Comparação entre provas de função pulmonar, escore de Shwachman-Kulczycki e escore de Brasfield em pacientes com fibrose cística

Freire, Ivanice Duarte; Silva, Fernando Antonio de Abreu e; Araújo, Manuel Ângelo de

doi:10.1590/s1806-37132008000500006

Cited by 12 publications

(10 citation statements)

References 17 publications

(26 reference statements)

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“…Similar to our findings and despite having a better FEV 1 , two retrospective studies in patients with CF in Brazil also found significant correlations between the SK score and FEV 1 (r = 0.5 and 0.75) 18,19. …”

Section: Discussionsupporting

confidence: 91%

“…Note that the correlation they found is higher than that found in our study (r = 0.71). Freire et al19 while evaluating 40 children with CF also found a significant correlation between the SK score and the Brasfield score (r = 0.62). The highest correlation observed in our study was between the SK score and CT (r = −0.78, p < 0.001).…”

Section: Discussionmentioning

confidence: 89%

“…Another limitation of the SK score is that its development was based on pediatric patients and some of the domains are specifically focused on children. Nevertheless, it is still used in many CF clinics and studies, even in older patients 18,19,24. …”

Section: Discussionmentioning

confidence: 99%

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Shwachman-Kulczycki score still useful to monitor cystic fibrosis severity

Stollar

Adde

Cunha

et al. 2011

Clinics

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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 (FEV1), 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 FEV1 was 59 ± 26 (as percentage of predicted values). The following significant correlations versus the Shwachman-Kulczycki score were found: FEV1 (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 FEV1, a statistically significantly correlation with the Shwachman-Kulczycki score was found only in patients with FEV1 <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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Section: Discussionsupporting

confidence: 91%

Section: Discussionmentioning

confidence: 89%

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Shwachman-Kulczycki score still useful to monitor cystic fibrosis severity

Stollar

Adde

Cunha

et al. 2011

Clinics

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“…Previous research verified a good correlation between the SK score and lung function [22,23]. Therefore, the SK score might be a useful tool in countries where CF-specific HRQoL questionnaires and spirometry are not accessible in clinical practice.…”

Section: Discussionmentioning

confidence: 85%

Factors influencing quality of life and disease severity in Hungarian children and young adults with cystic fibrosis

et al. 2014

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BackgroundThe aim of our study was to evaluate factors affecting cystic fibrosis (CF) patients’ health-related quality of life (HRQoL) and to assess the level of agreement on HRQol between children and their parents.MethodsFifty-nine patients (mean age: 14.03 ± 4.81 years) from 5 Hungarian CF centres completed the survey. HRQoL was measured using The Cystic Fibrosis Questionnaire-Revised (CFQ-R). Parents were asked to fill out a questionnaire about their smoking habits, educational level and history of chronic illness. Disease severity was assessed using the physician-reported Shwachman-Kulczycki (SK) score system. Spirometry, Body Mass Index (BMI) percentile (pc), hospitalisation and Pseudomonas aeruginosa (PA) infection were examined as physiologic parameters of CF, and the impact of these factors on HRQoL was assessed. A multivariate regression analysis was performed to identify the most important factors affecting HRQoL. The level of significance was set to 0.05.ResultsPassive smoking and parental educational level and chronic diseases status did not have a significant impact on the patients’ HRQoL (p > 0.05). Significantly lower SK scores and spirometry values were found in low BMI pc patients (p < 0.001), in hospitalised (p < 0.01) and in PA-infected patients (p < 0.01), than in the adequate-weight, non-hospitalised and PA culture-negative subgroup. Lower CFQ-R scores were detected in hospitalised patients than in non-hospitalised patients in their Physical functioning domain. PA-infected patients had HRQoL scores that were significantly worse in the Body image (p < 0.01) and Respiratory symptoms (p < 0.05) domains than the PA culture-negative patients. Patients with a low BMI pc (<25th BMI pc) had significantly lower scores in the Eating, Body image and Treatment burden domains, than the adequate-weight patients (>25th BMI pc) (p < 0.01). A strong child–parent agreement was found in the Physical functioning domain (r = 0.77, p < 0.01).ConclusionsPassive smoking, parental educational level and chronic diseases of parents do not affect the HRQoL of CF patients. In contrast, hospitalisation, PA infection and malnutrition have a significant and negative impact on patients’ HRQoL and the clinical severity of the disease. Parents and children were consistent in their scoring of symptoms and behaviours that were observable.

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“…Estudos prévios que incluíram pacientes pediátricos de maior média de idade (abrangendo adolescentes) encontraram importante relação entre escore de Shwachman e testes de função pulmonar, especialmente VEF 1 [20][21][22]. No presente estudo, embora verificada diferença nesse escore na comparação entre os grupos, não se observaram melhores parâmetros espirométricos no grupo IRT.…”

Section: Discussionunclassified

Comparação entre a evolução de pré-escolares com fibrose cística identificados por triagem neonatal ou por sintomatologia clínica

Lumertz¹,

Moura²,

Pinto³

et al. 2018

Sci Med

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Este artigo está licenciado sob forma de uma licença Creative Commons Atribuição 4.0 Internacional, que permite uso irrestrito, distribuição e reprodução em qualquer meio, desde que a publicação original seja corretamente citada. RESUMOOBJETIVOS: Comparar dados clínicos, laboratoriais e espirométricos de pacientes pré-escolares (idade entre três e seis anos) com fibrose cística, identificados pela alteração do tripsinogênio imunorreativo no teste de triagem neonatal (grupo IRT) com os dados de pacientes cuja suspeita diagnóstica ocorreu a partir de sinais e sintomas clínicos (grupo DC). MÉTODOS: Estudo do tipo coorte retrospectiva, com amostra obtida a partir dos prontuários dos pacientes que faziam acompanhamento ambulatorial especializado e multidisciplinar. Todas as crianças incluídas tinham diagnóstico confirmado de fibrose cística por teste de eletrólitos no suor e/ou sequenciamento genético. Como variáveis incluíram-se sexo, peso, altura, índice de massa corporal, número de internações por ano, colonização por Pseudomonas aeruginosa, presença de insuficiência pancreática e escore de Shwachman. Este escore abrange quatro domínios: atividade geral, nutrição, exame radiológico e avaliação física, cada um pontuado entre 5 e 25 pontos, sendo que o escore ≤40 pontos indica estado grave e ≥86 pontos excelente estado. Para comparação da função pulmonar utilizaram-se os dados da última espirometria realizada antes dos seis anos de idade. Os desfechos foram comparados por qui-quadrado ou teste t de Student, com limite de significância de 0,05. RESULTADOS: Foram incluídos no estudo 24 pacientes, sete no grupo IRT e 17 no grupo DC. As crianças do grupo IRT iniciaram acompanhamento com média de idade de 1,51±2,04 meses e as do grupo DC com 3,77±1,80 meses (p=0,014). A média do escore de Shwachman foi 94,40±2,19 nos pacientes do grupo IRT vs. 87,67±8,00 no grupo DC (p=0,018). A média do escore Z para o índice de massa corporal foi 0,95±1,02 no grupo IRT vs. 0,51±1,05 no grupo DC (p=0,051). Foram encontrados valores ligeiramente superiores nas variáveis espirométricas no grupo IRT, sem diferença estatisticamente significativa entre os grupos. CONCLUSÕES: Os resultados sugerem que os pacientes diagnosticados com fibrose cística a partir da suspeita pelo teste de triagem neonatal beneficiaram-se de uma intervenção precoce, podendo iniciar os testes de função pulmonar e receber tratamento e orientações mais cedo. A avaliação de parâmetros clínicos com o escore de Shwachman mostrou que os benefícios puderam ser observados já na idade pré-escolar. DESCRITORES: fibrose cística; espirometria; triagem neonatal; triagem do recém-nascido; nutrição da criança. ABSTRACTAIMS: To compare clinical, laboratory and spirometric parameters of preschoolers (ages from three to six years old) with cystic fibrosis identified by abnormal newborn screening test (NS group), with data of patients whose diagnostic suspicion arose from characteristic clinical signs and symptoms (CS group). METHODS: Retrospective cohort study, with a sample obtained...

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Comparação entre provas de função pulmonar, escore de Shwachman-Kulczycki e escore de Brasfield em pacientes com fibrose cística

Abstract: These two scoring systems are complementary, correlating with each other, as well as with pulmonary function tests. The radiology domain of the S-K scoring system is a good alternative to the Brasfield score.

Cited by 12 publications

References 17 publications

Shwachman-Kulczycki score still useful to monitor cystic fibrosis severity

Shwachman-Kulczycki score still useful to monitor cystic fibrosis severity

Factors influencing quality of life and disease severity in Hungarian children and young adults with cystic fibrosis

Comparação entre a evolução de pré-escolares com fibrose cística identificados por triagem neonatal ou por sintomatologia clínica

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