Source: Annual Congress 2009 - Cystic fibrosis: advances in clinical research

Abstract

Early diagnosis and proper care can improve survival in Cystic Fibrosis (CF).
Aim: to determine if cetain risk factors (RF) may impact outcomes.
Methods: retrospective study of clinical files of CF patients (age range 0-24 years) followed up bertween 1999-2009 in a Children‘ s Hospital, without CF care facilities. RF: late follow up (LF), poor socioeconomic status (PSS), malnutrition at diagnosis (MD), del F508 homozygous (HDF), frequent pulmonary exacerbations (PE), early Paeruginosa colonisation (EPC), associated conditions (AC) were correlated with clinical scores. Funding for CF care (2004-) was also considered. We analysed group A (25/38 patients) diagnosed before 1 year of age, group B (5/38)- after 1 year; excluding meconium ileus (MI) patients.
Results: 10 patients died (70% before 2004, mean age at death 1.1 year). 21% presented with MI and underwent surgery. Age when started follow up differed between groups (0.38 months vs. 4.57 years) which impacted on recurrence of PE (53% vs83%) (p<0.001). 81% patients were genotyped; 57% of the 28 currently followed up were HDF. 35% (10/28) patients had malnutrition at evaluation; this correlated in 35% (8/28) with PSS and impacted on Schwachmann scores. Early CFRLD (4/38), ABPA (2/38) and reintervention for complicated MI (6/8) influenced the outcomes. 85% patients had EPC. PE were more frequent in patients who associated PSS, MD, AC. Lung function was poorer over the study period in the late followed up patients who had more RF.
Conclusion: Analysed RF negatively impacted the outcomes; some of them could have been influnced by early diagnosis. High standards of care, staffed CF units and funding of care should be priorities when aiming for better outlook.

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Citations should be made in the following way:
L. Dracea, L. Pop, L. Tamas (Brasov, Timisoara, Romania). Poor outcomes in cystic fibrosis – a challenging experience in eastern Europe. Eur Respir J 2009; 34: Suppl. 53, 1252

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