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Mutation Classes Linked to Cystic Fibrosis Outcomes

Radiography may be more sensitive for assessing groups than pulmonary function tests

WEDNESDAY, Dec. 2 (HealthDay News) -- In cystic fibrosis patients, the classification of severity of mutations that is applied to the pancreas may also help predict pulmonary outcomes, according to research published in the December issue of Radiology.

Robert H. Cleveland, M.D., of the Children's Hospital Boston, and colleagues analyzed data from 93 patients with cystic fibrosis. Patients were grouped by CFTR mutation classes, which have been applied to pancreatic sufficiency. Patients were put into a severe-disease group (further divided into three subgroups) or a mild-disease group.

Based on chest radiographs and forced vital capacity, the researchers found that the severe group declined faster than the mild group. In the severe group, subgroup A (with two class I alleles) deteriorated faster than subgroup B (with class I and II or III) or subgroup C (class II and II or III) based on radiographs. Subgroup B progressed faster than C based on radiographs. Pulmonary function tests didn't differentiate between subgroups aside from a borderline difference between subgroups A and C on forced expiratory volume in one second.

"In conclusion, this inexpensive and noninvasive tool depicted decline in pulmonary health that may be more sensitive than conventional pulmonary function test measures, and study results confirmed that for mutations encountered in the current patient cohort, the severity classification of cystic fibrosis mutations typically applied to pancreatic phenotype may also apply to the pulmonary phenotype," the authors write.

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