A female patient aged 3 months and 10 days was admitted to the cardiology department because of symptoms of heart failure.According to the echocardiography results,the patient received a diagnosis of primary endocardi...A female patient aged 3 months and 10 days was admitted to the cardiology department because of symptoms of heart failure.According to the echocardiography results,the patient received a diagnosis of primary endocardial fi broelastosis and was treated withγ-globulin,prednisone,digoxin,and diuretics.Coronary computed tomographic angiography and coronary angiography were performed as there was no improvement after 2 months of treatment.Finally,the patient received a diagnosis of anomalous origin of the left coronary artery from the pulmonary artery(ALCAPA).ALCAPA is a rare congenital heart defect that can cause severe heart failure during infancy,and is easily misdiagnosed clinically.In this report,we show the process of misdiagnosis of the case and consult the relevant literature,hoping to improve the understanding and early diagnosis of ALCAPA.展开更多
Syncope belongs to the transient loss of consciousness(TLOC), characterized by a rapid onset, short duration, and spontaneous complete recovery. It is common in children and adolescents, accounting for 1% to 2% of eme...Syncope belongs to the transient loss of consciousness(TLOC), characterized by a rapid onset, short duration, and spontaneous complete recovery. It is common in children and adolescents, accounting for 1% to 2% of emergency department visits.Recurrent syncope can seriously affect children's physical and mental health, learning ability and quality of life and sometimes cardiac syncope even poses a risk of sudden death. The present guideline for the diagnosis and treatment of syncope in children and adolescents was developed for guiding a better clinical management of pediatric syncope. Based on the globally recent development and the evidence-based data in China, 2018 Chinese Pediatric Cardiology Society(CPCS) guideline for diagnosis and treatment of syncope in children and adolescents was jointly prepared by the Pediatric Cardiology Society, Chinese Pediatric Society, Chinese Medical Association(CMA)/Committee on Pediatric Syncope, Pediatricians Branch, Chinese Medical Doctor Association(CMDA)/Committee on Pediatric Cardiology, Chinese College of Cardiovascular Physicians, Chinese Medical Doctor Association(CMDA)/Pediatric Cardiology Society, Beijing Pediatric Society, Beijing Medical Association(BMA). The present guideline includes the underlying diseases of syncope in children and adolescents, the diagnostic procedures, methodology and clinical significance of standing test and headup tilt test, the clinical diagnosis vasovagal syncope, postural orthostatic tachycardia syndrome, orthostatic hypotension and orthostatic hypertension, and the treatment of syncope as well as follow-up.展开更多
In the past studies,it is shown that cardiac troponin I(cTnI,encoded by TNNI3),as a cytoplasmic protein,is an inhibitory subunit in troponin complex,and involves in cardiomyocyte diastolic regulation.Here,we assessed ...In the past studies,it is shown that cardiac troponin I(cTnI,encoded by TNNI3),as a cytoplasmic protein,is an inhibitory subunit in troponin complex,and involves in cardiomyocyte diastolic regulation.Here,we assessed a novel role of cTnI as a nucleoprotein.Firstly,the nuclear translocation of cTnI was found in mouse,human fetuses and rat heart tissues.In addition,there were differences in percentage of intranuclear cTnI in different conditions.Based on weighted gene co-expression network analyses(WGCNA)and verification in cell experiments,a strong expression correlation was found between TNNI3 and Atp2a2,which encodes sarco-endoplasmic reticulum Ca2t ATPase isoform 2a(SERCA2a),and involves in ATP hydrolysis and Ca2t transient.TNNI3 gain and loss caused Atpa2a2 increase/decrease in a dosedependent manner both in mRNA and protein levels,in vivo and in vitro.By using ChIP-sequence we demonstrated specific binding DNA sequences of cTnI were enriched in ATP2a2 promoter239we889 region and the specific binding sequence motif of cTnI was analyzed by software as"CCAT",which has been reported to be required for YY1 binding to the promoter region of YY1-related genes.Moreover,it was further verified that pcDNA3.1()-TNNI3 could express cTnI proteins and increase the promoter activity of Atp2a2 through luciferase report assay.In the end,we evaluated beat frequencies,total ATP contents,Ca2t transients in TNNI3-siRNA myocardial cells.These findings indicated,for the first time,cTnI may regulate Atp2a2 in cardiomyocytes as a co-regulatory factor and participate in the regulation of intracellular Ca ions.展开更多
基金The project was funded by the Natural Science Foundation of Chongqing Science and Technology Commission(cstc2019jcyj-msxmX0866).
文摘A female patient aged 3 months and 10 days was admitted to the cardiology department because of symptoms of heart failure.According to the echocardiography results,the patient received a diagnosis of primary endocardial fi broelastosis and was treated withγ-globulin,prednisone,digoxin,and diuretics.Coronary computed tomographic angiography and coronary angiography were performed as there was no improvement after 2 months of treatment.Finally,the patient received a diagnosis of anomalous origin of the left coronary artery from the pulmonary artery(ALCAPA).ALCAPA is a rare congenital heart defect that can cause severe heart failure during infancy,and is easily misdiagnosed clinically.In this report,we show the process of misdiagnosis of the case and consult the relevant literature,hoping to improve the understanding and early diagnosis of ALCAPA.
文摘Syncope belongs to the transient loss of consciousness(TLOC), characterized by a rapid onset, short duration, and spontaneous complete recovery. It is common in children and adolescents, accounting for 1% to 2% of emergency department visits.Recurrent syncope can seriously affect children's physical and mental health, learning ability and quality of life and sometimes cardiac syncope even poses a risk of sudden death. The present guideline for the diagnosis and treatment of syncope in children and adolescents was developed for guiding a better clinical management of pediatric syncope. Based on the globally recent development and the evidence-based data in China, 2018 Chinese Pediatric Cardiology Society(CPCS) guideline for diagnosis and treatment of syncope in children and adolescents was jointly prepared by the Pediatric Cardiology Society, Chinese Pediatric Society, Chinese Medical Association(CMA)/Committee on Pediatric Syncope, Pediatricians Branch, Chinese Medical Doctor Association(CMDA)/Committee on Pediatric Cardiology, Chinese College of Cardiovascular Physicians, Chinese Medical Doctor Association(CMDA)/Pediatric Cardiology Society, Beijing Pediatric Society, Beijing Medical Association(BMA). The present guideline includes the underlying diseases of syncope in children and adolescents, the diagnostic procedures, methodology and clinical significance of standing test and headup tilt test, the clinical diagnosis vasovagal syncope, postural orthostatic tachycardia syndrome, orthostatic hypotension and orthostatic hypertension, and the treatment of syncope as well as follow-up.
基金This study was supported by research grants from Natural Science Foundation of China(No.81700214,and 81670212).
文摘In the past studies,it is shown that cardiac troponin I(cTnI,encoded by TNNI3),as a cytoplasmic protein,is an inhibitory subunit in troponin complex,and involves in cardiomyocyte diastolic regulation.Here,we assessed a novel role of cTnI as a nucleoprotein.Firstly,the nuclear translocation of cTnI was found in mouse,human fetuses and rat heart tissues.In addition,there were differences in percentage of intranuclear cTnI in different conditions.Based on weighted gene co-expression network analyses(WGCNA)and verification in cell experiments,a strong expression correlation was found between TNNI3 and Atp2a2,which encodes sarco-endoplasmic reticulum Ca2t ATPase isoform 2a(SERCA2a),and involves in ATP hydrolysis and Ca2t transient.TNNI3 gain and loss caused Atpa2a2 increase/decrease in a dosedependent manner both in mRNA and protein levels,in vivo and in vitro.By using ChIP-sequence we demonstrated specific binding DNA sequences of cTnI were enriched in ATP2a2 promoter239we889 region and the specific binding sequence motif of cTnI was analyzed by software as"CCAT",which has been reported to be required for YY1 binding to the promoter region of YY1-related genes.Moreover,it was further verified that pcDNA3.1()-TNNI3 could express cTnI proteins and increase the promoter activity of Atp2a2 through luciferase report assay.In the end,we evaluated beat frequencies,total ATP contents,Ca2t transients in TNNI3-siRNA myocardial cells.These findings indicated,for the first time,cTnI may regulate Atp2a2 in cardiomyocytes as a co-regulatory factor and participate in the regulation of intracellular Ca ions.
