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1.
JOURNAL OF RARE DISEASES ; (4): 57-62, 2024.
Article in English | WPRIM | ID: wpr-1006918

ABSTRACT

ObjectiveTuberous sclerosis complex (TSC) is a rare autosomal dominant genetic disease involving multiple organ system. Renal angiomyolipoma (RAML) is a leading cause of death for adult TSC patients. Our study aims at investigating the clinical manifestations of TSC-RAML to enable clinicians to have a better understanding of the disease in Chinese patients.MethodsWe reviewed retrospectively the data collected from patients with TSC-RAML in Peking Union Medical College Hospital from January 2014 to January 2023. We also collected information about the age, gender, imageological examination, RAML stage, and comorbidities.ResultsA total of 186 TSC-RAML patients were registered, 65 of whom were males and 121 were females(male-to-female ratio 1∶1.86). The median age of all patients was 31 years old. Totally, 117 cases (62.9%)of RAML were rated stage 6. Twenty-two cases (11.8%) had a history of tumor rupture and bleeding. Research shows statistical differences between high and low grade on RAML rupture bleeding(P=0.0475). Angiofibromas/fibrous cephalic plaque (155/186, 83.3%), subependymal nodules(103/146, 70.5%), lymphangioleiomyomatosis (102/157, 65.0%), hypomelanotic macules (114/186, 61.3%), and shagreen patch (83/186, 44.6%)were the most common clinical manifestations. All patients with lymphangioleiomyomatosis(LAM) were female(P < 0.0001).ConclusionsFemale patients predominated TSC-RAML patients. Most RAML were in stage 6. About 11.8% cases had a history of tumor rupture and hemorrhage. The higher grade meant the higher proportion of tumor rupture and hemorrhage. Dermatological and nervous lesions were the most common comorbidities. All patients with LAM in this study were female.

2.
China Pharmacy ; (12): 2868-2873, 2023.
Article in Chinese | WPRIM | ID: wpr-999219

ABSTRACT

OBJECTIVE To investigate the effects and mechanism of atractylodin on inflammatory injury of periodontal tissue and alveolar bone loss in periodontitis rats. METHODS A total of 144 SD rats were divided into control group (intragastric and intraperitoneal injection of normal saline), model group (intragastric and intraperitoneal injection of normal saline), atractylodin low-dose, medium-dose and high-dose groups (intraperitoneal injection of 6.665, 13.33, and 26.66 mg/kg atractylodin), metronidazole group (positive control group, intragastric injection of 0.05 g/kg metronidazole, intraperitoneal injection of normal saline), AMD3100 [stromal cell-derived factor-1 (SDF-1)/CXC chemokine receptor-4 (CXCR4) pathway inhibitor] group (intragastric injection of 1 mg/kg AMD3100, intraperitoneal injection of normal saline), atractylodin high-dose+AMD 3100 group (intraperitoneal injection of 26.66 mg/kg atractylodin, intragastric injection of 1 mg/kg AMD3100), with 18 rats in each group. Except for the control group, all other groups of rats were inoculated with Porphyromonas gingivalis to construct a periodontitis model. After successful modeling, they were given relevant medicine or normal saline, once a day, for 4 consecutive weeks. The gingival index of rats was detected; the levels of interleukin-6 (IL-6) and tumor necrosis factor α (TNF-α) in rat serum were also determined; alveolar bone resorption, periodontal histopathologic changes and the number of osteoclasts were detected by methylene blue staining, HE staining and TRAP staining, respectively. The expressions of osteoprotegerin (OPG), receptor activator of NF-κB ligand (RANKL), SDF-1 and CXCR4 proteins were determined. RESULTS Compared with the control group, serious pathological injury of periodontal tissue was found in the model group, the gingival index, the levels of IL-6 and TNF- α, alveolar bone absorption value, the number of osteoclasts, and the expression of RANKL protein were all increased significantly (P<0.05), while the expressions of OPG, SDF-1 and CXCR4 proteins were decreased significantly (P<0.05). Compared with the model group, pathological injury of periodontal tissue in rats was reduced; the gingival index, the levels of IL-6 and TNF-α, alveolar bone resorption value, osteoclast number and RANKL protein expression were decreased significantly, while protein expressions of OPG, SDF-1 and CXCR4 were increased significantly in atractylodin low-dose, medium-dose and high-dose groups and metronidazole group (P<0.05). The change trend of corresponding indexes in the AMD3100 group was opposite to the above (P<0.05). AMD3100 attenuated the inhibitory effect of high-dose atractylodin on inflammatory response and alveolar bone loss in rats with periodontitis (P<0.05). CONCLUSIONS Atractylodin may improve the inflammatory response and alveolar bone loss in periodontitis rats by activating the SDF-1/CXCR4 signaling pathway.

3.
Acta Pharmaceutica Sinica ; (12): 3032-3039, 2023.
Article in Chinese | WPRIM | ID: wpr-999042

ABSTRACT

The current study explored the hepatotoxicity among closed-ring genipin, open-ring tautomer of genipin and gardenia blue that generated from genipin and amino acid reaction using HepaRG cells to identify the material basis of genipin-induced hepatotoxicity in vitro. The effects of temperature, pH value and different kinds of amino acids on the chemical structure tautomerism between closed-ring and open-ring tautomer of genipin and the production of gardenia blue were investigated firstly, which aimed to explicit the conditions that could distinguish the closed-ring genipin and its open-ring tautomer, and the conditions generating gardenia blue, which were applied to prepare different kinds of gardenia blue; the CCK-8 kit was employed to analyze the hepatotoxicity of closed-ring genipin, open-ring tautomer of genipin and gardenia blue. From the results, it was found that, the structure transformation from close-ring to open-ring of genipin could be inhibited under the condition with acid environment; being essential groups to generate gardenia blue, the primary amino group and the open-ring tautomer of genipin reacting to generate the dihydropyridine ring was probably the key structure of gardenia blue; the structure characteristics existed apparent distinction at the reactive temperature of 37 ℃ and 80 ℃; compared to the culture condition with pH = 7.4, the concentration of genipin with close-ring in culture medium was significantly increased at pH = 5, but the cell viability did not decreased; the cell toxicity of gardenia blue was apparently lower than open-ring tautomer of genipin, and even some kinds of gardenia blue showed growth promoting effect on HepaRG cells. Here, it was suggested potentially that open-ring tautomer of genipin be the important material basis to induce hepatotoxicity, which could provide a cue and lay a foundation for the elucidation of the underlying mechanism of genipin-induced hepatotoxicity.

4.
International Eye Science ; (12): 2052-2058, 2023.
Article in Chinese | WPRIM | ID: wpr-998489

ABSTRACT

AIM:To observe the changes of macular morphology and microcirculation in myopic maculopathy(MM), and investigate theirs correlation and effects on vision.METHODS: Case-control study. A total of 165 patients(189 eyes)with high myopia and 154 healthy volunteers(154 eyes)from October 2016 to December 2018 were selected. According to the classification of Meta-analysis for pathologic myopia(META-PM), participants were divided into M0 group(category 0, 41 eyes), M1 group(category 1, 53 eyes), M2 group(category 2 and 3, 52 eyes), and myopic choroidal neovascularization(mCNV)group(43 eyes). All participants underwent optical coherence tomography angiography(OCTA)examination. Morphological and microcirculation parameters of retina at different layers were compared between groups. Pearson correlation was used to assess the correlation between morphological and microcirculation parameters. Correlations between vision and other parameters were analyzed using multiple linear regression analysis.RESULTS:Foveal full retinal thickness(FRT)and outer retinal thickness(ORT)were all lower in M0, M1 and M2 groups than those of control group(all P&#x003C;0.01). Foveal superficial capillary plexus vessel density(SVD)and deep capillary plexus vessel density(DVD)were all lower in M2 and mCNV groups than those of the control group(all P&#x003C;0.01). Parafoveal FRT and ORT were all lower in M0, M1, M2 and mCNV groups than those of the control group(all P&#x003C;0.01). Parafoveal inner retinal thickness(IRT), SVD and DVD were all lower in M2 and mCNV groups than those of the control group(all P&#x003C;0.01). Subfoveal choroidal thickness(SFCT)and choroid capillaries vessel density(CVD)were all lower in M0, M1, M2 and mCNV groups than those of the control group(all P&#x003C;0.01). Foveal vessel density of retina and choroid were positively correlated with its thickness in patients with MM without CNV(all P&#x003C;0.05). Multivariate analysis showed that axial length(AL), diffuse or patchy chorioretinal atrophy were influencing foctors of best corrected visual acuity(BCVA; all P&#x003C;0.01).CONCLUSION:Retinal morphological changes precede microcirculation changes in MM. Most of all, ORT changes precede IRT changes. Foveal vessel density of retina and choroid were positively correlated with its thickness. The main influencing factors of BCVA were AL and types of MM.

5.
Chinese Journal of Clinical Thoracic and Cardiovascular Surgery ; (12): 551-556, 2023.
Article in Chinese | WPRIM | ID: wpr-996347

ABSTRACT

@#Objective    To analyze the early outcomes of anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) patients with severe left ventricular dysfunction after surgical repair, and to explore the predictors for extracorporeal membrane oxygenation (ECMO) support for these patients. Methods    The clinical data of ALCAPA patients with severe left ventricular dysfunction (left ventricular ejection fraction<40%) who underwent coronary artery reimplantation in the pediatric center of our hospital from 2013 to 2020 were retrospectively analyzed. The patients were divided into an ECMO group and a non-ECMO group. Clinical data of the two groups were compared and analyzed. Results    A total of 64 ALCAPA patients were included. There were 7 patients in the ECMO group, including 4 males and 3 females aged 6.58±1.84 months. There were 57 pateints in the non-ECMO group, including 30 males and 27 females aged 4.34±2.56 months. The mortality of the patients was 6.25% (4/64), including 2 patients in the ECMO group, and 2 in the non-ECMO group. The postoperative complications rate was significantly higher in the ECMO group than that in the non-ECMO group (P=0.041). There were statistical differences in the cardiopulmonary bypass time [254 (153, 417) min vs. 106 (51, 192) min, P=0.013], aortic cross-clamping (ACC) time (89.57±13.66 min vs. 61.58± 19.57 min, P=0.039), and preoperative left ventricular end-diastolic diameter/body surface area (132.32±14.71 mm/m2 vs. 108.00±29.64 mm/m2, P=0.040) between the two groups. Multivariate logistic regression analysis showed that ACC time was an independent risk factor for postoperative ECMO support (P=0.005). Receiver operating characteristic (ROC) curve analysis showed that the area under the ROC curve was 0.757, the sensitivity was 85.70%, specificity was 66.70%, with the cut-off value of 66 min. Conclusion    ACC time is an independent risk factor for postoperative ECMO support. Patients with an ACC time>66 min have a significantly higher risk for ECMO support after the surgery.

6.
Chinese Journal of Medical Aesthetics and Cosmetology ; (6): 182-185, 2023.
Article in Chinese | WPRIM | ID: wpr-995922

ABSTRACT

Objective:To explore the application of nasal aesthetic polygon theory in the reconstruction of new domes on nasal tip with autogenous costal cartilage.Methods:From June 2019 to June 2021, 116 patients (26 males and 90 females) received rhinoplasty, CT-assisted examination of the costal cartilage, nasal bone and nasal cartilage was performed, and the autogenous costal cartilaginous cortex was used to make dome reconstruction grafts that were transplanted to the original alar cartilage and partially fixed to the original nasal septum cartilage to form a satisfactory and natural nasal shape.Results:All the patients were followed up for 6-24 months. No cartilaginous, overrotated or underrotated appearance was found in nasal tip. The shape of nasal tip was clear and good, with an obvious performance point and full lower lobule. The nose was tall and straight, and looked natural and beautiful in three dimensions.Conclusions:The nasal aesthetic polygon theory is used to guide the reconstruction of new domes with autogenous costal cartilage in nasal tip surgery. Compared to the cap and shield grafts, the nasal tip is more close to the normal anatomical structure, the shape and texture are more similiar to the natural state and the nasal tip is softer by using autogenous costal cartilage, and so it is an ideal surgical procedure for nasal tip reconstruction.

7.
Chinese Medical Journal ; (24): 1897-1909, 2023.
Article in English | WPRIM | ID: wpr-980976

ABSTRACT

Endometriosis, a heterogeneous, inflammatory, and estrogen-dependent gynecological disease defined by the presence and growth of endometrial tissues outside the lining of the uterus, affects approximately 5-10% of reproductive-age women, causing chronic pelvic pain and reduced fertility. Although the etiology of endometriosis is still elusive, emerging evidence supports the idea that immune dysregulation can promote the survival and growth of retrograde endometrial debris. Peritoneal macrophages and natural killer (NK) cells exhibit deficient cytotoxicity in the endometriotic microenvironment, leading to inefficient eradication of refluxed endometrial fragments. In addition, the imbalance of T-cell subtypes results in aberrant cytokine production and chronic inflammation, which contribute to endometriosis development. Although it remains uncertain whether immune dysregulation represents an initial cause or merely a secondary enhancer of endometriosis, therapies targeting altered immune pathways exhibit satisfactory effects in preventing disease onset and progression. Here, we summarize the phenotypic and functional alterations of immune cells in the endometriotic microenvironment, focusing on their interactions with microbiota and endocrine and nervous systems, and how these interactions contribute to the etiology and symptomology of endometriosis.


Subject(s)
Female , Humans , Endometriosis/metabolism , Killer Cells, Natural/metabolism , T-Lymphocytes/metabolism , Estrogens , Endometrium/metabolism
8.
Chinese Medical Journal ; (24): 1430-1438, 2023.
Article in English | WPRIM | ID: wpr-980942

ABSTRACT

BACKGROUND@#This study aimed to develop a comprehensive instrument for evaluating and ranking clinical practice guidelines, named Scientific, Transparent and Applicable Rankings tool (STAR), and test its reliability, validity, and usability.@*METHODS@#This study set up a multidisciplinary working group including guideline methodologists, statisticians, journal editors, clinicians, and other experts. Scoping review, Delphi methods, and hierarchical analysis were used to develop the STAR tool. We evaluated the instrument's intrinsic and interrater reliability, content and criterion validity, and usability.@*RESULTS@#STAR contained 39 items grouped into 11 domains. The mean intrinsic reliability of the domains, indicated by Cronbach's α coefficient, was 0.588 (95% confidence interval [CI]: 0.414, 0.762). Interrater reliability as assessed with Cohen's kappa coefficient was 0.774 (95% CI: 0.740, 0.807) for methodological evaluators and 0.618 (95% CI: 0.587, 0.648) for clinical evaluators. The overall content validity index was 0.905. Pearson's r correlation for criterion validity was 0.885 (95% CI: 0.804, 0.932). The mean usability score of the items was 4.6 and the median time spent to evaluate each guideline was 20 min.@*CONCLUSION@#The instrument performed well in terms of reliability, validity, and efficiency, and can be used for comprehensively evaluating and ranking guidelines.


Subject(s)
Humans , Reproducibility of Results , Surveys and Questionnaires , Practice Guidelines as Topic
9.
Chinese Journal of Clinical Thoracic and Cardiovascular Surgery ; (12): 422-427, 2023.
Article in Chinese | WPRIM | ID: wpr-979525

ABSTRACT

@#Objective     To explore the effects of intravenous treprostinil in different doses on the hemodynamics and postoperative outcomes after high-risk total cavo-pulmonary connection (TCPC). Methods    From 2018 to 2021, among 189 patients who underwent TCPC in the Department of Pediatric Cardiac Surgery of Fuwai Hospital, 26 high-risk patients who received the intravenous treprostinil therapy were retrospectively analyzed. There were 12 males and 14 females, with an age of 4 (3, 6) years and a weight of 17.6±6.2 kg. The patients were divided into two groups: a high-dose group [15 patients, maintaining dose>10 ng/(kg·min)] and a low-dose group [11 patients, maintaining dose≤ 10 ng/(kg·min)]. The hemodynamics before treprostinil using and during the first 24 hours after reaching the maintaining dose of treprostinil, and postoperative outcomes of the two groups were investigated. Results    The incidence of heterotaxia was higher in the high-dose group (66.7% vs. 18.2%, P=0.021). During the observation period, the mean pulmonary artery pressure decreased from 11.9±3.6 mm Hg to 11.0±3.3 mm Hg in the low-dose group (P=0.013), and from 12.9±4.7 mm Hg to 10.2±3.4 mm Hg in the high-dose group (P=0.001). The decreasing effect in the high-dose group was better than that in the low-dose group (P=0.010). There was no statistical difference in the postoperative outcomes between the two groups (P>0.05). In terms of side effects, patients needed temporarily increased dosage of vasoactive drugs to maintain stable blood pressure during 6-12 h after treprostinil therapy in the high-dose group. Conclusion    In patients after high-risk TCPC, intravenous high-dose treprostinil has a better therapeutic effect on reducing pulmonary artery pressure. However, it should be noted that increased dosage of vasoactive agents may be required to maintain blood pressure stability in patients with high-dose treprostinil.

10.
Chinese Journal of Trauma ; (12): 385-393, 2023.
Article in Chinese | WPRIM | ID: wpr-992613

ABSTRACT

Osteochondral lesion of talus (OLT) is a foot and ankle disease characterized by ankle pain, which may impact the joint function and life quality. If managed improperly, it may lead to a further ankle arthritis, severely compromising the prognosis. The therapeutic effect of conservative treatment for OLT is still uncertain. Surgery is still the main treatment modality for OLT with various techniques. However, the optimized surgical technique is still inconclusive, furthermore, regeneration and repair of cartilage after debridement is also a great challenge for the treatment of OLT. Platelet-rich plasma (PRP) with good repair effect on cartilage injury is gradually applied in the treatment of OLT. However, there still lacks the unified understanding of the technique and specification of PRP for the treatment of OLT. Therefore, National Orthopedics Center of Shanghai Sixth People′s Hospital allied Foot Ankle Basic Research & Orthopedics Group, Chinese Association of Orthopedic Surgeons; Foot and Ankle Committee of Chinese Association of Sports Medicine Physicians; and Foot and Ankle Group of Orthopedic Specialized Branch of Shanghai Medical Association to organize related experts to formulate the Expert consensus on platelet- rich plasma treatment for osteochondral lesion of talus ( version2023). Fifteen recommendations were put forward upon PRP preparation, indications, contraindications and treatment methods of PRP for OLT, so as to standardize the PRP treatment for OLT.

11.
Chinese Journal of Pharmacology and Toxicology ; (6): 540-540, 2023.
Article in Chinese | WPRIM | ID: wpr-992215

ABSTRACT

OBJECTIVE The N-methyl-D-aspartate(NMDA)receptor has been shown to be strongly associ-ated with rapid antidepressant effects.GW043 is a com-pound with a novel structure that we designed and syn-thesized to act on the NMDA receptor(NMDAR).METH-ODS In this study,we first confirmed the target of GW043 using a receptor binding assay.We observed the effect of GW043 on NMDAR currents in vivo and in vitro assays using a membrane clamp technique with a view to characterizing the function of GW043.We investi-gated the antidepressant effect of GW043 in rodent behavioral models such as FST,TST and CUMS.Fur-thermore,we explored the mechanism of GW043 onset using Western blotting,BrdU staining,Golgi staining and electrophysiological techniques.RESULTS GW043 interacts with high affinity only at the NMDAR.Electro-physiological studies have indicated that GW043 is a par-tial agonist of NMDAR.Meanwhile,behavioral experi-ments were conducted to confirm the antidepressant effect of GW043 in rodents.The mechanism study found that GW043 regulate synaptic plasticity through LTP and BDNF/mTOR pathways and increase the number of new-born neurons to cause antidepressant effects.GW043,a partial agonist of NMDAR,reversed depression-like behav-ior in rats by modulating synaptic plasticity,suggesting an antidepressant effect.CONCLUSION The results suggest that GW043 is a partial agonist of NMDA recep-tors and has significant antidepressant effects.

12.
Chinese Critical Care Medicine ; (12): 32-36, 2023.
Article in Chinese | WPRIM | ID: wpr-991974

ABSTRACT

Objective:To analyze the epidemic characteristics and clinical key indicators of the patients infected with SARS-CoV-2 of the local Omicron variant epidemic, to understand the clinical characteristics of mild and severe patients, and to provide a scientific basis for the effective treatment and prevention of severe disease.Methods:From January 2020 to March 2022, the clinical and laboratory data of COVID-19 patients admitted to the Fifth People's Hospital of Wuxi were retrospective analyzed, including virus gene subtypes, demographic information, clinical classification, main clinical symptoms, and key indicators of clinical testing, and the changes of clinical characteristics of the patients infected with SARS-CoV-2.Results:A total of 150 patients with SARS-CoV-2 infection were admitted, 78, 52 and 20 in 2020, 2021 and 2022, including 10, 1 and 1 severe patient, and the main infected virus strains were L, Delta, and Omicron variants. The relapse rate of patients infected with the Omicron variant was as high as 15.0% (3/20), the incidence of diarrhea decreased to 10.0% (2/20), the incidence of severe disease decreased to 5.0% (1/20), and the number of hospitalization days of mild patients increased compared with 2020 (days: 20.43±1.78 vs. 15.84±1.12); respiratory symptoms were reduced, and the proportion of pulmonary lesions decreased to 10.5%; the virus titer of severely ill patients with SARS-CoV-2 Omicron variant infection (day 3) was higher than that of L-type strain (Ct value: 23.92±1.16 vs. 28.19±1.54). The acute plasma cytokines interleukin (IL-6, IL-10) and tumor necrosis factor-α (TNF-α) were significantly lower in patients with severe Omicron variant new coronavirus infection than those with mild disease [IL-6 (ng/L): 3.92±0.24 vs. 6.02±0.41, IL-10 (ng/L): 0.58±0.01 vs. 4.43±0.32, TNF-α (ng/L): 1.73±0.02 vs. 6.91±1.25, all P < 0.05], while γ-interferon (IFN-γ) and IL-17A were significantly higher than patients with mild disease [IFN-γ (ng/L): 23.07±0.17 vs. 13.52±2.34, IL-17A (ng/L): 35.58±0.08 vs. 26.39±1.37, both P < 0.05]. Compared with previous epidemics (2020 and 2021), the proportion of CD4/CD8 ratio, lymphocyte count, eosinophil and serum creatinine decreased in patients with mild Omicron infection in 2022 (36.8% vs. 22.1%, 9.8%; 36.8% vs. 23.5%, 7.8%; 42.1% vs. 41.2%, 15.7%; 42.1% vs. 19.1%, 9.8%), the proportion of patients with elevated monocyte count and procalcitonin was large (42.1% vs. 50.0%, 23.5%; 21.1% vs. 5.9%, 0). Conclusion:The incidences of severe disease in patients with SARS-CoV-2 Omicron variant infection was significantly lower than that of previous epidemics, and the occurrence of severe diseases was still related to the underlying diseases.

13.
Chinese Journal of Medical Education Research ; (12): 1121-1126, 2023.
Article in Chinese | WPRIM | ID: wpr-991484

ABSTRACT

Objective:In order to improve the staff's ability to deal with public health emergencies, a third-class hospital in Beijing has made efforts to improve the emergency response ability of all staff members to achieve zero infection, and to carry out vocational training and assessment of all staff.Methods:The instructional system design (ISD) model system is used to design the training course. The online learning, electronic examination paper assessment and on-site training of people in the hospital were analyzed by Excel and SPSS 22.0.Results:After training, the average scores of trainees increased from 84 points to 100 points, and the average answering time was shortened from 308 s to 179 s. There were differences in the assessment scores before and after training for personnel with different professional titles, and there were differences in the assessment scores before and after training for personnel between different departments.Conclusion:In case of public health emergency, it is necessary to train and assess the whole staff. The application of ISD model is helpful to make training plan quickly. The application of online learning assessment is the first effective way of emergency training.

14.
Chinese Pediatric Emergency Medicine ; (12): 531-535, 2023.
Article in Chinese | WPRIM | ID: wpr-990555

ABSTRACT

Objective:To explore the risk factors of early delayed recovery after right ventricular-extrapulmonary arterial(RV-PA)conduit reconstruction.Methods:From 2017 to 2021, the children with RV-PA conduit reconstruction, who were treated in our hospital were retrospectively analyzed.The demographic data and peri-operative clinical data of the patients were collected for statistical analysis.Results:Fifty-five patients were included in the study.The patients were sequenced by the length of ICU stay.The time at the 75th percentile was defined as the critical value for grouping.According to the ICU stay time of the children, they were divided into normal recovery group(ICU stay ≤7 days, n=40)and delayed recovery group(ICU stay>7 days, n=15).The mechanical ventilator time in the whole group was 24(0, 1 408)h, and the ICU stay time was 4(1, 67)d.Six cases required extracorporeal membrane oxygenation (ECMO) support, and two cases died.In the multivariate Logistic regression analysis of two groups, long cardiopulmonary bypass(CPB) time( OR=1.034, 95% CI 1.009-1.061, P=0.009)and poor right ventricular function( OR=9.536, 95% CI 1.010-90.037, P=0.049)were independent risk factors for early delayed recovery. Conclusion:The risk of RV-PA conduit reconstruction is high.The proportion of ECMO support is increased.The mortality rate is higher.Right heart dysfunction and prolonged CPB time are risk factors for delayed postoperative recovery.

15.
Chinese Journal of Pediatrics ; (12): 1018-1023, 2023.
Article in Chinese | WPRIM | ID: wpr-1013216

ABSTRACT

Objectives: To investigate the current application status and implementation difficulties of extracorporeal cardiopulmonary resuscitation (ECPR) in children with sudden cardiac arrest. Methods: This cross-sectional survey was conducted in 35 hospitals. A Children's ECPR Information Questionnaire on the implementation status of ECPR technology (abbreviated as the questionnaire) was designed, to collect the data of 385 children treated with ECPR in the 35 hospitals. The survey extracted the information about development of ECPR, the maintenance of extracorporeal membrane oxygenation (ECMO) machine, the indication of ECPR, and the difficulties of implementation in China. These ECPR patients were grouped based on their age, the hospital location and level, to compare the survival rates after weaning and discharge. The statistical analysis used Chi-square test and one-way analysis of variance for the comparison between the groups, LSD method for post hoc testing, and Bonferroni method for pairwise comparison. Results: Of the 385 ECPR cases, 224 were males and 161 females. There were 185 (48.1%) survival cases after weaning and 157 (40.8%) after discharge. There were 324 children (84.2%) receiving ECPR for cardiac disease and 27 children (7.0%) for respiratory failure. The primary cause of death in ECPR patients was circulatory failure (82 cases, 35.9%), followed by brain failure (80 cases, 35.0%). The most common place of ECPR was intensive care unit (ICU) (278 cases, 72.2%); ECPR catheters were mostly inserted through incision (327 cases, 84.9%). There were 32 hospitals (91.4%) had established ECMO emergency teams, holding 125 ECMO machines in total. ECMO machines mainly located in ICU (89 pieces, 71.2%), and the majority of hospitals (32 units, 91.4%) did not have pre-charged loops. There were no statistically significant differences in the post-withdrawal and post-discharge survival rates of ECPR patients among different age groups, regions, and hospitals (all P>0.05). The top 5 difficulties in implementing ECPR in non-ICU environments were lack of ECMO machines (16 times), difficulty in placing CPR pipes (15 times), long time intervals between CPR and ECMO transfer (13 times), lack of conventional backup ECMO loops (10 times), and inability of ECMO emergency teams to quickly arrive at the site (5 times). Conclusion: ECPR has been gradually developed in the field of pediatric critical care in China, and needs to be further standardized. ECPR in non-ICU environment remains a challenge.


Subject(s)
Child , Female , Humans , Male , Aftercare , Cardiopulmonary Resuscitation/methods , Cross-Sectional Studies , Death, Sudden, Cardiac/prevention & control , East Asian People , Heart Arrest/therapy , Patient Discharge , Retrospective Studies , Surveys and Questionnaires
16.
Chinese Journal of Pediatrics ; (12): 983-988, 2023.
Article in Chinese | WPRIM | ID: wpr-1013212

ABSTRACT

Objective: To analyze the efficacy and safety of the sodium channel blockers (SCB) antiseizure medication in the treatment of focal epilepsy in infants under 6 months of age. Methods: This was a case series study. Infants with focal epilepsy with onset within 6 months of age and treated with SCB attending the Department of Neurology of Beijing Children's Hospital from June 2016 to April 2022 were collected. The clinical data, auxiliary examinations, SCB application, efficacy, adverse reactions, and prognosis were analyzed retrospectively. Patients were grouped according to type of seizure and epileptic syndrome, age of onset and etiology. Chi square test and Fisher exact test were used to analyze the differences between groups statistically. Results: A total of 118 infants were enrolled, 65 males and 53 females, with an age of epilepsy onset of 56 (4, 114) days. Developmental and epileptic encephalopathy was diagnosed in 60 infants, 39 had self-limited neonatal and (or) infantile epilepsy, and 19 had non-syndromic focal epilepsy. Application of SCB: 106 used oxcarbazepine, 2 used lacosamide, 9 switched from oxcarbazepine to lacosamide or a combination of 2 SCB, and 1 used oxcarbazepine, lacosamide, and lamotrigine successively; oxcarbazepine was the first choice in 46 cases. The age at which SCB was applied was 103 (53, 144) days. The children were followed up for 6 months to 6 years. SCB was effective in 89 cases (75.4%), including 70 cases (59.3%) who achieved seizure freedom. The seizure-free rate was higher in the focal epilepsy only group than in the group with other seizure types (64.4% (65/101) vs. 4/17, χ²=9.99, P<0.05). The responder and seizure-free rates were all higher in the group with the onset age of >3-6 months than the group >1-3 months (84.4% (38/45) vs. 62.5% (20/32), 73.3% (33/45) vs. 46.9% (15/32), χ²=4.85 and 5.58, both P<0.05). With the exception of variants in the PRRT2 gene, those with variants in sodium or potassium channels had higher responder and seizure-free rates than those with variants in other genes(86.2% (25/29) vs. 45.5% (10/22), 62.1% (18/29) vs. 22.7% (5/22), χ²=9.65 and 7.82,both P<0.05). The most common adverse event was transient hyponatremia, which happened in 66 cases (55.9%). There were 9 cases of rash, which subsided in 6 cases after discontinuing oxcarbazepine and switching to lacosamide, and 7 cases of electrocardiogram abnormalities, which improved after withdrawing oxcarbazepine and changing to lacosamide in 1 case. Conclusion: SCB are effective and tolerable in the treatment of focal epilepsy in infants under 6 months of age, with better efficacy in patients with genetic variants of the sodium or potassium channel, focal seizures only, and seizure onset >3-6 months of age.


Subject(s)
Child , Female , Male , Infant, Newborn , Humans , Infant , Sodium Channel Blockers/adverse effects , Oxcarbazepine , Lacosamide , Retrospective Studies , Epilepsies, Partial/drug therapy , Seizures , Sodium , Anticonvulsants/adverse effects
17.
Chinese Journal of Hematology ; (12): 906-910, 2023.
Article in Chinese | WPRIM | ID: wpr-1012255

ABSTRACT

Objective: To compare digital polymerase chain reaction (dPCR) and real-time quantitative PCR (qPCR) measurements of BCR::ABL (P210) mRNA expression in patients with chronic myeloid leukemia (CML) . Methods: In this non-interventional, cross-sectional study, BCR::ABL (P210) mRNA was simultaneously measured by dPCR and qPCR in peripheral blood samples collected from patients with CML who underwent tyrosine kinase inhibitor therapy and who achieved at least a complete cytogenetic response from September 2021 to February 2023 at Peking University People's Hospital. The difference, correlation, and agreement between the two methods were evaluated using the Wilcoxon signed-rank test, Spearman's correlation, and Bland-Altman analysis, respectively. Results: In total, 459 data pairs for BCR::ABL mRNA expression measured by dPCR and qPCR from 356 patients with CML were analyzed. There was a significant difference in BCR::ABL mRNA expression between the two methods (P<0.001). When analyzed by the depth of the molecular response (MR), a significant difference only existed for patients with ≥MR4.5 (P<0.001). No significant difference was observed for those who did not achieve a major MR (no MMR; P=0.922) or for those who achieved a major MR (MMR; P=0.723) or MR4 (P=0.099). There was a moderate correlation between the BCR::ABL mRNA expression between the two methods (r=0.761, P<0.001). However, the correlation gradually weakened or disappeared as the depth of the MR increased (no MMR: r=0.929, P<0.001; MMR: r=0.815, P<0.001; MR4: r=0.408, P<0.001; MR4.5: r=0.176, P=0.176). In addition, the agreement in BCR::ABL mRNA expression between the two methods in those with MR4.5 was weaker than other groups (no MMR: ▉= 0.042, P=0.846; MMR:▉=0.054, P=0.229; MR4:▉=-0.020, P=0.399; MR4.5:▉=-0.219, P<0.001) . Conclusions: dPCR is more accurate than qPCR for measuring BCR::ABL (P210) mRNA expression in patients with CML who achieve a stable deep MR.


Subject(s)
Humans , Cross-Sectional Studies , Cytogenetics , Leukemia, Myelogenous, Chronic, BCR-ABL Positive/genetics , Real-Time Polymerase Chain Reaction , RNA, Messenger/genetics
18.
Chinese Journal of Otorhinolaryngology Head and Neck Surgery ; (12): 111-116, 2023.
Article in Chinese | WPRIM | ID: wpr-971417

ABSTRACT

Objective: To reclassify the flat type sudden deafness according to the types of audiogram shape, and to explore the correlation between different pattern of hearing loss and prognosis. Methods: All of 1 024 patients with unilateral sudden deafness (492 males and 532 females, aged from 19 to 65 years, with an average age of 41.2 years old) admitted to 33 hospitals nationwide from August 2007 to October 2011 were divided into four types according to Chinese Guideline of Sudden Deafness(2015): low-frequency, high-frequency, flat and total deafness. Then, 402 patients with flat type sudden deafness were further divided into ascending type, descending type and consistent type according to the audiogram shapes. First, we compared the clinical characteristics and prognosis among these three subtypes of flat deafness, then compared the clinical characteristics and prognosis between ascending flat deafness and low-frequency deafness, descending flat deafness and high-frequency deafness, consistent flat deafness and total deafness, explored the factors related to the prognosis of flat deafness. SPSS 21.0 software, ANOVA, χ2 test, t-test and Logistic regression were used to analyze the data. Results: The cure rates of flat ascending, flat descending and flat consistent sudden deafness groups were 70.7%, 17.1% and 34.0% respectively, with a statistically significant difference (χ2=33.984, P<0.001); However, there was no significant difference in age, sex and affected side (all P>0.05). The independent related factors for the recovery of flat type sudden deafness were as follows: whether there was dizziness [OR=0.459; 95% confidence interval (CI): 0.271-0.777], the type of audiogram shape (OR=0.721; 95%CI: 0.530-0.981), and days from onset to therapy (OR=0.903, 95%CI: 0.835-0.978), all of which had P values<0.05. There was no significant difference in the cure rates between ascending flat sudden deafness and low-frequency descending sudden deafness, descending flat sudden deafness and high-frequency descending sudden deafness (all P>0.05). The pure tone average(PTA) of flat consistent sudden deafness and total deafness were (69.1±18.9) and (101.7±17.7) dB HL, respectively, with a statistically significant difference (t=20.890, P<0.001), and the cure rates were 34.0% and 14.5%, respectively, with a statistically significant difference (χ2=29.012, P<0.001). Conclusion: According to the audiogram shape, the flat type sudden deafness can be further divided into ascending flat sudden deafness, descending flat sudden deafness and consistent flat sudden deafness, which can more effectively evaluate the prognosis. The cure rate of ascending flat sudden deafness is similar to that of low-frequency sudden deafness, and the prognosis is well; The cure rate of descending flat sudden deafness is similar to that of high-frequency descending sudden deafness, and the prognosis is poor. The cure rate of consistent flat sudden deafness is higher than that of total deafness. PTA plays an important role in the prognosis of consistent flat sudden deafness and total deafness. Total deafness can be regarded as a single type of sudden deafness.


Subject(s)
Male , Female , Humans , Adult , Young Adult , Middle Aged , Aged , Hearing Loss, Sudden , Deafness , Hearing Tests , Prognosis , Vertigo , Hearing Loss, Unilateral , Hearing Loss, Sensorineural
19.
Chinese Journal of Clinical Thoracic and Cardiovascular Surgery ; (12): 106-112, 2023.
Article in Chinese | WPRIM | ID: wpr-953766

ABSTRACT

@#Objective    To explore the natural changes of procalcitonin (PCT) in the early period after pediatric cardiac surgery with cardiopulmonary bypass (CPB). Methods    A prospective and observational study was done on patients below 3 years of age, who underwent cardiac surgery involving CPB, with the risk adjustment of congenital heart surgery (RACHS) score of 2 to 5 and free from active preoperative infection or inflammatory disease. Blood samples for measurement of PCT, C-reactive protein (CRP) and white blood cell (WBC) were taken before surgery and daily for 7 days in postoperative period. Infections and complications within 7 days after operation were investigated. According to the presence or absence of infection and complications within 7 days after operation, the enrolled children were divided into an infection+complications group, a simple infection group, a simple complication group, and a normal group. Results     Finally, 429 children with PICU stay≥ 4 days were enrolled, including 268 males and 161 females, with a median age of 8.0 (0.7, 26.0) months. There were 145 children in the simple infection group, 38 children in the simple complication group, 230 children in the normal group and 16 children in the infection+complications group. The levels of PCT, CRP and WBC were significantly higher after CPB. CRP and WBC peaked on the second postoperative day (POD) and remained higher than normal until POD7. PCT peaked on POD1 and would generally decrease to normal on POD5 if without infection and complications. Age, body weight, RACHS scores, the duration of CPB and aortic cross-clamping time were correlated with PCT level. There was a statistical difference in PCT concentration between the simple infection group and the normal group on POD 3-7 (P<0.01) and a statistical difference between the simple complication group and the normal group on POD 1-7 (P<0.01). A statistical difference was found between the simple infection group and the simple complication group in PCT on POD 1-5 (P<0.05). Conclusion    WBC, CRP and PCT significantly increase after CPB in pediatric cardiac surgery patients. The factors influencing PCT concentration include age, weight, RACHS scores, CPB and aortic cross-clamping time, infection and complications.

20.
Chinese Journal of Reparative and Reconstructive Surgery ; (12): 782-787, 2023.
Article in Chinese | WPRIM | ID: wpr-981668

ABSTRACT

OBJECTIVE@#To investigate the short-term effectiveness of calcaneal lateral displacement osteotomy with lateral ligament repair in the treatment of Takakura stage Ⅱ varus-type ankle arthritis.@*METHODS@#A retrospective analysis was performed on the clinical data of 13 patients with Takakura stage Ⅱ varus-type ankle arthritis treated with calcaneal lateral displacement osteotomy with lateral ligament repair between January 2016 and December 2020. There were 6 males and 7 females aged 31-65 years, with an average age of 53.6 years. The preoperative tibial-ankle surface angle (TASA) was (88.13±1.01)°, medial distal tibial angle (MDTA) was (86.36±1.49)°, tibial talar surface angle (TTSA) was (6.03±1.63)°, talar tilting angle (TTA) was (81.95±2.15)°, and tibiocalcaneal axis angle (TCAA) was (-5.74±6.81)°. The preoperative American Orthopedic Foot and Ankle Society (AOFAS) score was 56.3±7.1 and the pain visual analogue scale (VAS) score was 3.7±0.5. AOFAS scores, VAS scores, TTSA, TTA, and TCAA were compared between pre- and post-operatively.@*RESULTS@#All 13 patients were followed up 14-41 months, with an average of 28.7 months. The osteotomies healed in all patients. The last follow-up revealed TTA, TTSA, and TCAA to be (88.27±1.19)°, (-0.13±1.37)°, and (2.09±5.10)° respectively, the AOFAS score was 84.3±4.2 and the VAS score was 0.7±0.5, all showing significant improvement when compared to preoperative values ( P<0.05).@*CONCLUSION@#For patients with Takakura stage Ⅱ varus-type ankle arthritis, calcaneal lateral displacement osteotomy with lateral ligament repair can correct the lower limb force line, regain ankle stability, and achieving good short-term effectiveness.


Subject(s)
Male , Female , Humans , Middle Aged , Ankle , Retrospective Studies , Ankle Joint/surgery , Osteoarthritis/surgery , Osteotomy , Collateral Ligaments , Treatment Outcome
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