
IMPROVING EARLY DIAGNOSIS AND A MULTIDISCIPLINARY APPROACH TO THE MANAGEMENT OF CHILDREN WITH CYSTIC FIBROSIS IN UZBEKISTAN
Talgatova Aygerim Talgatovna , Republican Specialized Scientific and Practical Medical Center of Pediatrics,Tashkent, UzbekistanAbstract
Objective: To evaluate the effectiveness of an integrated program for early diagnosis and a multidisciplinary team (MDT) approach in managing children with cystic fibrosis (CF) in Uzbekistan, aiming to improve clinical outcomes. Methods: A retrospective cohort study was conducted, analyzing the medical records of CF patients diagnosed at a national referral center. Patients were divided into two groups: Group 1 (n=50), diagnosed before the implementation of the new program (2015-2018), and Group 2 (n=55), diagnosed and managed under the new program (2019-2022), which included standardized diagnostic algorithms (sweat test, genetic testing) and coordinated care from an MDT (pediatric pulmonologist, gastroenterologist, physiotherapist, nutritionist). Key outcomes, including age at diagnosis, nutritional status (BMI-for-age z-score), and pulmonary function (FEV1%), were compared between the groups. Results: The mean age at diagnosis was significantly lower in Group 2 (0.8 ± 0.5 years) compared to Group 1 (4.2 ± 2.1 years) (p < 0.001). At age 8, children in Group 2 demonstrated significantly better clinical outcomes: a higher mean BMI-for-age z-score (-0.5 ± 0.4 vs. -1.8 ± 0.7, p < 0.001) and a higher mean FEV1% predicted (85% ± 10% vs. 68% ± 15%, p < 0.001). The annual rate of pulmonary exacerbations requiring hospitalization was also lower in Group 2 (0.7 per year) compared to Group 1 (2.5 per year). Conclusion: The implementation of a structured program for early diagnosis combined with a multidisciplinary approach to care significantly improves the nutritional status, and pulmonary function, and reduces morbidity in children with cystic fibrosis in Uzbekistan. These findings strongly support the nationwide expansion of newborn screening for CF and the establishment of specialized MDT centers to improve long-term prognosis.
Keywords
cystic fibrosis, multidisciplinary team, early diagnosis, newborn screening, sweat test, Uzbekistan, pediatrics, FEV1.
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