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1.
Respirology ; 29(4): 288-294, 2024 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-38153786

RESUMO

BACKGROUND AND OBJECTIVE: Australia introduced a partial ban on asbestos consumption in 1984. There is continuing concern about exposure to asbestos in the built environment and non-occupational exposures. The aim of this study was to describe epidemiological trends of mesothelioma in Western Australia (WA) over the 60 years since the first case was recorded. METHODS: Every case of mesothelioma notified to the WA Cancer Registry is reviewed by an expert panel. Data include demographic and clinical variables including principal mode of asbestos exposure and age at first exposure. Trends over time for survival, latency and pathological subtype of mesothelioma where analysed. Incidence rates for cases exposed during home renovation where calculated. RESULTS: Two thousand seven hundred ninety-six cases of mesothelioma were identified with males comprising the majority (n = 2368, 84.7%). The median (IQR) age at diagnosis was 70 (62-78) years, and median latency of 47 (38-55) years. Pleural mesothelioma was recorded in 2620 (93.7%) cases with the epithelioid subtype most prevalent (n = 1730, 61.9%). Overall, median survival was 298 (128-585) days and latency 46 (37-54) years, both effectively doubling over the study period. Non-occupational exposures were proportionally higher in females (52.6%), compared with males (9.5%). Home renovation was the primary exposure in 227 (8.1%) cases, with number of cases and incidence rate ratio peaking in 2005/09 but subsequently decreasing. CONCLUSION: The annual number of cases of mesothelioma in WA may have hit a plateau. The majority of females have non-occupational exposures and incidence rates from home renovation exposure may have peaked, suggesting the ban on asbestos has been effective.


Assuntos
Amianto , Mesotelioma Maligno , Mesotelioma , Neoplasias Pleurais , Masculino , Feminino , Humanos , Austrália Ocidental/epidemiologia , Austrália/epidemiologia , Mesotelioma/epidemiologia , Amianto/efeitos adversos , Neoplasias Pleurais/etiologia , Neoplasias Pleurais/complicações , Sistema de Registros , Incidência
2.
Respirology ; 28(6): 543-550, 2023 06.
Artigo em Inglês | MEDLINE | ID: mdl-36889745

RESUMO

BACKGROUND AND OBJECTIVE: Asbestos is a major risk factor for lung cancer, with or without tobacco smoke exposure. Low dose computed tomography (LDCT) screening for early lung cancer is effective but only when targeting high risk populations. This study aimed to analyse the effectiveness of LDCT screening in an asbestos exposed population and to compare lung cancer screening program (LCSP) eligibility criteria. METHODS: Participants in an asbestos health surveillance program, the Western Australia Asbestos Review Program, underwent at least one LDCT scan and lung function assessment as part of annual review between 2012 and 2017. Lung cancer cases were confirmed through linkage to the WA cancer registry. Theoretical eligibility for different screening programs was calculated. RESULTS: Five thousand seven hundred and two LDCT scans were performed on 1743 individuals. The median age was 69.8 years, 1481 (85.0%) were male and 1147 (65.8%) were ever-smokers (median pack-year exposure of 20.0). Overall, 26 lung cancers were detected (1.5% of the population; 3.5 cases per 1000 person-years of observation). Lung cancer was early stage in 86.4% and four (15.4%) cases were never smokers. Based on current lung screening program criteria, 1299 (74.5%) of this population, including the majority (17, 65.4%) of lung cancer cases, would not have been eligible for any LCSP. CONCLUSION: This population is at raised risk despite modest tobacco exposure. LDCT screening is effective at identifying early-stage lung cancer in this population and existing lung cancer risk criteria do not capture this population adequately.


Assuntos
Amianto , Neoplasias Pulmonares , Humanos , Masculino , Idoso , Feminino , Neoplasias Pulmonares/diagnóstico por imagem , Neoplasias Pulmonares/epidemiologia , Detecção Precoce de Câncer/métodos , Amianto/efeitos adversos , Fatores de Risco , Pulmão/diagnóstico por imagem , Programas de Rastreamento/efeitos adversos
3.
BMJ Open Respir Res ; 9(1)2022 12.
Artigo em Inglês | MEDLINE | ID: mdl-36581353

RESUMO

INTRODUCTION: Deliberate exposure to medical ionising radiation should be as low as reasonably practicable but the reduction of radiation from CT should be balanced against diagnostic image quality. The ability of ultra-low-dose CT (uLDCT: similar radiation to chest X-ray) to demonstrate low contrast abnormalities (emphysema and interstitial lung abnormality (ILA)) is unclear.The aim of this cross-sectional study was to analyse the lung parenchymal findings from uLDCT scans against physiological measures of respiratory function. METHODS: WA Asbestos Review Programme participants were eligible if they had an uLDCT scan and lung function assessment between Janary and December 2018. All scans were performed using a single CT machine and reported using a standardised, semiquantitative synoptic report which includes emphysema and linear fibrosis (ILA) scores. RESULTS: Of 1344 participants, median (IQR) age was 72.0 (65.0-78.0) years, the majority were males (84.9%) with mixed occupational asbestos exposure (68.1%). There were 721 (53.6%) with no abnormality, 158 (11.8%) with emphysema, 465 (34.6%) with ILA. Mean radiation dose was 0.12 mSv. There was statistically significant between group differences for all physiological parameters of lung function compared with controls. For instance, the emphysema score significantly correlated with obstructive forced expiratory volume in 1 s (FEV1)/forced vital capacity ratio (r=0.512), per cent predicted FEV1 (r=0.24) and lower diffusion of carbon monoxide (DLCO) (r=0.337). Multivariate modelling demonstrated that increasing age, emphysema and fibrosis scores predicted reduced DLCO (adjusted R2=0.30). DISCUSSION: uLDCT-detected parenchymal lung abnormalities correlate strongly with significant changes on lung function testing suggesting the observed CT abnormalities are of physiological and clinical significance.


Assuntos
Amianto , Enfisema , Pneumopatias , Enfisema Pulmonar , Masculino , Humanos , Idoso , Feminino , Estudos de Coortes , Estudos Transversais , Tomografia Computadorizada por Raios X/métodos , Pulmão/diagnóstico por imagem , Enfisema Pulmonar/diagnóstico por imagem , Enfisema Pulmonar/etiologia , Amianto/efeitos adversos , Fibrose
4.
PLoS One ; 16(7): e0255000, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-34280228

RESUMO

BACKGROUND: Most evidence for interpregnancy interval (IPI) and adverse birth outcomes come from studies that are prone to incomplete control for confounders that vary between women. Comparing pregnancies to the same women can address this issue. METHODS: We conducted an international longitudinal cohort study of 5,521,211 births to 3,849,193 women from Australia (1980-2016), Finland (1987-2017), Norway (1980-2016) and the United States (California) (1991-2012). IPI was calculated based on the time difference between two dates-the date of birth of the first pregnancy and the date of conception of the next (index) pregnancy. We estimated associations between IPI and preterm birth (PTB), spontaneous PTB, and small-for-gestational age births (SGA) using logistic regression (between-women analyses). We also used conditional logistic regression comparing IPIs and birth outcomes in the same women (within-women analyses). Random effects meta-analysis was used to calculate pooled adjusted odds ratios (aOR). RESULTS: Compared to an IPI of 18-23 months, there was insufficient evidence for an association between IPI <6 months and overall PTB (aOR 1.08, 95% CI 0.99-1.18) and SGA (aOR 0.99, 95% CI 0.81-1.19), but increased odds of spontaneous PTB (aOR 1.38, 95% CI 1.21-1.57) in the within-women analysis. We observed elevated odds of all birth outcomes associated with IPI ≥60 months. In comparison, between-women analyses showed elevated odds of adverse birth outcomes for <12 month and >24 month IPIs. CONCLUSIONS: We found consistently elevated odds of adverse birth outcomes following long IPIs. IPI shorter than 6 months were associated with elevated risk of spontaneous PTB, but there was insufficient evidence for increased risk of other adverse birth outcomes. Current recommendations of waiting at least 24 months to conceive after a previous pregnancy, may be unnecessarily long in high-income countries.


Assuntos
Intervalo entre Nascimentos , Resultado da Gravidez/epidemiologia , Nascimento Prematuro/epidemiologia , Adulto , Estudos de Coortes , Países Desenvolvidos , Feminino , Humanos , Recém-Nascido de Baixo Peso , Recém-Nascido , Recém-Nascido Pequeno para a Idade Gestacional , Estudos Longitudinais , Idade Materna , Gravidez , Nascimento Prematuro/patologia , Adulto Jovem
5.
BMC Cancer ; 21(1): 740, 2021 Jun 27.
Artigo em Inglês | MEDLINE | ID: mdl-34176480

RESUMO

BACKGROUND: NUT carcinoma (NC), previously known as NUT midline carcinoma, is a rare and very aggressive cancer that occurs in both children and adults. NC is largely chemoresistant, with an overall survival of less than 7 months. Because the carcinoma is not restricted to a particular organ, diagnosis is often a challenge. In the absence of a clearly determined incidence for NC, we sought to study the diagnosis of patients in a well-defined population. METHODS: We systematically reviewed records of all patients that presented to the Oncology Department of the Princess Margaret Hospital for Children from 1989 to 2014. This institution in the geographically isolated state of Western Australia has a catchment population of around 2 million. We then identified all high grade undifferentiated sarcomas or carcinomas in the 0-16 year age group. RESULTS: Over 26 years, we found 14 patients of 16 years or younger with undifferentiated malignant tumors. Of these, five tumors were positive by immunohistochemistry for the NUT/NUTM1 (Nuclear Protein in Testis) protein and/or the translocation t(15;19). Three patients presented with thoracic tumors, one with a para-spinal tumor, and one had an upper airway nasopharyngeal carcinoma. In all five cases, there was an initial response to therapy and then progression. This 26-year survey was conducted in a geographically isolated state with a well-defined population, and we determined an estimated incidence of NC of around 0.41 per million child years (0-16 yrs. of age) at risk. From three patients it was feasible to derive cell lines for further genetic analyses and drug screening. CONCLUSIONS: For the first time, the incidence of NC could be determined in a well-defined geographic area. The calculated rate of NC incidence is consistent with a history of under-recognition for this malignancy. These findings indicate that improved diagnostic detection of NC would enable better management and counselling of patients. Our findings emphasize the heterogeneity of NC, and they highlight the need to develop personalised therapy options, and to consider a diagnosis of NC in undifferentiated malignant tumors.


Assuntos
Neoplasias de Células Escamosas/epidemiologia , Sarcoma/epidemiologia , Adolescente , Criança , Pré-Escolar , Feminino , História do Século XX , História do Século XXI , Humanos , Lactente , Recém-Nascido , Masculino , Austrália Ocidental
6.
Disabil Health J ; 13(2): 100871, 2020 04.
Artigo em Inglês | MEDLINE | ID: mdl-31806497

RESUMO

BACKGROUND: Pregnancy in women with intellectual disability (ID) is increasingly recognised, along with their increased likelihood of experiencing risk factors for adverse pregnancy and infant outcomes. OBJECTIVES: We aimed to compare risks of socio-demographic, pregnancy and infant outcomes of women with ID to other women. METHODS: All mothers with children born in Western Australia from 1983 to 2012 were linked to the population-based Intellectual Disability Exploring Answers database. Mothers with ID were matched by age and Aboriginality to a population sample of mothers without ID. Pregnancy and birth outcomes were compared for both groups and logistic regression was used to compare outcome risks. RESULTS: Compared to non-Aboriginal mothers without ID, non-Aboriginal mothers with ID were more likely to be of low socio-economic status, be without partner, smoke antenatally and have pre-existing asthma. They had a 1.5 times increased risk of pregnancy complications, specifically pre-eclampsia, urinary tract infection, threatened preterm labour and post-partum haemorrhage. After adjustment for maternal medical conditions and pregnancy complications, infants of Aboriginal mothers with ID had twice the risk of preterm birth and 1.6 times the risk of having percentage of optimal head circumference <95% compared to infants of Aboriginal mothers without ID. Infants of both Aboriginal and non-Aboriginal mothers with ID were more likely to have percentage of optimal birth weight <85% compared to those without ID. CONCLUSIONS: For mothers with ID, modifiable risk factors for adverse outcomes need addressing. They may require additional assistance during pregnancy, including more frequent consultations and support to assist with pregnancy management.


Assuntos
Pessoas com Deficiência/psicologia , Deficiência Intelectual/complicações , Deficiência Intelectual/psicologia , Mães/psicologia , Complicações na Gravidez/psicologia , Resultado da Gravidez/psicologia , Nascimento Prematuro/psicologia , Adulto , Criança , Feminino , Humanos , Lactente , Recém-Nascido , Modelos Logísticos , Mães/estatística & dados numéricos , Gravidez , Fatores de Risco , Adulto Jovem
7.
BMJ Open ; 9(1): e027941, 2019 01 29.
Artigo em Inglês | MEDLINE | ID: mdl-30700492

RESUMO

INTRODUCTION: Short interpregnancy interval (IPI) has been linked to adverse pregnancy outcomes. WHO recommends waiting at least 2 years after a live birth and 6 months after miscarriage or induced termination before conception of another pregnancy. The evidence underpinning these recommendations largely relies on data from low/middle-income countries. Furthermore, recent epidemiological investigations have suggested that these studies may overestimate the effects of IPI due to residual confounding. Future investigations of IPI effects in high-income countries drawing from large, population-based data sources are needed to inform IPI recommendations. We aim to assess the impact of IPIs on maternal and child health outcomes in high-income countries. METHODS AND ANALYSIS: This international longitudinal retrospective cohort study will include more than 18 million pregnancies, making it the largest study to investigate IPI in high-income countries. Population-based data from Australia, Finland, Norway and USA will be used. Birth records in each country will be used to identify consecutive pregnancies. Exact dates of birth and clinical best estimates of gestational length will be used to estimate IPI. Administrative birth and health data sources with >99% coverage in each country will be used to identify maternal sociodemographics, pregnancy complications, details of labour and delivery, birth and child health information. We will use matched and unmatched regression models to investigate the impact of IPI on maternal and infant outcomes, and conduct meta-analysis to pool results across countries. ETHICS AND DISSEMINATION: Ethics boards at participating sites approved this research (approval was not required in Finland). Findings will be published in peer-reviewed journals and presented at international conferences, and will inform recommendations for optimal IPI in high-income countries. Findings will provide important information for women and families planning future pregnancies and for clinicians providing prenatal care and giving guidance on family planning.


Assuntos
Intervalo entre Nascimentos , Internacionalidade , Complicações na Gravidez/epidemiologia , Austrália , Estudos de Coortes , Países Desenvolvidos , Feminino , Finlândia , Humanos , Noruega , Gravidez , Projetos de Pesquisa , Estados Unidos
8.
J Am Heart Assoc ; 7(24): e010223, 2018 12 18.
Artigo em Inglês | MEDLINE | ID: mdl-30561268

RESUMO

Background Acute rheumatic fever ( ARF ) and rheumatic heart disease cause substantial burdens worldwide. Long-term antibiotic secondary prophylaxis is used to prevent disease progression, but evidence for benefits of different adherence levels is limited. Using data from northern Australia, we identified factors associated with adherence, and the association between adherence and ARF recurrence, progression to rheumatic heart disease, worsening or improvement of rheumatic heart disease, and mortality. Methods and Results Factors associated with adherence (percent of doses administered) were analyzed using logistic regression. Nested case-control and case-crossover designs were used to investigate associations with clinical outcomes; conditional logistic regression was used to estimate odds ratios ( OR ) with 95% CIs Adherence estimates (7728) were analyzed. Being female, younger, having more-severe disease, and living remotely were associated with higher adherence. Alcohol misuse was associated with lower adherence. The risk of ARF recurrence did not decrease until ≈40% of doses had been administered. Receiving <80% was associated with a 4-fold increase in the odds of ARF recurrence (case-control OR : 4.00 [95% CI : 1.7-9.29], case-crossover OR : 3.31 [95% CI : 1.09-10.07]) and appeared to be associated with increased all-cause mortality (case-control OR : 1.90 [95% CI : 0.89-4.06]; case-crossover OR 1.91 [95% CI : 0.51-7.12]). Conclusions We show for the first time that increased adherence to penicillin prophylaxis is associated with reduced ARF recurrence, and a likely reduction in mortality, in our setting. These findings can motivate patients to receive doses since even relatively low adherence can be beneficial, and additional doses further reduce adverse clinical outcomes.


Assuntos
Antibacterianos/administração & dosagem , Adesão à Medicação , Penicilinas/administração & dosagem , Febre Reumática/prevenção & controle , Cardiopatia Reumática/prevenção & controle , Prevenção Secundária/métodos , Adolescente , Adulto , Idoso , Criança , Pré-Escolar , Esquema de Medicação , Feminino , Conhecimentos, Atitudes e Prática em Saúde , Humanos , Masculino , Adesão à Medicação/etnologia , Pessoa de Meia-Idade , Havaiano Nativo ou Outro Ilhéu do Pacífico/psicologia , Northern Territory/epidemiologia , Fatores de Proteção , Recidiva , Estudos Retrospectivos , Febre Reumática/etnologia , Febre Reumática/microbiologia , Febre Reumática/mortalidade , Cardiopatia Reumática/etnologia , Cardiopatia Reumática/microbiologia , Cardiopatia Reumática/mortalidade , Fatores de Risco , Fatores de Tempo , Resultado do Tratamento , Adulto Jovem
9.
Int J Chron Obstruct Pulmon Dis ; 13: 1927-1935, 2018.
Artigo em Inglês | MEDLINE | ID: mdl-29942125

RESUMO

Early identification of people at risk of developing COPD is crucial for implementing preventive strategies. We aimed to systematically review and assess the performance of all published models that predicted development of COPD. A search was conducted to identify studies that developed a prediction model for COPD development. The Checklist for Critical Appraisal and Data Extraction for Systematic Reviews of Prediction Modelling Studies was followed when extracting data and appraising the selected studies. Of the 4,481 records identified, 30 articles were selected for full-text review, and only four of these were eligible to be included in the review. The only consistent predictor across all four models was a measure of smoking. Sex and age were used in most models; however, other factors varied widely. Two of the models had good ability to discriminate between people who were correctly or incorrectly classified as at risk of developing COPD. Overall none of the models were particularly useful in accurately predicting future risk of COPD, nor were they good at ruling out future risk of COPD. Further studies are needed to develop new prediction models and robustly validate them in external cohorts.


Assuntos
Lista de Checagem , Doença Pulmonar Obstrutiva Crônica/etiologia , Doença Pulmonar Obstrutiva Crônica/prevenção & controle , Fatores Etários , Teorema de Bayes , Feminino , Humanos , Estudos Longitudinais , Masculino , Doença Pulmonar Obstrutiva Crônica/diagnóstico , Medição de Risco , Fatores de Risco , Fatores Sexuais
10.
Am J Ind Med ; 61(7): 615-620, 2018 07.
Artigo em Inglês | MEDLINE | ID: mdl-29797780

RESUMO

BACKGROUND: Studies comparing different forms of asbestos are rare, and limited by the failure to compare results with unexposed populations. We compare autoimmune responses among former workers and residents of the crocidolite mining and milling town of Wittenoom, Western Australia, with an unexposed population. METHODS: ANA testing using indirect immunofluorescence was performed on randomly selected serum samples from Wittenoom workers or residents and compared with those from participants of another unexposed cohort study. RESULTS: ANA scores were higher in the Wittenoom participants compared with Busselton and the odds of being ANA positive was fivefold greater among Wittenoom participants than Busselton (OR 5.5, 95%CI 2.3-13.0). CONCLUSIONS: This study is the first to report increased ANA positivity among persons exposed exclusively to crocidolite. This finding of a high frequency of positive ANA tests among crocidolite-exposed subjects may be an indicator for an increased risk of systemic autoimmune diseases and needs further scrutiny.


Assuntos
Anticorpos Antinucleares/imunologia , Asbesto Crocidolita , Mineradores , Mineração , Exposição Ocupacional/estatística & dados numéricos , Adulto , Idoso , Idoso de 80 Anos ou mais , Asma/epidemiologia , Autoanticorpos/imunologia , Estudos de Casos e Controles , Exposição Ambiental/estatística & dados numéricos , Feminino , Humanos , Modelos Logísticos , Masculino , Pessoa de Meia-Idade , Razão de Chances , Prevalência , Características de Residência , Rinite Alérgica Sazonal/epidemiologia , Austrália Ocidental/epidemiologia
11.
Child Neurol Open ; 4: 2329048X16688125, 2017.
Artigo em Inglês | MEDLINE | ID: mdl-28503625

RESUMO

The risk of autism spectrum disorder varies by maternal race-ethnicity, immigration status, and birth region. In this retrospective cohort study, Western Australian state registries and a study population of 134 204 mothers enabled us to examine the odds of autism spectrum disorder with intellectual disability in children born from 1994 to 2005 by the aforementioned characteristics. We adjusted for maternal age, parity, socioeconomic status, and birth year. Indigenous women were 50% less likely to have a child with autism spectrum disorder with intellectual disability than Caucasian, nonimmigrant women. Overall, immigrant women were 40% less likely to have a child with autism spectrum disorder with intellectual disability than nonimmigrant women. However, Black women from East Africa had more than 3.5 times the odds of autism spectrum disorder with intellectual disability in their children than Caucasian nonimmigrant women. Research is implicated on risk and protective factors for autism spectrum disorder with intellectual disability in the children of immigrant women.

12.
Br J Cancer ; 116(6): 703-708, 2017 Mar 14.
Artigo em Inglês | MEDLINE | ID: mdl-28196068

RESUMO

BACKGROUND: Survival with the epithelioid subtype of malignant mesothelioma (MM) is longer than the biphasic or sarcomatoid subtypes. There is concern that cytology-diagnosed epithelioid MM may underdiagnose the biphasic subtype. This study examines survival differences between patients with epithelioid MM diagnosed by cytology only and other subtypes diagnosed by histology. METHODS: Demographics, diagnosis method, MM subtype and survival were extracted from the Western Australia (WA) Mesothelioma Registry, which records details of all MM cases occurring in WA. RESULTS: A total of 2024 MM cases were identified over 42 years. One thousand seven hundred forty-four (86.2%) were male, median (IQR) age was 68.6 (60.4-77.0) years. A total of 1212 (59.9%) cases were identified as epithelioid subtype of which 499 (41.2%) were diagnosed using fluid cytology only. Those with a cytology-only diagnosis were older than the histology group (median 70.2 vs 67.6 years, P<0.001), but median survival was similar (cytology 10.6 (5.5-19.2) vs histology 11.1 (4.8-19.8) months, P=0.727) and Cox regression modelling adjusting for age, sex, site and time since first exposure showed no difference in survival between the different diagnostic approaches. CONCLUSIONS: Survival of cytologically and histologically diagnosed epithelioid MM cases does not differ. A diagnostic tap should be considered adequate to diagnose epithelioid MM without need for further invasive testing.


Assuntos
Citodiagnóstico/métodos , Imuno-Histoquímica/métodos , Neoplasias Pulmonares/mortalidade , Mesotelioma/mortalidade , Neoplasias Pleurais/mortalidade , Idoso , Feminino , Seguimentos , Humanos , Neoplasias Pulmonares/diagnóstico , Neoplasias Pulmonares/epidemiologia , Masculino , Mesotelioma/diagnóstico , Mesotelioma/epidemiologia , Mesotelioma Maligno , Pessoa de Meia-Idade , Estadiamento de Neoplasias , Neoplasias Pleurais/diagnóstico , Neoplasias Pleurais/epidemiologia , Prognóstico , Sistema de Registros , Taxa de Sobrevida , Austrália Ocidental
14.
Eur Radiol ; 27(8): 3485-3490, 2017 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-28083692

RESUMO

OBJECTIVES: The correlation between ultra low dose computed tomography (ULDCT)-detected parenchymal lung changes and pulmonary function abnormalities is not well described. This study aimed to determine the relationship between ULDCT-detected interstitial lung disease (ILD) and measures of pulmonary function in an asbestos-exposed population. METHODS: Two thoracic radiologists independently categorised prone ULDCT scans from 143 participants for ILD appearances as absent (score 0), probable (1) or definite (2) without knowledge of asbestos exposure or lung function. Pulmonary function measures included spirometry and diffusing capacity to carbon monoxide (DLCO). RESULTS: Participants were 92% male with a median age of 73.0 years. CT dose index volume was between 0.6 and 1.8 mGy. Probable or definite ILD was reported in 63 (44.1%) participants. Inter-observer agreement was good (k = 0.613, p < 0.001). There was a statistically significant correlation between the ILD score and both forced expiratory volume in 1 second (FEV1) and forced vital capacity (FVC) (r = -0.17, p = 0.04 and r = -0.20, p = 0.02). There was a strong correlation between ILD score and DLCO (r = -0.34, p < 0.0001). CONCLUSION: Changes consistent with ILD on ULDCT correlate well with corresponding reductions in gas transfer, similar to standard CT. In asbestos-exposed populations, ULDCT may be adequate to detect radiological changes consistent with asbestosis. KEY POINTS: • Interobserver agreement for the ILD score using prone ULDCT is good. • Prone ULDCT appearances of ILD correlate with changes in spirometric observations. • Prone ULDCT appearances of ILD correlate strongly with changes in gas transfer. • Prone ULDCT may provide sufficient radiological evidence to inform the diagnosis of asbestosis.


Assuntos
Asbestose/diagnóstico por imagem , Idoso , Asbestose/diagnóstico , Asbestose/fisiopatologia , Feminino , Volume Expiratório Forçado/fisiologia , Humanos , Pulmão/fisiopatologia , Masculino , Pessoa de Meia-Idade , Variações Dependentes do Observador , Doses de Radiação , Radiografia Torácica/métodos , Testes de Função Respiratória/métodos , Índice de Gravidade de Doença , Espirometria , Tomografia Computadorizada por Raios X/métodos , Capacidade Vital/fisiologia
15.
Neurology ; 87(21): 2206-2213, 2016 Nov 22.
Artigo em Inglês | MEDLINE | ID: mdl-27770071

RESUMO

OBJECTIVE: To investigate seizure outcomes and their relationships to genotype and functional abilities in individuals with the cyclin-dependent kinase-like-5 (CDKL5) disorder. METHODS: Using the International CDKL5 Disorder Database, we identified 172 cases with a pathogenic CDKL5 mutation. We categorized individual mutations into 4 groups based on predicted structural and functional consequences. Negative binomial regression was used to model the linear association between current seizure rate and mutation group, current level of assistance required to walk 10 steps, and the highest level of expressive communication used to convey refusal or request. RESULTS: All but 3 (169/172) patients had a history of epilepsy. The median age at seizure onset was 6 weeks (range 1 week-1.5 years) and the median seizure rate at ascertainment was 2 per day (range 0-20 per day). After adjusting for walking ability and confounders including use or otherwise of polytherapy, seizure rate was lower in those with truncating mutations between aa172 and aa781 compared to those with no functional protein (incidence rate ratio [IRR] 0.57; 95% confidence interval [CI] 0.35-0.93). Ability to walk and use of spoken language were associated with lower rates of current seizures when compared to those with the least ability after adjusting for genotype (walking: IRR 0.62; 95% CI 0.39-0.99, communication: IRR 0.48; 95% CI 0.23-1.02). At a median age at questionnaire completion of 5 years, those previously treated with corticosteroids had more frequent seizures than those who have never been treated, whether or not there was a history of infantile spasms. CONCLUSIONS: Epilepsy is pervasive but not mandatory for the CDKL5 disorder. Genotype and functional abilities were related to seizure frequency, which appears refractory to antiepileptic drugs.


Assuntos
Síndrome de Rett/genética , Síndrome de Rett/fisiopatologia , Convulsões/genética , Convulsões/fisiopatologia , Espasmos Infantis/genética , Espasmos Infantis/fisiopatologia , Adolescente , Adulto , Idade de Início , Anticonvulsivantes/uso terapêutico , Criança , Pré-Escolar , Síndromes Epilépticas , Feminino , Genótipo , Humanos , Lactente , Recém-Nascido , Estimativa de Kaplan-Meier , Masculino , Fenótipo , Proteínas Serina-Treonina Quinases/genética , Síndrome de Rett/tratamento farmacológico , Convulsões/tratamento farmacológico , Espasmos Infantis/tratamento farmacológico , Adulto Jovem
16.
Am J Med Genet A ; 170(11): 2860-2869, 2016 11.
Artigo em Inglês | MEDLINE | ID: mdl-27528505

RESUMO

Functional abilities in the CDKL5 disorder have been described as severely impaired, yet some individuals are able to run and use phrases for speech. Our study investigated gross motor, hand function, and expressive communication abilities in individuals with the CDKL5 disorder. Data for 108 females and 16 males registered with the International CDKL5 disorder database and with a pathogenic CDKL5 mutation were analyzed. Relationships between functional abilities, age, genotype, and gender were analyzed using regression models. Over half of the females could sit on the floor and nearly a quarter could walk 10 steps. Fewer males could complete these tasks although one boy was able to sit, walk, and run. Most females and few males were able to pick up a large object. Females mostly used gestures to communicate while males mostly used other forms of non-verbal communication. Compared to those with no functional CDKL5 protein, individuals with truncating variants after aa 781 were more likely to be able to stand (OR 5.7, 95%CI 1.2, 26.6) or walk independently (4.3, 95%CI 0.9, 20.5), and use more advanced communication methods such as words (OR 6.1, 95%CI 1.5-24.2). Although abilities were markedly impaired for the majority with the CDKL5 disorder, some females and a few males had better functional abilities. This variability may be related to underlying gene variants, with females with a late truncating variant having better levels of ability than those with no functional protein. © 2016 Wiley Periodicals, Inc.


Assuntos
Estudos de Associação Genética , Proteínas Serina-Treonina Quinases/genética , Síndrome de Rett/diagnóstico , Síndrome de Rett/genética , Espasmos Infantis/diagnóstico , Espasmos Infantis/genética , Adolescente , Adulto , Criança , Pré-Escolar , Transtornos da Comunicação/diagnóstico , Transtornos da Comunicação/genética , Síndromes Epilépticas , Feminino , Genótipo , Humanos , Lactente , Deficiência Intelectual/diagnóstico , Deficiência Intelectual/genética , Masculino , Destreza Motora , Músculo Esquelético/fisiopatologia , Fenótipo , Fatores Sexuais , Adulto Jovem
17.
Public Health Res Pract ; 26(3)2016 Jul 15.
Artigo em Inglês | MEDLINE | ID: mdl-27421348

RESUMO

The Western Australian Data Linkage System is one of a few comprehensive, population-based data linkage systems worldwide, creating links between information from different sources relating to the same individual, family, place or event, while maintaining privacy. The Raine Study is an established cohort study with more than 2000 currently active participants. Individual consent was obtained from participants for information in publicly held databases to be linked to their study data. A waiver of consent was granted where it was impracticable to obtain consent. Approvals to link the datasets were obtained from relevant ethics committees and data custodians. The Raine Study dataset was subsequently linked to academic testing data collected by the Western Australian Department of Education. Examination of diet and academic performance showed that children who were predominantly breastfed for at least 6 months scored higher academically at age 10 than children who were breastfed for less than 6 months. A further study found that better diet quality at ages 1, 2 and 3 years was associated with higher academic scores at ages 10 and 12 years. Examination of nutritional intake at 14 years of age found that a better dietary pattern was associated with higher academic performance. The detailed longitudinal data collected in the Raine Study allowed for adjustment for multiple covariates and confounders. Data linkage reduces the burden on cohort participants by providing additional information without the need to contact participants. It can give information on participants who have been lost to follow-up; provide or complement missing data; give the opportunity for validation studies comparing recall of participants with administrative records; increase the population sample of studies by adding control participants from the general population; and allow for the adjustment of multiple covariates and confounders. The Raine Study dataset is extensive and detailed, and can be further improved by linking to other external data sources. By linking educational outcomes to the Raine Study database, it was shown across three different age groups that a healthy diet was consistently associated with higher academic performance.


Assuntos
Logro , Fenômenos Fisiológicos da Nutrição Infantil , Bases de Dados Factuais , Dieta Saudável , Armazenamento e Recuperação da Informação , Adolescente , Criança , Pré-Escolar , Avaliação Educacional , Feminino , Humanos , Lactente , Recém-Nascido , Estudos Longitudinais , Masculino , Austrália Ocidental
18.
Respirology ; 21(8): 1419-1424, 2016 11.
Artigo em Inglês | MEDLINE | ID: mdl-27312516

RESUMO

BACKGROUND AND OBJECTIVE: Computed tomography (CT)-based studies of asbestos-exposed individuals report a high prevalence of lung cancer, but the utility of low dose CT (LDCT) to screen asbestos-exposed populations is not established. We aimed to describe the prevalence of indeterminate pulmonary nodules and incidental findings on chest LDCT of asbestos-exposed subjects in Western Australia. METHODS: A total of 906 subjects from the Western Australian Asbestos Review Programme underwent LDCT of the chest as part of regular annual review. An indeterminate (solid) nodule was defined as >50 mm3 and part-solid/non-solid nodules >5 mm. The presence of asbestos-related diseases was recorded with a standardized report. RESULTS: Subjects were mostly (81%) men with a median age of 70 years. Fifty-eight (6.5%) participants were current smokers, 511 (56.4%) ex-smokers and 325 (36.4%) never-smokers. One hundred and four indeterminate nodules were detected in 77 subjects (8.5%); of these, eight cases had confirmed lung cancer (0.88%). Eighty-seven subjects (9.6%) had incidental findings that required further investigation, 42 (4.6%) from lower airways inflammation. The majority of nodules were solid, 4-6 mm and more common with age. Five hundred and eighty (64%) subjects had pleural plaques, and 364 (40.2%) had evidence of interstitial lung disease. CONCLUSION: The prevalence of LDCT-detected indeterminate lung nodules in 906 individuals with significant asbestos exposure was 8.5%, lower than many other CT studies. Clinically important incidental findings were found in 9.4%, predominantly related to lower respiratory tract inflammation. LDCT appears to effectively describe asbestos-related diseases and is likely to be an acceptable modality to monitor asbestos-exposed individuals.


Assuntos
Amianto , Achados Incidentais , Exposição por Inalação , Doenças Pulmonares Intersticiais/epidemiologia , Neoplasias Pulmonares/epidemiologia , Doenças Pleurais/epidemiologia , Idoso , Amianto/efeitos adversos , Amianto/análise , Feminino , Humanos , Exposição por Inalação/efeitos adversos , Exposição por Inalação/análise , Exposição por Inalação/prevenção & controle , Masculino , Pessoa de Meia-Idade , Prevalência , Serviços Preventivos de Saúde/métodos , Tomografia Computadorizada por Raios X/métodos , Austrália Ocidental/epidemiologia
19.
BMC Public Health ; 16: 461, 2016 06 01.
Artigo em Inglês | MEDLINE | ID: mdl-27246328

RESUMO

BACKGROUND: Maternal loss can have a deep-rooted impact on families. Whilst a disproportionate number of Aboriginal women die from potentially preventable causes, no research has investigated mortality in Aboriginal mothers. We aimed to examine the elevated mortality risk in Aboriginal mothers with a focus on external causes. METHODS: We linked data from four state administrative datasets to identify all women who had a child from 1983 to 2010 in Western Australia and ascertained their Aboriginality, socio-demographic details, and their dates and causes of death prior to 2011. Comparing Aboriginal mothers with other mothers, we estimated the hazard ratios (HRs) for death by any external cause and each of the sub-categories of accident, suicide, and homicide, and the corresponding age of their youngest child. RESULTS: Compared to non-Aboriginal mothers and after adjustment for parity, socio-economic status and remoteness, Aboriginal mothers were more likely to die from accidents [HR = 6.43 (95 % CI: 4.9, 8.4)], suicide [HR = 3.46 (95 % CI: 2.2, 5.4)], homicide [HR = 17.46 (95 % CI: 10.4, 29.2)] or any external cause [HR = 6.61 (95 % CI: 5.4, 8.1)]. For mothers experiencing death, the median age of their youngest child was 4.8 years. CONCLUSION: During the study period, Aboriginal mothers were much more likely to die than other mothers and they usually left more and younger children. These increased rates were only partly explained by socio-demographic circumstances. Further research is required to examine the risk factors associated with these potentially preventable deaths and to enable the development of informed health promotion to increase the life chances of Aboriginal mothers and their children.


Assuntos
Acidentes/estatística & dados numéricos , Causas de Morte , Homicídio/estatística & dados numéricos , Morte Materna/estatística & dados numéricos , Havaiano Nativo ou Outro Ilhéu do Pacífico/estatística & dados numéricos , Suicídio/estatística & dados numéricos , Adolescente , Adulto , Pré-Escolar , Estudos de Coortes , Feminino , Humanos , Paridade , Gravidez , Modelos de Riscos Proporcionais , Estudos Retrospectivos , Fatores de Risco , Fatores Socioeconômicos , Austrália Ocidental/epidemiologia , Adulto Jovem
20.
Medicine (Baltimore) ; 95(21): e3737, 2016 May.
Artigo em Inglês | MEDLINE | ID: mdl-27227936

RESUMO

To investigate the prevalence of intellectual disability (ID) and/or autism spectrum disorders (ASDs) in Western Australia (WA).A cohort of children born from 1983 to 2010 in WA with an ID and/or ASD were identified using the population-based IDEA (Intellectual Disability Exploring Answers) database, which ascertains cases through the Disability Services Commission (DSC) as well as education sources. Information on race, gender, mother's residence at birth and deaths was obtained through linkage to the Midwives Notification System and the Mortality Register. Diagnostic information on the cause of ID was obtained through review of medical records where available and children were classified as biomedical cause, ASD, or unknown cause.An overall prevalence of ID of 17.0/1000 livebirths (95% CI: 16.7, 17.4) showed an increase from the 10-year previous prevalence of 14.3/1000. The prevalence for mild or moderate ID was 15.0 (95% CI: 14.6, 15.3), severe ID was 1.2 (95% CI: 1.1, 1.3), and unknown level of ID was 0.9 (95% CI: 0.8, 1.0)/1000 livebirths. The prevalence for Aboriginal children was 39.0/1000 compared with 15.7/1000 for non-Aboriginal children, giving a prevalence ratio of 2.5 (95% CI: 2.4, 2.6). Prevalence of all ASD was 5.1/1000 of which 3.8/1000 had ASD and ID.The prevalence of ID has risen in WA over the last 10 years with most of this increase due to mild or moderate ID. Whilst the prevalence of ASD has also increased over this time this does not fully explain the observed increase. Aboriginal children are at a 2.5-fold risk of ID but are less likely to be accessing disability services.


Assuntos
Transtorno do Espectro Autista/epidemiologia , Deficiência Intelectual/epidemiologia , Adolescente , Adulto , Idade de Início , Transtorno do Espectro Autista/etnologia , Criança , Pré-Escolar , Feminino , Humanos , Deficiência Intelectual/etnologia , Masculino , Havaiano Nativo ou Outro Ilhéu do Pacífico/estatística & dados numéricos , Prevalência , Características de Residência , Índice de Gravidade de Doença , Distribuição por Sexo , Fatores Socioeconômicos , Austrália Ocidental/epidemiologia , Adulto Jovem
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