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2.
Pediatr Obes ; 14(4): e12481, 2019 04.
Artigo em Inglês | MEDLINE | ID: mdl-30417593

RESUMO

BACKGROUND: Given evidence suggesting that sleep impacts on metabolic processes, interventions targeting sleep may improve child physical activity (PA). OBJECTIVES: To describe the potential effect of an intervention to increase sleep on young children's (3-6 years) moderate to vigorous PA (MVPA) and total PA. To determine adherence to the intervention, impact on sleep duration as well as feasibility, uptake and acceptability was also assessed. METHODS: Pilot randomized controlled trial with 76 parent-child dyads randomly allocated to an intervention (n = 38) or control group (n = 38). Parents in the intervention group received a 3-month theory-informed intervention consisting of an online video, a telephone call and two text messages. Child PA was assessed using accelerometers at baseline and approximately 3 months. Parents also completed a sleep log and a telephone interview. RESULTS: The consent rate was 41% (76/186). Estimated effect size for the intervention relative to control was 10.8 min/day for MVPA, 2.7 min/day for PA and 0.9 h for sleep. Sixteen (44%) parents accessed the video, and 18 (50%) received the telephone call. Over 40% of parents found the video and telephone call useful/very useful. CONCLUSIONS: This study reports promising effects that an intervention targeting sleep may improve child MVPA and sleep duration. Some modifications to data collection methods and intervention delivery are needed.


Assuntos
Exercício Físico/fisiologia , Promoção da Saúde/métodos , Sono/fisiologia , Acelerometria , Austrália , Criança , Pré-Escolar , Estudos de Viabilidade , Feminino , Humanos , Masculino , Pais , Projetos Piloto , Estudos Prospectivos
3.
Health Educ Res ; 32(2): 197-205, 2017 04 01.
Artigo em Inglês | MEDLINE | ID: mdl-28380629

RESUMO

Implementation of vegetable and fruit programs in schools is less than optimal. This study aimed to identify, using a theoretical framework, factors associated with implementation of a school vegetable and fruit program; that provides a time in class for children to consume a piece of vegetable or fruit they have brought from home. Three hundred and three randomly selected school principals across the state of New South Wales, Australia responded to a 25-min telephone survey. Principals were asked if their school had implemented a vegetable and fruit program, and which of 12 factors from Damschroder's consolidated framework for implementation research had facilitated or impeded implementation. Multiple logistic regression models examined the association between such factors and program implementation. Seventy-eight percent of schools had a vegetable and fruit program. Schools were significantly more likely to implement the program if the principal believed that: the program was effective (OR = 2.97; P < 0.02); they had sufficient resources to implement the program (OR = 4.22; P < 0.0001); the program would not be difficult to implement (OR = 10.16; P< 0.0001) and that the program was as important as other school priorities (OR = 2.45; P < 0.02). Realizing the intended benefits of vegetable and fruit programs requires widespread implementation by schools. Consideration of principal beliefs about the program effectiveness, resources, difficultly and relative importance in program implementation strategies appear key to increasing program implementation.


Assuntos
Frutas , Promoção da Saúde/métodos , Recursos em Saúde , Instituições Acadêmicas/estatística & dados numéricos , Verduras , Criança , Pré-Escolar , Feminino , Humanos , Masculino , New South Wales , Avaliação de Programas e Projetos de Saúde
4.
Health Educ Res ; 30(2): 262-71, 2015 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-25516479

RESUMO

Despite significant investment in many countries, the extent of schools' adoption of obesity prevention policies and practices has not been widely reported. The aims of this article are to describe Australian schools' adoption of healthy eating and physical activity policies and practices over an 8-year period and to determine if their adoption varies according to schools' size, geographic or socio-economic location. Between 2006 and 2013, a representative randomly selected cohort of primary schools (n = 476) in New South Wales, Australia, participated in four telephone interviews. Repeated measures logistic regression analyses using a Generalised Estimating Equation (GEE) framework were undertaken to assess change over time. The prevalence of all four of the healthy eating practices and one physical activity practice significantly increased, while the prevalence of one physical activity practice significantly decreased. The adoption of practices did not differ by school characteristics. Government investment can equitably enhance school adoption of some obesity prevention policies and practices on a jurisdiction-wide basis. Additional and/or different implementation strategies may be required to facilitate greater adoption of physical activity practices. Ongoing monitoring of school adoption of school policies and practices is needed to ensure the intended benefits of government investment are achieved.


Assuntos
Promoção da Saúde/estatística & dados numéricos , Obesidade/prevenção & controle , Serviços de Saúde Escolar/estatística & dados numéricos , Dieta , Exercício Físico , Promoção da Saúde/legislação & jurisprudência , Humanos , Estudos Longitudinais , New South Wales , Fatores Socioeconômicos
5.
Health Promot J Austr ; 25(2): 136-8, 2014 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-24988137

RESUMO

ISSUE ADDRESSED: School-based programs targeting the prevention of tobacco use are a key strategy for reducing the overall tobacco-related mortality and morbidity in the community. While substantial research investment has resulted in the identification of various effective tobacco prevention interventions in schools, this research investment will not result in public health benefits, unless effectively disseminated and implemented. This rapid review aimed to identify effective implementation or dissemination interventions, targeting the adoption of school-based tobacco prevention programs. METHODS: A systematic search was conducted to identify published systematic reviews that examined the effectiveness of implementation and dissemination strategies for facilitating the adoption of tobacco policies or programs in schools from 1992 to 2012. RESULTS: The search yielded 1028 results, with one relevant systematic review being identified. The review included two controlled studies examining the implementation and dissemination of tobacco prevention programs and guidelines. The two randomised trials examined the delivery of active face-to-face training to implement a school-based curriculum compared with video-delivered or mail-based training. Improvements in the implementation of the programs were reported for the face-to-face training arm in both trials. CONCLUSIONS: Little rigorous evidence exists to guide the implementation and dissemination of tobacco prevention programs in schools. SO WHAT? Few systematic reviews exist to inform the implementation of evidence-based tobacco prevention programs in schools. In the absence of a strong evidence base, health care policymakers and practitioners may need to draw on setting-based frameworks or parallel evidence from other settings to design strategies to facilitate the adoption of tobacco prevention initiatives.


Assuntos
Promoção da Saúde/organização & administração , Disseminação de Informação , Instituições Acadêmicas/organização & administração , Prevenção do Hábito de Fumar , Indústria do Tabaco/legislação & jurisprudência , Política de Saúde , Promoção da Saúde/legislação & jurisprudência , Promoção da Saúde/normas , Humanos , Literatura de Revisão como Assunto , Instituições Acadêmicas/legislação & jurisprudência , Instituições Acadêmicas/normas , Revisões Sistemáticas como Assunto
6.
Support Care Cancer ; 15(4): 405-15, 2007 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-17043776

RESUMO

GOALS OF WORK: Partners of cancer patients may experience significant distress at the time of treatment and many may experience persistent difficulties, although little research has examined their longer term psychosocial outcomes or supportive care needs. MATERIALS AND METHODS: One hundred and fifty-four cancer survivors who were 1-11 years post diagnosis and disease-free and their partners completed mailed questionnaires. MAIN RESULTS: A positive relationship was found between psychological distress and supportive care needs both within and between partner and survivor samples. Partners reported high levels of anxiety and supportive care needs, most frequently concerning relationships and the impact of the cancer illness. Partners within couples reported both shared and unique needs, although agreement on ratings of shared needs was low. Needs did not diminish over time although partners demonstrated psychological resilience and reported positive outcomes. Predictors of distress and unmet needs were explored: physical QOL, relationship satisfaction, and total needs contributed to variability in partners' distress; relationship satisfaction and total needs were associated with survivors' distress. Distress and relationship satisfaction were associated with partners' unmet needs; only distress was associated with survivors' unmet needs. CONCLUSIONS: Partners are not merely providers of support, but need support themselves many years after a cancer diagnosis and in the context of apparently cured disease. The quality of the dyadic relationship may be critical in determining both partner and survivor distress and needs, and may prove a useful target for psychosocial interventions.


Assuntos
Adaptação Psicológica , Neoplasias/psicologia , Apoio Social , Cônjuges/psicologia , Adulto , Idoso , Idoso de 80 Anos ou mais , Características da Família , Feminino , Humanos , Pessoa de Meia-Idade , Avaliação das Necessidades , Qualidade de Vida
7.
Expert Opin Pharmacother ; 2(11): 1849-57, 2001 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-11825321

RESUMO

Fungal eye infections are rare. Trauma associated with contamination by vegetative material, contact lens wear and long term corticosteroid use are common risk factors. The aims of treatment are to preserve visual function, which depends on the rapid diagnosis and efficient administration of appropriate antifungal therapy. This necessitates a clinical suspicion of fungal aetiology and the taking of appropriate smears and cultures as early as possible to identify the fungal organism. Currently there are three main classes of drugs available for use in fungal eye infections: polyenes, azoles as derivatives of imidazoles, and 5-fluorocytosine. Of the polyenes, amphotericin B, natamycin and nystatin are of clinical ophthalmic use. Based on better pharmacokinetic profiles and spectra of antifungal activity, the triazoles are the agents of choice. Successful treatment of fungal keratitis depends on early initiation of specific therapy consisting of topically-applied antifungal agents since topical administration is most likely to provide the best opportunity for achieving therapeutic corneal levels. Hence, the molecular weight of the various antifungal agents is of importance since it influences their ability to penetrate the corneal epithelium. Systemic administration may be necessary for resistant fungal ulcers. For fungal endophthalmitis, to preserve visual function and eliminate the fungal pathogen, topical, systemic and possibly intraocular antifungal therapy is used, although some do not recommend use of systemic agents for exogenous endophthalmitis.


Assuntos
Antifúngicos/uso terapêutico , Infecções Oculares Fúngicas/tratamento farmacológico , Anti-Inflamatórios/uso terapêutico , Antifúngicos/economia , Infecções Oculares Fúngicas/economia , Infecções Oculares Fúngicas/microbiologia , Infecções Oculares Fúngicas/patologia , Humanos , Esteroides
8.
Expert Opin Pharmacother ; 1(7): 1353-65, 2000 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-11249470

RESUMO

Management of patients undergoing cardiac surgery has evolved in recent years as more is understood about the physiological changes and responses that occur during and after cardiopulmonary bypass (CPB). In particular, our understanding of the mechanisms involved in haemostasis and in the inflammatory response to bypass surgery, has allowed significant refinements in patient management. Improvements in the pharmacological conservation of blood loss have been striking, particularly with the development of the serine protease inhibitor, aprotinin (Trasylol, Bayer). Aprotinin represents a significant improvement, especially for patients at high risk, since it reduces the need for allogeneic and (sometimes scarce) blood products. However, in view of its cost, making an appropriate selection of patients most at risk of serious blood loss is a major consideration in the use of aprotinin. While its mechanisms of action are not well understood, the use of aprotinin also appears to reduce inflammatory response to CPB.


Assuntos
Aprotinina/uso terapêutico , Procedimentos Cirúrgicos Cardíacos , Hemostáticos/uso terapêutico , Complicações Pós-Operatórias/tratamento farmacológico , Aprotinina/administração & dosagem , Aprotinina/economia , Perda Sanguínea Cirúrgica/prevenção & controle , Procedimentos Cirúrgicos Cardíacos/economia , Hemostáticos/administração & dosagem , Hemostáticos/economia , Humanos , Inflamação/patologia , Inflamação/prevenção & controle , Período Intraoperatório , Complicações Pós-Operatórias/economia , Complicações Pós-Operatórias/patologia
9.
Heart Lung Circ ; 9(1): 23-6, 2000 May.
Artigo em Inglês | MEDLINE | ID: mdl-16351989

RESUMO

Interest has recently been expressed in developing an Australian adult cardiac surgical registry. Complete national registries of adult cardiac surgery have already been established in many European countries, the USA, Canada and elsewhere. Participating centres contributing to a national registry benefit by being able to benchmark themselves against norms for their particular country. A risk-adjusted database can help surgeons advise their patients of the chances of a good operative outcome. For a surgeon or a surgical unit, the only way to obtain a relevant risk model is to use their own data, and data from units in their particular country. It is also useful to have comparative data from other national registries to compare one's own country with international benchmarks. Since 1996, the European Cardiac Surgical Registry (ECSUR) has put considerable effort into producing unified datasets, harmonised with each other for worldwide use. In 1997, ECSUR launched a minimum cardiac surgical dataset. The worldwide launch of the full international adult cardiac surgical dataset is scheduled for July/August 2000. This dataset would be highly useful for application in Australia. The ECSUR organisation has the capability to analyse data from other countries and could perform this for Australia if requested. However, a better approach would be a national centre in Australia. Funding for national registries around the world has been obtained from Ministries of Health, participating surgical centres, and surgical software vendors. If an Australian national registry is indeed established it will find a ready-made, highly appropriate international cardiac surgical dataset sponsored by ECSUR and the Society of Thoracic Surgeons waiting for adoption by Australia.

10.
Eur J Cardiothorac Surg ; 16(1): 2-8, 1999 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-10456394

RESUMO

OBJECTIVES: Cardiac surgery is the first of any therapeutic discipline to attempt to establish a major international registry on a patient-by-patient basis. Being the first necessarily imposes a challenge since no rules or guidelines exist. Possibilities and boundaries must be identified. METHODS: The EACTS database committee established ECSUR (The European Cardiac SUrgical Registry) with the aim of collecting patient-by-patient information from centres across Europe. It was decided to collect patient data in three areas; adult cardiac surgery, paediatric cardiac surgery and thoracic surgery. Currently, these three areas are in different stages of development, with multinational data collection now having already commenced in adult cardiac surgery. RESULTS: After just over a year of data collection ECSUR has now received data from 98 centres in 30 countries, encompassing approximately 100,000 patients. Three national cardiac surgical registries currently send their data to ECSUR, and agreement has been reached with three other countries for prospective data sharing during 1999. CONCLUSION: This paper describes how ECSUR was established, the on-going status of the project, and how the project has now developed a global perspective through a joint European and US initiative to undertake international dataset unification for surgeons worldwide, by the definition of core adult cardiac, paediatric cardiac and thoracic surgical datasets.


Assuntos
Desenvolvimento de Programas , Sistema de Registros , Cirurgia Torácica , Coleta de Dados , Europa (Continente) , Humanos
11.
Science ; 274(5288): 701, 1996 Nov 01.
Artigo em Inglês | MEDLINE | ID: mdl-8966548
12.
Eur J Cardiothorac Surg ; 10(8): 595-8, 1996.
Artigo em Inglês | MEDLINE | ID: mdl-8875164

RESUMO

OBJECTIVE: To evaluate the results of closure of muscular ventricular septal defects through a left thoracotomy. METHODS: Records of 23 children operated consecutively between 1972 and 1990 were studied. Age of patients was 2.8 +/- 3 years (2 months-10 years), weight 8.9 +/- 5.7 kg (2.6-22 kg). Ten patients (43%) had undergone one and 4 patients (17%) two previous cardiac operations. Late follow-up was obtained from direct examination of patients or from reports of their referring physicians. Bypass time was 89 +/- 28 min (66-167 min). The aorta was cross-clamped for 44 +/- 15 min (21-66 min). Until 1977 operations were performed with moderate hypothermia and intermittent aortic cross-clamping. After 1978 deep hypothermia (20-25 degrees C) and cold crystalloid cardioplegia was used. Ventricular septal defects not accessible from other approaches were closed through a small fish-mouth incision in the apex of the left ventricle. Patients' data were sampled and stored in a computerised database. Risk factors were evaluated by stepwise logistic regression. RESULTS: Four patients died in the hospital (17%); two died later. Two required reoperation for residual/recurrent defects. All patients, except two from abroad, were available for follow-up, which ranged from 36 months to 18 years (mean 11.3 years). All were in NYHA class I. Only two risk factors were identified: the number of ventricular septal defects (P < 0.05) and associated atrial septal defect (P < 0.02). Early echocardiographic evaluation showed good LV size and function in all except one patient, who had a perioperative septal infarction. Late echocardiography performed in six patients demonstrated normal LV shortening without evidence of regional wall abnormality. CONCLUSIONS: Left ventriculotomy is a useful approach for closure of low muscular ventricular septal defects in selected patients.


Assuntos
Procedimentos Cirúrgicos Cardíacos/métodos , Comunicação Interventricular/cirurgia , Complicações Pós-Operatórias/fisiopatologia , Cateterismo Cardíaco , Criança , Pré-Escolar , Estudos de Avaliação como Assunto , Feminino , Seguimentos , Comunicação Interventricular/diagnóstico , Comunicação Interventricular/fisiopatologia , Humanos , Lactente , Masculino , Fatores de Risco , Taxa de Sobrevida
13.
Eur J Cardiothorac Surg ; 10(6): 443-8, 1996.
Artigo em Inglês | MEDLINE | ID: mdl-8817141

RESUMO

OBJECTIVE: Discordant atrioventricular (AV) connection is a rare congenital cardiac anomaly. Associated cardiac defects modify the physiology, clinical presentation, and surgical management of these patients. We have reviewed our overall experience with 90 patients operated for lesions associated with AV discordance between 1975-1990. METHODS: 90 patients, consecutively operated between 1975-1990, were reviewed. Patients' charts, angiograms and echocardiograms were studied. Follow-up was obtained from our records or was requested from referring cardiologists/paediatricians. It was completed in December 1992. For the analysis of risk factors of early death we used P values by chi-squared and Fisher's exact test. For the analysis of the triangulated events, we used the Kaplan-Meier method. Variables associated with P value over 0.20 were eliminated. RESULTS: 90 patients aged 6 months-30 years (mean 9.1 year) underwent repair of cardiac anomalies associated with AV discordance. Three patients had total cavopulmonary connection; the remainder received biventricular repair. Most important associated malformations were ventricular septal defect (77), subpulmonary obstruction (68) and tricuspid valve anomaly (21). 59 patients received extracardiac valved conduit, 10 had tricuspid valve replacement. Thirteen patients died in hospital (14%). One year and 10 year actuarial survival rate was 84% and 70% respectively. The most significant factors for early death were: anatomically abnormal tricuspid valve (P < 0.0001), tricuspid valve regurgitation (P < 0.002), date of operation (P < 0.012), preoperative or perioperative complete heart block (P < 0.015), and tricuspid valve surgery (P < 0.05). Complete heart block developed in 17 (20%) out of 85 patients who did not have it preoperatively. During the follow-up, 22 intracardiac reoperations were necessary (4 died). 63 patients of 73 survivors (86%) remain in NYHA Class 1, 6 in Class 2 and 4 in Class 3. CONCLUSIONS: The operative mortality was 14%. Twenty per cent of patients developed complete heart block. These results improved in the later part of the series (1985-1990); mortality decreased to 5% and incidence of heart block to 14%. Cardiac anomalies associated with AV discordance still present a surgical challenge. The data reported in our study should serve as a useful baseline for evaluation of newer surgical techniques, such as the "double switch" operation.


Assuntos
Átrios do Coração/anormalidades , Cardiopatias Congênitas/cirurgia , Defeitos dos Septos Cardíacos/cirurgia , Ventrículos do Coração/anormalidades , Adolescente , Adulto , Causas de Morte , Criança , Pré-Escolar , Feminino , Átrios do Coração/cirurgia , Bloqueio Cardíaco/diagnóstico , Bloqueio Cardíaco/mortalidade , Cardiopatias Congênitas/diagnóstico , Cardiopatias Congênitas/mortalidade , Defeitos dos Septos Cardíacos/diagnóstico , Defeitos dos Septos Cardíacos/mortalidade , Ventrículos do Coração/cirurgia , Humanos , Lactente , Masculino , Complicações Pós-Operatórias/diagnóstico , Complicações Pós-Operatórias/mortalidade , Taxa de Sobrevida
14.
Ann Hum Genet ; 59(3): 253-69, 1995 07.
Artigo em Inglês | MEDLINE | ID: mdl-7486833

RESUMO

Genetic variation in the COL6A1-COL6A2 gene cluster on chromosome 21 was studied in 113 controls and 58 European families (including control and family subgroups of British/Irish origin) having a child with trisomy 21. There were statistically significant differences among subgroups of trisomic children with and without congenital heart defects (CHD) in distributions of definitive, 3-RFLP haplotype classes received from their nondisjoining and disjoining parents. Haplotypes received by trisomic children with CHD from their disjoining parents were not a random sample of controls' haplotypes. Analysis of parental single-RFLP genotypes and linkage disequilibrium patterns confirmed this parent subgroup differed from a random sample of controls. There were no significant differences in parent subgroup genotype distribution at any of nine control loci distributed along chromosome 21q. This sample showed an association between genetic variation in the COL6A1 gene region and congenital heart defects in trisomy 21.


Assuntos
Síndrome de Down/genética , Cardiopatias Congênitas/genética , Adolescente , Adulto , Criança , Pré-Escolar , Feminino , Variação Genética , Genótipo , Haplótipos , Humanos , Lactente , Desequilíbrio de Ligação , Masculino , Família Multigênica/genética , Polimorfismo de Fragmento de Restrição , Sequências Repetitivas de Ácido Nucleico
15.
Hum Genet ; 93(4): 443-6, 1994 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-7909528

RESUMO

Collagen type VI is a candidate for a role in the pathogenesis of congenital heart defects (CHD) in Down's syndrome. Three restriction fragment length polymorphisms of the COL6A1 gene were used to determine COL6A1 genotypes in 50 families of affected children with trisomy 21 (29 with congenital heart defects and 21 without) and 37 unrelated volunteers. We found seven unusual genotypes in the parents of affected children with Down's syndrome, five being unique to the parents of children with trisomy 21 and CHD. There were no unusual genotypes associated with other chromosome 21 loci. No single COL6A1 genotype was associated with CHD. Thus, the unusual genotypes unique to parents of affected children suggest that genetic variation in the COL6A1 gene region contributes to the pathogenesis of CHD in Down's syndrome.


Assuntos
Colágeno/genética , Síndrome de Down , Cardiopatias Congênitas/genética , Criança , Genótipo , Humanos , Polimorfismo de Fragmento de Restrição
16.
Am J Hum Genet ; 53(2): 462-71, 1993 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-8101041

RESUMO

We have used DNA polymorphisms to study meiotic crossovers of chromosome 21q in 27 nuclear families. Each family had a child with Down syndrome and a congenital heart defect. Twenty DNA polymorphisms on chromosome 21 were used to determine parental and meiotic origin of nondisjunction and to identify crossovers. Twenty-four cases were of maternal origin, and three were of paternal origin. Twenty-two unequivocal crossover events were identified. Sixteen crossovers were observed in 22 chromosome pairs nondisjoining at the second meiotic division. Fifty percent of crossover events in MI nondisjunction are detectable by molecular genetic means. Thus, the results suggest that, in this sample, each nondisjoined chromosome 21 pair has been involved in at least one crossover event.


Assuntos
Troca Genética , Síndrome de Down/complicações , Síndrome de Down/genética , Cardiopatias Congênitas/complicações , Cardiopatias Congênitas/genética , Não Disjunção Genética , Adolescente , Criança , Pré-Escolar , Cromossomos Humanos Par 21 , Pai , Feminino , Genótipo , Humanos , Masculino , Meiose , Mães , Polimorfismo de Fragmento de Restrição , Sequências Repetitivas de Ácido Nucleico
17.
Pediatr Cardiol ; 14(2): 75-81, 1993 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-8469635

RESUMO

This study reviews the spectrum of congenital heart disease and associated anomalies in 59 patients with the CHARGE association. We have analyzed our clinical experience in managing the cardiovascular anomalies and have reviewed outcome and risk factors for mortality. This study also highlights problems of cardiac management in children born with multiple system involvement. Twenty patients have died; actuarial survival was 78% at 1 year and 60% at 10 years. In only four of the nonsurvivors could their demise be ascribed to their underlying congenital heart disease. We found the outlook for survival was poor if more than one of the following three features were present; cyanotic cardiac lesions, bilateral posterior choanal atresia, or tracheoesophageal fistula. However, mortality was largely due not to the structural heart or choanal abnormalities, but instead reflected the underlying pharyngeal and laryngeal incoordination which resulted in aspiration of secretions. Furthermore, outcome is likely to be improved if collaboration between specialist surgical teams allows necessary procedures to be performed using the minimum of anesthetics. Examination of both the short- and long-term management of these children has stressed the importance of a multidisciplinary approach to their care. The pattern of cardiac defects was not random; lesions within the Fallot spectrum accounted for 33% of their congenital heart disease. Atrioventricular septal defects were also overrepresented. Not all cardiovascular defects could be explained by hypothesizing a neural crest etiology.


Assuntos
Anormalidades Múltiplas/diagnóstico , Cardiopatias Congênitas/diagnóstico , Anormalidades Múltiplas/mortalidade , Anormalidades Múltiplas/cirurgia , Adolescente , Causas de Morte , Criança , Pré-Escolar , Feminino , Seguimentos , Cardiopatias Congênitas/mortalidade , Cardiopatias Congênitas/cirurgia , Humanos , Lactente , Recém-Nascido , Masculino , Equipe de Assistência ao Paciente , Complicações Pós-Operatórias/diagnóstico , Complicações Pós-Operatórias/mortalidade , Complicações Pós-Operatórias/cirurgia , Reoperação , Análise de Sobrevida , Síndrome
18.
Arch Otolaryngol Head Neck Surg ; 119(1): 49-54, 1993 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-8417743

RESUMO

A comprehensive evaluation of the otolaryngological abnormalities in 50 patients with colobomata, heart defect, atresia of the choanae, retarded growth or development, genital hypoplasia, and ear anomalies or deafness (CHARGE) was performed. All the patients had ear abnormalities; 96% (48/50) had malformed pinnae, and 54% (27/50) had facial nerve palsies. Only 8% (4/50) had normal hearing, the commonest hearing defect being severe conductive or mixed loss. Eighty-four percent (42/50) of computed tomographic scans of the temporal bone were abnormal, the characteristic abnormality being the combination of a hypoplastic incus and absent semicircular canals. Eighty-six percent (43/50) of patients had upper airway abnormalities. Posterior choanal abnormalities occurred in 56% (28/50), and 42% (21/50) had retrognathia leading to intubation difficulties. Laryngotracheal abnormalities occurred in 38% (19/50), and 14% (7/50) required tracheostomies. Careful upper airway assessment is essential to avoid potentially lethal complications such as aspiration.


Assuntos
Anormalidades Múltiplas , Atresia das Cóanas , Orelha Externa/anormalidades , Laringe/anormalidades , Micrognatismo , Anormalidades Múltiplas/patologia , Atresia das Cóanas/patologia , Coloboma/patologia , Feminino , Transtornos da Audição/complicações , Humanos , Lactente , Recém-Nascido , Masculino , Micrognatismo/patologia , Síndrome
19.
J Pediatr Surg ; 27(5): 558-60, 1992 May.
Artigo em Inglês | MEDLINE | ID: mdl-1625119

RESUMO

CHARGE association was diagnosed in 61 infants, 20 of whom died, mainly during the first 2 years of life. Esophageal atresia and/or tracheoesophageal fistula were present in 10 neonates. Axial skeletal anomalies occurred in 7 of the 10, but none had preaxial limb defects typical of the VATER association. All had major cardiac anomalies, predominantly tetralogy of Fallot. The majority of patients had primary repair of the esophagus. The postoperative course was stormy in all patients, with a high incidence of complications and 70% died. The recognition of features of the CHARGE association is important because it has major prognostic and therapeutic implications.


Assuntos
Anormalidades Múltiplas/diagnóstico , Atresia das Cóanas/complicações , Coloboma/complicações , Surdez/complicações , Orelha Externa/anormalidades , Atresia Esofágica/complicações , Transtornos do Crescimento/complicações , Cardiopatias Congênitas/complicações , Hipogonadismo/complicações , Fístula Traqueoesofágica/complicações , Atresia das Cóanas/diagnóstico , Coloboma/diagnóstico , Surdez/diagnóstico , Atresia Esofágica/diagnóstico , Feminino , Transtornos do Crescimento/diagnóstico , Cardiopatias Congênitas/diagnóstico , Humanos , Hipogonadismo/diagnóstico , Lactente , Recém-Nascido , Masculino , Fístula Traqueoesofágica/diagnóstico
20.
J Am Coll Cardiol ; 19(5): 1041-6, 1992 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-1552092

RESUMO

The presentation and outcome of 50 patients with neonatal Ebstein's anomaly seen from 1961 to 1990 were reviewed. The majority (88%) presented in the 1st 3 days of life; cyanosis (80%) was the most common presenting feature. Associated defects, present in 27 infants (54%), included pulmonary stenosis in 11 and atresia in 7. Nine patients (18%) died in the neonatal period; there were 15 late deaths (due to hemodynamic deterioration in 9, sudden death in 5 and a noncardiac cause in 1) at a mean age of 4.5 years (range 4 months to 19 years). Actuarial survival at 10 years was 61%. A new echocardiographic grade (1 to 4 in order of increasing severity of the defect) was devised with use of the ratio of the area of the right atrium and atrialized right ventricle to the area of the functional right ventricle and left heart chambers. Cardiac death occurred in 0 of 4 infants with grade 1, 1 (10%) of 10 with grade 2, 4 (44%) of 9 with grade 3 and 5 (100%) of 5 with grade 4. In a multivariate analysis of clinical and investigational features at presentation, echocardiographic grade of severity was the best independent predictor of death. Neonates with Ebstein's anomaly have a high early mortality rate and those surviving the 1st month of life remain at high risk of late hemodynamic deterioration or sudden death. Echocardiographic grading of severity of the defect permits prognostic stratification.


Assuntos
Anormalidades Múltiplas/mortalidade , Anomalia de Ebstein/mortalidade , Anormalidades Múltiplas/diagnóstico , Anormalidades Múltiplas/terapia , Cateterismo Cardíaco , Anomalia de Ebstein/classificação , Anomalia de Ebstein/diagnóstico por imagem , Anomalia de Ebstein/terapia , Ecocardiografia , Seguimentos , Cardiopatias Congênitas/diagnóstico , Cardiopatias Congênitas/mortalidade , Cardiopatias Congênitas/cirurgia , Humanos , Recém-Nascido , Análise Multivariada , Prognóstico , Estudos Retrospectivos , Fatores de Risco , Análise de Sobrevida
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