Ankylosing spondylitis is a chronic immunoinflammatory disease that mainly affects the spine and the sacroiliac joint， the mechanism of which is closely related to signaling pathways， such as osteoprotegerin （OPG）/receptor activator of nuclear factor-κB （RANK）/RANK ligand， mitogen-activated protein kinase （MAPK）， Wnt/β-catenin （β-catenin）， phosphoinositide 3- kinase/protein kinase B/mammalian target of rapamycin （PI3K/Akt/mTOR）. Traditional Chinese medicine has the characteristics of multiple components and targets， and is widely used for the treatment of autoimmune diseases due to its low toxicity， strong specificity， and high efficacy. This review found that monomers and compounds of traditional Chinese medicine can exert anti ankylosing spondylitis effects by intervening in the aforementioned signaling pathways， regulating immune inflammatory responses， and inhibiting biological processes such as bone destruction， ectopic osteogenic differentiation， cell apoptosis， and autophagy.

OBJECTIVES: To explore the efficacy of DSA-guided intrathecal drug delivery system combined with Zi Wu Liu Zhu Acupoint Therapy for management of cancer pain and provide reference for its standardized clinical application. Methods and. RESULTS: Recommendations were formulated based on literature review and expert group discussion, and consensus was reached following expert consultation. The consensus recommendations are comprehensive, covering the entire treatment procedures from preoperative assessment and preparation, surgical operation process, postoperative management and traditional Chinese medicine treatment to individualized treatment planning. The study results showed that the treatment plans combining traditional Chinese with Western medicine effectively alleviated cancer pain, reduced the use of opioid drugs, and significantly improved the quality of life and enhanced immune function of the patients. Postoperative follow-up suggested good treatment tolerance among the patients without serious complications. CONCLUSIONS The formulated consensus is comprehensive and can provide reference for clinicians to use DSA-guided intrathecal drug delivery system combined with Zi Wu Liu Zhu Acupoint Therapy. The combined treatment has a high clinical value with a good safety profile for management of cancer pain.
Humans ; Medicine, Chinese Traditional ; Cancer Pain/therapy* ; Drugs, Chinese Herbal/therapeutic use* ; Drug Delivery Systems ; Pain Management/methods* ; China

Objective:To compare the predictive ability of SpO 2/FiO 2(S/F) and ROX index on the failure of high-flow nasal cannula(HFNC)therapy in children with acute respiratory failure after congenital heart disease surgery, and to identify the best cut-off point. Methods:Through a case-control study, the clinical data of 371 children with acute respiratory failure after congenital heart surgery treated with HFNC admitted to Guangzhou Women and Children′s Medical Center from January 2018 to December 2021 were retrospectively analyzed.The primary outcome was the need for re-intubation within 48 h after extubation of invasive ventilation.The ability of S/F and ROX index to predict HFNC failure was compared, and the optimal cut-off point was determined based on the area under the curve (AUC) of receiver operating characteristic curve.Results:A total of 371 children were included, of whom 27 (7.3%) eventually required mechanical ventilation within 48 h. The S/F prediction accuracy was highest after 6 h of HFNC treatment(AUC=0.712, 95% CI 0.599-0.825, P=0.001), and the best cut-off point for S/F was 178 mmHg(1 mmHg=0.133 kPa)(sensitivity 74.9%, specificity 69.6%). Whereas the prediction accuracy of the ROX index was highest after 12 hours of HFNC treatment, the AUC was 0.737(95% CI 0.623-0.851, P=0.002), and the best cut-off point of the ROX index was 5.865(sensitivity 72.4% specificity 66.7%). The difference in AUC between S/F after 6 h of HFNC treatment and ROX after 12 h was not statistically significant ( P=0.444), with higher sensitivity and specificity, and earlier prediction time(6 hours) in the former. Conclusion:Children with acute respiratory failure after congenital heart surgery have a strong predictive ability of S/F after 6 h of HFNC treatment, and the risk of HFNC treatment failure is higher in children with S/F <178 mmHg.

Objective:To explore the normal ranges of perfusion parameters between cerebral hemisphere, cerebellar hemisphere and brain anatomical subregions (56 pairs) in different gender and age groups with multiple post labeling delay time (Multi-PLD) arterial spin labeling (ASL) imaging.Methods:From November 2020 to December 2020, 42 healthy adult volunteers (Male 25, Female 17) were recruited to perform 7 PLD ASL imaging, including 21 young adults (15 males and 6 females, aged 23—35 years) and 21 seniors (10 males and 11 females, aged 36—74 years). The data was processed offline by Cereflow software to obtain arterial arrival time (ATT) and corrected cerebral blood flow (CBF) and cerebral blood volume (CBV) perfusion parameters. SimpleITK standardization function was used to standardize the calculated perfusion image according to the anatomical automatic labeling (AAL) template. Therefore, CBF, ATT, CBV perfusion values of brain subregions were obtained. Paired samples t test, Wilcoxon rank sum test, independent samples t test and Mann-Whitney U test were used to compare the differences of perfusion parameters in the cerebral hemisphere, the cerebellar hemisphere, brain subregions depending on side, gender and age. Pearson correlation analysis was used to compare the correlations of perfusion parameters with age. Results:CBF in 62.5% (35/56) subregions and CBV in 44.6% (25/56) subregions were higher in right side than those in left side. ATT in most brain anatomical subregions (16/56) were higher in left side. The CBF [(35.30±8.31) vs. (34.34±7.53) ml·100g -1·min -1, P=0.021], CBV [(0.47±0.11) vs. (0.45±0.09) ml/100g, P<0.001], ATT [(1.30±0.10) vs. (1.24±0.11) s, P<0.001] in left cerebellar hemisphere were higher than that of right side. The CBF (28/56) of cerebral hemisphere, cerebellar hemisphere and brain subregions was higher in females than that in males, while ATT in 83.9% (47/56) subregions was lower than that in males (all P<0.05). CBV in female subjects was higher only in 5 brain regions (superior occipital gyrus, middle occipital gyrus, inferior occipital gyrus, superior parietal gyrus and cerebelum_7b) (all P<0.05). In young subjects, CBF in 44.6% (25/56) subregions and CBV in 33.9% (19/56) subregions were higher than those in the senior group (all P<0.05). The ATT in most subregions in young group were lower than those in senior group, but the difference was statistically significant only in rectus gyrus ( P=0.026) and paracentral lobule ( P=0.006). The CBF ( r=-0.430, P=0.005) and CBV ( r=-0.327, P=0.035) of cerebral hemisphere were negatively correlated with age. The CBF (24/25, r range:-0.497 —-0.343, all P<0.05) and CBV (16/19, r range:-0.474 —-0.322, all P<0.05) in most subregions were negatively correlated with age, while ATT was positively correlated (gyrus rectus: r=0.311, P=0.045; paracentral lobule: r=0.392, P=0.010). Conclusions:Multi-PLD ASL imaging could be applied for quantitative analysis of brain perfusion. The perfusion parameters of anatomical subregions are different depending on side, gender, and age.

Objective:To explore the influencing factors of all-cause death in patients with acute myocardial infarction (AMI) and renal insufficiency(RI), and establish a clinical prediction tool.Methods:Collected 727 patients who were hospitalized in Beijing Anzhen Hospital from January 1st 2014 to April 31th 2019, and diagnosed as AMI with RI. Recorded the patients' baseline characteristics, past medical history, current complications, laboratory and auxiliary examination results, treatment methods (included target vessel reconstruction methods, medications, etc.), and follow-up patients for all-cause deaths. Firstly, multivariate Cox regression analysis was used to construct the model in the training set(485 cases). Secondly, the receiver operating characteristic curve ( ROC), calibration curve and clinical decision curve analysis (DCA) were drawn in the validation set(242 cases) to further verify the effect of the prediction model. Finally, a nomogram was developed based on the verified risk factors. Results:Multivariate Cox regression analysis found that there were nine predictors for the prognosis of all-cause death in patients with AMI & RI, the dangerous factors included women, comorbidities, high direct bilirubin, cardiogenic shock, respiratory failure, co-infection and hemofiltration; the protective factors included PCI and taking aspirin. Model evaluation results showed that the AUC of the validation set was 0.82; the calibration line was close to the ideal calibration line, and the slope≈1, the intercept≈0, and the fitting effect was good. Clinical decision-making when the all-cause mortality rate was 35%, the net benefit of active intervention patients based on the Cox model was 38%. A visual nomogram model was developed based on the prognostic risk factors that have been evaluated and predicted to be good. Conclusion:Women, with a history of comorbidities, direct high bilirubin, combined with cardiogenic shock, combined with respiratory failure, combined infection, hemofiltration, PCI and taking aspirin, these nine factors will affect all causes of AMI & RI. For the probability of death, the model developed in this research has high accuracy.

Objective:To evaluate the medium and long-term clinical efficacy of the treatment of lumbar degenerative diseases in Dynesys dynamic internal fixation combined with decompression.Methods:From March 2008 to March 2015, 145 patients (84 males and 61 females, mean age 55.9±7.1 years old) with symptoms of lumbar degenerative diseases (69 lumbar disc herniation, 53 lumbar spinal stenosis and 23 I grade lumbar degenerative spondylolisthesis) were treated by the lumbar discectomy using Dynesys dynamic internal fixation combined with decompression. The clinical symptoms before and after surgery were assessed by visual analogue scale (VAS), Japanese Orthopaedic Association (JOA) and Oswestry disability index (ODI). Lumbar lateral radiographs were used to measure the height of intervertebral space between the surgical segment and the adjacent segment. The range of motion (ROM) between the surgical segment and the adjacent segment was measured by lumbar dynamic position X-ray. Surgical and adjacent segments degenerative were classified according to the Pfirrmann grade classification.Results:The VAS score, ODI and JOA score of lower back and lower limbs in patients with lumbar disc herniation were improved from 6.6±1.7, 7.1±1.4, 63.1%±10.2%, 12.5±2.4 preoperatively to 2.6±1.0, 2.8±0.9, 30.9%±9.8%, 22.4±2.1 at the latest follow-up. The differences were statistically significant. The VAS score, ODI score and JOA score of lower back and lower limbs in patients with lumbar spinal stenosis were improved from 6.3±2.2, 6.9±1.3, 63.4%±8.5%, 12.8±2.7 preoperatively to 2.4±1.2, 2.8±1.0, 35.1%±12.0%, 22.2±2.2 at the latest follow-up. The differences were statistically significant. The VAS score, ODI score and JOA score of lower back and lower limbs in patients with I degree lumbar degenerative spondylolisthesis were improved from 5.7±2.3, 6.7±0.9, 65.7%±10.0%, 12.5±2.7 preoperatively to 2.2±1.2, 2.7±1.1, 37.0%±11.8%, 22.4±2.6 at the latest follow-up. The differences were statistically significant. Comparing to preoperational value, the height of the operative segment and caudal intervertebral space were decreased at the 1 year postoperatively and last follow-up. But the difference was not significant. As for cranial adjacent segment, the height of intervertebral space preoperatively was decreased from 12.1±1.9 mm preoperatively to 11.7±1.6 mm at 1 year postoperatively, and to 11.3±1.8 mm at the latest follow-up. The difference between them was statistically significant ( F=6.46, P=0.001). The ROM of surgical segments was decreased from 7.6°±2.2° preoperatively to 5.5°±1.6° at 1 year postoperatively, and to 2.9°±1.4° at the latest follow-up. The difference between them was statistically significant ( F=267.9, P<0.001). Conversely, the ROM of cranial and caudal segments was increased from 8.2°±2.4°, 6.5°±1.6° preoperatively to 9.1°±2.1°, 7.1°±1.9° at 1 year postoperatively, and to 10.6°±2.5°, 7.2°±1.8° at the latest follow-up. The difference between them was statistically significant ( F=38.66, 3.81, P<0.001, 0.023). At the latest follow-up, 120 (51.9%) adjacent segments were to be defined adjacent segment degeneration which includes 103 radiological adjacent segment degeneration and 17 symptomatic adjacent segment degenerations. Conclusion:Dynesys dynamic internal fixation combined with decompression could achieve satisfying mid- and long-term therapeutic effect in the treatment of lumbar degenerative diseases. The ROM of surgical segments decreased with time, although part of the ROM was still retained at the latest follow-up. However, it does not seem to avoid the degeneration of adjacent segment.

Objective:To retrospectively analyze the blood leukocytes (WBC), lymphocytes (LYM), lymphocyte% (LYM%), and serum total immunoglobulin (IGA, IGG, IGM) and complement (C3, C4) index levels to explore its predictive value for the outcome of COVID-19 severe pneumonia.Methods:Eighty-five COVID-19 patients with severe pneumonia diagnosed in our hospital were randomly selected and were divided into good outcome group (50 cases) and poor outcome group (35 cases). WBC, LYM, LYM%, IGA, IGG, IGM, and C3, C4 level data, and analyze the differences between the two groups, the correlation of each indicator, and ROC curves of single and joint detection to explore relationship between indicators and outcomes, and the predictive efficacy of indicators on outcomes.Results:Differences in WBC, LYM, LYM%, IGG, and IGA levels were significant between the two groups ( P=0.000, 0.015, 0.000, 0.000, 0.001), among them with significant differences, LYM and LYM% were significantly positively correlated ( r=0.669, P=0.000), while WBC and LYM% levels were significantly negatively correlated ( r=-0.600, P=0.000), WBC and IGA levels were significantly positively correlated ( r=0.283, P=0.009) and IGG and IGA levels were also significantly positively correlated ( r=0.0.442, P=0.000); After logistic regression analysis, WBC, LYM, LYM%, IGG, and IGA are all important influencing factors ( P=0.001, 0.022, 0.000, 0.000, 0.003); but only the levels of WBC, IGG, and LYM% are Independent risk factors ( P=0.034, 0.004, 0.001), the ROC curve of the single detection and joint detection of their predicted outcome performance, respectively, and the max AUC (AUC=0.890, P=0.000) at the time of joint testing of WBC, LYM% and IGG, index YI=0.657, it has the greatest predictive power for adverse outcomes, with a sensitivity of 77.10% and a specificity of 88.00%. IGM, C3, C4, IGG/IGM, and C3/C4 levels were not significantly different( P=0.066, 0.204, 0.076, 0.310, 0.156). Conclusions:The levels of WBC, LYM, LYM%, IGG, and IGA in the early admission of COVID-19 infected patients with severe pneumonia have important predictive value for the outcome of them. WBC, LYM% and IGG levels are independent risks and joint detection of the three indexes have the best predictive performance.

OBJECTIVE: To investigate the incidence,clinical,biochemical and genetic characteristics of isovaleric acidemia (IVA) in Zhejiang province. METHODS: Between January 2009 and December 2019, a total of 3 510 004 newborns were screened for IVA using tandem mass spectrometry. Patients of IVA were confirmed by urine organic acid and RESULTS: A total of 15 patients with IVA were diagnosed, with an incidence of 1/234 000. Three patients had acute neonatal IVA, and the rest were asymptomatic. The isovalerylcarnitine (C5) levels were increased in all patients. Twelve children underwent urinary organic acid analysis, of which 11 cases had elevated isovalerylglycine levels, 4 cases with 3-hydroxyisovalerate increased simultaneously. Eleven IVA patients underwent genetic testing, 9 patients were compound heterozygous variants in CONCLUSIONS The clinical manifestations of IVA are non-specific, and the gene spectrum is scattered. Newborn patients screened by tandem mass spectrometry can receive early diagnosis and treatment, so as to correct metabolic defects and pathophysiological changes.
Amino Acid Metabolism, Inborn Errors/epidemiology* ; Child ; China/epidemiology* ; Humans ; Infant, Newborn ; Isovaleryl-CoA Dehydrogenase/genetics* ; Mutation ; Neonatal Screening ; Tandem Mass Spectrometry

OBJECTIVE: To explore effects of different delivery and storage conditions on concentrations of amino acids and carnitines in neonatal dried blood spots (DBS), so as to provide evidence for improving accurate and reliable detection by tandem mass spectrometry. METHODS: A total of 1 254 616 newborn DBS samples in Newborn Screening Center of Zhejiang Province were delivered and stored at room temperature (group A, RESULTS: The concentrations of amino acids and carnitines in the three groups were skewed, and the differences in amino acid and carnitine concentrations among groups were statistically significant (all CONCLUSIONS Cold-chain logistics system and storage in low temperature and low humidity can effectively reduce degradation of some amino acids and carnitines in DBS, improve the accuracy and reliability of detection, and thus ensures the quality of screening for neonatal metabolic diseases.
Amino Acids/analysis* ; Carnitine/analysis* ; Dried Blood Spot Testing/standards* ; Humans ; Humidity ; Infant, Newborn ; Neonatal Screening ; Reproducibility of Results ; Specimen Handling/standards* ; Tandem Mass Spectrometry ; Temperature ; Time Factors

Objective:To investigate the application feasibility of Region 4 Stork (R4S) system, an international collaborative newborn screening data platform, combined with cut-off value analysis in the neonatal screening for very long chain acyl-CoA dehydrogenase deficiency (VLCADD) by tandem mass spectrometry (MS/MS).Methods:The retrospective study was performed in 2, 040 072 neonates screened by MS/MS in Neonatal Screening Center of Zhejiang Province, China from October 2013 to July 2018. Nine hundred and ten cases were determined and identified as suspected positive VLCADD neonates by traditional cut-off method of tandem mass spectrometry. The original data of these 910 screened neonates were further analyzed by R4S system. Based on clinical diagnosis and ACADL gene test results, the screening efficiency between two methods was statistically compared.Results:The data of 910 suspected VLCADD-positive cases interpreted by cut-off method were further analyzed by R4S system, and the positive interpretation was reduced to 238 cases (including 9 confirmed positive cases). A total of 16 different mutations were found in ACADL gene sequencing among the confirmed children. The screening false positive rate (FPR) declined from 0.44‰ (901/2 040 072) to 0.11‰ (229/2 040 072), the rate of positive predictive value (PPV) increased from 0.99% (9/910) to 3.78% (9/238), and the specificity increased from 99.96% (2 039 162/2 040 063) to 99.99% (2 039 834/2 040 063). There was a statistically significant difference between cut-off method alone and cut-off method combined R4S system analysis (χ2=393.5, P<0.05). Conclusions:The R4S system combined with cut-off method applied in VLCADD neonatal screening by MS/MS can effectively improve screening performance, reduce false positive rate, and has certain value in clinical application.