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

“…severely impaired) to 25 (i.e. 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 …”

Neutrophil lymphocyte ratio, mean platelet volume, and immunoreactive trypsinogen as early inflammatory biomarkers for cystic fibrosis in infancy: A retrospective cohort study

“…Demographic features of all children, pancreatic sufficiency/insufficiency status, z‐scores for weight and height measurements, and body mass indexes (BMI) for children over 2 years which were calculated using reference values issued by the Centers for Disease Control were recorded at first admission, and first, second, third and 6 years of ages 24 . If present, radiological findings at first admission, and first, second, third and 6 years of ages were noted.…”

“…Control were recorded at first admission, and first, second, third and 6 years of ages. 24 If present, radiological findings at first admission, and first, second, third and 6 years of ages were noted. PFT were noted in patients who could perform the tests, and mean forced expiratory volume in 1 s (FEV 1 ), forced vital capacity (FVC), and forced expiratory flow (FEF 25−75 ) were recorded as percentages.…”

Evaluation of miR-155 Expression in Cystic Fibrosis Patients

“…In this cross-sectional study, there were 30 CF patients (based on clinical manifestations, sweat test, and genetic testing of the homozygous F508del mutation) aging from The study protocol was performed according to our previous research (5). Demographic data including age, sex, BMI, nutrition, clinical symptoms, sputum culture, and spirometry examination were collected for patients older than 4, and subsequently, the Shwachman-Kulczycki score was calculated.…”

“…Despite the improved treatment of CF and increased life expectancy, respiratory failure is still a principal cause of mortality in these patients (4). Therefore, to assess the disease progression, it is important to evaluate the patients and understand the involved parameters in the manifestation of CF signs and symptoms (5).…”