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Shwachman-Kulczycki score still useful to monitor cystic fibrosis severity

INTRODUCTION: The Shwachman-Kulczycki score was the first scoring system used in cystic fibrosis to assess disease severity. Despite its subjectivity, it is still widely used. OBJECTIVE: To study correlations among forced expiratory volume in one second (FEV(1)), chest radiography, chest computed to...

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Autores principales: Stollar, Fabíola, Villac Adde, Fabíola, Cunha, Maristela T, Leone, Claudio, Rodrigues, Joaquim C
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo 2011
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3129961/
https://www.ncbi.nlm.nih.gov/pubmed/21808862
http://dx.doi.org/10.1590/S1807-59322011000600010
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author Stollar, Fabíola
Villac Adde, Fabíola
Cunha, Maristela T
Leone, Claudio
Rodrigues, Joaquim C
author_facet Stollar, Fabíola
Villac Adde, Fabíola
Cunha, Maristela T
Leone, Claudio
Rodrigues, Joaquim C
author_sort Stollar, Fabíola
collection PubMed
description INTRODUCTION: The Shwachman-Kulczycki score was the first scoring system used in cystic fibrosis to assess disease severity. Despite its subjectivity, it is still widely used. OBJECTIVE: To study correlations among forced expiratory volume in one second (FEV(1)), chest radiography, chest computed tomography, 6-minute walk test, and Shwachman-Kulczycki score in patients with cystic fibrosis and to test whether the Shwachman-Kulczycki score is still useful in monitoring the severity of the disease. METHODS: A cross-sectional prospective study was performed to analyze the correlations (Spearman). Patients with clinically stable cystic fibrosis, aged 3-21 years, were included. RESULTS: 43 patients, 19F/24M, mean age 10.5 ± 4.7 years, with a median Shwachman-Kulczycki score of 70 were studied. The median Brasfield and Bhalla scores were 17 and 10, respectively. The mean Z score for the 6-minute walk test was −1.1 ± 1.106 and the mean FEV(1) was 59 ± 26 (as percentage of predicted values). The following significant correlations versus the Shwachman-Kulczycki score were found: FEV(1) (r  =  0.76), 6-minute walk test (r  =  0.71), chest radiography (r  =  0.71) and chest computed tomography (r  =  −0.78). When patients were divided according to FEV(1), a statistically significantly correlation with the Shwachman-Kulczycki score was found only in patients with FEV(1) <70% (r  =  0.67). CONCLUSIONS: The Shwachman-Kulczycki score remains an useful tool for monitoring the severity of cystic fibrosis, adequately reflecting the functional impairment and chest radiography and tomography changes, especially in patients with greater impairment of lung function. When assessing patients with mild lung disease its limitations should be considered and its usefulness in such patients should be evaluated in larger populations.
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spelling pubmed-31299612011-07-06 Shwachman-Kulczycki score still useful to monitor cystic fibrosis severity Stollar, Fabíola Villac Adde, Fabíola Cunha, Maristela T Leone, Claudio Rodrigues, Joaquim C Clinics (Sao Paulo) Clinical Science INTRODUCTION: The Shwachman-Kulczycki score was the first scoring system used in cystic fibrosis to assess disease severity. Despite its subjectivity, it is still widely used. OBJECTIVE: To study correlations among forced expiratory volume in one second (FEV(1)), chest radiography, chest computed tomography, 6-minute walk test, and Shwachman-Kulczycki score in patients with cystic fibrosis and to test whether the Shwachman-Kulczycki score is still useful in monitoring the severity of the disease. METHODS: A cross-sectional prospective study was performed to analyze the correlations (Spearman). Patients with clinically stable cystic fibrosis, aged 3-21 years, were included. RESULTS: 43 patients, 19F/24M, mean age 10.5 ± 4.7 years, with a median Shwachman-Kulczycki score of 70 were studied. The median Brasfield and Bhalla scores were 17 and 10, respectively. The mean Z score for the 6-minute walk test was −1.1 ± 1.106 and the mean FEV(1) was 59 ± 26 (as percentage of predicted values). The following significant correlations versus the Shwachman-Kulczycki score were found: FEV(1) (r  =  0.76), 6-minute walk test (r  =  0.71), chest radiography (r  =  0.71) and chest computed tomography (r  =  −0.78). When patients were divided according to FEV(1), a statistically significantly correlation with the Shwachman-Kulczycki score was found only in patients with FEV(1) <70% (r  =  0.67). CONCLUSIONS: The Shwachman-Kulczycki score remains an useful tool for monitoring the severity of cystic fibrosis, adequately reflecting the functional impairment and chest radiography and tomography changes, especially in patients with greater impairment of lung function. When assessing patients with mild lung disease its limitations should be considered and its usefulness in such patients should be evaluated in larger populations. Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo 2011-06 /pmc/articles/PMC3129961/ /pubmed/21808862 http://dx.doi.org/10.1590/S1807-59322011000600010 Text en Copyright © 2011 Hospital das Clínicas da FMUSP http://creativecommons.org/licenses/by-nc/3.0/ This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/3.0/) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.
spellingShingle Clinical Science
Stollar, Fabíola
Villac Adde, Fabíola
Cunha, Maristela T
Leone, Claudio
Rodrigues, Joaquim C
Shwachman-Kulczycki score still useful to monitor cystic fibrosis severity
title Shwachman-Kulczycki score still useful to monitor cystic fibrosis severity
title_full Shwachman-Kulczycki score still useful to monitor cystic fibrosis severity
title_fullStr Shwachman-Kulczycki score still useful to monitor cystic fibrosis severity
title_full_unstemmed Shwachman-Kulczycki score still useful to monitor cystic fibrosis severity
title_short Shwachman-Kulczycki score still useful to monitor cystic fibrosis severity
title_sort shwachman-kulczycki score still useful to monitor cystic fibrosis severity
topic Clinical Science
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3129961/
https://www.ncbi.nlm.nih.gov/pubmed/21808862
http://dx.doi.org/10.1590/S1807-59322011000600010
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