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Pediatrics |

Cystic Fibrosis in the 21st Century: Are There Still Reasons for Poor Outcomes and Early Deaths in a Pediatric Cystic Fibrosis Cohort?

Laura Dracea, PhD
Author and Funding Information

University Children's Hospital, Brasov, Romania


Chest. 2013;144(4_MeetingAbstracts):780A. doi:10.1378/chest.1703138
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Abstract

SESSION TITLE: Pediatric Pulmonary Posters

SESSION TYPE: Original Investigation Poster

PRESENTED ON: Wednesday, October 30, 2013 at 01:30 PM - 02:30 PM

PURPOSE: Outcomes and survival in Cystic Fibrosis in absence of a cure, depend on a multitude of factors as phenotype-genotype correlations,environment, time of intervention, quality of care. To identify risk factors for poor outcomes in a pediatric cohort of CF patients in order to prevent rapid decline of clinical status and early deaths.

METHODS: Retrospective clinical file analysis of CF patients (birth-18 years) followed up during 1998-2013 in a Clinical Children's Hospital with no specialized CF facility for care. Risk factors for poor outcomes as socioeconomic status, late diagnosis, lack of education, lack of funding were correlated with clinical evaluation, related conditions and age at death.

RESULTS: 55 CF patients were diagnosed and followed up, mean age at diagnosis 11 months. 75% of patients had at least one del F508 mutation (homozigous status 66%). 13 patients had meconium ileus (MI), all underwent surgery with high complication rate (80%) due to feeding problems in the early postoperative phase. 21/55 patients died, 12 before the age of 1 year. Low socioeconomic status, malnutrition at diagnosis, poor understanding of disease with emphasis on nutrition were statistic relevant RF in the deceased group (p<0.05). Comorbidities/ disease complications (early CFRLD 8/55, ABPA 4/55, diabetes 1/55 impacted on clinical outcomes. In 13/55 patients,diagnosed with other chronic diseases (juvenile myoclonic epilepsy, craniostenosis, cardiac malformations, asthma and allergic rhinitis, ADHD, renal malformations), lower QoL scores were observed, burden of treatment interventions was higher. (p<0.05).

CONCLUSIONS: Early diagnosis and interventions, proper care in specialized CF units, educational programs for families and medical staff, proper funding, availability of multidisciplinary care can make a difference in QoL and survival of CF patients.

CLINICAL IMPLICATIONS: Improve clinical care for CF patients, set the background for developing specialized CF Centers, develop education for CF in the 21st century. Prevent poor outcomes through implementing treatment strategies adjusted to local conditions.

DISCLOSURE: The following authors have nothing to disclose: Laura Dracea

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