Pediatric patients with cystic fibrosis (CF) who present crackling noises in the lungs or nail clubbing at two or more encounters with their doctor have 5.5 and 3.7 times increased risk of early mortality, respectively, according to a report published in the journal Pediatric Pulmonology.
Improved treatments and care of children with CF have reduced mortality rates. From a clinical point of view, it is still very important to recognize potential risk factors to identify those patients who may have a higher risk of a poorer prognosis.
Previous studies have identified FEV1 — forced expiratory volume in one second (a measure of lung function) — as a strong predictor of mortality in CF patients. But this outcome measure can be a challenge to assess in children.
To overcome this, other techniques to determine long-term outcome in children younger than 6 have been tested, including preschool breathing tests and multiple breath washout, but their predictive value has not been fully proven.
Evaluating CF signs and symptoms is current practice in the clinic, but their relevance in patients’ prognosis has never been accurately determined.
In a study titled “Risk factors for mortality before age 18 years in cystic fibrosis,” researchers combined information collected from 1994 to 2005 in the scope of the Epidemiologic Study of Cystic Fibrosis (ESCF) and integrated it with the Cystic Fibrosis Foundation Patient Registry (CFFPR) demographic and mortality data from 2013.
The team evaluated data from a total of 5,365 CF patients at preschool age who participated in the ESCF. Among those, 72% were linked to data from the CFFPR.
Researchers found that about 6% of the CF children included in the cohort died before they were 18. A detailed analysis of clinical signs and symptoms showed that those presenting crackling noises and nail clubbing between 3 and 5 years of age were associated with increased risk of early mortality. These two clinical factors are well-known signs of poor lung function, which explains the association with poorer outcome, the authors said.
As with other studies results, the researchers also found an association between early death and other risk factors, including female gender, racial or ethnic minority status, Medicaid status (as indication of lower socioeconomic groups), whether the underlying CFTR mutation was known, low weight for age, and Pseudomonas aeruginosa respiratory tract infection.
“Signs and symptoms that predict early mortality should be systematically evaluated in clinical trials of therapies in young children,” the researchers wrote. “The elimination of childhood deaths from CF should be a high priority for researchers and clinicians; identifying children who are at highest risk, and providing appropriate therapies, will be critical to achieving this goal.”
Overall, the study identified risk factors that are not commonly assessed in clinical practice, such as the presence of crackles and clubbing, that can provide valuable information on CF pediatric patients’ long-term prognosis.
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