کد مقاله | کد نشریه | سال انتشار | مقاله انگلیسی | نسخه تمام متن |
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6220826 | 1607442 | 2015 | 9 صفحه PDF | دانلود رایگان |
ObjectiveTo describe the clinical course of children who have intermediate sweat chloride values on initial screening for cystic fibrosis (CF).Study designWe performed a retrospective review of children with intermediate sweat chloride values (raised immunoreactive trypsinogen/1 copy of p.F508del CF mutation on newborn screening (NBS)/sweat chloride value of 30-59Â mmol/L) presenting to The Children's Hospital at Westmead over 15Â years. Patients with an intermediate sweat chloride evolving to a formal diagnosis of CF (termed “delayed CF”) were matched (2:1) with NBS positive patients with CF (termed “NBS positive CF”). Clinical outcomes were compared.ResultsFourteen of 29 (48%, 95% CI 0.3-0.66) patients with intermediate sweat chloride value evolved to a diagnosis of CF and were matched with 28 NBS positive patients with CF. Delayed CF had less pancreatic insufficiency (OR 0.06, 95% CI 0.01-0.44, PÂ =Â .006), less colonization with nonmucoid Pseudomonas aeruginosa (OR 0.04, 95% CI 0.01-0.38, PÂ =Â .005), milder obstructive lung disease (forced expiratory volume in 1Â second/forced vital capacity ratio), and overall disease severity (Shwachman scores) at 10Â years (mean difference 5.93, 95% CI 0.39-11.46, PÂ =Â .04; mean difference 4.72, 95% CI 0.9-8.53, PÂ =Â .015, respectively). Nutritional outcomes were better at 2Â years for delayed CF but did not persist to later ages.ConclusionsIn this cohort, approximately one-half of infants with intermediate sweat chloride value were later diagnosed with CF. The clinical course of delayed CF was milder in some aspects compared with NBS positive CF. These results emphasize the importance of ongoing follow-up of infants with intermediate sweat chloride values.
Journal: The Journal of Pediatrics - Volume 166, Issue 6, June 2015, Pages 1469-1474.e3