Zhonghua yi xue za zhi最新文献

Objective: To analyze the imaging characteristics and surgical effect for symmetrical lumbar hemivertebrae in pediatric patients. Methods: The data of 13 patients with hemivertebrae locating in the lumbar spine symmetrically were retrospectively analyzed, and all the patients were treated in Beijing Children's Hospital from January 2015 to September 2021. The mean age of the patients was 6.2 (2.9, 9.3) years. There were 8 males and 5 females. The data of coronal/sagittal plane including segmental Cobb angle, cranial/caudal compensatory curve, thoracic kyphosis, thoracolumbar kyphosis, sacral obliquity, and lumbar lordosis were recorded through long cassette spinal radiographs. Associated anomalies and the relationship between hemivertebrae and posterior component were recorded through computerized tomography (CT) and magnetic resonance imaging (MRI). All the patients received surgery, and their pre-and postoperative imaging data were compared. Results: A total of 26 hemivertebraes were found, in which 80.8% (21/26) located below L₂. Hemivertebraes in 10 patients were separated by a mean 1-2 normal vertebrae. Most hemivertebraes along with the corresponding posterior component were unison (21/26, 80.8%). The Cobb angles of cranial compensatory curve (13.9°±7.2°) was more serious than that of caudal compensatory curve (5.5°±5.0°)(P=0.04). The lumbar lordosis and thoracic kyphosis was 20.2°±15.0° and 18.7°±9.2°, respectively. Six patients complicated with sacral obliquity, while 7 patients complicated with thoracolumbar lordosis. Associated anomalies were found in 6 (46.2%) patients through CT and MRI. Eleven patients received one-or two-stage posterior hemivertebrae resection with short segmental fusion, and 2 patients received one-stage hemivertebrae resection with long segmental fusion. All the surgery were completed successfully without serious complications such as nerve injury, infection, and implant failure. The mean follow-up period was (42.4±10.2) months. At the last follow-up point, the correction rate of segmental Cobb angle and cranial compensatory curve was 83.3%±15.6% and 38.1%±10.4%, respectively, showing significant improvement (P<0.05). Although the caudal compensatory curve, sacral obliquity, and thoracic kyphosis improved after surgery, the data showed no significant difference compared to that before surgery. Thoracolumbar lordosis in all patients were corrected. Conclusions: Most hemivertebraes in such spinal deformity locate in lower lumbar region with a high incidence of anomalies. Individualized treatment based on patients' condition is essential for the complicated spinal deformity.

Objective: To summarize the long-term efficacy of Beijing Children's Hospital-2009-lymphoblastic lymphoma (BCH-2009-LBL) in the treatment of T-lymphoblastic lymphoma (T-LBL) in children and adolescents and to explore the prognostic factors. Methods: T-LBL children admitted to Beijing Children's Hospital Affiliated to Capital Medical University from January 2009 to April 2017 were retrospectively included. According to clinical stage, prognostic genes and treatment response, the children were divided into low, intermediate and high risk groups, and stratified treatment was performed according to the BCH-2009-LBL protocol, with follow-up until December 31, 2023. The clinical characteristics and therapeutic effect of each group were compared. Survival curve was drawn by Kaplan-Meier method, and the difference in survival rate between groups was compared by log-rank test. Multivariate Cox regression model was used to analyze the prognostic factors. Results: A total of 146 patients were included, the age of disease onset [M(Q₁, Q₃)] was 8.0 (1.5, 14.0) years old. There were 107 (73.3%) males and 39 (26.7%) females. Clinical staging: 1 case in stage Ⅰ and 1 case in stage Ⅱ (0.7% each), 41 cases (28.1%) cases in stage Ⅲ and 103 cases(70.5%) in stage Ⅳ. There were 1 case (0.7%), 93 cases (63.7%), and 52 cases (35.6%) in the low, intermediate, and high-risk groups, respectively. The follow-up time was 121 (80, 180) months, and the 5-year and 10-year event-free survival (EFS) rates were 76.4% and 75.0%, respectively. The 5-year EFS rates of low, intermediate and high risk groups were 100.0%, 81.3% and 67.3%, respectively. There was significant difference in remission between the middle-risk group and the high-risk group on the 8th day of hormone pretreatment and at the end of induction (both P<0.05). Recurrence/progression occurred in 29 cases (recurrence rate 19.9%), and the recurrence time was 15 (3, 74) months, in which 26 cases died and only 3 cases survived. Infection-related death occurred in 6 cases (4.1%). The failure or progression of hormone pretreatment at d8 (HR=10.089, 95%CI: 1.266-80.387, P=0.029) and the failure to achieve complete remission at the end of induction (mid-term evaluation) (HR=7.638, 95%CI: 2.411-24.199, P=0.001) were the risk factors for EFS rate of intermediate risk group. The above indexes had no statistical significance on EFS rate in high-risk groups (all P>0.05). Conclusions: BCH-2009-LBL regimen shows good efficacy in the treatment of pediatric T-LBL. The failure or progression of hormone pretreatment at d8 and the failure to achieve complete remission at the end of induction (mid-term evaluation) were the risk factors for EFS rate.

Objective: To investigate the current status and challenges of carrying out the four objective indicators which are necessary for the Sjögren's syndrome (SS) diagnosis in hospitals all over China. Methods: A questionnaire survey was conducted online by Questionstar from May to July 2023 among rheumatologists nationwide, to investigate whether unstimulated salivary flow (UWSF), Van Bijsterveld score (VBS), Schirmer test and labial gland focus score (FS) are carried out in their hospitals and the challenges that hinder their development. A cohort of patients with established SS was enrolled to verify the importance of the four objective indicators in diagnosing SS. Statistical analyses were performed using the chi-square test. Results: The questionnaire was completed by rheumatologists from 660 hospitals in 225 cities of 32 provinces, autonomous regions and municipalities all over China (one doctor from each hospital completed the questionnaire), of which 548 (83.0%) were tertiary care hospitals. The rate of carrying out the objective indicators in 660 hospitals was low: UWSF (290/660, 43.9%), FS (497/660, 75.3%) and VBS (393/660, 59.5%). The percentage of hospitals who consider it difficult to carry out UWSF, VBS, minor labial gland biopsy and Schirmer test was 92.6%(611/660), 69.4%(458/660), 59.8%(395/660) and 58.6%(387/660), respectively. All four objective indicators mentioned above could be carried out in only 139 (21.1%) hospitals. In 521 hospitals in which less than four objective indicators could be carried out, 23.2% (121/521) of rheumatologists selected clinical experience to diagnose SS. A total of 180 patients with SS diagnosed by perfecting all objective indices and meeting the 2016 the American College of Rheumatology/European League Against Rheumatism (ACR/EULAR) criteria were included, 173 females (96%), aged (46.6±13.6) years, with the missed diagnosis rate was 17.8% (32/180) assuming their labial FS was unavailable. In 166 patients with established SS who met the classic 2002 AECG criteria, 160 females (96%), aged (47.0±13.6) years, the missed diagnosis rate was 52.4% (87/166) assuming their labial FS was unavailable; or 10.8% (18/166) assuming their UWSF was unavailable. SS diagnosis couldn't be estimated according to 2002 AECG criteria, assuming both labial FS and UWSF were unavailable in 156 (94.0%) patients with positive anti-SSA/Ro; or assuming either labial FS or UWSF was unavailable in 10 (6.0%) patients with negative anti-SSA/Ro. Conclusion: The application rates of four objective indicators necessary for SS diagnosis are low, the rate of carrying out labial gland biopsy should be increased, and the labial FS reports and UWSF test should be standardized.

Objective: To analyze the consistency of the anesthesia index (AI) and Narcotrend index (NI) in monitoring the depth of anesthesia in pediatric tonsil/adenoidectomy. Methods: A total of 129 children who underwent elective tonsil/adenoidectomy at Xijing Hospital Affiliated to Air Force Medical University from July 2022 to October 2023 were prospectively enrolled. Both AI monitoring by ConView monitor and NI monitoring by Narcotrend monitor were conducted for children after they were admitted to the operating room, while Propofol-remifentanil combined with total intravenous anesthesia was used during the operation and the anesthetic dose was adjusted to maintain the NI value at 40-60. In addition, the AI and NI values were recorded at following time points: quiet eyes closing (T₁), the beginning of induction (T₂), loss of consciousness (T₃), tracheal intubation (T₄), the beginning of surgery (T₅), during tonsil excision (T₆), adenoidectomy (T₇), the end of surgery (T₈), consciousness recovery (T₉) and tracheal extubation (T₁₀). Bland-Altman consistency analysis was used to evaluate the consistency of the results of the two types of anesthesia depth monitoring, AI value and NI value. Receiver operator characteristic (ROC) curve and area under curve (AUC) were used to analyze the predictive efficacy of AI and NI values for the state of consciousness of children. The intraoperative awareness was followed up at 24 hours, 1 week and 1 month after surgery. Results: In 129 cases, there were 78 boys and 51 girls, with an average age of (7.2±3.2) years and an operation time of (72.8±14.0) min. No AI data were missing, but 13 NI data were missing, with a missing rate of 10.1% (13/129). At time points T₁-T₁₀, the AI values were 67.5±19.2 and the NI values were 67.2±19.2. Bland-Altman consistency analysis showed that the difference in value between AI and NI was 0.3 (95%CI:-0.1-0.6, P=0.120), with an out-of-consistency range of 4.0% (51/1 290). Before anesthesia induction, AI and NI values were maintained at a high level; with the beginning of anesthesia induction and the disappearance of the children's consciousness, both AI and NI values gradually decreased; after discontinuing the infusion of anesthetics, the values of AI and NI gradually increased, and the change trend of AI and NI in each stage of the perioperative period was consistent, and there was no significant difference between the two groups at each time point (all P>0.05). ROC curve analysis showed that the cut-off value for predicting the state of consciousness using AI was 72.5, with an AUC of 0.73 (95%CI: 0.70-0.75), and the cut-off value of NI was 79.5 and the AUC was 0.74 (95%CI: 0.72-0.77). There was no significant difference in AUC between the two indices (P=0.310). None of the children had i

The clinical and neuroimaging findings of Alzheimer's disease (AD) are significantly heterogeneous. There are uncertaintie in the current widely available clinical and imaging diagnosis of AD. In the era of amyloid-targeted monoclonal antibody therapy, accurate diagnosis of Alzheimer's disease is urgently needed. Positron emission tomography (PET) and cerebrospinal fluid (CSF) examination can effectively reflect the β-amyloid and neurofibrillary tangles pathology in AD, thus effectively improving the accuracy of AD diagnosis. Understanding the value of various CSF and PET biomarkers in diagnosing/staging AD is a prerequisite for clinical application of biomarkers. The selection and interpretation of biomarkers should fully consider the clinical features and structural imaging of dementia patients, while paying attention to the degree of concordance between the clinical stages and the biological stages reflected by the biomarkers. Thus, biomarkers are expected to play more additional value in AD diagnosis and treatment.

Objective: To investigate long-term health-related quality of life (HRQoL) and related factors in children with severe hemophilia A (HA) who received regular low-dose prophylaxis. Methods: Clinical data of severe HA children who began to receive regular low-dose coagulation factor Ⅷ (FⅧ) prophylaxis in Peking Union Medical College Hospital from January 1, 2008 to December 31, 2011 were retrospectively enrolled. The longest last follow-up period was May 31, 2023. The attendance of school or work and daily physical activity during the last follow-up were investigated. The patients were divided into full attendance group and incomplete attendence group according to attendance. The patients were divided into into exercise attainment group (reached Chinese sports recommendation) and exercise nonattainment group according to the exercise status. Barthel score was used to assess activities of daily living and Haemo-QoL was used to assess quality of life. Long-term HRQoL for children aged 8-16 years and patients aged 17 years and above were assessed using Haemo-QoL SF and Haem-A-QoL versions, respectively. Spearman correlation analysis was used to examine the correlation between treatment conditions and Haemo-QoL scores. Results: A total of 22 cases were enrolled, the prophylaxis initiation age ranged from 1.8-17.9 (10.4±3.8) years old. The average prophylactic FⅧ dose during low-dose prophylaxis was 24.2 U/kg per week and the follow-up time was 6.3-15.1 (9.6±2.8) years. At the last follow-up, the age of the patients was (20.2±5.4) years, of which 14 (63.6%) were adults over 18 years old. There were 15 patients in the full attendance group and 7 patients in the incomplete attendence group. Compared with the full attendance group, the incomplete attendence group had a smaller preventive treatment dose [M(Q_1, Q₃), (28.4±11.1) vs (15.3±3.7) U/kg, P=0.012], shorter preventive treatment time [148. 1 (18.6, 346.5) vs 48.0 (32.0, 156.9) weeks, P=0.017], and higher annual joint bleeding rate (AJBR) [12.5 (6.0, 22.3) vs 14.2 (13.2, 17.8) times, P=0.017]. There were 7 cases in the exercise attainment group and 15 cases in the exercise nonattainment group. Compared to the exercise attainment group, the exercise nonattainment group had shorter preventive treatment time[313. 7 (156.9, 366.0) vs 48.0 (16.5, 108.9) weeks, P=0.006], a higher AJBR [7.0 (5.1, 10.0) vs 23.3 (12.5, 29.8), P=0.003] and a higher hemophilia joint health score (HJHS) [9.0 (2.0, 15.5) vs 23.0 (12.0, 27.8), P=0.014]. Barthel score showed 81.8% (18 cases) of the patients' living ability was not influenced by the illness. In Haemo-QoL score, the total score of Haemo-QoL SF in 7 cases was (47.6±17.0) scores, the total score of Haem-A-QoL in 15 cases was (45.2±22.6) scores. The daily activity dimension of the Haem-A-QoL score was the lowest [38.2 (10.9, 45.5) scores], which was positively c

The incidence of perioperative adverse events in children aged 0 to 15 years was 5.2%. Preoperative scientific and accurate anesthesia assessment is a crucial step in ensuring the safety of pediatric surgery. Perioperative risk prediction is a digital quantitative evaluation of the level of perioperative risk, which classifies the degree of danger. In order to further standardize the methods of anesthesia assessment and identify risk factors, Chinese Society of Anesthesiology organized experts in anesthesiology from children's specialty hospitals and general hospitals, along with statisticians from public health colleges, to jointly draft the "Chinese expert consensus on pediatric anesthesia assessment and perioperative risk prediction (2024 edition)".The anesthesia assessment includes history collection, physical examination, laboratory examination, American Society of Anesthesiologists physical status, difficult airway assessment, and identification and assessment of critically sick children. Perioperative risk prediction includes preoperative anxiety, perioperative respiratory adverse events, regurgitation and aspiration, emergence delirium, postoperative nausea and vomiting, postoperative ICU admission, postoperative acute kidney injury, perioperative mortality, and risk prediction for in-hospital mortality in children with congenital heart disease undergoing non-cardiac surgery.This consensus has formulated a total of 16 recommendations, aiming to promote anesthesiologists' familiarity with the content of pediatric anesthesia assessment, identify risk factors for adverse events during the perioperative period, and take targeted measures to reduce the occurrence of adverse events and improve the safety of children during the perioperative period.

Objective: To explore the correlation between serum free triiodothyronine (FT3) and C-peptide-based insulin resistance index (HOMA2 IR-CP) in euthyroid adults. Methods: A cross-sectional study. The clinical data of euthyroid adult participants who underwent physical examination in the Second Medical Center of Chinese PLA General Hospital from January to December in 2019 were retrospectively analyzed. According to the HOMA2 IR-CP level, the participants were divided into HOMA2 IR-CP>2.18 group (n=3 463) and HOMA2 IR-CP≤2.18 group (n=8 204). Univariate Pearson correlation analysis and multivariate logistic regression analysis were used to analyze the correlation between FT3 and HOMA2 IR-CP. The interaction model was used to analyze the interaction between FT3 and related factors, and the dose-response relationship between continuity variable FT3 and HOMA2 IR-CP was explored by using restricted cubic spline plots. Results: A total of 11 667 euthyroid adult participants aged (50.7±10.0)years were recruited according to the inclusion and exclusion criteria, with 7 756 males and 3 911 females. The proportion of males, body mass index, systolic blood pressure, glycated hemoglobin A1c, fasting plasma glucose, triglyceride, hemoglobin, alanine aminotransferase and FT3 levels in HOMA2 IR-CP>2.18 group were significantly higher than those in HOMA2 IR-CP≤2.18 group (all P<0.05). The levels of low-density lipoprotein cholesterol, high-density lipoprotein cholesterol, and free thyroxine in HOMA2 IR-CP>2.18 group were lower than those in HOMA2 IR-CP≤2.18 group (all P<0.001). Univariate Pearson correlation analysis showed that FT3 was associated with HOMA2 IR-CP (r=0.21, P<0.001). Multivariate logistic regression analysis suggested an association between FT3 and HOMA2 IR-CP after adjusting for confounding factors(P_{for trend}<0.001). Subgroup analysis showed an association between FT3 and HOMA2 IR-CP in different subgroups of gender, age and glucose metabolism status (P_{for trend}<0.05). Multiplication interaction analysis suggested that there was an interaction between FT3 and age (P_interaction<0.001). Restricted cubic spline model analysis demonstrated that the correlation between FT3 and HOMA2 IR-CP was linear (P_overall<0.001, P_nonlinear=0.479). Conclusions: There is a correlation between serum FT3 and HOMA2 IR-CP in euthyroid adults. With the increase of FT3 level, insulin resistance increases gradually.

Birth defect and perinatal death are major public issues threatening the health of women and children in China. However, perinatal death attributed to birth defects has not yet received sufficient attention. To minimize the occurrence of perinatal death caused by birth defects, this review article deeply analyzed the current status of epidemiology, clinical, and basic research on perinatal death attributed to birth defects both domestically and internationally, and proposed to encourage the conduct of national research on perinatal causes. We should also pay attention to the application of the perinatal cause of death classification system, and focus on accurate diagnosis and the three-level prevention and control of perinatal death attributed to birth defect.

Evaluation of neobladder function in patients with long-term survival and no recurrence after laparoscopic radical cystectomy and intracorporeal Xing's neobladder. The clinical data of laparoscopic radical cystectomy and intracorporeal Xing's neobladder in long-term survival patients with bladder cancer treated in Beijing Chaoyang Hospital from July 2013 to July 2018 were analyzed retrospectively. All 17 patients underwent the surgery by the same surgical team, including 15 males and 2 females, whose mean age at the time of operation was (55.9±7.6) years. Thepostoperative urinary function and renal function were summarized. All operations were successfully completed. The mean operative time was (340±62) min. All patients were followed up for a long time, with a median follow-up time of 80(70, 96) months, Urinary continence was achieved in 17 (100%)casesduring the day and 13 (76.5%) cases at night, with a median bladder volume of 350 (200, 400) ml. All patients had good urinary control after surgery, and no hydronephrosis or creatinine increase was found in reexamination.After the application of Xing's neobladder operation, the patient maintained acceptable urinary control status after the operation, and the long-term follow-up effect was satisfactory.