Shwachman Score in Clinical Evaluation of Cystic Fibrosis

Abstract

Background: Cystic fibrosis is a complex progressive disease which assessing its progression and severity is essential. For this purpose there are scoring systems available to evaluate the disease severity. Objectives:The aim of the present study was to determine the clinical status of CF patients using shwachman score system in the pediatric pulmonary ward of Masih Daneshvari Hospital. Patients and Methods:A cross-sectional study was conducted to evaluate the clinical status with shwachman score system. 23 patients ranging from 5 to23 years were enrolled in this study. All data was extracted objectively from Shwachman-Kulczycki scoring system. Results: The overall mean Shwachman-Kulczycki score was 53.48 ± 13.8. Total scores of < 40 (severe), 41-55, 56-70, and 71-85 were detected in 1.7%, 39.1%, 30.4% and 8.7% of patients respectively. None of the patients were categorized in the excellent range. We found no statistically significant correlation between mortality rate and clinical score (P = 0.136). However, the results showed a statistically significant correlation between mortality rate and Shwachman score, (P = 0.02). Conclusions: Shwachman-Kulczycki score is an easily applied scoring system which can reflect the clinical status of patients objectively. However, concurrent use of other evaluating tools such as pulmonary function test (PFT) and Computed tomography scoring systems provide a more robust monitoring and a reliable evaluation tool.

Evaluation of the psychological status of mothers of children with cystic fibrosis and the relationship between children's clinical status

“…normal), which resulted in a total score categorised as excellent (86-100), good (71-85), mild (56-70), moderate (41-55) and severe (<40). 11 Statistical Package for Social Sciences (SPSS) version 21.0 software (Chicago, IL, USA) was used to perform data analyses. Continuous variables were reported as mean AE standard error of the mean, median, minimum and maximum levels, while categorical variables were reported as rate and percentage values.…”

Long Term Outcome of Cystic Fibrosis Patients with Multisystem Evaluation

“…This may be explained by mutations with less severe forms of presentation of the disease. To evaluate the disease severity, scoring systems such as "Shwachman-Kulczycki score system" are available [22]. It also underscores this fact that higher socioeconomic status of the family with better provision of nutritional supplements may greatly influence the growth of the child.…”

Clinical Scoring Systems in Cystic Fibrosis – What Are the Options for Developing Countries?

“…The chosen clinical scoring system needs to reflect daily clinical practice and it needs to obtain objective clinical information in a standardized, repeatable manner (13). In this journal, Khalilzadeh et al (14) published a clinical evaluation of 23 CF patients admitted to a Pediatric Ward in an Iranian Hospital, using the S-K scoring system; including six patients who died.…”

“…The study by Khalilzadeh et al (14) was further limited by the small sample size, the fact that the score seemed to be retrospectively applied, and the "snapshot" nature of the study (as opposed to longitudinal analysis of changes in the score over time). The fact that none of the children had "excellent" scores is not surprising given that the samples were selected from children who were admitted to hospital, and therefore likely had a more severe phenotype of CF.…”

“…Khalilzadeh et al's (14) concern about the effects of late diagnosis due to lack of neonatal screening is valid (19), and is a concern shared with other poorly resourced countries, and/or those of which screening may be con-sidered impractical or inappropriate. We have previously reported the problem of late diagnosis and the potential impact of that on the outcome in a CF cohort from South Africa (5).…”