Your browser doesn't support javascript.
loading
Mostrar: 20 | 50 | 100
Resultados 1 - 20 de 1.901
Filtrar
1.
Ital J Pediatr ; 47(1): 2, 2021 Jan 06.
Artigo em Inglês | MEDLINE | ID: mdl-33407736

RESUMO

BACKGROUND: Cystic fibrosis (CF) is a life-threatening disease affecting about 1:3000 newborns in Caucasian populations. The introduction of newborn screening for cystic fibrosis (CF NBS) has improved the clinical outcomes of individuals with CF through early diagnosis and early treatment. NBS strategies have been implemented over time. CF NBS was introduced extensively in 1984 in Tuscany, a region with 3.7 million people, characterized by a high allelic heterogeneity of CFTR gene. AIM AND METHODS: The aim of the study is to present the results from 34 years (1984-2018) of CF NBS, retrospectively evaluating the sensitivity, specificity and predictive values of the tests. In particular, we studied the impact of the introduction of DNA molecular analysis in NBS in a region with high allelic heterogeneity, such as Tuscany. RESULTS: Over these 34 years, 919,520 neonates were screened, using four different NBS strategies. From 1984 to 1991, CF NBS was performed by the determination of albumin on dried meconium (sensitivity 68.75%; specificity 99.82%). Subsequently, the analysis of immunoreactive trypsinogen on a blood spot was adopted as CF NBS protocol (sensitivity 83.33%; specificity 99.77%). From 1992 to 2010, this strategy was associated with lactase meconium dosage: IRT1/IRT2 + LACT protocol (sensitivity 87.50%; specificity 99.82%). From 2011, when the existing algorithm was integrated by analysis of CF causing variants of the CFTR gene (IRT1/IRT2 + LACT + IRT1/DNA protocol), a substantial improvement in sensitivity was seen (senisitivity 96.15%; specificity 99.75%). Other improved parameters with DNA analysis in the NBS programme, compared with the previous method, were the diagnosis time (52 days vs. 38 days) and the recall rate (0.58 to 0.38%). CONCLUSION: The inclusion of DNA analysis in the NBS was a fundamental step in improving sensitivity, even in a region with high allelic variability.


Assuntos
Fibrose Cística/diagnóstico , Fibrose Cística/epidemiologia , Triagem Neonatal , Feminino , Testes Genéticos , Humanos , Recém-Nascido , Itália , Masculino , Estudos Retrospectivos , Sensibilidade e Especificidade
3.
J Cyst Fibros ; 20(1): 25-30, 2021 01.
Artigo em Inglês | MEDLINE | ID: mdl-33309057

RESUMO

BACKGROUND: The presence of co-morbidities, including underlying respiratory problems, has been identified as a risk factor for severe COVID-19 disease. Information on the clinical course of SARS-CoV-2 infection in children with cystic fibrosis (CF) is limited, yet vital to provide accurate advice for children with CF, their families, caregivers and clinical teams. METHODS: Cases of SARS-CoV-2 infection in children with CF aged less than 18 years were collated by the CF Registry Global Harmonization Group across 13 countries between 1 February and 7 August 2020. RESULTS: Data on 105 children were collated and analysed. Median age of cases was ten years (interquartile range 6-15), 54% were male and median percentage predicted forced expiratory volume in one second was 94% (interquartile range 79-104). The majority (71%) of children were managed in the community during their COVID-19 illness. Out of 24 children admitted to hospital, six required supplementary oxygen and two non-invasive ventilation. Around half were prescribed antibiotics, five children received antiviral treatments, four azithromycin and one additional corticosteroids. Children that were hospitalised had lower lung function and reduced body mass index Z-scores. One child died six weeks after testing positive for SARS-CoV-2 following a deterioration that was not attributed to COVID-19 disease. CONCLUSIONS: SARS-CoV-2 infection in children with CF is usually associated with a mild illness in those who do not have pre-existing severe lung disease.


Assuntos
/complicações , Fibrose Cística/complicações , Fibrose Cística/terapia , Adolescente , Criança , Fibrose Cística/epidemiologia , Progressão da Doença , Feminino , Humanos , Masculino , Prognóstico , Fatores de Risco
4.
Rev Esp Salud Publica ; 942020 Dec 16.
Artigo em Espanhol | MEDLINE | ID: mdl-33323918

RESUMO

Galician newborn screening program for early detection of endocrine and metabolic diseases began in 1978 and was a pioneer in expanded newborn screening in Spain with the incorporation of mass spectrometry in July 2000. As a primary objective, 28 diseases are screened, including those recommended SNS except sickle cell anemia which is in the inclusion phase. In its 20-year history, 404,616 newborns (nb) have been analyzed, identifying 547 cases affected by the diseases included, with a global incidence of 1: 739 newborns and 1: 1.237 of the screened inborn errors of metabolism (IEM) (1:1.580 nb if excluding benign hyperphenylalaninemia-HPA), with an average participation of 99.35%, progressively higher during the analyzed period. Among the pathologies screened, congenital hypothyroidism (1:2.211 nb), cystinuria (1:4.129 nb) and HPA (1:5.699 nb), followed by phenylketonuria and cystic fibrosis (1:10,936 nb) stand out for their incidence. Sixty-six cases of false positives were identified (seventeen of them in relation to maternal pathology) and five false negatives, being the overall PPV and NPV of the program respectively of 89.2% and 99.99%, with a sensitivity of 99.09% and a specificity of 99.98%. The mortality rate of diagnosed CME patients is 1.52%, with eleven cases presenting symptoms prior to the screening result (2%). The intelligence quotient of IEM patients at risk of neurological involvement is normal in more than 95% of cases.


Assuntos
Hipotireoidismo Congênito/diagnóstico , Fibrose Cística/diagnóstico , Erros Inatos do Metabolismo/diagnóstico , Triagem Neonatal , Hipotireoidismo Congênito/epidemiologia , Fibrose Cística/epidemiologia , Reações Falso-Positivas , Feminino , Humanos , Incidência , Recém-Nascido , Masculino , Erros Inatos do Metabolismo/epidemiologia , Triagem Neonatal/métodos , Triagem Neonatal/normas , Triagem Neonatal/tendências , Avaliação de Programas e Projetos de Saúde , Sensibilidade e Especificidade , Espanha/epidemiologia
5.
Rev Esp Salud Publica ; 942020 Dec 16.
Artigo em Espanhol | MEDLINE | ID: mdl-33323920

RESUMO

OBJECTIVE: The main justification of this study was to describe our experience in neonatal screening and to define the prevalence of the diseases included in the neonatal screening program in Andalusia, among which are congenital hypothyroidism, expanded screening (aminoacidopathies, mitochondrial beta-oxidation defects and organic acidurias), cystic fibrosis, and screening for sickle cell anemia. METHODS: The study was carried out in the Metabolopathies Unit of the Virgen del Rocío Hospital in Seville with samples of newborns from Western Andalusia (Cádiz, Córdoba, Huelva and Seville) and autonomous city of Ceuta. A total of 435,141 newborns were studied (from the period from April 1st 2009 to December 31st 2019) to rule out congenital hypothyroidism and expanded screening; 378,306 for cystic fibrosis from May 1st 2011 to the same date described above. Finally, sickle cell anemia screening was included, which comprised a total of 55,576 newborns from November 26th, 2018 to the same period as the previous ones. Statistical analysis was performed using IBM SPSS software (version 22, SPSS INC., USA). RESULTS: The study revealed a prevalence of 1:1565 newborns for congenital hypothyroidism, 1:1532 newborns for extended screening, 1:6.878 newborns for cystic fibrosis, and a 1:11.115 newborns for sickle cell disease. CONCLUSIONS: The neonatal screening program allows a large number of newborns to benefit from the early detection of certain serious congenital diseases. This aim improves the morbidity and mortality of those who suffer from them.


Assuntos
Anemia Falciforme/diagnóstico , Hipotireoidismo Congênito/diagnóstico , Fibrose Cística/diagnóstico , Erros Inatos do Metabolismo/diagnóstico , Triagem Neonatal , Anemia Falciforme/epidemiologia , Hipotireoidismo Congênito/epidemiologia , Fibrose Cística/epidemiologia , Diagnóstico Precoce , Humanos , Recém-Nascido , Estudos Longitudinais , Erros Inatos do Metabolismo/epidemiologia , Prevalência , Estudos Retrospectivos , Espanha/epidemiologia
6.
Ital J Pediatr ; 46(1): 143, 2020 Oct 06.
Artigo em Inglês | MEDLINE | ID: mdl-33023602

RESUMO

The Veneto region is one of the most affected Italian regions by COVID-19. Chronic lung diseases, such as chronic obstructive pulmonary disease (COPD), may constitute a risk factor in COVID-19. Moreover, respiratory viruses were generally associated with severe pulmonary impairment in cystic fibrosis (CF). We would have therefore expected numerous cases of severe COVID-19 among the CF population. Surprisingly, we found that CF patients were significantly protected against infection by SARS-CoV-2. We discussed this aspect formulating some reasonable theories.


Assuntos
Infecções por Coronavirus/epidemiologia , Fibrose Cística/epidemiologia , Pandemias/estatística & dados numéricos , Pneumonia Viral/epidemiologia , Doença Pulmonar Obstrutiva Crônica/epidemiologia , Adulto , Técnicas de Laboratório Clínico/métodos , Estudos de Coortes , Infecções por Coronavirus/diagnóstico , Infecções por Coronavirus/prevenção & controle , Fibrose Cística/diagnóstico , Feminino , Humanos , Incidência , Itália/epidemiologia , Masculino , Pessoa de Meia-Idade , Pandemias/prevenção & controle , Pneumonia Viral/prevenção & controle , Doença Pulmonar Obstrutiva Crônica/diagnóstico , Reação em Cadeia da Polimerase em Tempo Real/métodos , Valores de Referência , Estudos Retrospectivos , Medição de Risco
7.
Acta Biomed ; 91(3): e2020035, 2020 09 07.
Artigo em Inglês | MEDLINE | ID: mdl-32921729

RESUMO

The novel coronavirus SARS-CoV-2 was first identified in China in December 2019 and has since spread worldwide. People with Cystic Fibrosis (CF) have reduced survival mainly because of respiratory failure due to chronic pulmonary infections. Therefore, CF patients should be considered to have an increased risk of developing severe manifestations in case of SARS-CoV-2 infection. Surprisingly, the results of recent studies concerning SARS-CoV-2 infection in patients with CF show that in these patients the infection rate was lower than that of the general population. Various factors have been considered to explain a possible protective effect of CF against SARS-CoV-2 infection.


Assuntos
Betacoronavirus , Infecções por Coronavirus/epidemiologia , Fibrose Cística/epidemiologia , Pulmão/diagnóstico por imagem , Pandemias , Pneumonia Viral/epidemiologia , Tomografia Computadorizada por Raios X/métodos , Comorbidade , Fibrose Cística/diagnóstico , Humanos
9.
Respir Med ; 170: 106062, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-32843180

RESUMO

BACKGROUND: Given the high incidence of confirmed infection by SARS-CoV-2 and mortality by COVID-19 in the Spanish population, its impact was analysed among persons with Cystic Fibrosis (CF) as a group at risk of a worse evolution. The possible causes of the incidence observed in them are explained and how CF Units have faced this health challenge is detailed. METHODS: Retrospective descriptive observational study, for which a Spanish CF Patients with Confirmed COVID-19 Registry is created, requesting information on number of people affected between 8 March-16 May 2020 and their clinical-demographic characteristics from the CF Units participating in the European Cystic Fibrosis Society Patient Registry (ECFSPR). The accumulated incidence is calculated, compared with that of the general population. Additionally, a survey (CF-COVID19-Spain) is carried out on prevention of SARS-CoV-2 infection, workings of CF Units and possible reasons for the incidence observed. RESULTS: COVID-19 was diagnosed in eight CF patients, one of whom had received a lung transplant. The accumulated incidence was 32/10000 in CF patients and 49/10000 in the general population. General death rate was 5.85/10000 while no CF patients included in the ECFSPR died. The characteristics of those affected and the results of the survey are described. CONCLUSIONS: Despite being considered a disease at high risk of severe COVID-19, the low incidence and mortality in CF patients in Spain contrasts with the figures for the general population. The possible factors that would explain such findings are discussed, with the help of the results of the CF-COVID19-Spain survey.


Assuntos
Betacoronavirus/isolamento & purificação , Infecções por Coronavirus , Fibrose Cística/epidemiologia , Pandemias , Pneumonia Viral , Adulto , Técnicas de Laboratório Clínico/métodos , Técnicas de Laboratório Clínico/estatística & dados numéricos , Infecções por Coronavirus/diagnóstico , Infecções por Coronavirus/epidemiologia , Infecções por Coronavirus/prevenção & controle , Infecções por Coronavirus/terapia , Feminino , Humanos , Incidência , Masculino , Mortalidade , Pandemias/prevenção & controle , Pneumonia Viral/epidemiologia , Pneumonia Viral/prevenção & controle , Pneumonia Viral/terapia , Sistema de Registros/estatística & dados numéricos , Estudos Retrospectivos , Medição de Risco , Espanha/epidemiologia
11.
Ann Biol Clin (Paris) ; 78(3): 314-318, 2020 Jun 01.
Artigo em Inglês | MEDLINE | ID: mdl-32540817

RESUMO

BACKGROUND: Cystic fibrosis (CF) is the most common autosomal recessive disease in Caucasians, caused by mutation in cystic fibrosis transmembrane conductance regulator (CFTR). The analysis of some extra and intragenic markers within or closely linked to CFTR gene is useful as a molecular method in clinical linkage analysis. Indeed, knowing that the molecular basis of CF is highly heterogeneous in our population is explained in the present study. In this work, we are interested for the first time to study the polymorphic marker IVS6a GATT in a CF Tunisian population. METHODS: Our study involved 80 CF Tunisian patients with a positive sweat test. A cohort of 90 healthy controls was also enrolled. The analysis of the variant IVS6a GATT was conducted by analysis of the fragments on automatic sequencer (ABI Prism 310). A statistical analysis was performed on Statistical Package for the Social Sciences (SPSS) version 20 software. RESULTS: The analysis of genotypic distribution of IVS6aGATT showed a significant difference between the control and CF groups suggesting the involvement of this marker in cystic fibrosis. Furthermore, we noted that the 6 GATT repetition in the homozygous state is more common in CF patients than in the control group (p <0.05). This while the 7GATT/7GATT genotype is more common among controls compared to CF patients (p = 0.002). Regarding the interest of this polymorphism on the clinical expression of cystic fibrosis, we have noted no significant association between 6/6 genotype with different clinical conditions in CF patients outside the CFTR mutation. While a significant association was found between respiratory involvement and mixed (respiratory and digestive) and the 6/6 genotype in patients with the mutation F508del homozygous (p <0.05). In addition, a significant association was also noted with gastrointestinal involvement for non F508del patients/F508del not (p = 0.014). Given that, phenotypic and genotypic heterogeneity of cystic fibrosis, several studies have sought to highlight the role of genetic markers linked to the CFTR gene in the expression and evolution of the disease. CONCLUSION: Our study on the implication of polymorphic marker IVS6a GATT is one of the first works carried out in the Tunisian population and confirms the usefulness of this marker in the clinical expression of cystic fibrosis.


Assuntos
Regulador de Condutância Transmembrana em Fibrose Cística/genética , Fibrose Cística/genética , Repetições de Microssatélites/genética , Polimorfismo Genético , Adolescente , Estudos de Casos e Controles , Criança , Pré-Escolar , Fibrose Cística/epidemiologia , Feminino , Frequência do Gene , Estudos de Associação Genética , Predisposição Genética para Doença , Genética Populacional , Genótipo , Humanos , Lactente , Masculino , Tunísia/epidemiologia
12.
BMC Med Genet ; 21(1): 89, 2020 05 01.
Artigo em Inglês | MEDLINE | ID: mdl-32357917

RESUMO

BACKGROUND: The aim was to establish the true risk of having an affected child with Cystic Fibrosis (CF) in the Sicilian infertile population. METHODS: A longitudinal CFTR screening of 1279 Sicilian infertile patients for all CFTR mutations sequencing the entire gene by Next Generation Sequencing (NGS) was performed from patient's blood. RESULTS: One patient out of 16 was a carrier of a CFTR mutation. Twenty-four mutations were found. Theoretically one couple out of 256 was at risk of CF transmission. CONCLUSIONS: The risk of CF transmission is unexpectedly high in Sicily and with a high heterogeneity. Sequencing an entire and long gene such as CFTR makes accessible the true panel of mutations in a specific population and helps better to understand the true risk of having an affected child.


Assuntos
Regulador de Condutância Transmembrana em Fibrose Cística/genética , Fibrose Cística/genética , Genética Populacional , Sequenciamento de Nucleotídeos em Larga Escala , Alelos , Fibrose Cística/epidemiologia , Fibrose Cística/patologia , Feminino , Genótipo , Humanos , Masculino , Mutação/genética , Análise de Sequência de DNA , Sicília/epidemiologia
13.
Pediatr Pulmonol ; 55(8): 1974-1983, 2020 08.
Artigo em Inglês | MEDLINE | ID: mdl-32364318

RESUMO

BACKGROUND: Studies have shown that sleep disorders occur in cystic fibrosis (CF) patients and may be present before daytime clinical manifestations. OBJECTIVES: To evaluate the presence of sleep disorders among children and adolescents with CF, attempting to identify associations with pulmonary function, nutritional status, days in hospital, and days taking antibiotics. METHODS: Individuals with a diagnosis of CF aged between 6 and 18 years were included. Information on sociodemographic, clinical profile, history of hospitalizations, and use of antibiotics in the last year were collected. Spirometry, bioimpedance, and polysomnography were performed. The presence of nocturnal hypoxemia and obstructive sleep apnea syndrome (OSAS) were evaluated and participants divided according to their presence. RESULTS: Thirty-one patients were included. The prevalence of OSAS was 32.3% and nocturnal hypoxemia was 29.0%. Average nocturnal peripheral oxyhemoglobin saturation (SpO2 ) correlated (P < .001) with forced vital capacity (r = .55) and forced expiratory volume in the first second (r = .62). The higher the percentage of total sleep time (TST) with SpO2 less than 90%, the lower the pulmonary function. Individuals with OSAS and nocturnal hypoxemia had lower spirometric values compared to patients without these disorders, but the nocturnal hypoxemia group also had lower Shwachman-Kulczycki score, longer hospitalization time and antibiotic use. TST with SpO2 less than 90% was associated with length of hospitalization (r2 = .53). CONCLUSION: Children and adolescents with CF have sleep disorders, including OSAS (32.3%) and nocturnal hypoxemia (29%). Individuals with nocturnal hypoxemia presented lower lung function, worse clinical score, and higher morbidity. TST with SpO2 less than 90% was associated with length of hospitalization.


Assuntos
Fibrose Cística/fisiopatologia , Hipóxia/fisiopatologia , Síndromes da Apneia do Sono/fisiopatologia , Adolescente , Biomarcadores , Criança , Fibrose Cística/epidemiologia , Feminino , Volume Expiratório Forçado , Humanos , Hipóxia/epidemiologia , Masculino , Morbidade , Estado Nutricional , Polissonografia , Prevalência , Síndromes da Apneia do Sono/epidemiologia , Transtornos do Sono-Vigília , Espirometria , Capacidade Vital
14.
Sociol Health Illn ; 42(5): 972-986, 2020 06.
Artigo em Inglês | MEDLINE | ID: mdl-32406081

RESUMO

With significant relevance to the Covid-19 pandemic, this paper contributes to emerging 'aerographic' research on the socio-materialities of air and breath, based on an in-depth empirical study of three hospital-based lung infection clinics treating people with cystic fibrosis. We begin by outlining the changing place of atmosphere in hospital design from the pre-antibiotic period and into the present. We then turn to the first of three aerographic themes where air becomes a matter of grasping and visualising otherwise invisible airborne infections. This includes imagining patients located within bodily spheres or 'cloud bodies', conceptually anchored in Irigaray's thoughts on the 'forgetting of the air' and Sloterdijk's immunitary 'spherology' of the body. Our second theme explores the material politics of air, air conditioning, window design and the way competing 'air regimes' come into conflict with each other at the interface of buildings, bodies and the biotic. Our final theme attends to the 'cost of air', the aero-economic problem of atmospheric scarcity within modern high-rise, deep-density healthcare architectures.


Assuntos
Ar , Instituições de Assistência Ambulatorial/organização & administração , Fibrose Cística/epidemiologia , Respiração , Infecções Respiratórias/epidemiologia , Microbiologia do Ar , Instituições de Assistência Ambulatorial/normas , Antibacterianos/uso terapêutico , Arquitetura de Instituições de Saúde , Comportamentos Relacionados com a Saúde , Humanos , Infecções Respiratórias/tratamento farmacológico
15.
PLoS One ; 15(4): e0231285, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-32302349

RESUMO

Cystic fibrosis (CF) is a rare genetic disease that affects the respiratory and digestive systems. Lung disease is variable among CF patients and associated with the development of comorbidities and chronic infections. The rate of lung function deterioration depends not only on the type of mutations in CFTR, the disease-causing gene, but also on modifier genes. In the present study, we aimed to identify genes and pathways that (i) contribute to the pathogenesis of cystic fibrosis and (ii) modulate the associated comorbidities. We profiled blood samples in CF patients and healthy controls and analyzed RNA-seq data with Weighted Gene Correlation Network Analysis (WGCNA). Interestingly, lung function, body mass index, the presence of diabetes, and chronic P. aeruginosa infections correlated with four modules of co-expressed genes. Detailed inspection of networks and hub genes pointed to cell adhesion, leukocyte trafficking and production of reactive oxygen species as central mechanisms in lung function decline and cystic fibrosis-related diabetes. Of note, we showed that blood is an informative surrogate tissue to study the contribution of inflammation to lung disease and diabetes in CF patients. Finally, we provided evidence that WGCNA is useful to analyze-omic datasets in rare genetic diseases as patient cohorts are inevitably small.


Assuntos
Fibrose Cística/epidemiologia , Fibrose Cística/genética , Diabetes Mellitus/genética , Genes Modificadores , Adulto , Comorbidade , Fibrose Cística/sangue , Regulador de Condutância Transmembrana em Fibrose Cística/genética , Diabetes Mellitus/sangue , Feminino , Humanos , Pulmão/metabolismo , Masculino , Mutação , Infecções por Pseudomonas/patologia , Transcriptoma
17.
JAMA Netw Open ; 3(3): e201737, 2020 03 02.
Artigo em Inglês | MEDLINE | ID: mdl-32219405

RESUMO

Importance: Sickle cell disease (SCD) and cystic fibrosis (CF) are severe autosomal recessive disorders associated with intermittent disease exacerbations that require hospitalizations, progressive chronic organ injury, and substantial premature mortality. Research funding is a limited resource and may contribute to health care disparities, especially for rare diseases that disproportionally affect economically disadvantaged groups. Objective: To compare disease-specific funding between SCD and CF and the association between funding and research productivity. Design, Setting, and Participants: This cross-sectional study examined federal and foundation funding, publications indexed in PubMed, clinical trials registered in ClinicalTrials.gov, and new drug approvals from January 1, 2008, to December 31, 2018, in an estimated US population of approximately 90 000 individuals with SCD and approximately 30 000 individuals with CF. Main Outcomes and Measures: Federal and foundation funding, publications indexed in PubMed, clinical trial registrations, and new drug approvals. Results: From 2008 through 2018, federal funding was greater per person with CF compared with SCD (mean [SD], $2807 [$175] vs $812 [$147]; P < .001). Foundation expenditures were greater for CF than for SCD (mean [SD], $7690 [$3974] vs $102 [$13.7]; P < .001). Significantly more research articles (mean [SD], 1594 [225] vs 926 [157]; P < .001) and US Food and Drug Administration drug approvals (4 vs 1) were found for CF compared with SCD, but the total number of clinical trials was similar (mean [SD], 27.3 [6.9] vs 23.8 [6.3]; P = .22). Conclusions and Relevance: The findings show that disparities in funding between SCD and CF may be associated with decreased research productivity and novel drug development for SCD. Increased federal and foundation funding is needed for SCD and other diseases that disproportionately affect economically disadvantaged groups to address health care disparities.


Assuntos
Anemia Falciforme/economia , Pesquisa Biomédica , Fibrose Cística/economia , Apoio à Pesquisa como Assunto , Anemia Falciforme/epidemiologia , Pesquisa Biomédica/economia , Pesquisa Biomédica/estatística & dados numéricos , Estudos Transversais , Fibrose Cística/epidemiologia , Desenvolvimento de Medicamentos/economia , Desenvolvimento de Medicamentos/estatística & dados numéricos , Fundações , Humanos , Apoio à Pesquisa como Assunto/economia , Apoio à Pesquisa como Assunto/organização & administração , Estados Unidos
18.
Respir Med ; 161: 105854, 2020 01.
Artigo em Inglês | MEDLINE | ID: mdl-32056728

RESUMO

BACKGROUND: Viral respiratory infections (VRI) in people living with Cystic fibrosis (CF) is less well understood than respiratory bacterial infections, particularly adults with CF and few studies have compared children with adults. This study evaluated the frequency of respiratory viruses in patients with cystic fibrosis (CF) in Western Australia (WA). We determined the VRI in CF and compared them with non-CF patients. Further, we compared CF patients that were hospitalised with those that were not. PATIENTS/METHODS: Nucleic acid from sputum of 157 CF and 348 non-CF patients was analysed for influenzavirus A (Flu A) and B, (Flu B), respiratory syncytial virus (RSV), human metapneumovirus (hMPV), human rhinovirus (RV), and parainfluenza viruses (PIV 1-3) by RT-PCR, during the 2016 winter respiratory season. RESULTS: No significant difference in the frequency of respiratory virus detection between CF and non-CF patients was found. RV was the most frequently detected virus in CF patients, and in hospitalised CF. RSV and hMPV were found less frequently in CF patients and RSV was not found in any hospitalised CF patient. A trend for fewer influenzavirus detections in adult CF patients was observed, however the trend was opposite for paediatric patients. RV and Flu A were the most common viruses detected in hospitalised CF patients. CONCLUSION: There was no significant difference in VRI between CF and non-CF patients. RV and influenza A were most commonly found in hospitalised CF patients, suggesting that infection with these viruses may contribute to hospitalisation for CF respiratory exacerbations.


Assuntos
Fibrose Cística/complicações , Infecções Respiratórias/etiologia , Viroses/etiologia , Adulto , Austrália/epidemiologia , Criança , Fibrose Cística/epidemiologia , Fibrose Cística/virologia , Feminino , Hospitalização , Humanos , Influenzavirus A/isolamento & purificação , Masculino , Estudos Prospectivos , Vírus Sinciciais Respiratórios/isolamento & purificação , Infecções Respiratórias/epidemiologia , Infecções Respiratórias/virologia , Estações do Ano , Viroses/epidemiologia , Viroses/virologia
19.
Rev Mal Respir ; 37(3): 235-242, 2020 Mar.
Artigo em Francês | MEDLINE | ID: mdl-31955962

RESUMO

INTRODUCTION: The aim of this study was to evaluate the impact of the transition period from childhood to adulthood in patients with cystic fibrosis (CF) being followed up in our reference center. METHODS: The clinical, functional, inflammatory and microbiological parameters of all transition patients were compared two years before (T-2) and two years after the transfer (T+2) from paediatric to adult centers and further analysed according to whether the transition conditions were optimal or suboptimal. RESULTS: Twenty-eight patients were included. The mean age at the transfer visit was 19.5 years (±3.5). There were no deaths during the study period. Consultations were more numerous at T-2 [14.5 (±5.9) vs. 12.0 (±5.1), P<0.004]. Chronic colonization with Pseudomonas aeruginosa was more frequent at T+2 (46.4% vs. 17.9%, P=0.021). A progressive decrease in FEV1 and FVC was observed between T-2 and T+2. The number of pulmonary exacerbations was lower in the optimal transition group. CONCLUSION: The period of transition from childhood to adulthood in patients with CF appears to be associated with functional and microbiological changes.


Assuntos
Envelhecimento/fisiologia , Fibrose Cística/epidemiologia , Fibrose Cística/terapia , Cuidado Transicional , Adolescente , Adulto , Comorbidade , Fibrose Cística/diagnóstico , Fibrose Cística/fisiopatologia , Feminino , Volume Expiratório Forçado , Hospitalização/estatística & dados numéricos , Humanos , Estudos Longitudinais , Pulmão/microbiologia , Masculino , Prognóstico , Infecções por Pseudomonas/complicações , Infecções por Pseudomonas/epidemiologia , Pseudomonas aeruginosa/isolamento & purificação , Infecções Respiratórias/complicações , Infecções Respiratórias/epidemiologia , Estudos Retrospectivos , Fatores de Tempo , Cuidado Transicional/normas , Cuidado Transicional/estatística & dados numéricos , Adulto Jovem
SELEÇÃO DE REFERÊNCIAS
DETALHE DA PESQUISA
...