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Internal physiological states influence behavioral decisions. We have investigated the underlying cellular and molecular mechanisms at the first olfactory synapse for starvation modulation of food-search behavior in Drosophila. We found that a local signal by short neuropeptide F (sNPF) and a global metabolic cue by insulin are integrated at specific odorant receptor neurons (ORNs) to modulate olfactory sensitivity. Results from two-photon calcium imaging show that starvation increases presynaptic activity via intraglomerular sNPF signaling. Expression of sNPF and its receptor (sNPFR1) in Or42b neurons is necessary for starvation-induced food-search behavior. Presynaptic facilitation in Or42b neurons is sufficient to mimic starvation-like behavior in fed flies. Furthermore, starvation elevates the transcription level of sNPFR1 but not that of sNPF, and insulin signaling suppresses sNPFR1 expression. Thus, starvation increases expression of sNPFR1 to change the odor map, resulting in more robust food-search behavior.
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Proteínas de Drosophila/metabolismo , Drosophila/fisiologia , Neuropeptídeos/metabolismo , Receptores de Neuropeptídeos/metabolismo , Receptores Odorantes/metabolismo , Transdução de Sinais , Animais , Antenas de Artrópodes/metabolismo , Feminino , Odorantes , Células Receptoras Sensoriais/metabolismo , Inanição/metabolismo , Sinapses/metabolismoRESUMO
This study aimed to compare the clinical characteristics and courses of pediatric patients with cardiac tumors in nonoperative and operative groups to help guide treatment decisions. We reviewed the medical records of patients diagnosed with primary pediatric cardiac tumors at our institution between 2003 and 2020. Demographic data, clinical characteristics, and follow-up data between the operation and nonoperation groups were compared. A total of 56 patients were included in the study. Thirteen patients underwent surgery. The median age was 1.4 months (range, 1 to 18 years). The patients in the operation group had more frequent symptoms or signs, such as desaturation, respiratory difficulty, murmur, a higher mass area/chamber area (MC) ratio, decreased ventricular contractility, and significant ventricular outflow tract obstruction (VOTO). An MC ratio of 0.568 was the cutoff value for differentiating patients with symptoms or signs of heart failure and decreased ventricular contractility. At the last follow-up, all patients had good ventricular contractility except one patient in the operative group with fibroma. In the non-operative group, rhabdomyomas often regressed spontaneously, while fibromas often increased in size. Two patients in the nonoperative group died. In the operative group, there was no early or late mortality or tumor recurrence. In this study, patients had good outcomes with or without surgery, even when the tumor was large, or surgery was performed in early infancy.
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PURPOSE: The objective of this study was to demonstrate that the gastric cross-sectional area (CSA) in the right lateral decubitus position (RLDP) during a 2-h fasting period is not larger than that during a conventional 4-h fasting period prior to pediatric echocardiography. METHODS: 93 patients aged under 3 years scheduled for echocardiography under sedation were enrolled and randomly allocated into two groups; 2-h fasting vs 4-h fasting. For group 4 h (n = 46), the patients were asked to be fasted for all types of liquid for more than 4 h, while group 2 h (n = 47) were asked to be fasted for all types of liquid for 2 h before echocardiography. Gastric ultrasound was performed before echocardiography, and CSARLDP was measured. We compared CSARLDP, incidence of at-risk stomach, fasting duration, and the incidence of major (pulmonary aspiration, aspiration pneumonia) and minor complications (nausea, retching, and vomiting, apnea, and bradycardia) between two groups. RESULTS: The mean difference of CSARLDP (group 2 h-group 4 h) was 0.49 (- 0.18 to 1.17) cm2, and it was within the non-inferiority margin (Δ = 2.1 cm2). There was no difference in the incidence of at-risk stomach (P = 0.514). There was no significant difference in the incidence of major and minor complications between the two groups. CONCLUSION: Two-hour fasting in pediatric patients who need an echocardiography did not increase major and minor complications and CSA significantly.
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Ecocardiografia , Jejum , Estômago , Ultrassonografia , Humanos , Feminino , Masculino , Ecocardiografia/métodos , Lactente , Pré-Escolar , Estômago/diagnóstico por imagem , Ultrassonografia/métodos , Fatores de Tempo , Pneumonia Aspirativa/prevenção & controleRESUMO
BACKGROUND: Few studies have examined the incidence of chronic kidney disease (CKD) and metabolic syndrome (MS) and their combined prognostic effects in adult congenital heart disease (ACHD). Our aims were to identify the incidence and prognostic implications of CKD and MS in ACHD. METHODS: This is retrospective cohort study. We included 2,462 ACHD ≥ 20 years of age who were treated at a tertiary hospital in Korea from 2006 to 2018. CKD was defined as an estimated glomerular filtration rate < 60 mL/min/1.73m². MS was diagnosed based on the presence of abnormal metabolic parameters: blood sugar level, obesity, dyslipidemia, and hypertension. The primary outcome was all-cause mortality from 2006 through 2019 using data from the Ministry of the Interior and Safety in Korea. RESULTS: The incidence of CKD and MS in ACHD was 7.6% and 35.9%, respectively. The coexistence rate of CKD and MS was 4.6%. Although MS was not independently associated with mortality in the multiple analysis (adjusted hazard ratio [aHR], 1.07; 95% confidence interval [CI], 0.79-1.46), it was closely related to the presence of CKD (adjusted odds ratio, 2.62; 95% CI, 1.89-3.63). ACHD patients with CKD had a significantly increased risk of mortality compared with those without CKD (aHR, 2.84; 95% CI, 2.00-4.04). CONCLUSIONS: In patients with ACHD, the distribution of MS is higher, and both MS and its components were associated with CKD. Given the CKD was independently associated with mortality, close monitoring and management of renal dysfunction and metabolic parameters in ACHD patients is needed.
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Cardiopatias Congênitas , Síndrome Metabólica , Insuficiência Renal Crônica , Adulto , Humanos , Síndrome Metabólica/complicações , Síndrome Metabólica/diagnóstico , Síndrome Metabólica/epidemiologia , Estudos Retrospectivos , Cardiopatias Congênitas/complicações , Cardiopatias Congênitas/diagnóstico , Prognóstico , Insuficiência Renal Crônica/complicações , Insuficiência Renal Crônica/diagnóstico , Taxa de Filtração Glomerular , Fatores de RiscoRESUMO
INTRODUCTION: This study evaluated the effect of music intervention on the anxiety and stress responses of patients who underwent an interventional cardiac catheterisation. METHODS: The study design was a pre- and post-test randomised controlled trial that included 94 patients who underwent a transcatheter atrial septal defect closure. Patients were allocated to receive either music intervention (n = 47) or usual care (n = 47) during the interventional cardiac catheterisation. Music intervention effectiveness was examined in terms of anxiety, salivary cortisol level, and heart rate variability. RESULTS: The average age of participants was 45.40 years (±16.04) in the experimental group and 47.26 years (±13.83) in the control group. Two-thirds (66.0%) of the participants in each group were women. State anxiety (F = 31.42, p < 0.001), anxiety-numerical rating scale (F = 20.08, p < 0.001), salivary cortisol levels (F = 4.98, p = 0.021), and low-frequency component/high-frequency component ratio (F = 17.31, p < 0.001) in the experimental group were significantly reduced compared with those in the control group at the end of the music intervention. CONCLUSION: This study provides practical evidence of a reduction in anxiety and stress response from music intervention preceding an interventional cardiac catheterisation, indicating that this intervention should be considered in clinical management.
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Musicoterapia , Música , Humanos , Adulto , Feminino , Pessoa de Meia-Idade , Masculino , Hidrocortisona , Ansiedade/etiologia , Ansiedade/terapia , Cateterismo Cardíaco , Projetos de PesquisaRESUMO
BACKGROUND: Although many studies have described an increased risk of necrotizing enterocolitis in duct dependent congenital heart diseases, very few have investigated its occurrence in full-term infants with duct dependent congenital heart diseases. METHODS: To evaluate the characteristics and risk factors of necrotizing enterocolitis, we performed a retrospective review of 355 full-term infants with duct dependent congenital heart diseases who received prostaglandin E1 therapy from April 2000 to May 2020. RESULTS: Necrotizing enterocolitis was observed in 10 patients (3.0%). Their average gestational age and birth weight were 38.2 weeks and 2783.5 g, respectively. The median age at diagnosis was 8.0 days (2-70 days). One patient was diagnosed with necrotizing enterocolitis stage IIA, five with stage IIB, two with stage IIIA, and two with stage IIIB; two (20%) received surgical treatment. The duct dependent pulmonary circulation group had higher frequencies of necrotizing enterocolitis (4.4%) than the duct dependent systemic circulation (2.0%) and parallel circulation (1.3%) groups. The necrotizing enterocolitis and the other groups had significantly different birth weight (2783.5 g vs 3170.9 g, respectively) and gestational age (38.2 weeks vs 39.1 weeks, respectively). Gestational age under 38 weeks (OR 8.87, p = 0.002), birth weight of < 2500 g (OR 5.1, p = 0.042), need for mechanical ventilation (OR 4.6, p = 0.021), parenteral nutrition (OR 107.7, p < 0.001), and functional single ventricle (OR 5.8, p = 0.009) were significant risk factors. The case-fatality rate was higher in the necrotizing enterocolitis (40.0%) than in the other group (8.3%, p = 0.009). CONCLUSIONS: Three percent of full-term infants with duct dependent congenital heart diseases developed necrotizing enterocolitis. Neonates with low birth weight, gestational age less than 38 weeks, functional single ventricle, or receiving assisted mechanical ventilation or parenteral nutrition are at increased risk.
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Enterocolite Necrosante , Cardiopatias Congênitas , Doenças do Recém-Nascido , Peso ao Nascer , Enterocolite Necrosante/epidemiologia , Enterocolite Necrosante/etiologia , Enterocolite Necrosante/cirurgia , Cardiopatias Congênitas/complicações , Cardiopatias Congênitas/epidemiologia , Humanos , Lactente , Recém-Nascido de Baixo Peso , Recém-NascidoRESUMO
BACKGROUND: Few studies used nationwide data to assess the survival rates (SRs) and death risk for idiopathic pulmonary arterial hypertension (IPAH; ICD-10 I27.0) in Korea. METHODS: IPAH data (N = 9,017; female:male = 6:4) were collected from the National Health Insurance Service in Korea, from 2006 through 2017. The data consisted of primary diagnoses related to IPAH. The Kaplan-Meier method and Cox proportional-hazards analyses were carried out. RESULTS: The mean age was 62.3 (± 19.4) years, 64.2 (± 18.9) years in female and 59.4 (± 19.8) years in male (P < 0.001). The one-, three-, five- and 10-year SRs for IPAH were 89.0%, 79.8%, 72.3% and 57.0%, respectively. The adjusted hazard ratio (HR) of IPAH was 1.81 (95% confidence interval [CI], 1.26-2.59) in 60-69 age group, 3.42 (95% CI, 2.40-4.87) in 70-79, and 7.73 (95% CI, 5.43-11.0) in 80s. Other risk factors were male, low-income status, diabetes, myocardial infarction, atrial fibrillation, ischemic stroke, hemorrhagic stroke, and malignant neoplasm. CONCLUSION: The 10-year SR of IPAH was 57% in Korea. The HR for IPAH was significantly high in patients with older age and other risk factors.
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Hipertensão Pulmonar Primária Familiar , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Modelos de Riscos Proporcionais , Estudos Retrospectivos , Fatores de Risco , Taxa de SobrevidaRESUMO
BACKGROUND: The transcatheter closure of atrial septal defect could be completed without the balloon-sizing technique, so we evaluated long-term outcomes compared with closure using balloon sizing, which was the conventional method. Even without using the balloon-sizing technique, transcatheter closure of atrial septal defect might be safe and effective. METHODS: We included 124 patients with isolated atrial septal defects who underwent device closure without balloon sizing between 2012 and 2016, and we further included 257 patients as a control group. Patients who received closure with multiple devices or who experienced postoperative residual defects were excluded. Immediate procedural results, as well as long-term outcomes for closure without balloon sizing, were investigated and compared with the control group. RESULTS: The procedural success rate was 96.7%, and there were no mortalities. No embolization or cardiac erosions were observed; however, one patient experienced residual shunt, and another developed progressed mitral regurgitation during the follow-up period (983±682 days). Newly onset persistent atrial fibrillation developed in one patient (1.0%). There were no significant differences in procedures or follow-up between the study and control groups. Despite the shorter procedural time in the study group, fluoro time was not different. Atrial arrhythmias were more frequently observed in the control group, but the difference was not significant. Persistent atrial fibrillation was observed in two patients in the control group (0.8%). CONCLUSIONS: Transcatheter closure of atrial septal defect can be performed safely and effectively without using the balloon-sizing technique. The long-term outcomes were similar to outcomes with balloon sizing.
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Fibrilação Atrial , Comunicação Interatrial , Insuficiência da Valva Mitral , Cateterismo Cardíaco/métodos , Ecocardiografia Transesofagiana , Comunicação Interatrial/diagnóstico , Comunicação Interatrial/cirurgia , HumanosRESUMO
OBJECTIVES: The aim of this study is to present the mid-term outcomes of Pulsta valve. BACKGROUND: The Pulsta valve is a Self-expandable knitted nitinol-wire stent mounted with a treated tri-leaflet α-Gal-free porcine pericardial valve for percutaneous pulmonary valve implantation (PPVI) in patients with native right ventricular outflow tract (RVOT) lesions. METHODS: A multi-center clinical trial using Pulsta valve® was designed for patients with severe pulmonary regurgitation (PR) in the native RVOT in multiple centers in South Korea and 25 patients were enrolled. Before PPVI, severe PR (mean PR fraction: 45.5 ± 6.9%) and enlarged RV volume (mean indexed RV end-diastolic volume; 169.7 ± 13.0 ml/m2 ) was present. The mean age was 21.6 ± 6.6 years old. RESULTS: All patients were successfully implanted with 26, 28, or 32 mm diameter of Pulsta valve loaded on the 18 or 20 French delivery catheters. At 6 months follow up, indexed RV end-diastolic volume was decreased to 126.9 ± 16.9 ml/m2 . At mean 33.1 ± 14.3 months follow-up, the mean value of mean pressure gradient in Pulsta valve was 6.5 ± 3.0 mmhg without significant PR. There was no serious device-related adverse event. CONCLUSIONS: A multi-center clinical trial was completed successfully with planned Pulsta valve implantation and demonstrated good mid-term effectiveness without device-related serious adverse events.
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Implante de Prótese de Valva Cardíaca , Próteses Valvulares Cardíacas , Valva Pulmonar , Adolescente , Adulto , Animais , Implante de Prótese de Valva Cardíaca/efeitos adversos , Humanos , Desenho de Prótese , Valva Pulmonar/diagnóstico por imagem , Valva Pulmonar/cirurgia , Suínos , Resultado do Tratamento , Adulto JovemRESUMO
BACKGROUND: Several reports described the repair of sinus of Valsalva aneurysms (SVAs); however, there is still debate regarding the optimal method of operation. We investigated the determinants of the development of significant aortic regurgitation (AR) and long-term survival after surgical repair. METHODS: Between January 1995 and December 2016, 71 patients (31 females; median age: 33.3 years) underwent surgical SVA repair with (n = 60) or without (n = 11) rupture. Aortic valvuloplasty (AVP) was performed using Trusler's technique in 28 patients (39.4%), and 11 patients (15.5%) underwent aortic valve replacement during the first operation. RESULTS: There was no early mortality, and three deaths occurred during follow-up (median: 65.4 months). Patients with grade II preoperative AR who underwent AVP tended to develop significant postoperative AR, but freedom from significant AR did not differ statistically (p = 0.387). Among patients who underwent AVP, freedom from significant AR did not differ statistically between those with grades I and II and those with grades III and IV (p = 0.460). CONCLUSION: Surgical repair of SVA with or without rupture can be performed safely using the dual approach technique. Concomitant aortic valve repair can be performed without difficulty and should be recommended not only for patients with moderate or severe preoperative AR (grades III and IV) but also for those with minimal or mild preoperative AR (grades I and II), whose aortic valve geometry needs correction.
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Aneurisma Aórtico/cirurgia , Ruptura Aórtica/cirurgia , Insuficiência da Valva Aórtica/cirurgia , Valva Aórtica/cirurgia , Implante de Prótese de Valva Cardíaca , Seio Aórtico/cirurgia , Procedimentos Cirúrgicos Vasculares , Adolescente , Adulto , Aneurisma Aórtico/diagnóstico por imagem , Aneurisma Aórtico/mortalidade , Ruptura Aórtica/diagnóstico por imagem , Ruptura Aórtica/mortalidade , Valva Aórtica/diagnóstico por imagem , Insuficiência da Valva Aórtica/diagnóstico por imagem , Insuficiência da Valva Aórtica/mortalidade , Criança , Feminino , Implante de Prótese de Valva Cardíaca/efeitos adversos , Implante de Prótese de Valva Cardíaca/mortalidade , Humanos , Masculino , Pessoa de Meia-Idade , Complicações Pós-Operatórias/etiologia , Estudos Retrospectivos , Medição de Risco , Fatores de Risco , Seio Aórtico/diagnóstico por imagem , Fatores de Tempo , Resultado do Tratamento , Procedimentos Cirúrgicos Vasculares/efeitos adversos , Procedimentos Cirúrgicos Vasculares/mortalidade , Adulto JovemRESUMO
BACKGROUND: Despite advancements in heart transplantation for pediatric patients in Korea, the waiting list mortality has not been reported. Therefore, we investigated the waiting list mortality rate and factors associated with patient mortality. METHODS: We reviewed the medical records of pediatric patients who were registered for heart transplantation at three major hospitals in Korea from January 2000 to January 2020. All patients who died while waiting for heart transplantation were investigated, and we identified the waiting list mortality rate, causes of mortality and median survival periods depending on the variable risk factors. RESULTS: A total of 145 patients received heart transplantations at the three institutions we surveyed, and the waiting list mortality rate was 26%. The most common underlying diseases were cardiomyopathy (66.7%) and congenital heart disease (30.3%). The leading causes that contributed to death were heart failure (36.3%), multi-organ failure (27.2%), and complications associated with extracorporeal membrane oxygenation (ECMO) (25.7%). The median survival period was 63 days. ECMO was applied in 30 patients. The different waiting list mortality percentages according to age, cardiac diagnosis, use of ECMO, and initial Korean Network of Organ Sharing (KONOS) level were determined using univariate analysis, but age was the only significant factor associated with waiting list mortality based on a multivariate analysis. CONCLUSION: The waiting list mortality of pediatric heart transplantation candidates was confirmed to be considerably high, and age, underlying disease, the application of ECMO, and the initial KONOS level were the factors that influenced the survival period.
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Cardiomiopatias/mortalidade , Cardiopatias Congênitas/mortalidade , Transplante de Coração , Cardiomiopatias/terapia , Criança , Pré-Escolar , Oxigenação por Membrana Extracorpórea/efeitos adversos , Feminino , Cardiopatias Congênitas/terapia , Coração Auxiliar , Humanos , Lactente , Estimativa de Kaplan-Meier , Masculino , Insuficiência de Múltiplos Órgãos/etiologia , Análise Multivariada , Sistema de Registros , República da Coreia , Estudos Retrospectivos , Fatores de Risco , Fatores de Tempo , Listas de EsperaRESUMO
BACKGROUND: The number of adults with congenital heart disease (ACHD) with atrial fibrillation (AF) is expected to increase. We sought to assess the impact of AF on survival in Korean ACHD. METHODS: Records of AF in ACHD were extracted from the records of the Korea National Health Insurance Service from 2006 through 2015. Multiple Cox proportional hazards analyses were carried out after adjustment for age, sex, income level, AF, and comorbidities. Survival rates (SRs) with and without AF were compared. The death records from 2006 through 2016 were included. RESULTS: A total of 3,999 ACHD had AF (51.4% were male) and 62,691 ACHD did not have AF (43.5% were male); the proportion of ACHD who were 60 years and older was 53.0% and 27.0% in those with and without AF, respectively (P < 0.001). The age-standardized incidence rate for AF was 1,842.0 persons per 100,000 people in the Korean general population from 2006 through 2015. For AF in ACHD, it was 5,996.4 persons per 100,000 ACHD during the same period, which was higher than that in the general population (P < 0.001). Significantly higher proportion of death (20.9%) occurred in ACHD with AF than without AF (8.3%) (P < 0.001). The adjusted hazard ratio for AF of death in ACHD was 1.39 (95% confidence interval, 1.29-1.50). The ten-year SR of ACHD with AF was 69.7% whereas it was 87.5% in non-AF (P < 0.001). CONCLUSION: In ACHD, AF occurs more frequently and has a worse prognosis than seen in the non-valvular general population in Korea. AF is associated with increased death in ACHD, especially with aging.
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Fibrilação Atrial/mortalidade , Cardiopatias Congênitas/patologia , Adulto , Idoso , Idoso de 80 Anos ou mais , Fibrilação Atrial/complicações , Fibrilação Atrial/epidemiologia , Fibrilação Atrial/patologia , Comorbidade , Bases de Dados Factuais , Feminino , Cardiopatias Congênitas/complicações , Humanos , Incidência , Masculino , Pessoa de Meia-Idade , Prognóstico , Modelos de Riscos Proporcionais , República da Coreia/epidemiologia , Estudos Retrospectivos , Análise de Sobrevida , Adulto JovemRESUMO
Fragmented QRS (fQRS) is an easily evaluated noninvasive parameter in electrocardiograms (ECGs) for predicting cardiac adverse events. Our study aimed to evaluate whether fQRS could be used as a risk factor to predict a major adverse cardiac event (MACE) in children with idiopathic dilated cardiomyopathy (DCM). Our retrospective study enrolled 63 patients aged ≤ 19 years who were newly diagnosed with idiopathic DCM at Samsung Medical Center from 2003 to 2014, and followed up until December 2018. Demographic data, ECGs, and echocardiography were reviewed and analyzed for their possible links with adverse outcomes. The median age was 14.0 months and the median length of follow-up was 65.0 months. Age, QRS duration, and ejection fraction in M-mode at diagnosis were significantly different between the fQRS and non-fQRS groups (P = 0.026; P = 0.001; and P = 0.020). Significant difference between the fQRS and non-fQRS groups was found in patients with a MACE (P = 0.016, odd ratio 3.643) or any arrhythmias (P = 0.008, odd ratio 6.563). The MACE-free survival rate showed a significant difference in terms of fQRS (P = 0.003; P = 0.007; and P = 0.027). In univariate analyses, age, QRS duration, corrected QT, positive fQRS, and the number of leads with fQRS were significant predictors of MACEs. Among the above factors, positive fQRS at diagnosis was a strongly significant predictor of adverse outcomes in multivariate analyses (hazards ratio 94.529, P = 0.001). Fragmented QRS complex at diagnosis could be used as a strong predictor for cardiac adverse outcomes in pediatric patients with idiopathic DCM.
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Cardiomiopatia Dilatada/complicações , Eletrocardiografia/métodos , Cardiopatias/diagnóstico , Arritmias Cardíacas/diagnóstico , Cardiomiopatia Dilatada/mortalidade , Criança , Pré-Escolar , Feminino , Cardiopatias/etiologia , Humanos , Lactente , Recém-Nascido , Masculino , Análise Multivariada , Prognóstico , Modelos de Riscos Proporcionais , Estudos Retrospectivos , Fatores de RiscoRESUMO
We present a case of percutaneous coronary intervention in a 4-month-old infant with both severe coronary stenosis and acute heart failure after arterial switch operation for transposition of the great arteries. Under extracorporeal membrane oxygenation, balloon angioplasty of the left coronary artery with a 2.0 × 15-mm balloon and stent implantation on the right coronary artery with a 2.25 × 26-mm stent were performed successfully. Echocardiography after the intervention showed recovered cardiac function and no complications.
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Transposição das Grandes Artérias , Intervenção Coronária Percutânea , Transposição dos Grandes Vasos , Transposição das Grandes Artérias/efeitos adversos , Constrição Patológica , Vasos Coronários/diagnóstico por imagem , Vasos Coronários/cirurgia , Humanos , Lactente , Transposição dos Grandes Vasos/diagnóstico por imagem , Transposição dos Grandes Vasos/cirurgiaRESUMO
BACKGROUND: Infracardiac obstructive total anomalous pulmonary venous return (TAPVR) has a poor outcome following surgical correction. We compared the surgical outcomes of obstructive TAPVR between non-infracardiac and infracardiac types. METHODS: Among 51 patients who underwent surgical repair for obstructive TAPVR, 23 with infracardiac type and 28 with non-infracardiac type were included in this investigation. The study compared the immediate postoperative courses in the intensive care unit and long-term mortality and pulmonary vein stenosis. The risk factors for long-term survival in obstructive TAPVR also were investigated. RESULTS: The postoperative follow-up period was 79.8 ± 81.5 months. Immediate major operative complications were observed in 22 patients (43.1%); 10 patients (19.6%) died, and eight patients (15.7%) experienced pulmonary vein stenosis during the follow-up period. The Kaplan-Meier curve showed better cumulative survival in patients with infracardiac TAPVR (P = 0.308). The significant factors for survival after surgical repair of obstructive TAPVR did not include anatomical type but instead were postoperative course of ventilator care and lengths of intensive care unit and hospital stays. CONCLUSION: Patients with non-infracardiac TAPVR with obstruction had a longer postoperative course and experienced more complications. Their survival rate was poorer, and postoperative pulmonary vein stenosis was more frequent in those patients compared with infracardiac TAPVR patients. However, a large-scale study is mandatory to gather more data and confirm our findings.
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Procedimentos Cirúrgicos Cardíacos/métodos , Complicações Pós-Operatórias/epidemiologia , Veias Pulmonares/anormalidades , Síndrome de Cimitarra/cirurgia , Seguimentos , Humanos , Incidência , Veias Pulmonares/cirurgia , República da Coreia/epidemiologia , Estudos Retrospectivos , Fatores de Risco , Síndrome de Cimitarra/diagnóstico , Síndrome de Cimitarra/mortalidade , Taxa de Sobrevida/tendências , Resultado do TratamentoRESUMO
BACKGROUND: A certain degree of pulmonary stenosis after total correction of tetralogy of Fallot has been considered acceptable. But the long-term outcomes are not well understood. We observed the natural course of immediate pulmonary stenosis and investigated related factors for progression. METHODS: Fifty-two patients with acceptable pulmonary stenosis immediately after operation were enrolled. Acceptable pulmonary stenosis was defined as peak pressure gradient between 15 and 45 mmHg by Doppler echocardiography. Latent class linear mixed model was used to differentiate patients with progressed pulmonary stenosis, and the factors related to progression were analysed. RESULTS: Pulmonary stenosis progressed in 14 patients (27%). Between the progression group and no progression group, there were no significant differences in operative age, sex, and the use of the transannular patch technique. However, immediate gradient was higher in the progression group (32.1 mmHg versus 25.7 mmHg, p = 0.009), and the cut-off value was 26.8 mmHg (sensitivity = 65.3%, specificity = 65.8%). Main stenosis at the sub-valve was observed more frequently in the progression group (85.7% versus 52.6%, p = 0.027). Despite no difference in the preoperative pulmonary valve z value, the last follow-up pulmonary valve z value was significantly lower in the progression group (-1.15 versus 0.35, p = 0.002). CONCLUSIONS: Pulmonary stenosis immediately after tetralogy of Fallot total correction might progress in patients with immediate pulmonary stenosis higher than ≥26.8 mmHg and the main site was sub-valve area.
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Progressão da Doença , Estenose da Valva Pulmonar/diagnóstico por imagem , Estenose da Valva Pulmonar/fisiopatologia , Tetralogia de Fallot/cirurgia , Criança , Pré-Escolar , Ecocardiografia Doppler , Feminino , Humanos , Lactente , Modelos Lineares , Masculino , Valva Pulmonar/anormalidades , Valva Pulmonar/diagnóstico por imagem , Estenose da Valva Pulmonar/etiologia , República da Coreia , Tetralogia de Fallot/complicações , Tetralogia de Fallot/fisiopatologia , Resultado do TratamentoRESUMO
OBJECTIVE: Three-weekly docetaxel causes a high rate of febrile neutropenia, especially in the Asian population. Two-weekly docetaxel has been shown to reduce rate of febrile neutropenia in castrate-resistant prostate cancer patients. We conducted a preliminary study to investigate the safety of two-weekly docetaxel in advanced breast cancer patients. METHODS: We recruited 10 patients with advanced breast cancer with ECOG (Eastern Cooperative Oncology Group) performance status score of zero to two, who needed chemotherapy in the first or second-line setting to receive two-weekly docetaxel for 8 cycles. The primary endpoint was safety and secondary endpoints were response rate and progression free survival. RESULTS: The most reported adverse events were haematological (anaemia 100% and neutropenia 90%). The febrile neutropenia rate was 10%. The overall response rate was 20%. The median progression free survival was 5.0 months. CONCLUSION: Two-weekly docetaxel may be a reasonable alternative treatment regimen for patients with advanced breast cancer in the first or second-line setting. This regimen is yet to be compared with standard 3-weekly schedule in a phase 3 randomised clinical trial.
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Antineoplásicos/administração & dosagem , Neoplasias da Mama/tratamento farmacológico , Neoplasias da Mama/patologia , Docetaxel/administração & dosagem , Adolescente , Adulto , Idoso , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Feminino , Humanos , Pessoa de Meia-Idade , Intervalo Livre de Progressão , Resultado do Tratamento , Adulto JovemRESUMO
The role of astrocytes in brain plasticity has not been extensively studied compared with that of neurons. Here we adopted integrative translational and reverse-translational approaches to explore the role of an astrocyte-specific major water channel in the brain, aquaporin-4 (AQP4), in brain plasticity and learning. We initially identified the most prevalent genetic variant of AQP4 (single nucleotide polymorphism of rs162008 with C or T variation, which has a minor allele frequency of 0.21) from a human database (n=60 706) and examined its functionality in modulating the expression level of AQP4 in an in vitro luciferase reporter assay. In the following experiments, AQP4 knock-down in mice not only impaired hippocampal volumetric plasticity after exposure to enriched environment but also caused loss of long-term potentiation after theta-burst stimulation. In humans, there was a cross-sectional association of rs162008 with gray matter (GM) volume variation in cortices, including the vicinity of the Perisylvian heteromodal language area (Sample 1, n=650). GM volume variation in these brain regions was positively associated with the semantic verbal fluency. In a prospective follow-up study (Sample 2, n=45), the effects of an intensive 5-week foreign language (English) learning experience on regional GM volume increase were modulated by this AQP4 variant, which was also associated with verbal learning capacity change. We then delineated in mice mechanisms that included AQP4-dependent transient astrocytic volume changes and astrocytic structural elaboration. We believe our study provides the first integrative evidence for a gliogenetic basis that involves AQP4, underlying language-associated brain plasticity.
Assuntos
Aquaporina 4/metabolismo , Astrócitos/citologia , Desenvolvimento da Linguagem , Aprendizagem/fisiologia , Neuroglia/citologia , Plasticidade Neuronal/fisiologia , Adulto , Animais , Aquaporina 4/biossíntese , Aquaporina 4/genética , Astrócitos/metabolismo , Encéfalo/metabolismo , Estudos Transversais , Modelos Animais de Doenças , Feminino , Seguimentos , Frequência do Gene , Substância Cinzenta/citologia , Substância Cinzenta/metabolismo , Hipocampo/metabolismo , Humanos , Masculino , Camundongos , Camundongos Knockout , Neuroglia/metabolismo , Neurônios/metabolismo , Polimorfismo de Nucleotídeo Único , Estudos ProspectivosRESUMO
Objectives: We aimed to investigate the outcome of tricuspid valve repair (TVR) performed concomitantly with pulmonary valve replacement in repaired tetralogy of Fallot (TOF) patients. Design: This retrospective study included all patients who underwent pulmonary vale replacement from 2000 to 2016 after TOF correction. TVR patient data were compared to those of patients who underwent pulmonary vale replacement alone. Results: Thirty-eight patients were enrolled. The degree of tricuspid regurgitation was significantly decreased after operation in the TVR group. Tricuspid valve annulus and annuloectasia before operation did not vary between groups (21.1 ± 6.3 and 41.4% in no TVR vs. 21.3 ± 4.8 and 52.6% in TVR). However pre-operative right ventricular volumes were larger in the TVR group. Normal tricuspid valve coaptation (body to body) was observed less frequently in the TVR group than in the other group (52.6% vs. 93.1%, p < .001). Pre-operative tricuspid regurgitation had a linear correlation with right ventricular volume, but not with tricuspid annulus size. Conclusion: Tricuspid annulus diameter decreased significantly regardless of TVR. Abnormal coaptations were observed more in patients group and the degree of pre-operative tricuspid regurgitation was linearly correlated with right ventricular volume rather than tricuspid annulus size.
Assuntos
Procedimentos Cirúrgicos Cardíacos , Implante de Prótese de Valva Cardíaca , Insuficiência da Valva Pulmonar/cirurgia , Valva Pulmonar/cirurgia , Tetralogia de Fallot/cirurgia , Insuficiência da Valva Tricúspide/cirurgia , Valva Tricúspide/cirurgia , Adolescente , Adulto , Procedimentos Cirúrgicos Cardíacos/efeitos adversos , Criança , Feminino , Implante de Prótese de Valva Cardíaca/efeitos adversos , Humanos , Masculino , Pessoa de Meia-Idade , Valva Pulmonar/fisiopatologia , Insuficiência da Valva Pulmonar/etiologia , Insuficiência da Valva Pulmonar/fisiopatologia , Recuperação de Função Fisiológica , Estudos Retrospectivos , Tetralogia de Fallot/complicações , Tetralogia de Fallot/fisiopatologia , Resultado do Tratamento , Valva Tricúspide/fisiopatologia , Insuficiência da Valva Tricúspide/etiologia , Insuficiência da Valva Tricúspide/fisiopatologia , Adulto JovemRESUMO
BACKGROUND: This study aimed to determine the effect of a multidisciplinary approach on the birth rate of fetuses with prenatally diagnosed congenital heart diseases (CHDs). METHODS: Among the fetuses of 724 gravidas who underwent fetal echocardiography in Samsung Medical Center from January 2013 to June 2017, 463 fetuses with normal cardiac structure, arrhythmia or simple left-to-right shunt were excluded, and the remaining 261 were included in the study. The subjects were subdivided into groups based on whether they were consulted multidisciplinarily, that is, consulted simultaneously by pediatric cardiologists, obstetricians and pediatric cardiac surgeons or not. They were also categorized based on the initial fetal echocardiogram results. RESULTS: Among the fetuses in the multidisciplinary group, 64.5% of the fetuses were given birth to, and the proportion was not different from that in the non-multidisciplinary group (68.6%, P = 0.48). The delivery rate in the multidisciplinary consultation group were 69.2% in the transposition of the great arteries group, 63.6% in the tetralogy of Fallot group, 68.8% in the pulmonary atresia or interrupted aortic arch group, 62.5% in the coarctation of aorta group, 60.0% in the atrioventricular septal defect group, 70.0% in the functional single ventricle group, and 55.6% in the hypoplastic left heart syndrome group; there were no significant differences between the 10 echocardiogram groups. However, when the subjects were categorized into Fontan repair group and biventricular repair group, the Fontan repair group showed a significant increase in the likelihood of delivery when a multidisciplinary approach was taken (P = 0.035). CONCLUSION: When a fetus was diagnosed with a CHD where Fontan repair should be considered, a multidisciplinary approach resulted in increased possibility of delivery.