ArticleArchives of disease in childhood2007
Pancreatic phenotype in infants with cystic fibrosis identified by mutation screening.
Article in Archives of disease in childhood, 2007. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 11 papers, 1 of them a synthesis that pooled it.
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The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
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Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
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Who cites it
11 citing papers in PubMed, 1 synthesis or guideline pooled it, 33 citations in OpenAlex.
- Guidelines for diagnosis of cystic fibrosis in newborns through older adults: Cystic Fibrosis Foundation consensus report.The Journal of pediatrics · 2008Guideline
- Utility of Fecal Elastase-1 in Estimating Exocrine Pancreatic Function in Cystic Fibrosis: A Scoping Review.Pediatric pulmonology · 2026Article
- Choline in cystic fibrosis: relations to pancreas insufficiency, enterohepatic cycle, PEMT and intestinal microbiota.European journal of nutrition · 2021Review
- Assessing the impact of cystic fibrosis on the antipyretic response of ibuprofen in children: Physiologically-based modeling as a candle in the dark.British journal of clinical pharmacology · 2020Article
- Growth failure and treatment in cystic fibrosis.Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society · 2019Review
- Cystic fibrosis: a clinical view.Cellular and molecular life sciences : CMLS · 2017Review
- Pathophysiology of cystic fibrosis and drugs used in associated digestive tract diseases.World journal of gastroenterology · 2013Review
- The cystic fibrosis intestine.Cold Spring Harbor perspectives in medicine · 2013Review
- Nutritional care in children with cystic fibrosis: are our patients becoming better?European journal of clinical nutrition · 2013Review
- Consensus on the use and interpretation of cystic fibrosis mutation analysis in clinical practice.Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society · 2008Review
- What and when to collect from infants with cystic fibrosis.Archives of disease in childhood · 2007Article
Corrections and comments
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Authors and funding
9 authors at 2 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
objectiveTo determine the pancreatic phenotype of infants with cystic fibrosis (CF) diagnosed in the first week of life by a combined immunoreactive trypsin/mutation screening program.
designA prospective evaluation of pancreatic function in infants with CF at the time of neonatal diagnosis and up to the age of 12.
settingTwo different centres (Verona, Italy and Westmead, Australia) to enable comparison of results between two regions where <60% or > or =90% of patients, respectively, have at least one single DeltaF508 a mutation. PATIENTS: 315 children with CF including 149 at Verona and 166 at Westmead.
interventionsFat balance studies over 3-5 days and pancreatic stimulation tests with main outcome measures being faecal fat or pancreatic colipase secretion. PATIENTS with malabsorption are pancreatic insufficient (PI) or with normal absorption and pancreatic sufficient (PS).
results34 infants (23%) at Verona and 46 (28%) at Westmead were PS at diagnosis. 15% of those with two class I, II or III "severe" mutations and 26/28 (93%) of those with class IV or V mutations were PS at this early age. Of the 80 infants with PS, 20 became PI before the age of 12. All 20 had two severe mutations.
conclusionNeonatal mutational screening programs for CF are less likely to detect PS patients with non-DeltaF508 mutations. Of PS patients who are detected, those with two severe class I, II or III mutations are at particularly high risk of becoming PI during early childhood.
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Registered trials
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