1.Experts consensus on standard items of the cohort construction and quality control of temporomandibular joint diseases (2024)
Min HU ; Chi YANG ; Huawei LIU ; Haixia LU ; Chen YAO ; Qiufei XIE ; Yongjin CHEN ; Kaiyuan FU ; Bing FANG ; Songsong ZHU ; Qing ZHOU ; Zhiye CHEN ; Yaomin ZHU ; Qingbin ZHANG ; Ying YAN ; Xing LONG ; Zhiyong LI ; Yehua GAN ; Shibin YU ; Yuxing BAI ; Yi ZHANG ; Yanyi WANG ; Jie LEI ; Yong CHENG ; Changkui LIU ; Ye CAO ; Dongmei HE ; Ning WEN ; Shanyong ZHANG ; Minjie CHEN ; Guoliang JIAO ; Xinhua LIU ; Hua JIANG ; Yang HE ; Pei SHEN ; Haitao HUANG ; Yongfeng LI ; Jisi ZHENG ; Jing GUO ; Lisheng ZHAO ; Laiqing XU
Chinese Journal of Stomatology 2024;59(10):977-987
Temporomandibular joint (TMJ) diseases are common clinical conditions. The number of patients with TMJ diseases is large, and the etiology, epidemiology, disease spectrum, and treatment of the disease remain controversial and unknown. To understand and master the current situation of the occurrence, development and prevention of TMJ diseases, as well as to identify the patterns in etiology, incidence, drug sensitivity, and prognosis is crucial for alleviating patients′suffering.This will facilitate in-depth medical research, effective disease prevention measures, and the formulation of corresponding health policies. Cohort construction and research has an irreplaceable role in precise disease prevention and significant improvement in diagnosis and treatment levels. Large-scale cohort studies are needed to explore the relationship between potential risk factors and outcomes of TMJ diseases, and to observe disease prognoses through long-term follw-ups. The consensus aims to establish a standard conceptual frame work for a cohort study on patients with TMJ disease while providing ideas for cohort data standards to this condition. TMJ disease cohort data consists of both common data standards applicable to all specific disease cohorts as well as disease-specific data standards. Common data were available for each specific disease cohort. By integrating different cohort research resources, standard problems or study variables can be unified. Long-term follow-up can be performed using consistent definitions and criteria across different projects for better core data collection. It is hoped that this consensus will be facilitate the development cohort studies of TMJ diseases.
2.Sagittal splitting osteotomy of the mandibular outer cortex and autologous bone grafting for the treatment of hemifacial microsomia
Lai GUI ; Feng NIU ; Bing YU ; Jianfeng LIU ; Ying CHEN ; Xi FU ; Shixing XU ; Jia QIAO ; Qi JIN ; Yu HE ; Xuebing LIANG ; Lei CUI ; Fuhuan CHEN ; Qi CHEN
Chinese Journal of Plastic Surgery 2024;40(3):249-257
Objective:To investigate a new method for the reconstruction of hemifacial microsomia by sagittal osteotomy of the affected mandibular outer cortex combined with bone graft of mandibular outer cortex from healthy side.Methods:From March 2006 to March 2023, the clinical data of patients with hemifacial microsomia admitted to the Department of Craniomaxillofacial Surgery, Plastic Surgery Hospital, Chinese Academy of Medical Sciences were analyzed retrospectively. Preoperative diagnosis and surgical design were performed based on clinical manifestations and imaging findings. All cases were operated under general anesthesia. The affected mandibular outer cortex was previously split by an intraoral approach, and then the mandibular outer cortex of appropriate shape and size on the healthy side was harvested and grafted into the split bone space according to the preoperative design, following by internal rigid fixation. Complications, facial appearance improvement, and patient satisfaction were followed up. Photographs were taken preoperative, immediately postoperative and at the long-term(last) postoperative follow-up, and the severity of the deformity was analyzed. CT data from preoperative, immediate postoperative, and long-term follow-up visits were imported into Surgicase Proplan medical three-dimensional image workstation in Dicom format. The mandible was reconstructed using Segmentation, and the thickness of the mandible was measured during pre-operative, immediate post-operative and long-term follow-up visits. Anova with repeated measurement design was used to compare measurements and LSD test was used for multiple comparisons. The Kruskal-Wallis rank sum test were used to statistically analyze malformation severity. P< 0.05 is considered statistically significant. Results:A total of 39 patients were included in this study, including 13 females and 26 males, with an average age of (22.21±4.57) years (15-27 years). All patients were followed up for an average of (45.56±39.41) months (6-153 months) after surgery. The grafted mandibular outer cortex grows well with the adjacent bone tissue, and the mandibular angle and mandibular body are significantly wider. Of the 39 cases, 1 developed an infection 1 year after surgery, the titanium plate was exposed, and the patient healed after debridement and removal of the immobilizing splint. The facial appearance of the other patients improved significantly. Preoperative, immediate postoperative and long term follow up of mandibular thickness measurements were compared in pairs, and the differences were statistically significant (all P<0.05). The patient’s appearance satisfaction score: the preoperative score was [2.0(1.5, 2.0)] points, the immediate postoperative score was [4.0(4.0, 4.0)] points, the score of the last postoperative follow up was [4.0(4.0, 4.0)] points. There was statistical difference in satisfaction among the three groups ( P<0.01). The preoperative scores were compared with the scores of the immediate postoperative and the last postoperative follow-up respectively, and the differences were statistically significant( P<0.01). There was no statistical significance in satisfaction between the immediate postoperative score and the score of the last postoperative follow up ( P>0.05). Conclusion:The sagittal splitting osteotomy of the mandibular outer cortex is consistent with the features of mandibular anatomy, and provides a good condition for the grafting and healing of autogenous bone. Removing the outer cortex of the mandible on the healthy side not only increases the thickness of the affected side, but also decreases the width of the angle of the mandible on the healthy side, so as to effectively correct the asymmetric deformity of the mandible. The method is simple, with few complications and good results, and is one of the ideal treatments to correct hemofacial microsomia.
3.Correlation analysis between Pirani score and talo-navicular angle,calcaneo-cuboid angle and tibio-calcaneall angle of infant clubfoot under ultrasound
Wenjing WANG ; Bing XIA ; Yingmei DONG ; Panpan HE ; Zhiwei CHENG ; Fengqun MA ; Chaohua WANG ; Fuyun LIU ; Weiming HU ; Feipeng WANG ; Yufeng ZHAO ; Hezhou LI ; Jiale FU
Chinese Journal of Surgery 2024;62(3):210-215
Objective:To explore the evaluation effect of ultrasonography and Pirani score on tarsal deformity, treatment effect and pseudo-correction of congenital clubfoot in infants and young children, and the correlation between the two methods.Methods:This is a retrospective case series study. The clinical data of 26 children (40 feet) with congenital clubfoot who were evaluated by ultrasonography in the Third Affiliated Hospital of Zhengzhou University from January 2020 to January 2023 were retrospectively collected. There were 16 males and 10 females. The age at the first ultrasound examination was ( M(IQR)) 9.0 (18.0) days (range: 1 to 46 days). All patients were treated with Ponseti method by the same physician. The Pirani scores before and after treatment and at the last examination, and the talonavicular angle, calcaneocuboid angle and tibiocalcaneal angle measured by ultrasound were collected, and the treatment and follow-up were recorded. Paired sample t test, repeated measures analysis of variance or Kruskal-Wallis test were used for data comparison, and Spearman correlation analysis was used for correlation analysis. The receiver operating characteristic curve was used to calculate the efficacy of ultrasound in evaluating different Pirani scores. Results:The number of plaster fixation in 26 children was 4.0 (1.0) times (range: 2 to 8 times). The medial talonavicular angle and posterior tibiocalcaneal angle were significantly improved after treatment and at the last follow-up compared with those before treatment, and the differences were statistically significant (all P<0.01). There was no difference in lateral calcaneocuboid angle before and after treatment and at the last follow-up ( F=1.971, P>0.05). Pseudo-correction occurred in 2 cases (2 feet) during the treatment, with an incidence of 5%. Correlation analysis showed that there was a moderate positive correlation between talonavicular angle and Pirani midfoot score ( r=0.480, P<0.01). There was no correlation between calcaneocuboid angle and Pirani midfoot score ( r=0.114, P=0.105). There was a moderate negative correlation between tibial heel angle and Pirani hindfoot score ( r=-0.566, P<0.01). The cut-off point of Pirani midfoot score of 1.5 was 38.78°, the sensitivity was 0.90, the specificity was 0.56, and the area under the curve was 0.75. The cut-off value of angle was 27.51 °, the sensitivity was 0.16, the specificity was 0.92, and the area under the curve was 0.44.The cut-off points of Pirani midfoot score of 3.0 were 45.08°and 9.96°, the sensitivity was 0.94 and 0.91, the specificity was 0.37 and 0.42, and the area under the curve was 0.59 and 0.62, respectively. The cut-off values of Pirani hindfoot score of 2.0 and 3.0 were 167.46° and 160.15°, respectively. The sensitivity was 0.75 and 0.67, the specificity was 0.81 and 0.83, and the area under the curve was 0.78 and 0.71, respectively. Conclusion:Ultrasound can complement with Pirani score, visually and dynamically observe the morphology and position changes of talonavicular joint, calcaneocuboid joint and tibiotalocalcaneal joint, monitor the recovery and pseudo-correction of tarsal bones, and better evaluate the therapeutic effect.
4.Risk factors of cognitive dysfunction and its relationship with poor prognosis in elderly hypertensive pa-tients with frailty
Can ZHAO ; Shan-Shan LIU ; Jing-Bing LI ; Shao-Ying WANG ; Li-Di WANG ; Jun-Fu LIU
Chinese Journal of cardiovascular Rehabilitation Medicine 2024;33(4):391-394
Objective:To investigate the risk factors of cognitive dysfunction and its relationship with poor prognosis in elderly hypertensive patients with frailty.Methods:A total of 150 elderly hypertensive patients with frailty trea-ted in Eighth People's Hospital of Hebei Province from July 2019 to March 2021 were selected.According to pres-ence of cognitive dysfunction,they were divided into normal cognition group(n=92)and cognitive dysfunction group(n=58),and general data and prognosis condition were collected in two groups.Multivariate Logistic regres-sion analysis was used to analyze the risk factors of cognitive dysfunction in elderly hypertensive patients with frail-ty;Spearman correlation analysis was used to analyze the correlation between cognitive dysfunction degree and prog-nosis.Results:Compared with normal cognition group,cognitive dysfunction group was significantly older and pos-sessed significant higher proportions of diabetes,coronary heart disease,stroke,hyponatremia,multiple medication and malnutrition(P<0.05 or<0.01).Multivariate Logistic regression analysis indicated that age ≥75 years,stroke,multiple medication and malnutrition were independent risk factors for cognitive dysfunction in elderly hy-pertensive patients with frailty(OR=2.804~6.434,P<0.05 or<0.01).Incidence rate of poor prognosis events in cognitive dysfunction group was significantly higher than that of normal cognition group(51.72%vs.11.96%,P<0.001).Spearman correlation analysis showed that cognitive dysfunction was significant positively correlated with poor prognosis in these patients(r=0.435,P<0.001).Conclusion:Age,stroke,multiple medication and malnu-trition are independent risk factors for cognitive dysfunction in elderly hypertensive patients with frailty.Cognitive dysfunction is closely related to poor prognosis in these patients.
5.Nutritional status of pediatric patients undergoing allogeneic hematopoietic stem cell transplantation
Mei YAN ; Wei-Bing TANG ; Yong-Jun FANG ; Jie HUANG ; Ting ZHU ; Jin-Yu FU ; Xiao-Na XIA ; Chang-Wei LIU ; Yuan-Yuan WAN ; Jian PAN
Parenteral & Enteral Nutrition 2024;31(5):257-261
Objective:To observe the changes in the nutritional status of pediatric patients after allogeneic hematopoietic stem cell transplantation(allo-HSCT)for one year,and to analyze the risk factors.Methods:We collected data from 88 pediatric patients who underwent allo-HSCT at the Department of Hematology and Oncology in Children's Hospital of Nanjing Medical University between May 2018 and November 2022.All pediatric patients underwent nutritional status analysis before transplantation,at enrollment,3 months,6 months and 1 year after allo-HSCT.Linear regression model was used to analyze the risk factors for growth rate.Results:The body mass index Z score(BMI-Z)before allo-HSCT was(0.096±1.349),and decreased to(-0.258±1.438)、(-0.715±1.432)、(-0.584±1.444)at enrollment,3 months,6 months after allo-HSCT,and(-0.130±1.317)at 1 year after allo-HSCT(P<0.001).There was no significant change in BMI-Z between pre-transplantation and 1 year after transplantation(P=1.000).Height for age Z score(HAZ)before transplantation was(0.137±1.305)and decreased to(-0.083±1.267)、(-0.221±1.299)、(-0.269±1.282)in 3 months,6 months and 1 year after allo-HSCT(P<0.001).Multivariate linear regression showed that age≥10 years old(P=0.015)and chronic graft-versus-host disease(cGVHD)(P=0.005)were independent risk factors for change in HAZ.Conclusion:The BMI-Z of pediatric patients treated with allo-HSCT returned to the pre-transplantation level after one year,while HAZ continued to decrease.Allo-HSCT may cause impaired growth rate in pediatric patients.Attention should be paid to HAZ changes in pediatric patients before and after allo-HSCT,especially in pediatric patients≥10 years old of age and those with cGVHD.Effective nutritional intervention should be provided in time.
6.Correlation analysis between Pirani score and talo-navicular angle,calcaneo-cuboid angle and tibio-calcaneall angle of infant clubfoot under ultrasound
Wenjing WANG ; Bing XIA ; Yingmei DONG ; Panpan HE ; Zhiwei CHENG ; Fengqun MA ; Chaohua WANG ; Fuyun LIU ; Weiming HU ; Feipeng WANG ; Yufeng ZHAO ; Hezhou LI ; Jiale FU
Chinese Journal of Surgery 2024;62(3):210-215
Objective:To explore the evaluation effect of ultrasonography and Pirani score on tarsal deformity, treatment effect and pseudo-correction of congenital clubfoot in infants and young children, and the correlation between the two methods.Methods:This is a retrospective case series study. The clinical data of 26 children (40 feet) with congenital clubfoot who were evaluated by ultrasonography in the Third Affiliated Hospital of Zhengzhou University from January 2020 to January 2023 were retrospectively collected. There were 16 males and 10 females. The age at the first ultrasound examination was ( M(IQR)) 9.0 (18.0) days (range: 1 to 46 days). All patients were treated with Ponseti method by the same physician. The Pirani scores before and after treatment and at the last examination, and the talonavicular angle, calcaneocuboid angle and tibiocalcaneal angle measured by ultrasound were collected, and the treatment and follow-up were recorded. Paired sample t test, repeated measures analysis of variance or Kruskal-Wallis test were used for data comparison, and Spearman correlation analysis was used for correlation analysis. The receiver operating characteristic curve was used to calculate the efficacy of ultrasound in evaluating different Pirani scores. Results:The number of plaster fixation in 26 children was 4.0 (1.0) times (range: 2 to 8 times). The medial talonavicular angle and posterior tibiocalcaneal angle were significantly improved after treatment and at the last follow-up compared with those before treatment, and the differences were statistically significant (all P<0.01). There was no difference in lateral calcaneocuboid angle before and after treatment and at the last follow-up ( F=1.971, P>0.05). Pseudo-correction occurred in 2 cases (2 feet) during the treatment, with an incidence of 5%. Correlation analysis showed that there was a moderate positive correlation between talonavicular angle and Pirani midfoot score ( r=0.480, P<0.01). There was no correlation between calcaneocuboid angle and Pirani midfoot score ( r=0.114, P=0.105). There was a moderate negative correlation between tibial heel angle and Pirani hindfoot score ( r=-0.566, P<0.01). The cut-off point of Pirani midfoot score of 1.5 was 38.78°, the sensitivity was 0.90, the specificity was 0.56, and the area under the curve was 0.75. The cut-off value of angle was 27.51 °, the sensitivity was 0.16, the specificity was 0.92, and the area under the curve was 0.44.The cut-off points of Pirani midfoot score of 3.0 were 45.08°and 9.96°, the sensitivity was 0.94 and 0.91, the specificity was 0.37 and 0.42, and the area under the curve was 0.59 and 0.62, respectively. The cut-off values of Pirani hindfoot score of 2.0 and 3.0 were 167.46° and 160.15°, respectively. The sensitivity was 0.75 and 0.67, the specificity was 0.81 and 0.83, and the area under the curve was 0.78 and 0.71, respectively. Conclusion:Ultrasound can complement with Pirani score, visually and dynamically observe the morphology and position changes of talonavicular joint, calcaneocuboid joint and tibiotalocalcaneal joint, monitor the recovery and pseudo-correction of tarsal bones, and better evaluate the therapeutic effect.
7.Rhabdomyolysis and related organ damage in new recruits during military training under high temperature and humidity environment
Jian SHEN ; Xin ZHANG ; Henan LIU ; Boning ZHOU ; Ying LI ; Yang JIAO ; Bing LIU ; Xiaolai ZHENG ; Zhenhong FU
Journal of Army Medical University 2024;46(10):1063-1067
Objective To explore the possibility of rhabdomyolysis and related organ damage in new recruits training in high temperature and high humidity environment by comparing the effects of different training environments on the laboratory indicators and electrocardiogram.Methods A total of 250 new recruits from a unit in Beijing and another 250 ones from a unit in Hainan were recruited and assigned into conventional environment group and high temperature and high humidity environment group,respectively.All of them were male,with an average age of 21.36±2.59 years.Before and in 4 weeks after training in the same subjects,their general information,blood and urine indicators,and electrocardiogram were collected.All data were statistically analyzed.Results The incidences of inflammatory reaction,myocardial injury,muscle injury,liver injury,and kidney injury were 45.76%,3.39%,12.71%,25.42%,and 12.71%,respectively,in the high temperature and high humidity environment group,which were all significantly higher than those in the conventional environment group(P<0.05).The former group had an incidence rate of 6.78%,5.93%,8.47%,6.78%,and 2.54%,respectively,in sinus bradycardia,atrial premature beats,unspecific ST-T changes,left ventricular hypertension and short PR interval,and all of the rates were higher than those of the conventional environment group(P<0.05).Conclusion The incidences of inflammatory reaction,myocardial injury,muscle injury,liver injury,kidney injury,and ECG abnormalities are more common in new recruits after military training in high temperature and high humidity environment.
8.Sagittal splitting osteotomy of the mandibular outer cortex and autologous bone grafting for the treatment of hemifacial microsomia
Lai GUI ; Feng NIU ; Bing YU ; Jianfeng LIU ; Ying CHEN ; Xi FU ; Shixing XU ; Jia QIAO ; Qi JIN ; Yu HE ; Xuebing LIANG ; Lei CUI ; Fuhuan CHEN ; Qi CHEN
Chinese Journal of Plastic Surgery 2024;40(3):249-257
Objective:To investigate a new method for the reconstruction of hemifacial microsomia by sagittal osteotomy of the affected mandibular outer cortex combined with bone graft of mandibular outer cortex from healthy side.Methods:From March 2006 to March 2023, the clinical data of patients with hemifacial microsomia admitted to the Department of Craniomaxillofacial Surgery, Plastic Surgery Hospital, Chinese Academy of Medical Sciences were analyzed retrospectively. Preoperative diagnosis and surgical design were performed based on clinical manifestations and imaging findings. All cases were operated under general anesthesia. The affected mandibular outer cortex was previously split by an intraoral approach, and then the mandibular outer cortex of appropriate shape and size on the healthy side was harvested and grafted into the split bone space according to the preoperative design, following by internal rigid fixation. Complications, facial appearance improvement, and patient satisfaction were followed up. Photographs were taken preoperative, immediately postoperative and at the long-term(last) postoperative follow-up, and the severity of the deformity was analyzed. CT data from preoperative, immediate postoperative, and long-term follow-up visits were imported into Surgicase Proplan medical three-dimensional image workstation in Dicom format. The mandible was reconstructed using Segmentation, and the thickness of the mandible was measured during pre-operative, immediate post-operative and long-term follow-up visits. Anova with repeated measurement design was used to compare measurements and LSD test was used for multiple comparisons. The Kruskal-Wallis rank sum test were used to statistically analyze malformation severity. P< 0.05 is considered statistically significant. Results:A total of 39 patients were included in this study, including 13 females and 26 males, with an average age of (22.21±4.57) years (15-27 years). All patients were followed up for an average of (45.56±39.41) months (6-153 months) after surgery. The grafted mandibular outer cortex grows well with the adjacent bone tissue, and the mandibular angle and mandibular body are significantly wider. Of the 39 cases, 1 developed an infection 1 year after surgery, the titanium plate was exposed, and the patient healed after debridement and removal of the immobilizing splint. The facial appearance of the other patients improved significantly. Preoperative, immediate postoperative and long term follow up of mandibular thickness measurements were compared in pairs, and the differences were statistically significant (all P<0.05). The patient’s appearance satisfaction score: the preoperative score was [2.0(1.5, 2.0)] points, the immediate postoperative score was [4.0(4.0, 4.0)] points, the score of the last postoperative follow up was [4.0(4.0, 4.0)] points. There was statistical difference in satisfaction among the three groups ( P<0.01). The preoperative scores were compared with the scores of the immediate postoperative and the last postoperative follow-up respectively, and the differences were statistically significant( P<0.01). There was no statistical significance in satisfaction between the immediate postoperative score and the score of the last postoperative follow up ( P>0.05). Conclusion:The sagittal splitting osteotomy of the mandibular outer cortex is consistent with the features of mandibular anatomy, and provides a good condition for the grafting and healing of autogenous bone. Removing the outer cortex of the mandible on the healthy side not only increases the thickness of the affected side, but also decreases the width of the angle of the mandible on the healthy side, so as to effectively correct the asymmetric deformity of the mandible. The method is simple, with few complications and good results, and is one of the ideal treatments to correct hemofacial microsomia.
9.Clinical effects of Yiqi Shengjin Prescription on patients with primary Sj?gren's syndrome due to Dual Deficiency of Qi and Yin
Zhong-Fu TANG ; Chuan-Bing HUANG ; Li-Li CHENG ; Jun-Jie CHEN ; Shuang-Shuang SHANG ; Si-Di LIU ; Ming LI
Chinese Traditional Patent Medicine 2024;46(6):1857-1861
AIM To investigate the clinical effects of Yiqi Shengjin Prescription on patients with primary Sj?gren's syndrome due to Dual Deficiency of Qi and Yin.METHODS Ninety-two patients were randomly assigned into control group(46 cases)for 8-week intervention of conventional treatment,and observation group(46 cases)for 8-week intervention of both Yiqi Shengjin Prescription and conventional treatment.The changes in clinical effects,TCM syndrome scores,ESSDAI score,ESSPRI score,salivary flow rate,immunoinflammatory indices(ESR,hs-CRP,IgG,C3,C4),SAS score,SDS score,T lymphocytes(CD4+,CD8+,Treg,Th17)and safety indices were detected.RESULTS The observation group demonstrated higher total effective rate than the control group(P<0.05).After the treatment,the observation group exhibited decreased TCM syndrome scores(P<0.05),which was lower than those in the control group(P<0.05);the two groups displayed decreased ESSDAI score,ESSPRI score,ESR,hs-CRP,IgG,SAS score,SDS score,CD8+,Th17(P<0.05),and increased salivary flow rate,C3,C4,Treg(P<0.05),especially for the observation group(except for CD4+)(P<0.05).No obvious adverse reactions were observable in the two groups.CONCLUSION For the patients with primary Sjogren's syndrome due to Deficiency of Qi and Yin,Yiqi Shengjin Prescription can safely and effectively improve dry mouth and eye symptoms,and reduce inflammatory indices,whose mechanism may contribute to the regulation of T lymphocyte balance in vivo.
10.Analysis of the effects of low/intermediate dose of coagulation factor Ⅷ on 30 adult patients with severe hemophilia A in a single center.
Yan Hui YUAN ; Pei Pei XU ; Yue Yi XU ; Sha LIU ; Xiao Yan SHAO ; Wei Jing ZHANG ; Li GONG ; Min ZHOU ; Bing CHEN ; Rong Fu ZHOU
Chinese Journal of Hematology 2023;44(1):38-42
Objective: To evaluate the clinical effects of low- and intermediate-dose factor Ⅷ (F Ⅷ) prophylaxis in Chinese adult patients with severe hemophilia A. Methods: Thirty adult patients with severe hemophilia A who received low- (n=20) /intermediate-dose (n=10) F Ⅷ prophylaxis at Nanjing Drum Tower Hospital affiliated with Nanjing University Medical College were included in the study. The annual bleeding rate (ABR), annual joint bleeding rate (AJBR), number of target joints, functional independence score of hemophilia (FISH), quality of life score, and health status score (SF-36) before and after preventive treatment were retrospectively analyzed and compared. Results: The median follow-up was 48 months. Compared with on-demand treatment, low- and intermediate-dose prophylaxis significantly reduced ABR, AJBR, and the number of target joints (P<0.05) ; the improvement in the intermediate-dose prophylaxis group was better than that in the low-dose prophylaxis group (P<0.05). Compared with on-demand treatment, the FISH score, quality of life score, and SF-36 score significantly improved in both groups (P<0.05), but there was no significant difference between the two groups (P>0.05) . Conclusion: In Chinese adults with severe hemophilia A, low- and intermediate-dose prophylaxis can significantly reduce bleeding frequency, delay the progression of joint lesions, and improve the quality of life of patients as compared with on-demand treatment. The improvement in clinical bleeding was better with intermediate-dose prophylaxis than low-dose prophylaxis.
Humans
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Hemophilia A/drug therapy*
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Factor VIII/therapeutic use*
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Quality of Life
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Retrospective Studies
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Hemarthrosis/prevention & control*
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Hemorrhage/drug therapy*

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