title: Shwachman-Kulczycki score still useful to monitor cystic fibrosis severity.
abstract: BACKGROUND
  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.
authors.affiliation: Instituto da Criança, Hospital das Clínicas, University of São Paulo, São Paulo, Brazil. fabipediatria@hotmail.com
authors.forename: Fabíola
authors.initials: F
authors.lastname: Stollar
authors.forename: Fabíola Villac
authors.initials: FV
authors.lastname: Adde
authors.forename: Maristela T
authors.initials: MT
authors.lastname: Cunha
authors.forename: Claudio
authors.initials: C
authors.lastname: Leone
authors.forename: Joaquim C
authors.initials: JC
authors.lastname: Rodrigues
mesh_terms: D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D003430:Cross-Sectional Studies; D003550:Cystic Fibrosis / Q000000981:diagnostic imaging / Q000503:physiopathology*; D005260:Female; D005541:Forced Expiratory Volume; D006801:Humans; D016014:Linear Models; D008168:Lung / Q000503:physiopathology*; D008297:Male; D011446:Prospective Studies; D011859:Radiography; D012129:Respiratory Function Tests; D012720:Severity of Illness Index; D016138:Walking / Q000502:physiology; D055815:Young Adult