the first test. Spearman correlation analysis was used for reliability and validity analysis.Results:The EFPT scale showed good internal consistency, as Cronbach′s α coefficient was 0.73 to 0.83. Test-retest reliability was good, as the internal correlation coefficients ranged from 0.50 to 0.85. The results of the EFPT correlated moderately well ( r=0.29-0.57) with the total number of responses, the number of completed collocations, persistent responses and non-persistent errors, as well as with the percentage of the generalized level of the WCST. Moreover, all of the EFPT results correlated moderately well with those of the IADL except for the task of taking medication. Compared with the control group, the brain-injured group got significantly higher sub-test and total scores on the EFPT. Conclusions:The Chinese version of the EFPT has demonstrated adequate reliability and validity with brain-injured Chinese persons. It is a good tool for evaluating the executive functioning of such persons and can reflect any executive dysfunction in their daily living activities.

Objective:To observe the effect of different temperature rewarming on intraoperative brain protection in children with deep hypothermia circulatory arrest (DHCA).Methods:Totally 24 cases of children undergoing deep hypothermia circulatory arrest under general anesthesia were selected, and randomly divided into 38 ℃ and 43 ℃ groups including 12 cases respectely. Children patients in each group were given forced air rewarming at 38 ℃ and 43 ℃ respectively during the rewarming phase after cross-clamping remission. The age of patients, weight, operation time, intraoperative blood loss, intraoperative fluid infusion and postoperative length of stay were recorded. The arterial blood was taken to determine the serum neuron-specific enolase (NSE) , Platelet activating factor (PAF), and blood lactic acid immediately when the patients were entering into operating room (T 0), before Extracorporeal Circulation (T 1), before aortic cross-clamping (T 2), before DHCA (T 3), rewarming (T 4), temperature to 30 ℃ (T 5), temperature to 33 ℃ (T 6), temperature to 35 ℃ (T 7), CPB was terminated (T 8) and at the end of operation (T 9). And the body temperature were recorded at T 0, T 1, T 3 and T 8. Results:At T 0, T 1, T 3, T 8 and T 9, the temperature difference between the two groups was not statistically significant ( P>0.05). The body temperature first decreased and then increased in the group of 38℃ and 43℃ ( P<0.01). And the serum PAF and NSE significantly increased during the operation ( F values were 5 172.087, 3 535.935, P<0.01). The content of PAF, NSE and lactate in 38 ℃ group and 43 ℃ group increased with time from T 0 to T 9 ( P<0.01).The PAF content of children in the 43℃ group at T 8 and T 9 were (20.34±4.70) μg/L and (25.46±7.75) μg/L respectively, lower than that in the 38℃ group (25.20±3.80) μg/L and (34.97±8.52) μg/L, the differences were statistically significant ( F value was 7.769, P=0.011; F value was 8.193, P=0.009). At T 8 and T 9, NSE content of children in the 43℃ group were (22.13±5.13) μg/L and (28.15±7.90) μg/L respectively, lower than those in the 38℃ group (26.92±3.89) μg/L and (36.57±8.90) μg/L, the differences were statistically significant ( F value was 6.656, P=0.017; F value was 6.012, P=0.023). Blood lactic acid content of children in the 43℃ group at T 4-T 9 respectively (2.77±0.70), (2.93±0.69), (3.13±0.77), (3.39±0.64), (3.77±0.86), (3.83±0.62) mmol/L, lower than 38 ℃ group of children (3.57±0.87), (3.82±0.80), (4.35±0.89), (4.60±0.95), (4.79±0.94), (4.92±0.92) mmol/L, the differences were statistically significant ( F values were 6.125-11.551, P values were 0.022-0.003). Conclusion:Both 38 ℃ and 43 ℃ can ensure that the body temperature of the children increases steadily during the intraoperative rewarming phase in deep hypothermic circulatory arrest operation,but 43℃ can effectively reduce the serum PAF and NSE in children with brain protection, its mechanism may be associated with reduced blood lactic acid.