Lactentes com fibrose cística do programa de triagem neonatal do Rio de Janeiro: avaliação clínica, microbiológica e radiológica
Autor(a) principal: | |
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Data de Publicação: | 2018 |
Outros Autores: | |
Tipo de documento: | Tese |
Idioma: | por |
Título da fonte: | Biblioteca Digital de Teses e Dissertações da UERJ |
Texto Completo: | http://www.bdtd.uerj.br/handle/1/18286 |
Resumo: | Cystic fibrosis (CF) is a progressive, genetic disease that affects 1:2500 caucasian live births. Diagnosis criteria are well defined and newborn screening programs (NBS) using immunoreactive trypsinogen (IRT) has been widely established worldwide and, more recently, in Brazil. Newborn infants with high risk of presenting CF are identified by NBS and, once diagnosis is confirmed, follow up and management must be initiated early. The benefits of NBS for CF have been studied and show evidences of improvement in nutritional status and lung function in infants, beside the contribution for long term survival. High resolution computed tomography (HRCT) is a sensitive method for detecting structural lung abnormalities. HRCT evaluation through reproducible scoring systems can be used to quantify chest computed tomography (CT) findings in cystic fibrosis (CF) patients. The aim of the study was to describe early HRCT findings according to a validated score system in cystic fibrosis infants diagnosed through newborn screening (NBS) followed at Instituto Nacional de Saúde da Mulher, da Criança e do Adolescente Fernandes Figueira – FIOCRUZ. This is a cross-sectional study including NBS infants with CF born between 2013-2017. Clinical, nutritional and microbiological data were evaluated at the moment of diagnosis. The subjects were submitted to HRCT scans within the first year after diagnosis, when clinically stable. The CT scans were evaluated through the modified Bhalla score. 35 patients were included in the study, with male predominance and mean age at diagnosis was 3.8 months. Regarding clinical presentation, 20% were asymptomatic, while respiratory signs were present in 45.7% and digestive in 65.7%, with anthropometric data revealing nutritional risk in 68.5% of patients. Microbiological data showed that 51.4% of patients had no CF pathogenic bacteria in respiratory secretion, while 17.1% presented Pseudomonas aeruginosa isolation and 31.4% showed other pathogenic bacteria. 32 subjects were submitted to HRCT scans. The mean total modified Bhalla score was 3.6 ± 2.1 and 93.8% scans were abnormal. Bronchial wall thickening was the most common parameter (90.6%), followed by collapse/consolidation (59.4%), mosaic attenuation/perfusion (50%), bronchiectasis (37.5%) and mucus plugging (15.6%). Bronchial wall thickening was diffuse in most patients. A substantial proportion of infants diagnosed with CF after detection by NBS already have evidence of lung disease. P. aeruginosa colonization was associated to higher Bhalla scores, highlighting the importance of this CF pathogen in early structural lung disease. The presence of bronchial wall thickening in most subjects in such early age patients may already reflect the presence of airway inflammatory process. Detection and quantification of structural abnormalities through modified Bhalla score may contribute to acknowledgment lung disease before clinically apparent. |
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Leão, Robson de Souzahttp://lattes.cnpq.br/5369029097197832Boechat, Marcia Cristina Bastoshttp://lattes.cnpq.br/4311911721179399Costa, Cláudia Henrique dahttp://lattes.cnpq.br/7858045844671281Marques, Elizabeth de Andradehttp://lattes.cnpq.br/5959485578597640Daltro, Pedro Augusto Nascimentohttp://lattes.cnpq.br/4593055652374668Fonseca, Vania de Matoshttp://lattes.cnpq.br/1601115011359365http://lattes.cnpq.br/7598766589760207Cohen, Renata Wrobel Folescurenatawfc@gmail.com2022-08-26T15:36:42Z2018-08-10COHEN, Renata Wrobel Folescu. Lactentes com fibrose cística do programa de triagem neonatal do Rio de Janeiro: avaliação clínica, microbiológica e radiológica. 2018. 90 f. Tese (Doutorado em Ciências Médica) – Faculdade de Ciências Médicas, Universidade do Estado do Rio de Janeiro, Rio de Janeiro, 2018.http://www.bdtd.uerj.br/handle/1/18286Cystic fibrosis (CF) is a progressive, genetic disease that affects 1:2500 caucasian live births. Diagnosis criteria are well defined and newborn screening programs (NBS) using immunoreactive trypsinogen (IRT) has been widely established worldwide and, more recently, in Brazil. Newborn infants with high risk of presenting CF are identified by NBS and, once diagnosis is confirmed, follow up and management must be initiated early. The benefits of NBS for CF have been studied and show evidences of improvement in nutritional status and lung function in infants, beside the contribution for long term survival. High resolution computed tomography (HRCT) is a sensitive method for detecting structural lung abnormalities. HRCT evaluation through reproducible scoring systems can be used to quantify chest computed tomography (CT) findings in cystic fibrosis (CF) patients. The aim of the study was to describe early HRCT findings according to a validated score system in cystic fibrosis infants diagnosed through newborn screening (NBS) followed at Instituto Nacional de Saúde da Mulher, da Criança e do Adolescente Fernandes Figueira – FIOCRUZ. This is a cross-sectional study including NBS infants with CF born between 2013-2017. Clinical, nutritional and microbiological data were evaluated at the moment of diagnosis. The subjects were submitted to HRCT scans within the first year after diagnosis, when clinically stable. The CT scans were evaluated through the modified Bhalla score. 35 patients were included in the study, with male predominance and mean age at diagnosis was 3.8 months. Regarding clinical presentation, 20% were asymptomatic, while respiratory signs were present in 45.7% and digestive in 65.7%, with anthropometric data revealing nutritional risk in 68.5% of patients. Microbiological data showed that 51.4% of patients had no CF pathogenic bacteria in respiratory secretion, while 17.1% presented Pseudomonas aeruginosa isolation and 31.4% showed other pathogenic bacteria. 32 subjects were submitted to HRCT scans. The mean total modified Bhalla score was 3.6 ± 2.1 and 93.8% scans were abnormal. Bronchial wall thickening was the most common parameter (90.6%), followed by collapse/consolidation (59.4%), mosaic attenuation/perfusion (50%), bronchiectasis (37.5%) and mucus plugging (15.6%). Bronchial wall thickening was diffuse in most patients. A substantial proportion of infants diagnosed with CF after detection by NBS already have evidence of lung disease. P. aeruginosa colonization was associated to higher Bhalla scores, highlighting the importance of this CF pathogen in early structural lung disease. The presence of bronchial wall thickening in most subjects in such early age patients may already reflect the presence of airway inflammatory process. Detection and quantification of structural abnormalities through modified Bhalla score may contribute to acknowledgment lung disease before clinically apparent.A fibrose Cística (FC) é uma doença autossômica recessiva multissistêmica que atinge 1:2500 nascidos vivos caucasianos. Os critérios diagnósticos para FC são bem definidos e o programa de triagem neonatal (PTN) através da dosagem da tripsina imunorreativa (TIR) vem sendo amplamente estabelecido mundialmente e, mais recentemente, no Brasil. Recém-nascidos com alto risco de apresentar FC são identificados através do PTN e, uma vez que o diagnóstico seja confirmado, acompanhamento e tratamento são iniciados precocemente. Os benefícios do PTN para FC tem sido amplamente estudados e existem evidências de melhora no estado nutricional e função pulmonar em lactentes, além de contribuir para sobrevida de pacientes a longo prazo. A tomografia computadorizada do tórax (TCAR) tem fornecido aos clínicos método mais sensível de detecção de lesões estruturais pulmonares. A análise da TCAR baseada em escore de pontuação tem sido útil para graduar objetivamente a gravidade e a extensão da doença pulmonar em FC. Este trabalho tem como objetivo avaliar lesão estrutural pulmonar por meio de TCAR em lactentes com FC diagnosticados a partir do programa de triagem neonatal vinculados ao Instituto Nacional de Saúde da Mulher, da Criança e do Adolescente Fernandes Figueira - FIOCRUZ. Trata-se de estudo transversal no qual, para cada paciente oriundo do PTN para FC do Estado do Rio de Janeiro de 2013 a 2017, foram avaliados dados clínicos, nutricionais e microbiológicos no momento do diagnóstico. Os exames de TCAR realizados, até 12 meses após diagnóstico, foram pontuados através escore de Bhalla modificado. 35 pacientes foram incluídos no estudo, havendo predomínio do gênero masculino, com média de idade no momento do diagnóstico de 3,8 meses. Quanto a apresentação clínica, 20% pacientes eram assintomáticos e os sinais respiratórios estavam presentes em 45,7% e digestivos em 65,7% da população, com 68,5% apresentando índices antropométricos indicativos de risco nutricional. Em relação ao aspecto microbiológico, 51,4% dos pacientes não evidenciaram patógenos relacionados a FC, 17,1% apresentaram isolamento de Pseudomonas aeruginosa e, 31,4%, outras bactérias relacionadas a FC. 32 dos 35 pacientes incluídos no estudo foram submetidos a TCAR. A média do escore de Bhalla modificado foi 3,6 (DP±2.1) e 93,8% das tomografias apresentavam alterações. O espessamento peribrônquico foi o parâmetro mais comum (90,6%), seguido de colapso/consolidação (59,4%), padrão de atenuação em mosaico (50%), bronquiectasias (37,5%) e tampões mucosos (15,6%). O espessamento peribrônquico foi difuso na maioria dos pacientes. A colonização das vias aéreas por P. aeruginosa esteve associada a maiores valores de escore de Bhalla modificado, destacando a importância deste patógeno na doença estrutural pulmonar precoce. A presença de espessamento peribrônquico na maioria dos pacientes em idade tão precoce pode já refletir a existência de processo inflamatório de vias aéreas. A detecção e quantificação de anormalidades estruturais através do escore de Bhalla modificado pode contribuir para o conhecimento da doença pulmonar antes de sintomas clínicos evidentes.Submitted by Heloísa CB/A (helobdtd@gmail.com) on 2022-08-26T15:36:42Z No. of bitstreams: 1 Renata Wrobel Folescu Cohen.pdf: 3167994 bytes, checksum: 2e3cd6514074f1bb9e2f736791b41371 (MD5)Made available in DSpace on 2022-08-26T15:36:42Z (GMT). No. of bitstreams: 1 Renata Wrobel Folescu Cohen.pdf: 3167994 bytes, checksum: 2e3cd6514074f1bb9e2f736791b41371 (MD5) Previous issue date: 2018-08-10application/pdfporUniversidade do Estado do Rio de JaneiroPrograma de Pós-Graduação em Ciências MédicasUERJBrasilCentro Biomédico::Faculdade de Ciências MédicasCystic fibrosisNewborn screeningComputed tomographyFibrose CísticaTriagem NeonatalTomografia ComputadorizadaCIENCIAS DA SAUDE::MEDICINA::CLINICA MEDICALactentes com fibrose cística do programa de triagem neonatal do Rio de Janeiro: avaliação clínica, microbiológica e radiológicaInfants with cystic fibrosis detected by newborn screening in Rio de Janeiro: clinical, microbiological and radiological evaluationinfo:eu-repo/semantics/publishedVersioninfo:eu-repo/semantics/doctoralThesisinfo:eu-repo/semantics/openAccessreponame:Biblioteca Digital de Teses e Dissertações da UERJinstname:Universidade do Estado do Rio de Janeiro (UERJ)instacron:UERJORIGINALTese - Renata Wrobel Folescu Cohen - 2018 - CompletaTese - Renata Wrobel Folescu Cohen - 2018 - Completaapplication/pdf3167994http://www.bdtd.uerj.br/bitstream/1/18286/2/Tese+-+Renata+Wrobel+Folescu+Cohen+-+2018+-+Completa2e3cd6514074f1bb9e2f736791b41371MD52LICENSElicense.txtlicense.txttext/plain; charset=utf-82123http://www.bdtd.uerj.br/bitstream/1/18286/1/license.txte5502652da718045d7fcd832b79fca29MD511/182862024-02-26 15:59:52.871oai:www.bdtd.uerj.br:1/18286Tk9UQTogTElDRU7Dh0EgUkVERSBTSVJJVVMKRXN0YSBsaWNlbsOnYSBkZSBleGVtcGxvIMOpIGZvcm5lY2lkYSBhcGVuYXMgcGFyYSBmaW5zIGluZm9ybWF0aXZvcy4KCkxJQ0VOw4dBIERFIERJU1RSSUJVScOHw4NPIE7Dg08tRVhDTFVTSVZBCgpDb20gYSBhcHJlc2VudGHDp8OjbyBkZXN0YSBsaWNlbsOnYSwgdm9jw6ogKG8gYXV0b3IgKGVzKSBvdSBvIHRpdHVsYXIgZG9zIGRpcmVpdG9zIGRlIGF1dG9yKSBjb25jZWRlIMOgIFVuaXZlcnNpZGFkZSAKZG8gRXN0YWRvIGRvIFJpbyBkZSBKYW5laXJvIChVRVJKKSBvIGRpcmVpdG8gbsOjby1leGNsdXNpdm8gZGUgcmVwcm9kdXppciwgIHRyYWR1emlyIChjb25mb3JtZSBkZWZpbmlkbyBhYmFpeG8pLCBlL291IApkaXN0cmlidWlyIGEgc3VhIHRlc2Ugb3UgZGlzc2VydGHDp8OjbyAoaW5jbHVpbmRvIG8gcmVzdW1vKSBwb3IgdG9kbyBvIG11bmRvIG5vIGZvcm1hdG8gaW1wcmVzc28gZSBlbGV0csO0bmljbyBlIAplbSBxdWFscXVlciBtZWlvLCBpbmNsdWluZG8gb3MgZm9ybWF0b3Mgw6F1ZGlvIG91IHbDrWRlby4KClZvY8OqIGNvbmNvcmRhIHF1ZSBhIFVFUkogcG9kZSwgc2VtIGFsdGVyYXIgbyBjb250ZcO6ZG8sIHRyYW5zcG9yIGEgc3VhIHRlc2Ugb3UgZGlzc2VydGHDp8OjbyAKcGFyYSBxdWFscXVlciBtZWlvIG91IGZvcm1hdG8gcGFyYSBmaW5zIGRlIHByZXNlcnZhw6fDo28uCgpWb2PDqiB0YW1iw6ltIGNvbmNvcmRhIHF1ZSBhIFVFUkogcG9kZSBtYW50ZXIgbWFpcyBkZSB1bWEgY8OzcGlhIGEgc3VhIHRlc2Ugb3UgCmRpc3NlcnRhw6fDo28gcGFyYSBmaW5zIGRlIHNlZ3VyYW7Dp2EsIGJhY2stdXAgZSBwcmVzZXJ2YcOnw6NvLgoKVm9jw6ogZGVjbGFyYSBxdWUgYSBzdWEgdGVzZSBvdSBkaXNzZXJ0YcOnw6NvIMOpIG9yaWdpbmFsIGUgcXVlIHZvY8OqIHRlbSBvIHBvZGVyIGRlIGNvbmNlZGVyIG9zIGRpcmVpdG9zIGNvbnRpZG9zIApuZXN0YSBsaWNlbsOnYS4gVm9jw6ogdGFtYsOpbSBkZWNsYXJhIHF1ZSBvIGRlcMOzc2l0byBkYSBzdWEgdGVzZSBvdSBkaXNzZXJ0YcOnw6NvIG7Do28sIHF1ZSBzZWphIGRlIHNldSAKY29uaGVjaW1lbnRvLCBpbmZyaW5nZSBkaXJlaXRvcyBhdXRvcmFpcyBkZSBuaW5ndcOpbS4KCkNhc28gYSBzdWEgdGVzZSBvdSBkaXNzZXJ0YcOnw6NvIGNvbnRlbmhhIG1hdGVyaWFsIHF1ZSB2b2PDqiBuw6NvIHBvc3N1aSBhIHRpdHVsYXJpZGFkZSBkb3MgZGlyZWl0b3MgYXV0b3JhaXMsIHZvY8OqIApkZWNsYXJhIHF1ZSBvYnRldmUgYSBwZXJtaXNzw6NvIGlycmVzdHJpdGEgZG8gZGV0ZW50b3IgZG9zIGRpcmVpdG9zIGF1dG9yYWlzIHBhcmEgY29uY2VkZXIgw6AgVUVSSiBvcyBkaXJlaXRvcyBhcHJlc2VudGFkb3MgbmVzdGEgbGljZW7Dp2EsIGUgcXVlIGVzc2UgbWF0ZXJpYWwgZGUgcHJvcHJpZWRhZGUgZGUgdGVyY2Vpcm9zIGVzdMOhIGNsYXJhbWVudGUgCmlkZW50aWZpY2FkbyBlIHJlY29uaGVjaWRvIG5vIHRleHRvIG91IG5vIGNvbnRlw7pkbyBkYSB0ZXNlIG91IGRpc3NlcnRhw6fDo28gb3JhIGRlcG9zaXRhZGEuCgpDQVNPIEEgVEVTRSBPVSBESVNTRVJUQcOHw4NPIE9SQSBERVBPU0lUQURBIFRFTkhBIFNJRE8gUkVTVUxUQURPIERFIFVNIFBBVFJPQ8ONTklPIE9VIApBUE9JTyBERSBVTUEgQUfDik5DSUEgREUgRk9NRU5UTyBPVSBPVVRSTyBPUkdBTklTTU8gUVVFIE7Dg08gU0VKQSBFU1RBClVOSVZFUlNJREFERSwgVk9Dw4ogREVDTEFSQSBRVUUgUkVTUEVJVE9VIFRPRE9TIEUgUVVBSVNRVUVSIERJUkVJVE9TIERFIFJFVklTw4NPIENPTU8gClRBTULDiU0gQVMgREVNQUlTIE9CUklHQcOHw5VFUyBFWElHSURBUyBQT1IgQ09OVFJBVE8gT1UgQUNPUkRPLgoKQSBVbml2ZXJzaWRhZGUgZG8gRXN0YWRvIGRvIFJpbyBkZSBKYW5laXJvIChVRVJKKSBzZSBjb21wcm9tZXRlIGEgaWRlbnRpZmljYXIgY2xhcmFtZW50ZSBvIHNldSBub21lIChzKSBvdSBvKHMpIG5vbWUocykgZG8ocykgCmRldGVudG9yKGVzKSBkb3MgZGlyZWl0b3MgYXV0b3JhaXMgZGEgdGVzZSBvdSBkaXNzZXJ0YcOnw6NvLCBlIG7Do28gZmFyw6EgcXVhbHF1ZXIgYWx0ZXJhw6fDo28sIGFsw6ltIGRhcXVlbGFzIApjb25jZWRpZGFzIHBvciBlc3RhIGxpY2Vuw6dhLgo=Biblioteca Digital de Teses e Dissertaçõeshttp://www.bdtd.uerj.br/PUBhttps://www.bdtd.uerj.br:8443/oai/requestbdtd.suporte@uerj.bropendoar:29032024-02-26T18:59:52Biblioteca Digital de Teses e Dissertações da UERJ - Universidade do Estado do Rio de Janeiro (UERJ)false |
dc.title.por.fl_str_mv |
Lactentes com fibrose cística do programa de triagem neonatal do Rio de Janeiro: avaliação clínica, microbiológica e radiológica |
dc.title.alternative.eng.fl_str_mv |
Infants with cystic fibrosis detected by newborn screening in Rio de Janeiro: clinical, microbiological and radiological evaluation |
title |
Lactentes com fibrose cística do programa de triagem neonatal do Rio de Janeiro: avaliação clínica, microbiológica e radiológica |
spellingShingle |
Lactentes com fibrose cística do programa de triagem neonatal do Rio de Janeiro: avaliação clínica, microbiológica e radiológica Cohen, Renata Wrobel Folescu Cystic fibrosis Newborn screening Computed tomography Fibrose Cística Triagem Neonatal Tomografia Computadorizada CIENCIAS DA SAUDE::MEDICINA::CLINICA MEDICA |
title_short |
Lactentes com fibrose cística do programa de triagem neonatal do Rio de Janeiro: avaliação clínica, microbiológica e radiológica |
title_full |
Lactentes com fibrose cística do programa de triagem neonatal do Rio de Janeiro: avaliação clínica, microbiológica e radiológica |
title_fullStr |
Lactentes com fibrose cística do programa de triagem neonatal do Rio de Janeiro: avaliação clínica, microbiológica e radiológica |
title_full_unstemmed |
Lactentes com fibrose cística do programa de triagem neonatal do Rio de Janeiro: avaliação clínica, microbiológica e radiológica |
title_sort |
Lactentes com fibrose cística do programa de triagem neonatal do Rio de Janeiro: avaliação clínica, microbiológica e radiológica |
author |
Cohen, Renata Wrobel Folescu |
author_facet |
Cohen, Renata Wrobel Folescu renatawfc@gmail.com |
author_role |
author |
author2 |
renatawfc@gmail.com |
author2_role |
author |
dc.contributor.advisor1.fl_str_mv |
Leão, Robson de Souza |
dc.contributor.advisor1Lattes.fl_str_mv |
http://lattes.cnpq.br/5369029097197832 |
dc.contributor.advisor-co1.fl_str_mv |
Boechat, Marcia Cristina Bastos |
dc.contributor.advisor-co1Lattes.fl_str_mv |
http://lattes.cnpq.br/4311911721179399 |
dc.contributor.referee1.fl_str_mv |
Costa, Cláudia Henrique da |
dc.contributor.referee1Lattes.fl_str_mv |
http://lattes.cnpq.br/7858045844671281 |
dc.contributor.referee2.fl_str_mv |
Marques, Elizabeth de Andrade |
dc.contributor.referee2Lattes.fl_str_mv |
http://lattes.cnpq.br/5959485578597640 |
dc.contributor.referee3.fl_str_mv |
Daltro, Pedro Augusto Nascimento |
dc.contributor.referee3Lattes.fl_str_mv |
http://lattes.cnpq.br/4593055652374668 |
dc.contributor.referee4.fl_str_mv |
Fonseca, Vania de Matos |
dc.contributor.referee4Lattes.fl_str_mv |
http://lattes.cnpq.br/1601115011359365 |
dc.contributor.authorLattes.fl_str_mv |
http://lattes.cnpq.br/7598766589760207 |
dc.contributor.author.fl_str_mv |
Cohen, Renata Wrobel Folescu renatawfc@gmail.com |
contributor_str_mv |
Leão, Robson de Souza Boechat, Marcia Cristina Bastos Costa, Cláudia Henrique da Marques, Elizabeth de Andrade Daltro, Pedro Augusto Nascimento Fonseca, Vania de Matos |
dc.subject.eng.fl_str_mv |
Cystic fibrosis Newborn screening Computed tomography |
topic |
Cystic fibrosis Newborn screening Computed tomography Fibrose Cística Triagem Neonatal Tomografia Computadorizada CIENCIAS DA SAUDE::MEDICINA::CLINICA MEDICA |
dc.subject.por.fl_str_mv |
Fibrose Cística Triagem Neonatal Tomografia Computadorizada |
dc.subject.cnpq.fl_str_mv |
CIENCIAS DA SAUDE::MEDICINA::CLINICA MEDICA |
description |
Cystic fibrosis (CF) is a progressive, genetic disease that affects 1:2500 caucasian live births. Diagnosis criteria are well defined and newborn screening programs (NBS) using immunoreactive trypsinogen (IRT) has been widely established worldwide and, more recently, in Brazil. Newborn infants with high risk of presenting CF are identified by NBS and, once diagnosis is confirmed, follow up and management must be initiated early. The benefits of NBS for CF have been studied and show evidences of improvement in nutritional status and lung function in infants, beside the contribution for long term survival. High resolution computed tomography (HRCT) is a sensitive method for detecting structural lung abnormalities. HRCT evaluation through reproducible scoring systems can be used to quantify chest computed tomography (CT) findings in cystic fibrosis (CF) patients. The aim of the study was to describe early HRCT findings according to a validated score system in cystic fibrosis infants diagnosed through newborn screening (NBS) followed at Instituto Nacional de Saúde da Mulher, da Criança e do Adolescente Fernandes Figueira – FIOCRUZ. This is a cross-sectional study including NBS infants with CF born between 2013-2017. Clinical, nutritional and microbiological data were evaluated at the moment of diagnosis. The subjects were submitted to HRCT scans within the first year after diagnosis, when clinically stable. The CT scans were evaluated through the modified Bhalla score. 35 patients were included in the study, with male predominance and mean age at diagnosis was 3.8 months. Regarding clinical presentation, 20% were asymptomatic, while respiratory signs were present in 45.7% and digestive in 65.7%, with anthropometric data revealing nutritional risk in 68.5% of patients. Microbiological data showed that 51.4% of patients had no CF pathogenic bacteria in respiratory secretion, while 17.1% presented Pseudomonas aeruginosa isolation and 31.4% showed other pathogenic bacteria. 32 subjects were submitted to HRCT scans. The mean total modified Bhalla score was 3.6 ± 2.1 and 93.8% scans were abnormal. Bronchial wall thickening was the most common parameter (90.6%), followed by collapse/consolidation (59.4%), mosaic attenuation/perfusion (50%), bronchiectasis (37.5%) and mucus plugging (15.6%). Bronchial wall thickening was diffuse in most patients. A substantial proportion of infants diagnosed with CF after detection by NBS already have evidence of lung disease. P. aeruginosa colonization was associated to higher Bhalla scores, highlighting the importance of this CF pathogen in early structural lung disease. The presence of bronchial wall thickening in most subjects in such early age patients may already reflect the presence of airway inflammatory process. Detection and quantification of structural abnormalities through modified Bhalla score may contribute to acknowledgment lung disease before clinically apparent. |
publishDate |
2018 |
dc.date.issued.fl_str_mv |
2018-08-10 |
dc.date.accessioned.fl_str_mv |
2022-08-26T15:36:42Z |
dc.type.status.fl_str_mv |
info:eu-repo/semantics/publishedVersion |
dc.type.driver.fl_str_mv |
info:eu-repo/semantics/doctoralThesis |
format |
doctoralThesis |
status_str |
publishedVersion |
dc.identifier.citation.fl_str_mv |
COHEN, Renata Wrobel Folescu. Lactentes com fibrose cística do programa de triagem neonatal do Rio de Janeiro: avaliação clínica, microbiológica e radiológica. 2018. 90 f. Tese (Doutorado em Ciências Médica) – Faculdade de Ciências Médicas, Universidade do Estado do Rio de Janeiro, Rio de Janeiro, 2018. |
dc.identifier.uri.fl_str_mv |
http://www.bdtd.uerj.br/handle/1/18286 |
identifier_str_mv |
COHEN, Renata Wrobel Folescu. Lactentes com fibrose cística do programa de triagem neonatal do Rio de Janeiro: avaliação clínica, microbiológica e radiológica. 2018. 90 f. Tese (Doutorado em Ciências Médica) – Faculdade de Ciências Médicas, Universidade do Estado do Rio de Janeiro, Rio de Janeiro, 2018. |
url |
http://www.bdtd.uerj.br/handle/1/18286 |
dc.language.iso.fl_str_mv |
por |
language |
por |
dc.rights.driver.fl_str_mv |
info:eu-repo/semantics/openAccess |
eu_rights_str_mv |
openAccess |
dc.format.none.fl_str_mv |
application/pdf |
dc.publisher.none.fl_str_mv |
Universidade do Estado do Rio de Janeiro |
dc.publisher.program.fl_str_mv |
Programa de Pós-Graduação em Ciências Médicas |
dc.publisher.initials.fl_str_mv |
UERJ |
dc.publisher.country.fl_str_mv |
Brasil |
dc.publisher.department.fl_str_mv |
Centro Biomédico::Faculdade de Ciências Médicas |
publisher.none.fl_str_mv |
Universidade do Estado do Rio de Janeiro |
dc.source.none.fl_str_mv |
reponame:Biblioteca Digital de Teses e Dissertações da UERJ instname:Universidade do Estado do Rio de Janeiro (UERJ) instacron:UERJ |
instname_str |
Universidade do Estado do Rio de Janeiro (UERJ) |
instacron_str |
UERJ |
institution |
UERJ |
reponame_str |
Biblioteca Digital de Teses e Dissertações da UERJ |
collection |
Biblioteca Digital de Teses e Dissertações da UERJ |
bitstream.url.fl_str_mv |
http://www.bdtd.uerj.br/bitstream/1/18286/2/Tese+-+Renata+Wrobel+Folescu+Cohen+-+2018+-+Completa http://www.bdtd.uerj.br/bitstream/1/18286/1/license.txt |
bitstream.checksum.fl_str_mv |
2e3cd6514074f1bb9e2f736791b41371 e5502652da718045d7fcd832b79fca29 |
bitstream.checksumAlgorithm.fl_str_mv |
MD5 MD5 |
repository.name.fl_str_mv |
Biblioteca Digital de Teses e Dissertações da UERJ - Universidade do Estado do Rio de Janeiro (UERJ) |
repository.mail.fl_str_mv |
bdtd.suporte@uerj.br |
_version_ |
1811728716693241856 |