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1.
Medicine (Baltimore) ; 100(9): e24899, 2021 Mar 05.
Artigo em Inglês | MEDLINE | ID: mdl-33655950

RESUMO

BACKGROUNDS: Many studies have evaluated the effect of maternal fever on the development risk of congenital heart diseases (CHDs) in offspring, but the findings were inconsistent. Furthermore, a complete overview of the existing data was also missing. Therefore, we intend to provide updated epidemiologic evidence to estimate the association between maternal fever and the risk of overall CHDs and specific CHD phenotypes in offspring. METHODS: Pubmed, Embase, and Web of Science were searched through March 2020 to identify eligible studies that assessed the association between maternal fever and CHDs risk in offspring. The summary risk estimates were calculated using random-effects models. Potential heterogeneity source was explored by subgroup analyses and potential publication bias was assessed by Begg funnel plots and Begg rank correlation test. RESULTS: Sixteen studies involving 31,922 CHDs cases among 183,563 participants were included in this meta-analysis. Overall, mothers who had a fever experience during preconception and conception periods had a significantly higher risk of overall CHDs in offspring (odds ratio [OR] = 1.45, 95% confidence interval [CI]: 1.21-1.73) when compared with those who did not have a fever experience. For specific CHD phenotypes in offspring, a statistically significant association was found between maternal fever and risk of conotruncal defects (CTD) (OR = 1.38, 95%CI: 1.01-1.89), atrial septal defects (ASD) (OR = 1.48, 95% CI: 1.01-2.17), transposition of the great vessels (TGA) (OR = 1.81, 95% CI: 1.14-2.88), and right ventricular outflow tract obstruction (RVOTO) (OR = 1.66, 95% CI: 1.04-2.65). Relevant heterogeneity moderators have been identified by subgroup analyses, and sensitivity analyses yielded consistent results. CONCLUSIONS: Although the role of potential bias and evidence of heterogeneity should be carefully evaluated, our review indicates that maternal fever is significantly associated with the risk of CHDs in offspring, which highlights that preventing maternal fever during the preconception and conception periods play an important role in decreasing the risk of CHDs in offspring. However, given the limited number of current case-control studies, larger-sample prospective studies are required to further confirm our results. Besides, due to the underlying mechanisms between maternal fever and the risk of specific CHD phenotypes in offspring are still unreported, more research is needed to explore the possible mechanisms.


Assuntos
Fertilização , Febre/complicações , Cardiopatias Congênitas/etiologia , Estudos Observacionais como Assunto , Complicações na Gravidez , Efeitos Tardios da Exposição Pré-Natal/epidemiologia , Medição de Risco/métodos , Feminino , Cardiopatias Congênitas/epidemiologia , Humanos , Mães , Gravidez , Fatores de Risco
2.
Pediatr Rheumatol Online J ; 19(1): 21, 2021 Feb 24.
Artigo em Inglês | MEDLINE | ID: mdl-33627147

RESUMO

IMPORTANCE: Active pediatric COVID-19 pneumonia and MIS-C are two disease processes requiring rapid diagnosis and different treatment protocols. OBJECTIVE: To distinguish active pediatric COVID-19 pneumonia and MIS-C using presenting signs and symptoms, patient characteristics, and laboratory values. DESIGN: Patients diagnosed and hospitalized with active COVID-19 pneumonia or MIS-C at Children's of Alabama Hospital in Birmingham, AL from April 1 through September 1, 2020 were identified retrospectively. Active COVID-19 and MIS-C cases were defined using diagnostic codes and verified for accuracy using current US Centers for Disease Control case definitions. All clinical notes were reviewed for documentation of COVID-19 pneumonia or MIS-C, and clinical notes and electronic medical records were reviewed for patient demographics, presenting signs and symptoms, prior exposure to or testing for the SARS-CoV-2 virus, laboratory data, imaging, treatment modalities and response to treatment. FINDINGS: 111 patients were identified, with 74 classified as mild COVID-19, 8 patients as moderate COVID-19, 8 patients as severe COVID-19, 10 as mild MIS-C and 11 as severe MIS-C. All groups had a male predominance, with Black and Hispanic patients overrepresented as compared to the demographics of Alabama. Most MIS-C patients were healthy at baseline, with most COVID-19 patients having at least one underlying illness. Fever, rash, conjunctivitis, and gastrointestinal symptoms were predominant in the MIS-C population whereas COVID-19 patients presented with predominantly respiratory symptoms. The two groups were similar in duration of symptomatic prodrome and exposure history to the SARS-CoV-2 virus, but MIS-C patients had a longer duration between presentation and exposure history. COVID-19 patients were more likely to have a positive SAR-CoV-2 PCR and to require respiratory support on admission. MIS-C patients had lower sodium levels, higher levels of C-reactive protein, erythrocyte sedimentation rate, d-dimer and procalcitonin. COVID-19 patients had higher lactate dehydrogenase levels on admission. MIS-C patients had coronary artery changes on echocardiography more often than COVID-19 patients. CONCLUSIONS AND RELEVANCE: This study is one of the first to directly compare COVID-19 and MIS-C in the pediatric population. The significant differences found between symptoms at presentation, demographics, and laboratory findings will aide health-care providers in distinguishing the two disease entities.


Assuntos
/fisiopatologia , Síndrome de Resposta Inflamatória Sistêmica/fisiopatologia , Dor Abdominal/fisiopatologia , Adolescente , Afro-Americanos , Asma/epidemiologia , Proteína C-Reativa/metabolismo , /metabolismo , Estudos de Casos e Controles , Criança , Pré-Escolar , Comorbidade , Conjuntivite/fisiopatologia , Doença da Artéria Coronariana , Diabetes Mellitus/epidemiologia , Diarreia/fisiopatologia , Dilatação Patológica , Ecocardiografia , Exantema/fisiopatologia , Feminino , Febre/fisiopatologia , Cardiopatias Congênitas/epidemiologia , Hispano-Americanos , Humanos , Hiponatremia/metabolismo , Masculino , Náusea/fisiopatologia , Neoplasias/epidemiologia , Transtornos do Neurodesenvolvimento/epidemiologia , Obesidade/epidemiologia , Índice de Gravidade de Doença , Distribuição por Sexo , Volume Sistólico , Síndrome de Resposta Inflamatória Sistêmica/epidemiologia , Síndrome de Resposta Inflamatória Sistêmica/metabolismo , Fatores de Tempo , Vômito/fisiopatologia
6.
MMWR Morb Mortal Wkly Rep ; 70(6): 197-201, 2021 Feb 12.
Artigo em Inglês | MEDLINE | ID: mdl-33571179

RESUMO

An estimated 1.4 million adults in the United States live with congenital heart defects (CHDs), yet their health outcomes are not well understood (1). Using self-reported, cross-sectional data from 1,482 respondents in the 2016-2019 Congenital Heart Survey To Recognize Outcomes, Needs, and well-beinG (CH STRONG) (2), CDC and academic partners estimated the prevalence of comorbidities among adults with CHDs aged 20-38 years born in Arizona (AZ), Arkansas (AR), and metropolitan Atlanta, Georgia (GA) compared with the general population (aged 20-38 years) from the National Health and Nutrition Examination Survey (NHANES) during 2015-2018 (3) and the AZ, AR, and GA Behavioral Risk Factor Surveillance Systems (BRFSS) during 2016-2018 (4). Adults with CHDs were more likely than those in the general population to report cardiovascular comorbidities, such as a history of congestive heart failure (4.3% versus 0.2%) and stroke (1.4% versus 0.3%), particularly those with severe CHDs (2). Adults with CHDs were more likely to report current depressive symptoms (15.1% versus 8.5%), but less likely to report previous diagnoses of depression (14.2% versus 22.6%), asthma (12.7% versus 16.9%), or rheumatologic disease (3.2% versus 8.0%). Prevalence of noncardiovascular comorbidities was similar between adults whose CHD was considered severe and those with nonsevere CHDs. Public health practitioners and clinicians can encourage young adults with CHDs to seek appropriate medical care to help them live as healthy a life as possible.


Assuntos
Cardiopatias Congênitas/epidemiologia , Adulto , Arizona/epidemiologia , Arkansas/epidemiologia , Cidades/epidemiologia , Comorbidade , Feminino , Georgia/epidemiologia , Necessidades e Demandas de Serviços de Saúde , Humanos , Masculino , Avaliação de Resultados em Cuidados de Saúde , Qualidade de Vida , Inquéritos e Questionários , Adulto Jovem
7.
Cardiol Young ; 31(3): 344-351, 2021 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-33407975

RESUMO

The COVID-19 pandemic has had a huge influence in almost all areas of life, affecting societies, economics, and health care systems worldwide. The paediatric cardiology community is no exception. As the challenging battle with COVID-19 continues, professionals from the Association for the European Paediatric and Congenital Cardiology receive many questions regarding COVID-19 in a Paediatric and Congenital Cardiology setting. The aim of this paper is to present the AEPC position on frequently asked questions based on the most recent scientific data, as well as to frame a discussion on how to take care of our patients during this unprecedented crisis. As the times are changing quickly and information regarding COVID-19 is very dynamic, continuous collection of evidence will help guide constructive decision-making.


Assuntos
Antiarrítmicos/uso terapêutico , Anticoagulantes/uso terapêutico , Antivirais/uso terapêutico , Arritmias Cardíacas/tratamento farmacológico , Cardiopatias Congênitas/terapia , Fatores Imunológicos/uso terapêutico , Monofosfato de Adenosina/análogos & derivados , Monofosfato de Adenosina/uso terapêutico , Alanina/análogos & derivados , Alanina/uso terapêutico , Arritmias Cardíacas/epidemiologia , Arritmias Cardíacas/fisiopatologia , Síndrome de Brugada/tratamento farmacológico , Síndrome de Brugada/epidemiologia , Síndrome de Brugada/fisiopatologia , /fisiopatologia , Procedimentos Cirúrgicos Cardíacos , Cardiologia , Cardiopatias Congênitas/epidemiologia , Cardiopatias Congênitas/fisiopatologia , Transplante de Coração , Humanos , Transmissão Vertical de Doença Infecciosa , Síndrome do QT Longo/tratamento farmacológico , Síndrome do QT Longo/epidemiologia , Síndrome do QT Longo/fisiopatologia , Miocardite/epidemiologia , Miocardite/fisiopatologia , Miocárdio , Pediatria , Medição de Risco , Sociedades Médicas , Síndrome de Resposta Inflamatória Sistêmica/tratamento farmacológico , Síndrome de Resposta Inflamatória Sistêmica/epidemiologia , Síndrome de Resposta Inflamatória Sistêmica/fisiopatologia , Taquicardia Ventricular/tratamento farmacológico , Taquicardia Ventricular/epidemiologia , Taquicardia Ventricular/fisiopatologia
9.
Niger J Clin Pract ; 24(1): 100-103, 2021 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-33473033

RESUMO

Background: Complex cardiac anomalies are a set of associated heart structural anomalies requiring some adaptive mechanism to maintain life. Objectives: This is to determine pattern and prevalence of common complex cardiac anomalies among children presenting for cardiac surgery in Enugu. Methodology: The children that present for cardiac surgery in our center over a six-month period were admitted and rescreened using a standardized echocardiographic method, information on biodata, oxygen saturation and 2D echo diagnosis were recorded in a proforma. Results: A hundred and one (101) children with different structural heart diseases presented for cardiac surgery, 53 females and 48 males, 99 (98%) had congenital heart defects (49 cyanotic and 50 acyanotic). Complex cardiac anomalies were noted among 49 and 50 were non-complex, 6 (12.2%) acyanotic, and 43 (87.8%) cyanotic complex P < 0.01. This was noted among 21 females and 28 males. Children less than 3 years had 31 cases (63.3%) of the complex heart diseases while children between 5 years to 18 years had 14 (28.6%). The top three complex congenital heart disease were complex Tetralogy of Fallot which was found among 26 (53%) subjects. Tricuspid atresia and Truncus arteriosus were found in 3 children with congenial heart disease each, Shone complex was found in a child, Hypoplastic left heart syndrome was also noted in a child. Conclusion: The incidence of complex congenital heart diseases among prospective Paediatric cardiac surgery patients is high. Efforts should be made to improve access to preventive cardiology in order to curb these diseases.


Assuntos
Procedimentos Cirúrgicos Cardíacos , Cardiopatias Congênitas , Criança , Feminino , Instalações de Saúde , Cardiopatias Congênitas/epidemiologia , Cardiopatias Congênitas/cirurgia , Humanos , Lactente , Masculino , Nigéria/epidemiologia , Estudos Prospectivos
10.
Acta Odontol Scand ; 79(3): 232-240, 2021 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-33415995

RESUMO

AIM: Poor oral health is related with bacteraemia that may lead to endocarditis in children with congenital heart disease (CHD). The aim of this study was to conduct a systematic literature review to compare caries prevalence in children (<18 years of age) with CHD with healthy children. MATERIAL AND METHODS: A literature search of studies from two online databases (Pubmed (MEDLINE) and Scopus) published from January 2000 to October 2019 using Medical subject heading terms Congenital Heart Disease, Congenital Heart Defect, and Caries was done, and manual search from the reference lists of selected publications. PICO (Patient/Population, Intervention, Comparison group, and Outcome) criteria was applied. Quality of the publications was assessed with the modified Newcastle-Ottawa scale. RESULTS: The search resulted in 151 articles of which nine fulfilled the inclusion criteria. Caries prevalence was significantly higher in children with CHD compared to healthy children in three out of nine studies. Information on predisposing background factors was limited but difference in caries prevalence was not attributed to SES. The quality of the studies varied. CONCLUSIONS: Although evidence is limited, the current literature indicates that children with CHD experience a higher caries prevalence compared to healthy children.


Assuntos
Cárie Dentária , Cardiopatias Congênitas , Criança , Cárie Dentária/epidemiologia , Cardiopatias Congênitas/complicações , Cardiopatias Congênitas/epidemiologia , Humanos , Saúde Bucal , Prevalência
11.
Pediatr Cardiol ; 42(1): 1-18, 2021 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-33373013

RESUMO

Congenital heart defects (CHD) is one of the most common types of birth defects. Thanks to advances in surgical techniques and intensive care, the majority of children with severe forms of CHD survive into adulthood. However, this increase in survival comes with a cost. CHD survivors have neurological functioning at the bottom of the normal range. A large spectrum of central nervous system dysmaturation leads to the deficits seen in critical CHD. The heart develops early during gestation, and CHD has a profound effect on fetal brain development for the remainder of gestation. Term infants with critical CHD are born with an immature brain, which is highly susceptible to hypoxic-ischemic injuries. Perioperative blood flow disturbances due to the CHD and the use of cardiopulmonary bypass or circulatory arrest during surgery cause additional neurological injuries. Innate patient factors, such as genetic syndromes and preterm birth, and postoperative complications play a larger role in neurological injury than perioperative factors. Strategies to reduce the disability burden in critical CHD survivors are urgently needed.


Assuntos
Encefalopatias/epidemiologia , Cardiopatias Congênitas/epidemiologia , Complicações Pós-Operatórias/epidemiologia , Adulto , Encéfalo/crescimento & desenvolvimento , Lesões Encefálicas/epidemiologia , Ponte Cardiopulmonar/métodos , Criança , Feminino , Cardiopatias Congênitas/cirurgia , Humanos , Hipóxia-Isquemia Encefálica/epidemiologia , Lactente , Recém-Nascido , Transtornos do Neurodesenvolvimento/epidemiologia , Gravidez , Nascimento Prematuro/epidemiologia , Sobreviventes
13.
J Assoc Physicians India ; 68(11): 42-45, 2020 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-33187036

RESUMO

Background: Nearly one third of all major congenital anomalies are due to congenital heart disease (CHD). Globally the prevalence of CHD in adults varies from 0.66 to 40.0 per 1000 study population. In India the prevalence of CHD is 1.09 to 543 per 1000 study population. CHD is a major global health burden because of increased mortality and morbidity associated with it. Early diagnosis and management can be helpful in improving survival rates, quality of life and prognosis in patients suffering from CHD. Aims and Objectives: The aim of this study was to assess the echocardiographic profile of CHD in Western Rajasthan, India in population above 12 years of age. Methods: This retrospective study was carried out at department of Cardiology and Pediatrics of Dr. S. N. Medical College and attached group of hospitals in Jodhpur, Rajasthan, India. The presence of CHD among all patients above 12 years of age who underwent transthoracic echocardiography over a period of around 4 years from July 2014 to April 2018 was analyzed. Results: A total of 256 patients above 12 years of age were identified as having CHD out of the 33,228 patients who underwent echocardiography during the study period, thus giving a prevalence of 7.7 per 1000 study population. Amongst the total diagnosed CHD cases, 137 (53.52%) patients were male with male to female ratio of 1.15:1. CHDs were diagnosed more commonly between 13 to 24 years of age (54.69%). The commonest type of CHD in the present study was atrial septal defect (27.34%) whereas the most common cyanotic CHD was tetralogy of Fallot (10.94%). Conclusion: Prevalence of CHD in study cohort of age more than 12 years in Western Rajasthan, India was 7.7 per 1000 study population. Profile of CHDs in the present study was similar to that in published literature. We propose to do larger and targeted studies in this age group because many CHDs will become inoperable or even if operated will leave some or other cardiac dysfunction beyond adolescence.


Assuntos
Cardiopatias Congênitas , Qualidade de Vida , Adolescente , Adulto , Criança , Ecocardiografia , Feminino , Cardiopatias Congênitas/diagnóstico por imagem , Cardiopatias Congênitas/epidemiologia , Humanos , Índia/epidemiologia , Masculino , Estudos Retrospectivos , Adulto Jovem
14.
Wiad Lek ; 73(9 cz. 2): 1926-1933, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-33148835

RESUMO

OBJECTIVE: Introduction: The work presents a research project carried out in John Paul II Hospital in Cracow in Clinical Department of Cardiac and Vascular Diseases with the Intensive Cardiac Supervision Subdivision, with participation of 100 (50 F, 50 M) patients with congenital heart defects. The purpose of the work is to resolve the issue of personality specifics, and thus the different characteristics of people who suffer from congenital heart defects. Therefore, the following questions should be answered: Is there a relationship between personality traits and the occurrence of a congenital heart defect? What personality traits are characteristic for patients with congenital heart defects? The aim: We aimed to assess personality traits of clients suffering from PFO and ASD. The article also talks about how coronavirus pandemic affects patients with congenital heart disease. PATIENTS AND METHODS: Materials and methods: The research was conducted by psychologist Adrianna Skoczek. We performed a psychological clinical assessment and conducted the psychological tests like Eysenck Personality Questionnaire-Revised Short Version [EPQ-R(S)] and Eysenck's Impulsivity Inventory [IVE] by Hans J. Eysenck and Sybil G. Eysenck, the State-Trait Anxiety Inventory [STAI] by C. D. Spielberger, R. L. Gorsuch, R. E. Lushene describing personality traits of patients. Patients (F=50, M=50), with ASD (n=70) and with PFO (n=30). RESULTS: Results: In people suffering from ASD, the level of declared empathy was statistically significantly higher than the levels of impulsiveness and tendency to risk-taking. In people suffering from PFO, a statistically significant difference was observed only between the level of psychoticism and other variables. The level of declared empathy was statistically significantly higher than the levels of impulsiveness and tendency to risk-taking, a statistically significant difference was found between the analyzed variables - anxiety as a trait and anxiety as a condition. CONCLUSION: Conclusions: The results of the study allowed us to isolate the specific personality traits of patients suffering from congenital heart defects.


Assuntos
Transtorno do Espectro Autista , Infecções por Coronavirus , Forame Oval Patente , Cardiopatias Congênitas , Pandemias , Pneumonia Viral , Betacoronavirus , Cardiopatias Congênitas/epidemiologia , Humanos , Personalidade
15.
J Biol Regul Homeost Agents ; 34(4 Suppl. 2): 99-106, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-33000608

RESUMO

This study examined the prevalence and distribution of elevated systolic pulmonary arterial pressure, measured by echocardiography, in young patients with down syndrome associated or not with congenital heart disease and surgical correction during childhood. Pulmonary artery systolic pressure, computed by regurgitant tricuspid flow velocity evaluation, is the most frequently used parameter for the screening of pulmonary hypertension. Down syndrome and congenital heart disease often coexist and the probability to detect elevated systolic pulmonary arterial pressure in this setting is high. However, little is known about the evaluation of pulmonary arterial pressure during growth of patients with down syndrome with or without congenital heart disease. We enrolled 47 young patients (55% of male sex; mean age: 18.4 ± 6.0 years), 40 with congenital heart disease and 7 without a cardiac defect. Systolic pulmonary arterial pressure was assessed by echocardiography. No difference was found in the population dichotomized by presence or absence of CHD. Only male sex (p=0.000), highly sensitive troponin-T (P=0.027), tricuspid annular plane systolic excursion (TAPSE, p=0.045) and sPAP (p=0.004) were elevated in surgical group. The ASD was found as, the most common structural abnormality in our patients (50%), followed by VSD (27.5%) and complex CHD (such as complete atrioventricular canal defect, CAVC = 25% and Fallot disease = 15%). Furthermore, about 45% of patients had the combined defect. Only 37.5% of patients underwent to corrective surgery during the first months of life. We observed a significantly increase of sPAP values in patients with complex CHD, such as CAVC (p=0.019) and Fallot disease (p=0.001) but, in the following multivariate analysis performed in the patients with CHD, only Fallot disease remains as independent predictors of elevated values of sPAP (p=0.022). An elevated systolic pulmonary arterial pressure may represent the key screening tool in the diagnostic assessment of suspect pulmonary arterial hypertension in high risk population with down syndrome regardless the presence of congenital heart disease.


Assuntos
Síndrome de Down , Cardiopatias Congênitas , Adolescente , Pressão Sanguínea , Criança , Síndrome de Down/complicações , Síndrome de Down/diagnóstico por imagem , Síndrome de Down/epidemiologia , Feminino , Cardiopatias Congênitas/complicações , Cardiopatias Congênitas/diagnóstico por imagem , Cardiopatias Congênitas/epidemiologia , Humanos , Masculino , Prevalência , Artéria Pulmonar/diagnóstico por imagem , Adulto Jovem
16.
J Biol Regul Homeost Agents ; 34(4 Suppl. 2): 91-97, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-33000607

RESUMO

Congenital hypothyroidism (CH) is the most common endocrine disease in children, according to literature, infants with CH have an increased risk of associated congenital malformations (CM), especially cardiac defects (CD), compared to the general population. We retrospectively analyzed medical records of 255 patients with a positive screening result for CH in the period 1991-2016 followed at our Center. At the time of enrollment, the clinical examination included looking for the presence of heart murmurs and dysmorphic features. In all patients an echocardiography with cardiological evaluation were performed. Of all patients, 191 were included in the final analysis. Of these, 51.3% (98/191) presented an eutopic normally sized thyroid gland while 48.7% (93/191) showed a thyroid dysgenesis. Among the studied infants, 13.6% (26/191) presented CD. The most frequent cardiac anomaly was atrial septal defect (ASD) which was found in 65.4% (17/26) of patients with CD. Other defects were ventricular septal defect (VSD), patent ductus arteriosus (PDA), pulmonary valve stenosis (PvS), transposition of the great vessels (TGV), aortic valve stenosis (AvS). Six patients had multiple defects. In the analysed group, there was no significant relation with sex, type of CH, median blood-TSH (b-TSH) and serum-TSH (s-TSH) values and frequency of CD. There is a high prevalence of CD in CH, indicating the need of routine echocardiography in these patients to achieve an early diagnosis and management of CD.


Assuntos
Hipotireoidismo Congênito , Cardiopatias Congênitas , Criança , Hipotireoidismo Congênito/diagnóstico por imagem , Hipotireoidismo Congênito/epidemiologia , Ecocardiografia , Cardiopatias Congênitas/complicações , Cardiopatias Congênitas/diagnóstico por imagem , Cardiopatias Congênitas/epidemiologia , Humanos , Estudos Retrospectivos
17.
Medicine (Baltimore) ; 99(42): e22740, 2020 Oct 16.
Artigo em Inglês | MEDLINE | ID: mdl-33080735

RESUMO

Prolonged heart rate-corrected QT (QTc) interval is an independent risk factor for sudden cardiac death, which is the hallmark of Timothy syndrome (TS). There are little data on children with syndactyly and QTc prolongation.To evaluate the characteristics and long-term outcomes in children with syndactyly, and to attempt to identify TS in patients with syndactyly and QTc prolongation.This is a retrospective case-control study of children with syndactyly who visited Beijing Jishuitan Hospital between July 2003 and February 2013. The patients with prolonged QTc intervals are matched 1:4 with patients without prolongation. Genetic testing of the CACNA1C gene is routinely performed in patients with QTc prolongation.The mean age at admission is 3.4 ±â€Š2.3 years. Compared with the normal QTc group, those with QTc prolongation showed higher frequencies of congenital heart disease (11.8% vs 1.5%, P = .042), mental retardation and facial dysmorphia (11.8% vs 0, P = .004), and T wave alternans (23.5% vs 4.4%, P = .01). In the multivariable analysis, only T wave alternans (OR = 10.61, 95%CI: 1.39-81.16, P = .023) is independently associated with QTc prolongation in patients with syndactyly. One child with QTc prolongation had a mutation in the CACNA1C gene. No patients with prolonged QTs interval met the threshold for TS.Children with syndactyly and prolonged QTc interval had more multisystem diseases and electrocardiography abnormalities. T wave alternans is independently associated with QTc prolongation in patients with syndactyly.


Assuntos
Síndrome do QT Longo/epidemiologia , Sindactilia/epidemiologia , Canais de Cálcio Tipo L/genética , Estudos de Casos e Controles , Pré-Escolar , China/epidemiologia , Anormalidades Craniofaciais/epidemiologia , Eletrocardiografia , Feminino , Cardiopatias Congênitas/epidemiologia , Humanos , Deficiência Intelectual/epidemiologia , Masculino , Análise Multivariada , Mutação , Estudos Retrospectivos
19.
PLoS One ; 15(9): e0239272, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-32976507

RESUMO

OBJECTIVE: The aim of the current study was to investigate how cerebral and splanchnic oxygen saturation (rSO2-C and rSO2-A) in critically ill children transported in air ambulance was affected by flight with cabin pressurization corresponding to ≥ 5000 feet. A second aim was to investigate any differences between cyanotic and non-cyanotic children in relation to cerebral and splanchnic oxygen saturation during flight ≥ 5000 feet. The variability of the cerebral and splanchnic Near Infrared Spectroscopy (NIRS) sensors was evaluated. DESIGN: NIRS was used to measure rSO2-C and rSO2-A during transport of critically ill children in air ambulance. rSO2 data was collected and stored by the NIRS monitor and extracted and analyzed off-line after the transport. Prior to evaluation of the NIRS signals all zero and floor-effect values were removed. SETTING: The Pediatric Intensive Care Unit (PICU) at Astrid Lindgren Children's Hospital, Karolinska University Hospital in Stockholm, Sweden. PATIENTS: In total, 44 critically ill children scheduled for inter-hospital transport by a specialized pediatric transport team were included in the study between January 2014 and January 2019 (convenience sampling). INTERVENTION: No interventions were conducted. MEASUREMENTS: All study patients were monitored with a cerebral NIRS-sensor placed over the forehead and an abdominal NIRS-sensor placed in the infra-umbilical area for cerebral and splanchnic regional oxygen saturation monitoring, rSO2-C and rSO2-A, respectively. MAIN RESULTS: Complete rSO2-C and rSO2-A data was obtained in 39 patients. Median age was 12 days. Cyanotic congenital heart malformations were present in 9 patients (23%). In 22 patients (56%) rSO2-C decreased at altitude ≥ 5000 feet and in 24 patients (61%) rSO2-A decreased at altitude ≥ 5000 feet compared to baseline (p<0.0001). In 25 patients (64%) the rSO2-C/rSO2-A ratio was greater at altitude ≥ 5000 feet than at baseline. A ratio ≥ 1 was seen in 77% of patients at altitude ≥ 5000 feet compared to in 67% of patients at baseline. CONCLUSION: Both cerebral and splanchnic oxygen saturation decreased at altitude ≥ 5000 feet compared to baseline. In most patients, both cyanotic and non-cyanotic, cerebral oxygen saturation was preserved more than splanchnic oxygen saturation.


Assuntos
Encéfalo/metabolismo , Cardiopatias Congênitas/epidemiologia , Monitorização Fisiológica , Oxigênio/metabolismo , Resgate Aéreo , Altitude , Encéfalo/patologia , Pré-Escolar , Estado Terminal/epidemiologia , Feminino , Cardiopatias Congênitas/metabolismo , Cardiopatias Congênitas/patologia , Humanos , Lactente , Unidades de Terapia Intensiva Pediátrica , Masculino , Oximetria , Suécia/epidemiologia
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