Clinical characteristics of patients requiring lung transplantation referral in national cystic fibrosis registry data
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Date
2023
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Abstract
Background. We aimed to determine the number of cystic fibrosis (CF) patients recorded in the Cystic Fibrosis Registry of Türkiye (CFRT) who were in need of lung transplantation (LT) referral and examine clinical differences between patients who were LT candidates due to rapid forced expiratory volume in one second (FEV₁) decline and LT candidates without rapid FEV₁ decline in the last year to identify a preventable cause in patients with such rapid FEV₁ decline. Methods. All CF patients recorded in the CFRT in 2018 were evaluated in terms of LT. Patients were divided into those with FEV₁ below 50% and in need of LT due to a decrease of 20% or more in the previous year (Group 1) and those who did not have FEV₁ decline of more than 20% in the previous year but had other indications for LT (Group 2). Demographic and clinical features were compared between the two groups. Results. Of 1488 patients registered in CFRT, 58 had a need for LT. Twenty patients were included in Group 1 and others in Group 2. Our findings did not reveal any significant variations in treatment, chronic infection status, or complications between the two groups. The average weight z-score was significantly higher in Group 1. Positive correlations were detected between weight z-score and FEV₁ in 2017 in Group 1 and between FEV₁ values in 2017 and 2018 in Group 2. Conclusions. There appears to be a relationship between the nutritional status and weight z-scores of CF patients and pulmonary function, which may indirectly affect the need for lung transplantation referral. © 2023, Turkish National Pediatric Society. All rights reserved.
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Cystic Fibrosis , Forced Expiratory Volume , Humans , Lung , Lung Transplantation , Referral and Consultation , Routinely Collected Health Data , azithromycin , bronchodilating agent , mannitol , proton pump inhibitor , ursodeoxycholic acid , Achromobacter , adolescent , adult , allergic bronchopulmonary aspergillosis , arterial embolization , arterial gas , Article , artificial ventilation , atypical mycobacteriosis , atypical Mycobacterium , body mass , Burkholderia cepacia , Burkholderia cepacia infection , chronic obstructive lung disease , clinical feature , controlled study , cystic fibrosis , echocardiography , enzyme replacement , female , forced expiratory volume , forced vital capacity , gene mutation , Haemophilus influenzae , Haemophilus influenzae infection , hemoptysis , human , intensive care unit , length of stay , liver disease , lung function , lung function test , lung transplantation , major clinical study , male , malnutrition , methicillin resistant Staphylococcus aureus , nonhuman , noninvasive ventilation , osteoporosis , positive end expiratory pressure ventilation , prophylaxis , Pseudomonas aeruginosa , Pseudomonas infection , spirometry , Staphylococcus aureus , Staphylococcus aureus infection , Stenotrophomonas maltophilia , complication , cystic fibrosis , lung , patient referral , routinely collected health data