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Prevalence of Anemia and its Relation with Shwachman Score in Children with Cystic Fibrosis
Mona Afifi1, Maryam Hassanzad1, Fatemeh Malek2
1Pediatric Respiratory Diseases Research Center, National Research Institute of Tuberculosis and Lung Diseases (NRITLD), Shahid Beheshti University of Medical Sciences, Tehran, Iran.
Insights
Cystic fibrosis (CF) disease severity correlates with anemia but not vitamin D deficiency in patients. Early diagnosis of CF is crucial for managing nutritional issues like anemia.
Area of Science:
- Medical Research
- Genetics
- Pulmonology
Background:
- Cystic fibrosis (CF) is a progressive genetic disorder with global impact.
- Early CF diagnosis aids in preventing nutritional complications such as anemia and vitamin deficiencies.
- Disease progression complicates CF management.
Purpose of the Study:
- To evaluate cystic fibrosis disease severity using the Shwachman-Kulczycki score.
- To investigate the relationship between CF disease severity and anemia.
- To determine the association between CF disease severity and vitamin D deficiency.
Main Methods:
- Cross-sectional study involving 57 diagnosed cystic fibrosis patients.
- Collected clinical and laboratory data from medical records.
- Assessed disease severity via Shwachman scoring system and blood sampling at diagnosis.
Main Results:
- Significant correlation found between cystic fibrosis disease severity and patient age (P=0.02).
- Disease severity was significantly higher in anemic CF patients (p=0.006).
- No significant difference observed between CF disease severity and vitamin D levels (P=0.150).
Conclusions:
- The Shwachman-Kulczycki scoring system effectively reflects the clinical status of CF patients.
- Further research with larger sample sizes and diverse methodologies is recommended for enhanced accuracy.
Background:
Cystic fibrosis is a chronic and progressive genetic disease with a worldwide prevalence. As the disease progresses, symptoms develop, and make its management more challenging. Accumulating evidence suggests that early diagnosis of CF can significantly contribute to preventing reported nutritional problems including anemia, vitamin deficiencies, and hypoalbuminemia. This cross-sectional study was conducted to assess disease severity in cystic fibrosis patients using the Shwachman-Kulczycki score, as well as to determine its relation with anemia and vitamin D deficiency.
Materials And Methods:
Clinical and CF-related laboratory data were collected from the medical records of 57 CF patients with a definitive diagnosis. At the time of diagnosis, physicians performed simultaneous, blood sampling and scoring of patients using the Shwachman scoring system.
Results:
The mean age of patients was 16.12±6.48 years. Total scores of 86-100, 71-85, 56-70, 41-55, and <40, were reported in 5.4%, 7.1%, 14.3%, 14.3%, and 58.9% of CF patients, respectively. A significant correlation was found between disease severity and patients' age (P=0.02). The analysis also showed that the disease severity was significantly higher in anemic patients when compared to non-anemics (p =0.006). Based on the results, 33 patients with normochromic, 11 patients with microcytic, and 6 patients with macrocytic anemia were diagnosed in this study. We did not find a significant difference between disease severity and vitamin D levels (P=0.150).
Conclusion:
The scoring system used in the current study could reflect properly the clinical status of CF patients. However, simultaneous use of various methods using a larger sample size for comparison of results is suggested to improve the accuracy of findings.
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