Assessment of cystic fibrosis severity

Abstract

Introduction. Cystic fibrosis (CF) is one of the most common hereditary diseases and being characterized by chronic lung injury, exocrine pancreatic insufficiency and nutrition disorders. In this disease the mutation of the CFTR gene lead to changes of sodium chloride metabolism inside and outside epithelial cells found in he lungs, liver, pancreas, digestive tract and reproductive system. Thus, the result of this malfunction is represented by sticky and thick mucus, salty taste of the sweat and thickened digestive juices which can clog the lumen and alveoli of the lungs (clinically difficult breathing, formation of the environment proned to bacteria growth) or may disturb (when the pancreas is mainly involved) the process or proper digestion and absorbtion of nutrients, leading even to organ failure insevere cases (lungs, pancreas). Pulmonary involvement in CF reflects the severity of the disease and represents the major cause of death.Major criteria used to assess CF severity are based on the evaluation of the lung function. Materials and methods. Our study included 60 patients (the average age 9.08±1.01 years) diagnosed with cystic fibrosis. CF severity was assessed using Shwachman-Kulczycki score, which is based on the following criteria: overall activity of the patient, physical examination results, nutritional status, and data of the chest X-ray examination. Each category was assigned from 1 to 25 points, while the total score ranged from 4 to 100 points maximum (severe ≤40 points, 40-55 points – moderate; mild – 56-70 points, 71-85 points – good, and excellent – 86-100 points). Results and discussions. The Shwachman-Kulczycki score of just 25.46±2.09 points, that indicates a severe evolution of CF, was registered at 46.81% of children with severe malnutrition, but also in older patients with advanced lung diseases. For 25.92% of children the score was 53.57±0.63 points, that means moderate evolution of cystic fibrosis. In 15.6% of patients the Shwachman-Kulczycki score showed a favorable clinical evolution, with a summary of 62.12±0.98 points. Only 12.77% of children had mild form of the diseases with a good score of 78.0±1.30 points. In the study group there were no children identified to have with excellent clinical condition, because of the presence of changes in clinical status and paraclinical tests. Conclusion. The Shwachman-Kulczycki score that includes clinical and imaging criteria, is a very simple to use tool, demonstrated to be highly informative in assessing the clinical status of patients with cystic fibrosis and is recommended to be used in the work of specialists in pediatrics.