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1.
Dis Model Mech ; 15(12)2022 12 01.
Artigo em Inglês | MEDLINE | ID: mdl-36518084

RESUMO

To model peripheral nerve degeneration and investigate molecular mechanisms of neurodegeneration, we established a cell system of induced pluripotent stem cell (iPSC)-derived sensory neurons exposed to vincristine, a drug that frequently causes chemotherapy-induced peripheral neuropathy. Sensory neurons differentiated from iPSCs exhibit distinct neurochemical patterns according to the immunocytochemical phenotypes, and gene expression of peripherin (PRPH, hereafter referred to as Peri) and neurofilament heavy chain (NEFH, hereafter referred to as NF). The majority of iPSC-derived sensory neurons were PRPH positive/NEFH negative, i.e. Peri(+)/NF(-) neurons, whose somata were smaller than those of Peri(+)/NF(+) neurons. On exposure to vincristine, projections from the cell body of a neuron, i.e. neurites, were degenerated quicker than somata, the lethal concentration to kill 50% (LC50) of neurites being below the LC50 for somata, consistent with the clinical pattern of length-dependent neuropathy. We then examined the molecular expression in the MAP kinase signaling pathways of, extracellular signal-regulated kinases 1/2 (MAPK1/3, hereafter referred to as ERK), p38 mitogen-activated protein kinases (MAPK11/12/13/14, hereafter referred to as p38) and c-Jun N-terminal kinases (MAPK8/9/10, hereafter referred to as JNK). Regarding these three cascades, only phosphorylation of JNK was upregulated but not that of p38 or ERK1/2. Furthermore, vincristine-treatment resulted in impaired autophagy and reduced autophagic flux. Rapamycin-treatment reversed the effect of impaired autophagy and JNK activation. These results not only established a platform to study peripheral degeneration of human neurons but also provide molecular mechanisms for neurodegeneration with the potential for therapeutic targets.


Assuntos
Células-Tronco Pluripotentes Induzidas , Humanos , Células-Tronco Pluripotentes Induzidas/metabolismo , Vincristina/toxicidade , Proteínas Quinases JNK Ativadas por Mitógeno/metabolismo , Proteínas Quinases p38 Ativadas por Mitógeno/metabolismo , Fosforilação
2.
Vaccines (Basel) ; 10(12)2022 Dec 17.
Artigo em Inglês | MEDLINE | ID: mdl-36560584

RESUMO

Although neurological complications after the administration of vaccines against coronavirus disease 2019 (COVID-19) are rare, they might result in long-term morbidity. This study was designed to determine the risk of peripheral nervous system (PNS) adverse events after the administration of mRNA vaccines against COVID-19. Large-scale randomized controlled trials (RCTs) and cohort studies were systematically searched in databases, and 15 cohort studies were included in the synthesis. Among all PNS adverse events, only Bell's palsy and Guillain-Barré syndrome (GBS) had sufficient data and were included for further analysis. Individuals who received mRNA vaccines had a higher risk of Bell's palsy than the unvaccinated group, and the risk of Bell's palsy after BNT162b2 was significantly higher than after mRNA-1273. Regarding GBS, no significant difference in the risk was observed between BNT162b2 and the unvaccinated group, but BNT126b2 introduced a higher risk of post-vaccinated GBS than mRNA-1273. In conclusion, PNS adverse events, especially Bell's palsy, should be carefully observed after mRNA vaccination against COVID-19. With the opportunity of vaccination campaigns on such a large scale, further investigation and surveillance of post-vaccination neurological adverse events should also be established.

3.
Lancet ; 400(10355): 822-831, 2022 09 10.
Artigo em Inglês | MEDLINE | ID: mdl-36049495

RESUMO

BACKGROUND: Angiotensin receptor blockers (ARBs) and ß blockers are widely used in the treatment of Marfan syndrome to try to reduce the rate of progressive aortic root enlargement characteristic of this condition, but their separate and joint effects are uncertain. We aimed to determine these effects in a collaborative individual patient data meta-analysis of randomised trials of these treatments. METHODS: In this meta-analysis, we identified relevant trials of patients with Marfan syndrome by systematically searching MEDLINE, Embase, and CENTRAL from database inception to Nov 2, 2021. Trials were eligible if they involved a randomised comparison of an ARB versus control or an ARB versus ß blocker. We used individual patient data from patients with no prior aortic surgery to estimate the effects of: ARB versus control (placebo or open control); ARB versus ß blocker; and indirectly, ß blocker versus control. The primary endpoint was the annual rate of change of body surface area-adjusted aortic root dimension Z score, measured at the sinuses of Valsalva. FINDINGS: We identified ten potentially eligible trials including 1836 patients from our search, from which seven trials and 1442 patients were eligible for inclusion in our main analyses. Four trials involving 676 eligible participants compared ARB with control. During a median follow-up of 3 years, allocation to ARB approximately halved the annual rate of change in the aortic root Z score (mean annual increase 0·07 [SE 0·02] ARB vs 0·13 [SE 0·02] control; absolute difference -0·07 [95% CI -0·12 to -0·01]; p=0·012). Prespecified secondary subgroup analyses showed that the effects of ARB were particularly large in those with pathogenic variants in fibrillin-1, compared with those without such variants (heterogeneity p=0·0050), and there was no evidence to suggest that the effect of ARB varied with ß-blocker use (heterogeneity p=0·54). Three trials involving 766 eligible participants compared ARBs with ß blockers. During a median follow-up of 3 years, the annual change in the aortic root Z score was similar in the two groups (annual increase -0·08 [SE 0·03] in ARB groups vs -0·11 [SE 0·02] in ß-blocker groups; absolute difference 0·03 [95% CI -0·05 to 0·10]; p=0·48). Thus, indirectly, the difference in the annual change in the aortic root Z score between ß blockers and control was -0·09 (95% CI -0·18 to 0·00; p=0·042). INTERPRETATION: In people with Marfan syndrome and no previous aortic surgery, ARBs reduced the rate of increase of the aortic root Z score by about one half, including among those taking a ß blocker. The effects of ß blockers were similar to those of ARBs. Assuming additivity, combination therapy with both ARBs and ß blockers from the time of diagnosis would provide even greater reductions in the rate of aortic enlargement than either treatment alone, which, if maintained over a number of years, would be expected to lead to a delay in the need for aortic surgery. FUNDING: Marfan Foundation, the Oxford British Heart Foundation Centre for Research Excellence, and the UK Medical Research Council.


Assuntos
Síndrome de Marfan , Antagonistas Adrenérgicos beta/uso terapêutico , Antagonistas de Receptores de Angiotensina/uso terapêutico , Inibidores da Enzima Conversora de Angiotensina/uso terapêutico , Aorta , Humanos , Síndrome de Marfan/complicações , Síndrome de Marfan/tratamento farmacológico , Ensaios Clínicos Controlados Aleatórios como Assunto
4.
Tzu Chi Med J ; 34(1): 44-48, 2022.
Artigo em Inglês | MEDLINE | ID: mdl-35233355

RESUMO

Marfan syndrome (MFS), a multisystemic connective disorder, caused by fibrillin 1 gene mutations with autosomal dominant inheritance. The disease spectrum is wide and the major causes of death are related to aortic root aneurysm or dissection. The purposes of medical treatment are to reduce structural changes in the aortic wall and slow aortic root dilatation. Advance in medical researches have provided new insights into the pathogenesis of disease and opened up new horizons for treatments. Several medications such as angiotensin II type I receptor blockers, ß-blockers, angiotensin-converting enzyme inhibitors, calcium channel blockers, tetracyclines, and statins have been studied for the purpose. Currently, the life expectancy of Marfan patients improves significantly and is closes to the general population with proper treatment. In this article, we review and update the medical treatments for patients with MFS.

5.
J Formos Med Assoc ; 121(6): 1093-1101, 2022 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-34456093

RESUMO

BACKGROUND: Thoracic aortic aneurysm and dissection (TAAD) is a devastating but treatable disease if detected early. The clinical manifestations and genetic characteristics underlying TAAD patients in Taiwan, however, remain unclear. METHODS: We consecutively recruited patients referred for TAAD screening and/or management at a tertiary medical center in Taiwan. All patients received a comprehensive survey of the clinical manifestations and a genetic testing with a 29-gene next-generation sequencing (NGS) panel. RESULTS: Patients (n = 107) were referred for different reasons, and could be grouped into 4 categories: known aortic aneurysm or dissection (AoAD) (n = 57), Marfanoid features (n = 36), having family members of suspected AoAD (n = 11), and ectopic lens (n = 3). AoAD were confirmed in 73 (68.2%) of the entire cohort. Among all the clinical manifestations, skin striae distensae was the only physical sign that showed significant association with AoAD (p = 0.007 after adjusted). Disease-causing genes/variants were identified in 46 patients (43.0%); FBN1 was the most prevalent disease-causing gene, followed by TGFBR1, TGFBR2 and FBN2. A positive genetic testing was not only an independent predictor of AoAD (hazard ratio (HR) 3.468, 95% confidence interval (CI) [1.541-7.807], p = 0.003), but also had a higher chance of dissection among the patients with known dilated aorta (HR 4.552, 95% CI [1.578-13.135], p = 0.005). CONCLUSION: The presence of skin striae distensae may serve as a clinical cue for physicians to search for AoAD in subjects who are at risk. The NGS panel test not only helps confirm the diagnosis, but also stratify the risk of dissection among patients with dilated aorta.


Assuntos
Aneurisma da Aorta Torácica , Dissecção Aórtica , Estrias de Distensão , Dissecção Aórtica/diagnóstico , Dissecção Aórtica/genética , Aneurisma da Aorta Torácica/diagnóstico , Aneurisma da Aorta Torácica/genética , Estudos de Coortes , Humanos , Estudos Prospectivos , Taiwan
6.
Healthcare (Basel) ; 8(4)2020 Oct 25.
Artigo em Inglês | MEDLINE | ID: mdl-33113863

RESUMO

COVID-19 has been impacting the Med-Tech industry dramatically since the beginning of 2020. Along with the pandemic continuously growing, the demand for major global medical products such as masks and protective clothing has surged. The Med-Tech industry is facing the huge challenge of a lack of production capacity, including raw material, production equipment, production line, professional human resources, and more. It would require not only the operators in the Med-Tech industry to enlarge their productivity, but also new investors from outside. This study focused on the entry strategy analysis of the Med-Tech industry, developing five driving factors, and conducting an opinion survey from three different aspects, including vendors, channels, and end-users, under COVID-19 impact. A total of 99 valid questionnaires were collected. After that, the Importance Accessibility Analysis-Network Relation Map (IAA-NRM) approach was used to verify the importance and implementation priority of the entry strategies. Then, the Decision Making Trial and Evaluation Laboratory (DEMATEL) technique is used to construct the NRM method. The research results showed that there is a common strategic path, from the regulatory system to operation resources and then marketing promotion. In addition, in these three viewpoints, vendors and end-users have similar priorities in terms of industry attributes and barriers to entry.

7.
Circ Cardiovasc Interv ; 13(6): e008813, 2020 06.
Artigo em Inglês | MEDLINE | ID: mdl-32519889

RESUMO

BACKGROUND: Transcatheter ventricular septal defect (VSD) closure is a safe and efficacious alternative to surgery. However, its benefits in asymptomatic or minimally symptomatic patients remain unknown. METHODS: Sixty patients with VSD aged 12 to 60 years underwent cardiopulmonary exercise test and echocardiography 1 day before transcatheter VSD closure and 6 months after intervention (closure group). Thirty patients who did not receive the intervention underwent the same evaluations over 6 months (observation group). RESULTS: No significant change in exercise function was observed after VSD closure, except for increased peak oxygen (O2) pulse (absolute increase: 0.4±1.4 mL/beat). Left ventricular end-diastolic dimension and mitral peak early filling velocity-to-early diastolic annular velocity ratio decreased (absolute decrease: 0.3±0.6 cm and 0.7±1.9, respectively). Despite unchanged overall peak O2 consumption, 33% of closure group patients exhibited clinically significant improvements in peak O2 consumption (>10% increase relative to baseline). This was not related to the pulmonary flow-to-systemic flow ratio or baseline exercise capacity. By contrast, all exercise function parameters deteriorated significantly in the observation group. Subgroup analysis revealed that patients with a baseline left ventricular end-diastolic dimension Z score of >2 exhibited a significantly greater improvement in peak O2 consumption, peak O2 pulse, and oxygen uptake efficiency slope than did the observation group. CONCLUSIONS: Compared with conservative management, transcatheter VSD closure prevents deterioration in exercise capacity and promotes left ventricular reverse remodeling in asymptomatic or minimally symptomatic patients. These benefits are most prominent in patients whose left ventricular end-diastolic dimension Z score before intervention is >2, irrespective of baseline peak O2 consumption and pulmonary flow/systemic flow ratio. Registration: URL: http://www.clinicaltrials.gov. Unique identifier: NCT03127748.


Assuntos
Cateterismo Cardíaco , Tolerância ao Exercício , Comunicação Interventricular/terapia , Função Ventricular Esquerda , Remodelação Ventricular , Adolescente , Adulto , Fatores Etários , Doenças Assintomáticas , Cateterismo Cardíaco/efeitos adversos , Criança , Diástole , Feminino , Seguimentos , Comunicação Interventricular/complicações , Comunicação Interventricular/fisiopatologia , Humanos , Masculino , Pessoa de Meia-Idade , Consumo de Oxigênio , Estudos Prospectivos , Recuperação de Função Fisiológica , Fatores de Tempo , Resultado do Tratamento , Adulto Jovem
9.
J Formos Med Assoc ; 119(1 Pt 1): 182-190, 2020 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-31003918

RESUMO

BACKGROUND AND PURPOSE: Variable effects of beta-blockers (BB) and/or angiotensin receptor blockers (ARB) were reported to retard aortic root growth in Marfan syndrome (MFS). This study aimed to compare the effects of BB therapy and ARB-related therapies on cardiovascular protection in MFS. METHODS: Studies of randomized control trials comparing the efficacy of only-BB and ARB-related (only-ARB or ARB-plus-BB) therapies for MFS published before July 31, 2018 in PubMed, Embase, and the Cochrane Library were selected. The outcomes included changes in aortic growth and cardiovascular events. RESULTS: Eight trials involving 1381 patients were included. Patients received only-BB and ARB-related therapies did not differ significantly in changes in aortic growth (aortic root diameter: standardized mean difference [SMD] = 0.04, 95% confidence interval [CI]: -0.11-0.19, p = 0.63) or cardiovascular events (aortic dissection: Peto odds ratio [OR] = 1.67, 95% CI: 0.42-6.72, p = 0.47; aortic surgery: risk ratio = 0.97, 95% CI: 0.66-1.41, p = 0.86; death: Peto OR = 2.78, 95% CI: 0.39-19.82, p = 0.31). Subgroup analysis revealed that ARB-plus-BB therapy exhibited nonsignificantly better outcomes than only-BB therapy (aortic root diameter: SMD = 0.11, 95% CI: -0.22-0.45, p = 0.52; ascending aorta diameter: SMD = 0.10, 95% CI: -0.07-0.27, p = 0.26; aortic surgery: Peto OR = 1.10, 95% CI: 0.75-1.61, p = 0.62). CONCLUSION: For cardiovascular protection in MFS, only-ARB therapy is not inferior to only-BB therapy. Moreover, the outcomes of ARB-plus-BB therapy seemed to be favourable to those of only-BB therapy.


Assuntos
Antagonistas Adrenérgicos beta/uso terapêutico , Antagonistas de Receptores de Angiotensina/uso terapêutico , Doenças da Aorta/tratamento farmacológico , Losartan/uso terapêutico , Síndrome de Marfan/tratamento farmacológico , Doenças da Aorta/etiologia , Cardiotônicos/uso terapêutico , Dilatação Patológica/tratamento farmacológico , Dilatação Patológica/etiologia , Quimioterapia Combinada , Humanos , Ensaios Clínicos Controlados Aleatórios como Assunto , Resultado do Tratamento
12.
Heart Vessels ; 32(2): 201-207, 2017 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-27272896

RESUMO

Parameters from cardiopulmonary exercise test (CPET) are useful prognostic factors for patients with repaired tetralogy of fallot (TOF). Its application in exercise prescription remains unclear. This study sought to define its role. We made current exercise recommendations in repaired TOF patients according to European Society of Cardiology (ESC) guideline, which were based on ventricular function, pressure/volume load, pulmonary artery pressure, hypoxemia and arrhythmic burden both at rest and during exercise. CPET parameters (peak oxygen consumption, oxygen uptake efficiency plateau, and E/CO2 slope), along with cardiothoracic ratio, ventricular arrhythmia, QRS duration and NYHA functional status, were used to calculate "score to exercise". 112 repaired TOF adolescent and adult aged 32.6 ± 10.8 (14.05- to 56.99-year-old, median 30.1) years received exercise recommendations by ESC guideline, which suggested high, moderate and low intensity sports for 45 (40.2 %), 45 (40.2 %), and 22 (19.6 %) patients, respectively. The optimal cut-off values were 67 and 77 % for peak VO2, 86 and 100 % for OUEP, 22 and 28 for E/CO2 slope to correlate to the exercise intensity recommendation. But, individual CPET parameter had low consistency (41-46 %) in making decisions of exercise intensity compared to ESC recommendations. Using the "score to exercise", the consistency rate could be increased to 74.1 %. With "score to exercise" recommended exercise intensity, follow-up result revealed no adverse event related to sports. Individual CPET parameter did not correlate well to the exercise recommendation from ESC. We proposed a scoring system, "score to exercise", which incorporates three CPET parameters with cardiothoracic ratio, ventricular arrhythmia, QRS duration and NYHA functional status. Score to exercise is easy to be assessed and provides useful information for exercise recommendation in patients with repaired TOF.


Assuntos
Arritmias Cardíacas/epidemiologia , Teste de Esforço/normas , Terapia por Exercício/métodos , Tolerância ao Exercício , Consumo de Oxigênio , Tetralogia de Fallot/terapia , Adolescente , Adulto , Ecocardiografia , Eletrocardiografia Ambulatorial , Terapia por Exercício/efeitos adversos , Feminino , Humanos , Imageamento por Ressonância Magnética , Masculino , Pessoa de Meia-Idade , Guias de Prática Clínica como Assunto , Curva ROC , Sociedades Médicas , Taiwan , Função Ventricular Direita , Adulto Jovem
13.
J Magn Reson Imaging ; 44(2): 500-8, 2016 08.
Artigo em Inglês | MEDLINE | ID: mdl-26854646

RESUMO

PURPOSE: To examine possible hemodynamic alterations in adolescent to adult Marfan syndrome (MFS) patients with aortic root dilatation. MATERIALS AND METHODS: Four-dimensional flow MRI was performed in 20 MFS patients and 12 age-matched normal subjects with a 3T system. The cross-sectional areas of 10 planes along the aorta were segmented for calculating the axial and circumferential wall shear stress (WSSaxial , WSScirc ), oscillatory shear index (OSIaxial , OSIcirc ), and the nonroundness (NR), presenting the asymmetry of segmental WSS. Pearson's correlation analysis was performed to present the correlations between the quantified indices and the body surface area (BSA), aortic root diameter (ARD), and Z score of the ARD. P < 0.05 indicated statistical significance. RESULTS: Patients exhibited lower WSSaxial in the aortic root and the WSScirc in the arch (P < 0.05-0.001). MFS patients exhibited higher OSIaxial and OSIcirc in the sinotubular junction and arch, but lower OSIcirc in the descending aorta (all P < 0.05). The NR values were lower in patients (P < 0.05). The WSSaxial or WSScirc exhibited moderate to strong correlations with BSA, ARD, or Z score (R(2) = 0.50-0.72) in MFS patients. CONCLUSION: The significant differences in the quantified indices, which were associated with BSA, ARD, or Z score, in MFS were opposite to previous reports for younger MFS patients, indicating that altered flows in MFS patients may depend on the disease progress. The possible time dependency of hemodynamic alterations in MFS patients strongly suggests that longitudinal follow-up of 4D Flow is needed to comprehend disease progress. J. Magn. Reson. Imaging 2016;44:500-508.


Assuntos
Doenças da Aorta/diagnóstico por imagem , Doenças da Aorta/fisiopatologia , Velocidade do Fluxo Sanguíneo , Angiografia por Ressonância Magnética/métodos , Síndrome de Marfan/diagnóstico por imagem , Síndrome de Marfan/fisiopatologia , Adulto , Doenças da Aorta/patologia , Dilatação Patológica/diagnóstico por imagem , Dilatação Patológica/patologia , Humanos , Síndrome de Marfan/patologia , Pessoa de Meia-Idade , Reprodutibilidade dos Testes , Sensibilidade e Especificidade , Adulto Jovem
14.
Am Heart J ; 169(5): 605-12, 2015 May.
Artigo em Inglês | MEDLINE | ID: mdl-25965707

RESUMO

RATIONALE: A number of randomized trials are underway, which will address the effects of angiotensin receptor blockers (ARBs) on aortic root enlargement and a range of other end points in patients with Marfan syndrome. If individual participant data from these trials were to be combined, a meta-analysis of the resulting data, totaling approximately 2,300 patients, would allow estimation across a number of trials of the treatment effects both of ARB therapy and of ß-blockade. Such an analysis would also allow estimation of treatment effects in particular subgroups of patients on a range of end points of interest and would allow a more powerful estimate of the effects of these treatments on a composite end point of several clinical outcomes than would be available from any individual trial. DESIGN: A prospective, collaborative meta-analysis based on individual patient data from all randomized trials in Marfan syndrome of (i) ARBs versus placebo (or open-label control) and (ii) ARBs versus ß-blockers will be performed. A prospective study design, in which the principal hypotheses, trial eligibility criteria, analyses, and methods are specified in advance of the unblinding of the component trials, will help to limit bias owing to data-dependent emphasis on the results of particular trials. The use of individual patient data will allow for analysis of the effects of ARBs in particular patient subgroups and for time-to-event analysis for clinical outcomes. The meta-analysis protocol summarized in this report was written on behalf of the Marfan Treatment Trialists' Collaboration and finalized in late 2012, without foreknowledge of the results of any component trial, and will be made available online (http://www.ctsu.ox.ac.uk/research/meta-trials).


Assuntos
Antagonistas de Receptores de Angiotensina/uso terapêutico , Síndrome de Marfan/tratamento farmacológico , Metanálise como Assunto , Feminino , Humanos , Masculino , Estudos Prospectivos , Ensaios Clínicos Controlados Aleatórios como Assunto , Projetos de Pesquisa
15.
Circ J ; 79(7): 1609-17, 2015.
Artigo em Inglês | MEDLINE | ID: mdl-25959432

RESUMO

BACKGROUND: Three risk estimation methods for predicting the cardiac outcomes of pregnancy in women with heart disease have been proposed. This study was designed to compare their prediction performance in an Asian cohort with congenital heart disease (CHD). METHODS AND RESULTS: This study enrolled pregnant women with CHD who delivered their babies after the 20th gestational week between 1985 and 2011. Of 268 pregnancies in 190 women with CHD, 18 (6.7%) had cardiac complications. The incidence of maternal cardiac events among women with a CARPREG index of 0, 1 or 2 was 3.4%, 27.3% and 100%. The incidence was 2.7%, 8.6%, 11.1%, 40% and 17.6% for those with a ZAHARA score 0-0.5, 0.51-1.5, 1.51-2.5, 2.51-3.5 and >3.5. Among patients with a modified World Health Organization (WHO) classification I, II, III and IV, the incidence of maternal cardiac events was 0%, 4.0%, 12.2% and 25.7%. The c-statistic was 0.732 (95% confidence interval (CI): 0.589, 0.876; P<0.001) for the CARPREG score, 0.737 (95% CI: 0.611, 0.864; P=0.001) for the ZAHARA score and 0.827 (95% CI: 0.745, 0.909; P<0.001) for the WHO classification. CONCLUSIONS: All 3 risk estimation methods had good performance in predicting maternal cardiac outcomes; however, the modified WHO classification demonstrated superior discrimination and calibration.


Assuntos
Cardiopatias Congênitas , Complicações Cardiovasculares na Gravidez , Adulto , Feminino , Cardiopatias Congênitas/epidemiologia , Cardiopatias Congênitas/patologia , Humanos , Masculino , Gravidez , Complicações Cardiovasculares na Gravidez/epidemiologia , Complicações Cardiovasculares na Gravidez/patologia , Estudos Retrospectivos , Medição de Risco
16.
Acta Cardiol Sin ; 31(6): 473-7, 2015 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-27122910

RESUMO

BACKGROUND: For children with a history of Kawasaki disease (KD), low grade inflammation was generally reported to be associated with persistent coronary artery lesions (CAL). However, this association has not been clearly demonstrated to hold true in KD adolescents and young adults (10-25 years of age). METHODS: We enrolled 104 subjects into our study, who were separated into the following 3 groups and controls: 1): 22 KD patients with angiography-confirmed CAL which persisted for an average of 12.5 years after onset of KD; 2) 38 KD patients with regressed aneurysms; 3) 44 KD patients without any coronary complications from the disease onset; and 4) 31 age-matched (18.7 ± 1.88 years old) healthy controls. Plasma levels of high-sensitivity C reactive protein (hs-CRP) were measured for all participants. RESULTS: Plasma levels of hs-CRP were significantly higher in KD patients than in the controls, regardless of their coronary severity. However, there was no significant difference in hs-CRP levels among KD patients with different severities of CAL. Of the candidate risk factors of elevated hs-CRP such as body mass index, gender, coronary severity, and levels of high-density lipoprotein-cholesterol, linear regression analysis showed the only independent predictor of hs-CRP levels was BMI (ß = 0.306, p = 0.01), rather than patient grouping (p = 0.091). CONCLUSIONS: Our study found that levels of hs-CRP are significantly higher in adolescent and young adult patients with a history of KD, compared with age-matched controls. Low grade inflammation may play a minor role when KD patients enter into adulthood. body mass index (BMI), rather than coronary severity, was independently associated with the elevation of hs-CRP levels, one of biomarkers for further cardiovascular event. Therefore, ongoing control and management of BMI may be one of beneficial strategies that can be employed to help avoid elevation of hs-CRP levels in KD patients. KEY WORDS: Adolescents; High sensitivity-C reactive protein; Kawasaki disease; Young adult.

17.
Acta Cardiol Sin ; 31(6): 478-84, 2015 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-27122911

RESUMO

BACKGROUND: Patients with repaired tetralogy of Fallot (TOF) usually experience progressive right ventricle (RV) dysfunction due to pulmonary regurgitation (PR). This could further worsen the cardiopulmonary function. This study aimed to compare the changes in patient exercise cardiopulmonary test and cardiac magnetic resonance imaging, and consider the implication of these changes. METHODS: Our study examined repaired TOF patients who underwent cardiopulmonary exercise test (CPET) to obtain maximal (peak oxygen consumption, peak VO2) and submaximal parameters (oxygen uptake efficiency plateau, oxygen uptake efficiency plateau (OUEP), and ratio of minute ventilation to carbon dioxide production, VE/VCO2 slope). Additionally, the hemodynamic status was assessed by using cardiac magnetic resonance. Criteria for exclusion included TOF patients with pulmonary atresia, atrioventricular septal defect, or absence of pulmonary valve syndrome. RESULTS: We enrolled 158 patients whose mean age at repair was 7.8 ± 9.1 years (range 0.1-49.2 years) and the mean patient age at CPET was 29.5 ± 12.2 years (range 7.0-57.0 years). Severe PR (PR fraction ≥ 40%) in 53 patients, moderate in 55, and mild (PR fraction < 20%) in 50 patients were noted. The mean RV end-diastolic volume index (RVEDVi) was 113 ± 35 ml/m(2), with 7 patients observed to have a RVEDVi > 163 ml/m(2). The mean left ventricular ejection fraction (LVEF) was 63 ± 8%, left ventricular end-diastolic volume index (LVEDVi) was 65 ± 12 ml/m(2), and LVESVi was 25 ± 14 ml/m(2). CPET revealed significantly decreased peak VO2 (68.5 ± 14.4% of predicted), and fair OUEP (90.3 ± 14.1% of predicted) and VE/VCO2 slope (27.1 ± 5.3). PR fraction and age at repair were negatively correlated with maximal and submaximal exercise indicators (peak VO2 and OUEP). Left ventricular (LV) function and size were positively correlated with peak VO2 and OUEP. CONCLUSIONS: The results of CPET showed that patients with repaired TOF had a low maximal exercise capacity (peak VO2), but a fair submaximal exercise capacity (OUEP and VE/VCO2 slope), suggesting limited exercise capability in high intensity circumstances. PR, LV function and age at total repair were the most important determinants of CPET performance. KEY WORDS: Cardiac magnetic resonance; Cardiopulmonary exercise function; Pulmonary regurgitation; Surgical age; Tetralogy of Fallot.

18.
Acta Cardiol Sin ; 31(6): 500-6, 2015 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-27122914

RESUMO

UNLABELLED: With advances that have been made over the recent decades in transcatheter and surgical interventions, most patients with congenital heart disease (CHD) can survive into adulthood. Overall, probably half of these surviving patients are female. When these female CHD patients reach childbearing age, however, pregnancy management will be a major issue. In order to meet the demands of fetal growth, the maternal cardiovascular system starts a series of adaptations beginning in early pregnancy. These adaptations include: decreased systemic and pulmonary vascular resistances, decreased blood pressure, expansion of the blood volume, increased heart rate and increased cardiac output. For women with CHD, this hemodynamic alteration may increase the risks of adverse cardiovascular events as well as the fetal and neonatal complications. Therefore, proper risk stratification and effective counseling for women with CHD who are planning their pregnancies is an important undertaking. KEY WORDS: Congenital heart disease; Pregnancy.

19.
Am Heart J ; 167(4): 555-61, 2014 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-24655705

RESUMO

BACKGROUND: Right ventricular (RV) outflow tract obstruction (RVOTO) might protect the RV from adverse remodeling caused by significant pulmonary regurgitation (PR) in patients with repaired tetralogy of Fallot (rTOF), but the underlying mechanisms and influences on exercise tolerance remain unclear. This study sought to investigate the impacts from mild RVOTO on ventricular remodeling and exercise capacity in rTOF. METHODS: Eighty-five rTOF patients with a PR fraction ≥20% were assessed with cardiac magnetic resonance, cardiopulmonary exercise test, and echocardiography. Patients with a peak RVOT pressure gradient 20-50 mmHg were considered to have mild RVOTO (n = 29), while those with a gradient <20 mmHg had isolated PR (n = 56). RESULTS: Comparing to patients with isolated PR, patients with combined PR and mild RVOTO had smaller RV and RVOT dimension, better RV and left ventricular (LV) ejection fraction (EF), and superior exercise capacity. PR severity and RV mass/volume ratio were similar between these 2 groups. LVEF coupled with RVEF only in patients with isolated PR. In multivariate analysis, smaller RVOT dimension was independently related to smaller RV dimension (P < .001) and higher RVEF (P = .005). Furthermore, mild RVOTO was independently associated with higher peak oxygen consumption (P = .014) and oxygen uptake efficiency slope (P = .005). CONCLUSIONS: Patients with combined PR and mild RVOTO had better RV remodeling and exercise capacity compared to those with isolated PR. Our findings confirm the benefits from mild residual RVOTO support a policy of conservative RVOTO relief at repair.


Assuntos
Procedimentos Cirúrgicos Cardíacos/efeitos adversos , Ventrículos do Coração/patologia , Complicações Pós-Operatórias , Insuficiência da Valva Pulmonar/diagnóstico , Tetralogia de Fallot/cirurgia , Função Ventricular Esquerda/fisiologia , Obstrução do Fluxo Ventricular Externo/diagnóstico , Adulto , Ecocardiografia , Feminino , Seguimentos , Ventrículos do Coração/diagnóstico por imagem , Ventrículos do Coração/fisiopatologia , Humanos , Imagem Cinética por Ressonância Magnética/métodos , Masculino , Prognóstico , Insuficiência da Valva Pulmonar/etiologia , Insuficiência da Valva Pulmonar/fisiopatologia , Estudos Retrospectivos , Índice de Gravidade de Doença , Tetralogia de Fallot/fisiopatologia , Obstrução do Fluxo Ventricular Externo/complicações , Obstrução do Fluxo Ventricular Externo/fisiopatologia , Pressão Ventricular , Remodelação Ventricular
20.
Mayo Clin Proc ; 89(1): 34-42, 2014 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-24388020

RESUMO

OBJECTIVE: To explore the current epidemiological profile of Marfan syndrome in a general population. PATIENTS AND METHODS: Patients who had received a diagnosis of Marfan syndrome were identified from the Taiwan National Health Insurance database records from January 1, 2000, through December 31, 2012 (average population size, 22,765,535). Cardiovascular events and interventions were identified by using the respective International Classification of Diseases codes. RESULTS: We identified 2329 patients (58% men) with Marfan syndrome. The overall prevalence was 10.2 (95% CI, 9.8-10.7) per 100,000 individuals, with peaks at the age of 15 to 19, 10 to 14, and 20 to 24 years. The minimal birth incidence of 23.3 (95% CI, 21.7-23.3) per 100,000 individuals was estimated in those aged 20 to 29 years. The average annual mortality was 0.23% (69 deaths), mostly owing to cardiac causes (including dissection and sudden death in 40 patients, 58%). Aortic dissection occurred in 226 patients (10%; 61% men) at a mean age of 36.6±10.7 years. The probability of freedom from dissection was 99%, 80%, and 66% at the age of 20, 40, and 50 years, respectively. Of the 69 deaths and 226 dissections during the follow-up period, more than half of the cases occurred before the age of 40 years. Cardiovascular intervention was performed in 360 patients, with early mortality being higher in the emergent operation group (8%) than in the elective group (0%). CONCLUSION: From this national cohort study, the minimal birth incidence was 23.3 per 100,000 individuals, that is, possibly 1 patient with Marfan syndrome per 4286 people. Despite medical advances, aortic dissection still occurs in about one-tenth of the patients and carries a high mortality risk. Early diagnosis and timely medical interventions are warranted.


Assuntos
Síndrome de Marfan/epidemiologia , Adolescente , Adulto , Distribuição por Idade , Idoso , Idoso de 80 Anos ou mais , Causas de Morte , Criança , Pré-Escolar , Bases de Dados Factuais , Feminino , Humanos , Incidência , Lactente , Recém-Nascido , Masculino , Pessoa de Meia-Idade , Prevalência , Taxa de Sobrevida , Taiwan/epidemiologia , Adulto Jovem
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