RESUMO
Thoracic paravertebral nerve block(TPVB)is a regional anesthesia technique that pro-vides ipsilateral somatosensory,motor and sympathetic nerves block segmentally by injecting local anesthetics in the paravertebral space.In recent years,there has been an increasing number of studies on the use of TPVB technique for anesthesia and analgesia in pediatric thoracic and upper abdominal surgery,showing good perioperative analgesic efficacy.This article intends to provide a review of the current applica-tion and progress of TPVB technique for pediatric perioperative analgesia in terms of medication regimens,drug diffusion routes,block methods,clinical application,and complications.
RESUMO
BACKGROUND AND AIM@#Remnant cholesterol (remnant-C) mediates the progression of major adverse cardiovascular events. It is unclear whether remnant-C, and particularly cumulative exposure to remnant-C, is associated with nonalcoholic fatty liver disease (NAFLD). This study aimed to explore whether remnant-C, not only baseline but cumulative exposure, can be used to independently evaluate the risk of NAFLD.@*METHODS@#This study included 1 cohort totaling 21,958 subjects without NAFLD at baseline who underwent at least 2 repeated health checkups and 1 sub-cohort totaling 2,649 subjects restricted to those individuals with at least 4 examinations and no history of NAFLD until Exam 3. Cumulative remnant-C was calculated as a timeweighted model for each examination multiplied by the time between the 2 examinations divided the whole duration. Cox regression models were performed to estimate the association between baseline and cumulative exposure to remnant-C and incident NAFLD.@*RESULTS@#After multivariable adjustment, compared with the quintile 1 of baseline remnant-C, individuals with higher quintiles demonstrated significantly higher risks for NAFLD (hazard ratio [HR] 1.48, 95%CI 1.31-1.67 for quintile 2; HR 2.07, 95%CI 1.85-2.33 for quintile 3; HR 2.55, 95%CI 2.27-2.88 for quintile 4). Similarly, high cumulative remnant-C quintiles were significantly associated with higher risks for NAFLD (HR 3.43, 95%CI 1.95-6.05 for quintile 2; HR 4.25, 95%CI 2.44-7.40 for quintile 3; HR 6.29, 95%CI 3.59-10.99 for quintile 4), compared with the quintile 1.@*CONCLUSION@#Elevated levels of baseline and cumulative remnant-C were independently associated with incident NAFLD. Monitoring immediate levels and longitudinal trends of remnant-C may need to be emphasized in adults as part of NAFLD prevention strategy.
Assuntos
Adulto , Humanos , Estudos de Coortes , Hepatopatia Gordurosa não Alcoólica/etiologia , Colesterol , Modelos de Riscos Proporcionais , Fatores de RiscoRESUMO
ObjectiveTo investigate the clinical effect of Guanxin Shutong capsules （GXSTC） on coronary heart failure with heart blood stasis. MethodA total of 98 coronary heart failure patients with heart blood stasis who visited the expert clinics of the Affiliated Hospital of Liaoning University of Traditional Chinese Medicine from June 2020 to August 2022 were selected. They were randomized into a control group and an observation group， with 49 in each group. The two groups were given standardized treatment according to the guidelines for coronary heart failure. On this basis， the observation group was treated with Chinese patent medicine GXSTC. The cardiac structural and functional indicators， total effective rate of symptoms， exercise tolerance， heart rate variability （HRV）， quality of life and inflammatory factor levels of the two groups before and after treatment were compared. ResultBefore treatment， there was no notable difference in high-sensitivity C-reactive protein （hs-CRP）， six minute walk test （6MWT）， minnesota living with heart failure questionnaire （MLHFQ） score， left ventricular ejection fraction （LVEF）， tumor necrosis factor-α （TNF-α）， left ventricular end-diastolic diameter （LVEDD）， N-terminal pro-brain natriuretic peptide （NT-proBNP） and HRV between the two groups， and thus the data were comparable. After treatment， the observation group had higher effective rates of traditional Chinese medicine （TCM） symptoms （Z=-2.362， P<0.05） and New York heart association class （NYHA） functional classes （Z=-2.175， P<0.05） than the control group. The two groups presented decreased LVEDD， TNF-α， NT-proBNP and hs-CRP （P<0.05， P<0.01） while increased MLHFQ， 6MWT and HRV（P<0.05，P<0.01） after treatment as compared with those before treatment， and the observation group had more decrease or increase than the control group （P<0.05， P<0.01）. ConclusionGXSTC combined with standardized treatment relieves the physical symptoms of coronary heart failure patients with heart blood stasis， improves exercise tolerance and quality of life， reduces inflammatory reaction， increases HRV， and improves cardiac structure and function.
RESUMO
@#Objective To analyse the consistency of perioperative self-reported pain scores of lung cancer patients with clinical records to provide a basis for optimal pain management. Methods The patients with lung cancer who underwent surgical treatment in the Department of Thoracic Surgery, Sichuan Cancer Hospital from November 2017 to January 2020 were selected. They were divided into two groups based on the source of pain data. The self-report group used a questionnaire in which patients self-reported their pain scores, and the pain scores for the clinical record group were extracted from the electronic medical record system. Kappa test was used to compare the concordance of pain scores between the two groups preoperatively, on postoperative 1-6 days and on the day of discharge. McNemar's paired χ2 test was used to compare the differences in pain intensity levels between the two groups. Binary logistic multi-factor regression was used to analyse the factors influencing the concordance of severe pain (7-10 points) between the two groups. Results Totally 354 patients were collected, including 191 males and 163 females, with an average age of 55.64± 10.34 years. The median postoperative hospital stay was 6 days. The consistency of pain scores between the two groups was poor (Kappa=–0.035 to 0.262, P<0.05), and the distribution of pain levels at each time point was inconsistent and statistically significant (P<0.001). The percentage of inconsistent severe pain assessment ranged from 0.28% to 35.56%, with the highest percentage of inconsistent severe pain assessment on postoperative day 1 (35.56%). Single-port thoracoscopic surgical access was an influencing factor for inconsistent assessment of severe pain on postoperative day 3 (OR=2.571, P=0.005). Conclusion Self-reported perioperative pain scores of lung cancer patients are poorly aligned with clinical records. Clinical measures are needed to improve the accuracy of patient pain data reporting by choosing the correct assessment method, increasing education, and developing effective quality control measures.
RESUMO
Hypersplenism is a common complication caused by liver cirrhosis and portal hypertension, and at present, splenectomy and partial splenic artery embolization (PSE) are the main methods for the treatment of hypersplenism. Splenectomy has a marked effect in the treatment of hypersplenism and can significantly improve the clinical symptoms of patients with hypersplenism. Compared with splenectomy, PSE causes partial splenic parenchymal infarction and thus achieve similar clinical efficacy as partial splenectomy while preserving the spleen and its function. Although PSE is an effective method for the treatment of hypersplenism, there are few reports on the effect of PSE on liver fibrosis, immunity, and liver regeneration in China and globally. This article summarizes the common causes of hypersplenism, the mechanism of PSE in the treatment of hypersplenism, the therapeutic effect of different embolization methods and materials, and the effect of PSE on liver fibrosis, immunity, and liver regeneration, so as to provide a theoretical basis and new ideas for the clinical treatment of hypersplenism.
RESUMO
@#The number of investigator initiated research (IIR) is increasing. But the recognition and management of IIR in China is still in its infancy, and there is a lack of specific and operable guidance for the implementation process. Based on our practical experiences, previous literature reports, and current policy regulations, the authors took prospective IIR as an example to summarize the implementation process of IIR into 14 steps, which are as the following: study initiation, ethical review, study registration, study filing, case report form design, database establishment, standard operating procedure making, investigator training, informed consent, data collection, data entry, data verification, data locking and data archiving.
RESUMO
Objective:To investigate the expression of WT1 gene in children with acute lymphoblastic leukemia (ALL), and explore its clinical characteristics and correlation with the prognosis of ALL.Methods:The clinical data of 183 children with newly diagnosed ALL in Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology from January 2015 to May 2019 were retrospectively analyzed. The expression level of WT1 gene in bone marrow samples was detected by real-time fluorescence quantitative polymerase chain reaction. The children were followed up to June 2021 with a median follow-up time of 46 months (0 to 63 months).Results:Among 183 children with ALL, the WT1 gene positive was in 130 cases (71.04%), and the expression level was 1.41% (0.26%, 6.73%); WT1 gene negative was in 53 cases (28.96%). The expression levels of WT1 gene in children with T-cell lymphoblastic leukemia (T-ALL), non-hyperdiploid and middle/high-risk were significantly increased, and there were statistical differences ( P<0.05 or <0.01); however, there were no statistical differences in the expression levels of WT1 gene between children with different gender, chromosome karyotype, hepatosplenomegaly and the first diagnosis white blood cell count ( P>0.05). There were no statistical differences in complete remission rate and recurrence rate after induction chemotherapy between WT1 gene positive children and WT1 gene negative children: 87.69% (114/130) vs. 86.79% (46/53) and 16.15% (21/130) vs. 18.87% (10/53), P>0.05. By the end of follow-up, 179 children were followed up, and there was no statistical difference in survival rate between WT1 gene positive children and WT1 gene negative children: 89.68% (113/126) vs. 86.79% (46/53), P>0.05. Among the children with WT1 gene positive, relapse was in 21 cases, and there was no statistical difference in the expression level of WT1 gene after complete remission or after relapse, compared with that while the first diagnosis ( P>0.05); among non-relapse children, 96 completed the detection, the expression level of WT1 gene after complete remission was significantly lower than the first diagnosis: 0.17% (0.04%, 0.49%) vs. 2.01% (0.41%, 8.82%), and there was statistical difference ( P<0.01). Kaplan-Meier survival curve analysis result showed there was no statistical difference in survival time between WT1 gene positive children and WT1 gene negative children ( P>0.05). According to the median expression level of WT1 gene (1.41%), the children with WT1 gene positive were divided into high expression (66 cases) and low expression (64 cases), there was no statistical difference in survival time between high expression children and low expression children ( P>0.05). Conclusions:WT1 gene is commonly expressed in children with ALL and is associated with some clinical features and prognosis of the children. Decreased WT1 gene expression may result in better prognosis.
RESUMO
@#Telephone follow-up is one of the important ways to follow up patients. High-quality follow-up can benefit both doctors and patients. However, clinical research-related follow-up is often faced with problems such as time-consuming, laborious and poor patient compliance. The authors belong to a team that has been committed to the study of patient-reported outcomes for a long time. The team has carried out long-term follow-up of symptoms, daily function and postoperative complications of more than 1 000 patients after lung cancer surgery, and accumulated certain experience. In this paper, the experience of telephone follow-up was summarized and discussed with relevant literatures from the aspects of clarifying the purpose of clinical research follow-up, understanding the needs of patients in follow-up, and using follow-up skills.
RESUMO
@#Objective To investigate the current status of constipation during postoperative hospitalization and the factors associated with moderate to severe constipation at discharge in lung cancer patients. Methods Lung cancer patients who underwent surgery in 6 tertiary hospitals in Sichuan Province from November 2017 to January 2020 were enrolled. The MD Anderson Symptom Scale-Lung Cancer Module was used to collect postoperative constipation scores. Unconditional logistic stepwise regression was used to analyze the related influencing factors for moderate to severe constipation on the day of discharge. Results Finally 337 patients were collected. There were 171 males and 166 females, with an average age of 55.0±10.3 years. Constipation scores of lung cancer patients increased from postoperative day 1 to day 3, and showed a decreasing trend from day 3 to day 7. Moderate to severe constipation was present in 68 (20.2%) patients at discharge. The postoperative hospital stay (OR=0.743, P<0.001) and the dose of morphine used during postoperative hospitalization (OR=1.002, P=0.015) were influencing factors for moderate to severe constipation at discharge in lung cancer patients. Conclusion Lung cancer patients have the most severe constipation on postoperative day 3. Moderate to severe constipation at discharge is associated with the postoperative hospital stay and the dose of morphine used during postoperative hospitalization.
RESUMO
Objective:To investigate the influencing factors of delayed methotrexate (MTX) elimination after high-dose methotrexate (HD-MTX) treatment in children with acute lymphoblastic leukemia (ALL) and the effects of delayed MTX elimination and HD-MTX reduction on the prognosis of children with ALL.Methods:The clinical data of 242 children with ALL diagnosed and treated in Tongji Hospital of Tongji Medical College of Huazhong University of Science and Technology from January 2015 to June 2020 in accordance with the Chinese Children's Cancer Group study ALL 2015 (CCCG-ALL 2015) were retrospectively analyzed. Low risk and intermediate/high risk children respectively received 3 g/m 2 and 5 g/m 2 HD-MTX for 4 times, and the serum MTX concentration was monitored. The serum MTX concentration > 1 μmol/L at 44 h of administration was considered as the delayed elimination, which was divided into mild (> 1 μmol/L and ≤ 5 μmol/L), moderate (> 5 μmol/L and ≤ 10 μmol/L) and severe (> 10 μmol/L) delayed elimination. Univariate and multivariate logistic regression analysis were used to analyze the influencing factors of delayed MTX elimination, and univariate Cox proportional hazards model was used to analyze the related factors of ALL relapse. Results:The 242 children with ALL completed 962 times of HD-MTX chemotherapy. The median serum MTX concentration [ M ( Q1, Q3)] at 44 h of administration was 0.45 μmol/L (0.33 μmol/L, 0.72 μmol/L). The total incidence of delayed MTX elimination was 17.7% (170/962). The incidence of mild, moderate and severe delayed elimination was 13.8% (133/962), 2.6% (25/962) and 1.2% (12/962), respectively. Logistic regression analysis showed that age ≥ 7 years old ( OR = 1.68, 95% CI 1.17-2.41, P = 0.005), MTX dose >3 g/m 2 at each course ( OR = 2.14, 95% CI 1.52-3.03, P < 0.001) and the first course of HD-MTX chemotherapy ( OR = 2.05, 95% CI 1.43-2.93, P < 0.001) were independent risk factors for delayed MTX elimination. The median follow-up time was 50 months (34 months, 68 months), 12.8% (31/242) of the children relapsed, and the median relapse time was 30 months (30 months, 39 months). Univariate Cox regression analysis showed that there were no significant differences in the relapse rates among children with different gender, immunophenotype, risk, the number of delayed MTX elimination, and the completion of HD-MTX chemotherapy (the ratio of MTX average dose to initial planned dose) (all P > 0.05). Conclusions:The independent risk factors of delayed elimination of MTX in children with ALL are age ≥ 7 years old, MTX dose > 3 g/m 2 at each course and the first course of HD-MTX chemotherapy. Delayed elimination of MTX and reduction of HD-MTX have no significant effect on ALL relapse.
RESUMO
Objective:To investigate the clinical features of acute megakaryocytic leukemia (AMKL) in children.Methods:The clinical data of 14 children with AMKL in Tongji Hospital of Tongji Medical College of Huazhong University of Science and Technology from January 2012 to July 2021 were retrospectively analyzed, and the related literature was reviewed.Results:Among 14 children with AMKL, there were 5 males and 9 females, and the median age of onset was 19 months (0.1-109 months); 1 case was Down syndrome-related AMKL, and 13 cases were non-Down syndrome-related AMKL. Most of the children presented with fever, anemia or bleeding symptoms, and a few patients presented with joint pain as the primary symptom. Some children were accompanied by extramedullary infiltration such as hepatomegaly, splenomegaly or lymphadenovarix. Initial investigations of 14 children showed that the median white blood count, hemoglobin concentration and platelet count were 10.67×10 9/L [(6.56-83.62)×10 9/L], 84 g/L (55-121 g/L), 37×10 9/L [(8-1443) ×10 9/L], respectively, and the median proportion of naive cells in peripheral blood was 0.09 (0.00-0.79). Bone marrow smear showed that the primitive megakaryocytes were characterized by various size and irregular form, a few of which had cytoplasmic vacuoles, and the median proportion of bone marrow primitive megakaryocytes was 0.636 (0.332-0.976); the nuclei were round or irregular, with multiple nucleoli or hidden nucleoli. RAS staining was partially positive, and immunohistochemical assay showed that POX, AS-DNCE and α-NBE were negative. Detection of megakaryocyte-associated antigens by flow cytometry showed 12 children expressed CD41a or CD61, and 10 children expressed CD42b. Among 3 children who completed chemotherapy, 1 case of Down syndrome-related AMKL and 1 case of non-Down syndrome-related AMKL were event-free survival, and 1 case of non-Down syndrome-related AMKL died after bone marrow relapse. Conclusions:The clinical manifestations and biological characteristics of children with AMKL are complicated and the prognosis is poor. Some children can achieve disease-free survival through chemotherapy alone.
RESUMO
Objective:To explore the clinical features and prognosis of childhood acute lymphoblastic leukemia(ALL) complicated with EB virus (EBV) infection.Methods:The results of detection of EBV antibody and EBV-DNA in peripheral blood mononuclear cells of 196 children with ALL diagnosed in Tongji Hospital Affiliated to Tongji Medical College of Huazhong University of Science and Technology from January 2015 to January 2019 were collected. According to the results, 196 children with ALL were divided into EBV infection group and non-EBV infection group. The hepatomegaly and splenomegaly, chromosome, peripheral blood routine, immunophenotyping, clinical risk, secondary infection during chemotherapy, minimal residual disease (MRD) of day 46 after chemotherapy, karyotype, and prognosis were compared between the two groups. The children were followed up until April 30, 2019.Results:Among 196 children with ALL, EBV infection rate was 72.96% (143/196). The EBV-DNA level [median ( P25, P75)] of peripheral blood mononuclear cells was 3.7×10 3 copies/L(1.6×10 3 copies/L, 8.8×10 3 copies/L). The incidence of hepatosplenomegaly (subcostal ≥ 5 cm) in EBV infection group was higher than that in non-EBV infected group [14.69% (21/143) vs. 3.77% (2/53), χ 2= 4.45, P= 0.035]. There was no significant difference in the number of white blood cells and the incidence of abnormal karyotype between EBV infection group and non-EBV infection group (both P > 0.05). The secondary infection rate in EBV infection group was higher than that in the non-EBV infection group [41.96% (60/143) vs.24.53% (13/53), χ2= 5.03, P= 0.025], and the remission rate of day 46 in EBV-infection group was lower than that in non-EBV infection group [80.42% (115/143) vs. 98.11% (52/53), χ2= 9.60, P= 0.020]. The recurrence rate in EBV-infection group was higher than that in non-EBV infectious group [11.89% (17/143) vs. 1.89% (1/53), χ2= 4.64, P= 0.031], and there was a significant difference in the component ratio of immunophenotyping and clinical risk between the two groups (both P < 0.05). Conclusions:The hepatosplenomegaly in children with ALL complicated with EBV infection is obvious, the secondary infection rate is high, the remission rate is low, the recurrence rate is high, and the prognosis is poor. EBV infection may be related to immunophenotyping and clinical risk in children with ALL, and has nothing to do with the abnormal karyotypes.
RESUMO
Objective:To explore the correlation between cerebrovascular hemodynamic index (CVHI) accumulative score and subclinical arteriosclerosis indicators.Methods:A total of 27 184 cases were collected from the Health Management Center,the Third Xiangya Hospital,Central South University.Linear regression analysis was carried out to confirm the correlations between CVHI accumulative score and the modified Framingham stroke profile (FSP),as well as between CVHI accumulative score and cerebrovascular diseases (ICVD) scale.The correlation between CVHI accumulative score and brachial-ankle pulse wave velocity (baPWV),carotid plaque orcarotid intima-media thickness (CIMT) was analyzed by multifactor logistic regression model in 11 580 cases.Moreover,the correlation between CVHI accumulative score and microalbuminuria or serum cystatin C was performed by multifactor logistic regression model in 9 860 cases.Results:In this study,the people whose CVHI accumulative score was less than 75 accounted for 12.98％.The CVHI accumulative score was negatively related with the modified FSP score (r=-0.484,P＜0.01) or ICVD score (r=-0.455,P＜0.01).The multifactor logistic regression model found that the baPWV,carotid plaque,microalbuminuria and serum cystatin C were independent predictors for CVHI accumulative score.Conclusion:The CVHI accumulative score is correlated with the modified FSP score,ICVD score and indexes of subclinical arteriosclerosis (baPWV,carotid plaque,microalbuminuria and serum cystatin C).The CVHI accumulative score could be used as a tool for zero-level and primary prevention of cerebral stroke.
RESUMO
Objective To study the clinical features and prognostic analysis of 36 children with relapsed acute lymphoblastic leukemia(ALL)treated with the ALL 2006 protocol.Methods The data of 308 children who were new-ly diagnosed as ALL at the Department of Pediatric Hematology,Tongji Hospital,Tongji Medical College,Huazhong Uni-versity of Science Technology treated with the ALL 2006 protocol between January 2007 and December 2012 were col-lected,and the clinical features and prognosis of 36 children with relapsed ALL were retrospectively analyzed.The date included initial treatment time,age,gender,immunophenotyping,the white blood cell count,the risk classification,the chromosome,the fusion gene(29 kinds of fusion genes including MLL rearrangement,BCR/ABL,E2A/PBX1,TEL/AML1)in relapsed patients with ALL on the initial diagnosis and recurrence time,recurrence site,as well as whether to get second complete remission(CR2),follow-up time,follow-up deadline condition.Results After treatment with ALL 2006 protocol,the recurrence rate was 11.7%(36/308 cases);the 3 years overall survival rate was(38.0 ± 9.0)%.The recurrence happened almost in the very early stage,about 75.0%(27/36 cases),and the recurrence rate of the early stage and the late stage patients was 16.7%(16/36 cases)and 8.3%(3/36 cases),respectively.The re-lapsed sites were mainly in the bone marrow alone(66.7%,24/36 cases),and the extramedullary recurrence and the combination of bone marrow with extramedullary recurrence was 16.7%(6/36 cases).The 3-year OS was(16.4 ± 8.0)% and(80.0 ± 18.0)% for those relapsed in the very early stage and early stage,respectively(P=0.002).The 3-year OS of the high-risk,medium-risk relapsed patients were(21.0 ± 11.0)%,(51.9 ± 16.0)% and(64.3 ± 21.0)%(P=0.022).Conclusions After the treatment with ALL 2006 protocol,the patients with recurrence were almost at the very early stage.The relapsed sites were mainly in bone marrow alone.The factor associated with survival time of children with relapsed ALL is the relapse at the very early stage.
RESUMO
Objective To explore the application value and clinical effect of the bi-directional quality control module for Surgical Package in artificial femoral head replacement. Methods A total of 360 patients undergoing artificial femoral head replacement in our hospital from June 2015 to June 2017 were selected and divided into observation group and control group, 180 cases in each group. The observation group,used bi-directional quality control module of surgical bag, while the control group used conventional management model of surgical bag . The operation time, the incidence of surgical bag, postoperative complications and postoperative satisfaction were compared. Results The average operation time in the observation group was (69.2 ± 11.6)min, the average operation time in the control group was (76.8 ± 14.5)min, P<0.01. The total defect rate in the observation group was 2.2% . The total defect rate in the control group was 22.2%, P<0.01; the observation group had less severe pain, incision split, fever, postoperative bleeding, incision infection than the control group, P<0.05. Patient satisfaction score was (89.38±7.83) points in the observation group, (79.18±5.55) points in the control group, P<0.01. Conclusions The bi-directional quality control module of surgical package can shorten the operation time, reduce the defect surgical packages, reduce the incidence of postoperative complications , improve the quality of operation, and improve patient satisfaction in the application of artificial femoral head replacement. It is worthy of clinical application.
RESUMO
Objective To discuss the clinical effect of normal saline flushing and heparinized saline flushing in invasive arterial blood pressure monitoring in the critical patients. Methods A total of 140 critical patients were randomly divided into the experimental group and the control group with 70 cases in each group according to the envelope method. The experimental group used normal saline for tube flushing, the control group used heparinized saline (5 U/ml) for tube flushing. The incidence of complications and coagulation index were compared between two groups. Results There was no statistically difference in the incidence of the catheter jam and local infection (P>0.05). The incidence of bleeding events was 14.3% (10/70) in the control group, which was higher than 2.9% (2/70) of the experimental group. The difference was statistically significant(χ2=4.466, P<0.05). The activated partial thromboplastin time, prothrombin time of the control group were (32.22 ± 4.22) s, (11.56 ± 2.12) s, which were longer than (29.84 ± 3.64) s, (10.66 ± 1.48) s of the experimental group. And international normalized ratio of the control group was 1.12±0.14, which was higher than 1.03±0.20 of the experimental group. The difference was statistically significant (t values were-3.573,-2.912,-3.084, P <0.05). Conclusions The normal saline flushing liquid was more safer and reliable in the invasive arterial blood pressure monitoring.
RESUMO
Objective@#To analyze the mutation type of FⅧ gene in children with hemophilia A and to explore the relationship among hemophilia gene mutation spectrum, gene mutation and clinical phenotype.@*Method@#Sixty-two children with hemophilia A from Department of Pediatric Hematology, Tongji Hospital of Tongji Medical College, Huazhong University of Science and Technology between January 2015 and March 2017 were enrolled. All patients were male, aged from 4 months to 7 years and F Ⅷ activity ranged 0.2%-11.0%. Fifty cases had severe, 10 cases had moderate and 2 cases had mild hemophilia A. DNA was isolated from peripheral blood in hemophilia A children and the target gene fragment was amplified by PCR, in combination with the second generation sequencing, 22 and 1 introns were detected. Negative cases were detected by the second generation sequencing and results were compared with those of the international FⅧ gene mutation database.@*Result@#There were 20 cases (32%) of intron 22 inversion, 2 cases (3%) of intron 1 inversion, 18 cases (29%) of missense mutation, 5 cases (8%) of nonsense mutation, 7 cases (11%) of deletion mutation, 1 case(2%)of splice site mutation, 2 cases (3%) of large fragment deletion and 1 case of insertion mutation (2%). No mutation was detected in 2 cases (3%), and 4 cases (7%) failed to amplify. The correlation between phenotype and genotype showed that the most common gene mutation in severe hemophilia A was intron 22 inversion (20 cases), accounting for 40% of severe patients, followed by 11 cases of missense mutation (22%). The most common mutation in moderate hemophilia A was missense mutation (6 cases), accounting for 60% of moderate patients.@*Conclusion@#The most frequent mutation type in hemophilia A was intron 22 inversion, followed by missense mutation, again for missing mutation. The relationship between phenotype and genotype: the most frequent gene mutation in severe hemophilia A is intron 22 inversion, followed by missense mutation; the most frequent gene mutation in medium hemophilia A is missense mutation.
RESUMO
OBJECTIVE:To provide reference for further promoting the clinical application of chronopharmacology and rational drug use.METHODS:The self-designed questionnairea was used to conduct on-site investigation among participants from 40 hospitals in Anhui province by dint of a meeting.The survey data were analyzed statistically.RESULTS:Totally 170 questionnaires were distributed,and 166 were retrieved,the total response rate was 97.65％.93.37％ of the interviewed pharmacists were aware of the circadian rhythms,and 87.35％ were aware of the circadian rhythms of more than two kinds;interviewed pharmacists expressed better awareness of indicators like gastric acid secretion (75.30％),blood pressure (67.47％) and growth hormone (66.87％) etc.while their awareness of other diseases like anaphylactic rhinitis (66.87％),gastric ulcer (50.60％) and migraine (50.00％) remained to improve.All the interviewed pharmacists recognized the better therapeutic efficacy (84.34％) and lower side effect (81.33％) of chronopharmacology-referred medication;chronopharmacology characteristics of glucocorticoid drugs were best known (34.34％),followed by antihypertensive drugs (25.30％).CONCLUSIONS:Pharmacists in Anhui province have certain knowledge of chronopharmacology such as biological rhythms,biological process or indicators,biological rhythms of disease and advantage of chronopharmacology-referred medication.It indicates that there has been a foundation for popularizing the concept of chronopharmacology in clinical medication in China;but not often contacted physiological processes or indexes,the understanding of biological rhythm of uncommon diseases are still insufficient,and the understanding of chronopharmacology characteristics of various drugs are far from ideal.
RESUMO
Objective To investigate the changes of cerebrovascular hemodynamic accumulative score in health checkup population and their influencing factors.Methods A total of 1 840 people aged 23-88 years were checked by CVHI and accumulative scores were calculated.The general information,blood biochemistry,highly sensitive C-reaction protein (hs-CRP),homocysteine (Hcy),carotid intima-media thickness (CIMT) and brachial-ankle pulse wave velocity (baPWV) were also collected during the corresponding period.Results The proportion of abnormal CVHI accumulative score (＜75 points) was increasing with age.Compared with the normal CVHI accumulative score (≥75 points) group,the proportion of hypertensive disease,diabetes and stroke family history was significantly higher in the abnormal group (P＜ 0.05).In addition,the levels of age,body mass index (BMI),waist circumference (WC),systolic blood pressure (SBP),diastolic blood pressure (DBP),triglyceride (TG),low density lipoprotein cholesterol (LDL-C),fasting plasma glucose (FPG),hs-CRP,Hcy,CIMT and baPWV were also significantly increased in the abnormal group (P＜0.05).Correlation analysis showed a negative correlation between CVHI accumulative score and age,history of hypertension,diabetes,BMI,WC,SBP,DBP,LDL-C,TG,FPG,hs-CRP,Hcy,baPWV (P＜ 0.05),but a positive correlation with HDL-C (P＜0.05).Multiple factor analysis determined that age [OR(95％C1):1.017(1.002-1.033)],history of hypertension [OR(95％CI):1.510(1.096-2.081)],BMI [OR(95％CI):1.235(1.135-1.344)],SBP [OR(95％CI):1.044(1.030-1.059)],LDL-C [OR(95％CI):1.673(1.444-1.937)],TG [OR(95％CI):1.116(1.035-1.204)],hs-CRP [OR(95％CI):1.037(1.003-1.073)],Hcy [OR(95％CI):1.099(1.062-1.136)],baPWV [OR(95％CI):1.001(1.000-1.002)] were being selected into the regression model (P＜0.05),and they were independent influencing factors of decreased CVHI accumulative scores.Conelusion The decreased CVHI accumulative score has a significant relationship with exposure levels of risk factors in stroke.Therefore,we conclude that the detection of CVHI is a convenient and feasible method to screen high risk individuals for stroke in middle and old aged population undergoing medical examination.
RESUMO
Objective To study the computed tomography(CT)manifestations of involvement of bridging septa in the perirenal space(BSPS)during acute pancreatitis(AP)and its correlation with extrapancreatic inflam-mation on abdominal computed tomography(EPICT)score. Methods 106 patients with acute pancreatitis were included in this study. Emphasis was placed on CT findings of BSPS and the EPICT score in all the patients. Results The EPICT score was 4 to 7 in 67 patients,and the EPICT score was 0 to 3 in 39 patients. BSPS involve-ment was shown in all the patients. The left or right BSPS involved in 102 patients and 98 patients,respectively. Thickening of the BSPS was shown as strip shadow with slightly higher density and hazy border;fluid collection of the BSPS was shown as liquid density with hazy border. The involvement of BSPS showed a statistically significant association with the EPICT score in the AP patients(r=0. 703,P<0.01). Conclusion BSPS involved by acute pancreatitis is shown as a strip shadow with slightly higher density or as liquid density with hazy border on CT images, reflecting the severity of the acute pancreatitis.