Background Tuberculosis (TB) infection has been reported to have a possible relationship with the occurrence and clinical course of Takayasu arteritis (TA). We aimed to describe the characteristics of TB in a large po...Background Tuberculosis (TB) infection has been reported to have a possible relationship with the occurrence and clinical course of Takayasu arteritis (TA). We aimed to describe the characteristics of TB in a large population of TA patients. Methods We included a total of 1105 patients with TA, who were hospitalized between January 1992 and December 2017. Comparisons of clinical features were made according to the presence of TB. Results Among the 1105 patients, 109 (9.9%) had TB, including 53 patients (48.6%) diagnosed with TB before the onset of TA, 23 (21.1%) with a concurrent diagnosis of TB and TA, and 24 patients (22.0%) who developed TB after TA. Pulmonary TB was the most frequently identified (97 patients, 89.0%). Patients with TB had more frequent involvement of the pulmonary artery and experienced more chest discomfort and constitutional symptoms but had less interventional treatment. Demographic characteristics, comorbid diseases, and use of steroids were similar between patients with and without TB. Conclusions The proportion of Chinese TA patients with TB was not low, and about half of the patients had TB before TA. Pulmonary TB was the most common. Pulmonary artery involvement and pulmonary hypertension was more frequent in TA patients with TB.展开更多
Background Anemia is a common comorbidity of patients with Takayasu arteritis(TA). This study evaluated the prevalence, clinical characteristics, and treatment in Chinese TA patients with anemia. Methods This retrospe...Background Anemia is a common comorbidity of patients with Takayasu arteritis(TA). This study evaluated the prevalence, clinical characteristics, and treatment in Chinese TA patients with anemia. Methods This retrospective study included 533 consecutive patients hospitalized for TA from January 2009 to April 2018. Anemia was diagnosed on the basis of hemoglobin level, according to World Health Organization criteria. Results A total of 194 patients(36.4%) were diagnosed with anemia. Most had mild anemia(177, 91.2%). Female patients were predominant(92.8% of anemic patients). Normocytic anemia(62.9%) was the most common pattern. Anemic patients were more likely than non-anemic patients to have dizziness(29.4% vs. 21.2%), low body mass index(22.0 ± 3.6 vs. 22.9 ± 3.4 kg/m2), and active disease stage(64.9% vs. 50.1%);pulmonary involvement(12.4% vs. 26.8%), pulmonary hypertension(12.9% vs. 20.1%) and pulmonary hypertensive-target drugs(2.8% vs. 11.6%) were less common among anemic than non-anemic patients(all P < 0.05). Larger left ventricular end-diastolic diameter and lower left ventricular ejection fraction were observed in anemic patients. Over a median follow-up of four months, the increase of hemoglobin in anemic patients was associated with the use of iron supplementation. Conclusions Anemia is a very common concurrent condition in TA, especially in young, female patients. Patients with anemia are more likely to be in the active disease stage. Iron supplementation helps increase hemoglobin.展开更多
Population aging has become a major challenge for the healthcare in China. More than 23 million Chinese are cur- rently ≥ 80 years, with an annual increase of 5%. The Chi- nese population of 80 years or older is expe...Population aging has become a major challenge for the healthcare in China. More than 23 million Chinese are cur- rently ≥ 80 years, with an annual increase of 5%. The Chi- nese population of 80 years or older is expected to reach 30.67 million by 2020 and 74 million by 2040.展开更多
OBJECTIVE To analyze the causes of renal artery stenosis(RAS)and compare the clinical characteristics in accordance with the primary disease among patients aged from 30 to 50.METHODS Patients were grouped by etiologie...OBJECTIVE To analyze the causes of renal artery stenosis(RAS)and compare the clinical characteristics in accordance with the primary disease among patients aged from 30 to 50.METHODS Patients were grouped by etiologies of RAS.Groups were retrospectively examined and compared regarding demographic data,clinical manifestations,laboratory findings,and imaging findings.RESULTS A total of 152 patients(74 females,78 males;mean age:40.70±6.01 years)were enrolled,including 84 patients(55.3%)with atherosclerosis(AS),46 patients(30.3%)with Takayasu arteritis(TA),18 patients(11.8%)with fibromuscular dysplasia(FMD),and four patients(2.6%)with other etiologies.Patients in AS group had greater body mass index,higher prevalence of comorbidities and higher rate of smoking and drinking history.TA patients showed more constitutional symptoms and vascular findings,and higher erythrocyte sedimentation rate.RAS in both AS group and TA group mainly located on ostia and proximal segments,but RAS in FMD group mainly involved middle to distal segment of renal artery.The AS group had significantly lesser stenosis than the other groups.Although renal function evaluated by the estimated glomerular filtration rate did not significantly differ among the groups,the incidence of kidney shrinkage was significantly higher in the TA and FMD groups(39.1%and 50%,respectively)than in the AS group(8.3%).The FMD group had milder cardiac damage than other groups.CONCLUSIONS AS was the most common cause of RAS in patients aged from 30 to 50,followed by TA and FMD.The etiology of RAS should be carefully distinguished based on clinical manifestations,laboratory findings,and imaging to ensure that proper treatment is provided.展开更多
Platelet-derived growth factor receptor alpha （PDGFRct） is a marker of oligodendrocyte precursor cells in the central nervous system. NG2 is also considered a marker of oligodendrocyte precursor cells. However, whet...Platelet-derived growth factor receptor alpha （PDGFRct） is a marker of oligodendrocyte precursor cells in the central nervous system. NG2 is also considered a marker of oligodendrocyte precursor cells. However, whether there are differences in the distribution and morphol- ogy of oligodendrocyte precursor cells labeled by NG2 or PDGFRa in the developing neonatal rat brain remains unclear. In this study, by immunohistochemical staining, NG2 positive （NG2＋） cells were ubiquitous in the molecular layer, external pyramidal layer, internal pyramidal layer, and polymorphic layer of the cerebral cortex, and corpus callosum, external capsule, piriform cortex, and medial septal nucleus. NG2~ cells were stellate or fusiform in shape with long processes that were progressively decreased and shortened over the course of brain development. The distribution and morphology of PDGFRct positive （PDGFRa＋） cells were coincident with NG2＋ cells. The co- localization of NG2 and PDGFRu in the cell bodies and processes of some cells was confirmed by double immunofluorescence labeling. Moreover, cells double-labeled for NG2 and PDGFRa were predominantly in the early postnatal stage of development. The numbers of NG2＋/PDGFRa＋ cells and PDGFRa＋ cells decreased, but the number of NG2＋ cells increased from postnatal days 3 to 14 in the developing brain. In addition, amoeboid microglial cells of the corpus callosum, newborn brain macrophages in the normal developing brain, did not express NG2 or PDGFRu, but NG2 expression was detected in amoeboid microglia after hypoxia. The present results suggest that NG2 and PDGFRct are specific markers of oligodendrocyte precursor cells at different stages during early development. Additionally, the NG2 protein is involved in inflammatory and pathological processes of amoeboid microglial cells.展开更多
Coarctation of the aorta(CoA)refers to the congenital coarctation near the isthmus,ductus arteriosus or ligamentum arteriosus,with an incidence of 5%–10%in children with congenital heart defects.[1]It can be isolated...Coarctation of the aorta(CoA)refers to the congenital coarctation near the isthmus,ductus arteriosus or ligamentum arteriosus,with an incidence of 5%–10%in children with congenital heart defects.[1]It can be isolated or coexisted with ventricular septal defects,subaortic stenosis,patent ductus arteriosus,and mitral aortic valve.[2]CoA has a poor prognosis.Without intervention,the median age of death with aortic coarctation is 38 years[3]and 75%death of those patients is at the age of 46 years.[4]Causes of death included congestive heart failure(26%),aortic rupture(21%),bacterial endocarditis(18%),and intracranial hemorrhage(12%).[5]Therefore,early diagnosis and optimal treatment are the key to improve the prognosis of this disease.The aim of this report is underlining the importance of early and accurate diagnose of CoA as a cause of systemic hypertension in young patients and also emphasizing the genetic factors of CoA in twins.展开更多
FLNB encodes the protein filamin B(FLNB),which is expressed in chondrocytes of the human growth plate[1].To date,pathogenic mutations in the FLNB gene have solely been found to cause skeletal deformities,indicating th...FLNB encodes the protein filamin B(FLNB),which is expressed in chondrocytes of the human growth plate[1].To date,pathogenic mutations in the FLNB gene have solely been found to cause skeletal deformities,indicating the crucial role of FLNB in skeletal development.FLNB-related disorders include spondylocarpotarsal synostosis(SCT,OMIM:272,460).展开更多
Background： Takayasu arteritis （TA） is a rare inflammatory arteriopathy of unknown etiology. The aim of this study was to investigate the genetic susceptibility to TA in a Chinese population. Methods： Four single ...Background： Takayasu arteritis （TA） is a rare inflammatory arteriopathy of unknown etiology. The aim of this study was to investigate the genetic susceptibility to TA in a Chinese population. Methods： Four single nucleotide polymorphisms （SNPs） those locate in the IL12B region （rs56167332）, the MLX region （rs665268）, the FCGR2A/FCGR3A locus （rsi0919543）, and the HLA-B/M1CA locus （rs12524487）, associated with TA in different population, were genotyped in 123 Chinese TA patients and 147 healthy controls from January 2013 to August 2014. A Chi-square test was used to test for genotype/allele frequencies variants. Results： Among the four SNPs, rs 10919543 was found to be significantly associated with TA in the studied population. The GG genotype of rs 10919543 at the FCGR2A/FCGR3A locus is a high risk factor （odds ratio [OR] = 6.532, 95% confidence interval [C1] = 2.402 - 17.763, P 〈 0.001 ） for TA. Among TA patients, the level of eosinophil granulocytes （Eos） in the peripheral blood was observed to be higher in the GG group of rs 10919543 （n = 23, Eos = 0. I 1 [0.08, 0.17] x 109/L） than the GA ＋ AA group （n = 100, Eos = 0.08 [0.05, 0.13] 10/L, P = 0.028）. No correlation between the genotypes of the other three SNPs and TA patients was observed. Conclusions： Our findings revealed unique genetic pattern in Chinese TA patients that may be partly responsible for the higher risk of TA in this population. FCGR2A/FCGR3A-related immune disorder might contribute to the etiology of TA.展开更多
基金supported by the National Key Research and Development Program of China (2016YFC1300100)CAMS Innovation Fund for Medical Sciences (2016I2M-1-002)
文摘Background Tuberculosis (TB) infection has been reported to have a possible relationship with the occurrence and clinical course of Takayasu arteritis (TA). We aimed to describe the characteristics of TB in a large population of TA patients. Methods We included a total of 1105 patients with TA, who were hospitalized between January 1992 and December 2017. Comparisons of clinical features were made according to the presence of TB. Results Among the 1105 patients, 109 (9.9%) had TB, including 53 patients (48.6%) diagnosed with TB before the onset of TA, 23 (21.1%) with a concurrent diagnosis of TB and TA, and 24 patients (22.0%) who developed TB after TA. Pulmonary TB was the most frequently identified (97 patients, 89.0%). Patients with TB had more frequent involvement of the pulmonary artery and experienced more chest discomfort and constitutional symptoms but had less interventional treatment. Demographic characteristics, comorbid diseases, and use of steroids were similar between patients with and without TB. Conclusions The proportion of Chinese TA patients with TB was not low, and about half of the patients had TB before TA. Pulmonary TB was the most common. Pulmonary artery involvement and pulmonary hypertension was more frequent in TA patients with TB.
基金funded by the National Key Research and Development Program of China (2016YFC1300100)CAMS Innovation Fund for Medical Sciences (2016-I2M1-002)
文摘Background Anemia is a common comorbidity of patients with Takayasu arteritis(TA). This study evaluated the prevalence, clinical characteristics, and treatment in Chinese TA patients with anemia. Methods This retrospective study included 533 consecutive patients hospitalized for TA from January 2009 to April 2018. Anemia was diagnosed on the basis of hemoglobin level, according to World Health Organization criteria. Results A total of 194 patients(36.4%) were diagnosed with anemia. Most had mild anemia(177, 91.2%). Female patients were predominant(92.8% of anemic patients). Normocytic anemia(62.9%) was the most common pattern. Anemic patients were more likely than non-anemic patients to have dizziness(29.4% vs. 21.2%), low body mass index(22.0 ± 3.6 vs. 22.9 ± 3.4 kg/m2), and active disease stage(64.9% vs. 50.1%);pulmonary involvement(12.4% vs. 26.8%), pulmonary hypertension(12.9% vs. 20.1%) and pulmonary hypertensive-target drugs(2.8% vs. 11.6%) were less common among anemic than non-anemic patients(all P < 0.05). Larger left ventricular end-diastolic diameter and lower left ventricular ejection fraction were observed in anemic patients. Over a median follow-up of four months, the increase of hemoglobin in anemic patients was associated with the use of iron supplementation. Conclusions Anemia is a very common concurrent condition in TA, especially in young, female patients. Patients with anemia are more likely to be in the active disease stage. Iron supplementation helps increase hemoglobin.
文摘Population aging has become a major challenge for the healthcare in China. More than 23 million Chinese are cur- rently ≥ 80 years, with an annual increase of 5%. The Chi- nese population of 80 years or older is expected to reach 30.67 million by 2020 and 74 million by 2040.
基金This study was supported by the National Key Research Research and Development Plan of China(2016YFC1300100)the CAMS Innovation for Medical Science(2016-I2M-1-002)and the Non-profit Central Research Institute Fund of Chinese Academy of Medical Sciences(2019XK320035).
文摘OBJECTIVE To analyze the causes of renal artery stenosis(RAS)and compare the clinical characteristics in accordance with the primary disease among patients aged from 30 to 50.METHODS Patients were grouped by etiologies of RAS.Groups were retrospectively examined and compared regarding demographic data,clinical manifestations,laboratory findings,and imaging findings.RESULTS A total of 152 patients(74 females,78 males;mean age:40.70±6.01 years)were enrolled,including 84 patients(55.3%)with atherosclerosis(AS),46 patients(30.3%)with Takayasu arteritis(TA),18 patients(11.8%)with fibromuscular dysplasia(FMD),and four patients(2.6%)with other etiologies.Patients in AS group had greater body mass index,higher prevalence of comorbidities and higher rate of smoking and drinking history.TA patients showed more constitutional symptoms and vascular findings,and higher erythrocyte sedimentation rate.RAS in both AS group and TA group mainly located on ostia and proximal segments,but RAS in FMD group mainly involved middle to distal segment of renal artery.The AS group had significantly lesser stenosis than the other groups.Although renal function evaluated by the estimated glomerular filtration rate did not significantly differ among the groups,the incidence of kidney shrinkage was significantly higher in the TA and FMD groups(39.1%and 50%,respectively)than in the AS group(8.3%).The FMD group had milder cardiac damage than other groups.CONCLUSIONS AS was the most common cause of RAS in patients aged from 30 to 50,followed by TA and FMD.The etiology of RAS should be carefully distinguished based on clinical manifestations,laboratory findings,and imaging to ensure that proper treatment is provided.
基金supported by grants from the National Natural Science Foundation of China,No.31100769
文摘Platelet-derived growth factor receptor alpha （PDGFRct） is a marker of oligodendrocyte precursor cells in the central nervous system. NG2 is also considered a marker of oligodendrocyte precursor cells. However, whether there are differences in the distribution and morphol- ogy of oligodendrocyte precursor cells labeled by NG2 or PDGFRa in the developing neonatal rat brain remains unclear. In this study, by immunohistochemical staining, NG2 positive （NG2＋） cells were ubiquitous in the molecular layer, external pyramidal layer, internal pyramidal layer, and polymorphic layer of the cerebral cortex, and corpus callosum, external capsule, piriform cortex, and medial septal nucleus. NG2~ cells were stellate or fusiform in shape with long processes that were progressively decreased and shortened over the course of brain development. The distribution and morphology of PDGFRct positive （PDGFRa＋） cells were coincident with NG2＋ cells. The co- localization of NG2 and PDGFRu in the cell bodies and processes of some cells was confirmed by double immunofluorescence labeling. Moreover, cells double-labeled for NG2 and PDGFRa were predominantly in the early postnatal stage of development. The numbers of NG2＋/PDGFRa＋ cells and PDGFRa＋ cells decreased, but the number of NG2＋ cells increased from postnatal days 3 to 14 in the developing brain. In addition, amoeboid microglial cells of the corpus callosum, newborn brain macrophages in the normal developing brain, did not express NG2 or PDGFRu, but NG2 expression was detected in amoeboid microglia after hypoxia. The present results suggest that NG2 and PDGFRct are specific markers of oligodendrocyte precursor cells at different stages during early development. Additionally, the NG2 protein is involved in inflammatory and pathological processes of amoeboid microglial cells.
文摘Coarctation of the aorta(CoA)refers to the congenital coarctation near the isthmus,ductus arteriosus or ligamentum arteriosus,with an incidence of 5%–10%in children with congenital heart defects.[1]It can be isolated or coexisted with ventricular septal defects,subaortic stenosis,patent ductus arteriosus,and mitral aortic valve.[2]CoA has a poor prognosis.Without intervention,the median age of death with aortic coarctation is 38 years[3]and 75%death of those patients is at the age of 46 years.[4]Causes of death included congestive heart failure(26%),aortic rupture(21%),bacterial endocarditis(18%),and intracranial hemorrhage(12%).[5]Therefore,early diagnosis and optimal treatment are the key to improve the prognosis of this disease.The aim of this report is underlining the importance of early and accurate diagnose of CoA as a cause of systemic hypertension in young patients and also emphasizing the genetic factors of CoA in twins.
基金supported by the Suzhou Personnel Planning Project(GSWS2020046)the Suzhou Science and Technology Development Project(SKY2023007)+3 种基金the Research Project of Jiangsu Commission of Health(M2021082)the Summit Project of Clinical Medicine(ML13100523)awarded to Ting ChenIt’s also supported by the Jiangsu Provincial Maternal and Child Health Care Program(F202119)awarded to Hong-Ying Wangthe Suzhou Clinical enter for Rare Disease(Szlcyxzxj202105).
文摘FLNB encodes the protein filamin B(FLNB),which is expressed in chondrocytes of the human growth plate[1].To date,pathogenic mutations in the FLNB gene have solely been found to cause skeletal deformities,indicating the crucial role of FLNB in skeletal development.FLNB-related disorders include spondylocarpotarsal synostosis(SCT,OMIM:272,460).
基金This study wass supported by grants from the National Natural Science Foundation of China （No. 81470503 and No. 81470380）, and grant from the Ministry of Science and Technology of China （No. 2015AA020407）.
文摘Background： Takayasu arteritis （TA） is a rare inflammatory arteriopathy of unknown etiology. The aim of this study was to investigate the genetic susceptibility to TA in a Chinese population. Methods： Four single nucleotide polymorphisms （SNPs） those locate in the IL12B region （rs56167332）, the MLX region （rs665268）, the FCGR2A/FCGR3A locus （rsi0919543）, and the HLA-B/M1CA locus （rs12524487）, associated with TA in different population, were genotyped in 123 Chinese TA patients and 147 healthy controls from January 2013 to August 2014. A Chi-square test was used to test for genotype/allele frequencies variants. Results： Among the four SNPs, rs 10919543 was found to be significantly associated with TA in the studied population. The GG genotype of rs 10919543 at the FCGR2A/FCGR3A locus is a high risk factor （odds ratio [OR] = 6.532, 95% confidence interval [C1] = 2.402 - 17.763, P 〈 0.001 ） for TA. Among TA patients, the level of eosinophil granulocytes （Eos） in the peripheral blood was observed to be higher in the GG group of rs 10919543 （n = 23, Eos = 0. I 1 [0.08, 0.17] x 109/L） than the GA ＋ AA group （n = 100, Eos = 0.08 [0.05, 0.13] 10/L, P = 0.028）. No correlation between the genotypes of the other three SNPs and TA patients was observed. Conclusions： Our findings revealed unique genetic pattern in Chinese TA patients that may be partly responsible for the higher risk of TA in this population. FCGR2A/FCGR3A-related immune disorder might contribute to the etiology of TA.
