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 (FEV1), chest radiography, chest computed tomo...
| Main Authors: | , , , , |
|---|---|
| Format: | Article |
| Language: | English |
| Published: |
Elsevier España
2011-01-01
|
| Series: | Clinics |
| Subjects: | |
| Online Access: | http://www.scielo.br/scielo.php?script=sci_arttext&pid=S1807-59322011000600010 |
| _version_ | 1828781345041547264 |
|---|---|
| author | Fabíola Stollar Fabíola Villac Adde Maristela T. Cunha Claudio Leone Joaquim C. Rodrigues |
| author_facet | Fabíola Stollar Fabíola Villac Adde Maristela T. Cunha Claudio Leone Joaquim C. Rodrigues |
| author_sort | Fabíola Stollar |
| collection | DOAJ |
| 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 (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. |
| first_indexed | 2024-12-11T17:32:01Z |
| format | Article |
| id | doaj.art-1e538b804b3c45b89a88d372b02d8fe2 |
| institution | Directory Open Access Journal |
| issn | 1807-5932 1980-5322 |
| language | English |
| last_indexed | 2024-12-11T17:32:01Z |
| publishDate | 2011-01-01 |
| publisher | Elsevier España |
| record_format | Article |
| series | Clinics |
| spelling | doaj.art-1e538b804b3c45b89a88d372b02d8fe22022-12-22T00:56:47ZengElsevier EspañaClinics1807-59321980-53222011-01-0166697998310.1590/S1807-59322011000600010Shwachman-Kulczycki score still useful to monitor cystic fibrosis severityFabíola StollarFabíola Villac AddeMaristela T. CunhaClaudio LeoneJoaquim C. RodriguesINTRODUCTION: 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.http://www.scielo.br/scielo.php?script=sci_arttext&pid=S1807-59322011000600010Chest radiographySix minute walk testSpirometryChest tomographyClinical status |
| spellingShingle | Fabíola Stollar Fabíola Villac Adde Maristela T. Cunha Claudio Leone Joaquim C. Rodrigues Shwachman-Kulczycki score still useful to monitor cystic fibrosis severity Clinics Chest radiography Six minute walk test Spirometry Chest tomography Clinical status |
| 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 | Chest radiography Six minute walk test Spirometry Chest tomography Clinical status |
| url | http://www.scielo.br/scielo.php?script=sci_arttext&pid=S1807-59322011000600010 |
| work_keys_str_mv | AT fabiolastollar shwachmankulczyckiscorestillusefultomonitorcysticfibrosisseverity AT fabiolavillacadde shwachmankulczyckiscorestillusefultomonitorcysticfibrosisseverity AT maristelatcunha shwachmankulczyckiscorestillusefultomonitorcysticfibrosisseverity AT claudioleone shwachmankulczyckiscorestillusefultomonitorcysticfibrosisseverity AT joaquimcrodrigues shwachmankulczyckiscorestillusefultomonitorcysticfibrosisseverity |