ArticlePediatric gastroenterology, hepatology & nutrition2018
Cystic Fibrosis: Clinical Phenotypes in Children and Adolescents.
Article in Pediatric gastroenterology, hepatology & nutrition, 2018. 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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Who cites it
11 citing papers in PubMed, 1 synthesis or guideline pooled it.
- Prevalence of constipation in cystic fibrosis patients: a systematic review of observational studies.Jornal de pediatriaPooled it
- Identifying Gaps and Disparities in Screening for Cystic Fibrosis Associated Liver Disease: Insights From a CF Center Analysis.Pediatric pulmonology · 2025Article
- Mutational spectrum of CFTR in cystic fibrosis patients with gastrointestinal and hepatobiliary manifestations.Molecular biology reports · 2024Article
- The current state of gastrointestinal motility evaluation in cystic fibrosis: a comprehensive literature review.Translational gastroenterology and hepatology · 2024Review
- Understanding Cystic Fibrosis Comorbidities and Their Impact on Nutritional Management.Nutrients · 2022Review
- Intestinal Inflammation and Alterations in the Gut Microbiota in Cystic Fibrosis: A Review of the Current Evidence, Pathophysiology and Future Directions.Journal of clinical medicine · 2022Review
- Cystic fibrosis associated liver disease in children.World journal of hepatology · 2021Review
- Hyperinflammation and airway surface liquid dehydration in cystic fibrosis: purinergic system as therapeutic target.Inflammation research : official journal of the European Histamine Research Society ... [et al.] · 2021Review
- Mystery of a Family with Recurrent Male Infant Deaths- Solved by Autopsy and Molecular Tests.Indian journal of pediatrics · 2021Article
- MiRNA Expression Profile in the Airways is Altered during Pulmonary Exacerbation in Children with Cystic Fibrosis-A Preliminary Report.Journal of clinical medicine · 2020Article
- Unusual presentation of CF in an infant.Respiratory medicine case reports · 2020Article
Corrections and comments
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Authors and funding
8 authors.
Funding
No grant is acknowledged in the PubMed record.
Abstract
purposeThe objective of this study was to describe the clinical phenotypes of children and adolescents with cystic fibrosis (CF); and to assess the role of pancreatic insufficiency and neonatal screening in diagnosis.
methodsA cross-sectional study was conducted, which included 77 patients attending a reference center of CF between 2014 and 2016. Epidemiological data, anthropometric measurements, and the presence of pulmonary, pancreatic, gastrointestinal and hepatobiliary manifestations were evaluated based on clinical data and complementary examinations.
resultsOf the 77 patients, 51.9% were male, with a median age of 147 months (7.0-297.0 months), and the majority showed adequate nutritional status. The most common phenotype was pulmonary (92.2%), followed by pancreatic (87.0%), with pancreatic insufficiency in most cases. Gastrointestinal manifestation occurred in 46.8%, with constipation being the more common factor. Hepatobiliary disease occurred in 62.3% of patients. The group with pancreatic insufficiency was diagnosed earlier (5.0 months) when compared to the group with sufficiency (84.0 months) (
conclusionThe pulmonary phenotype was the most common, although extrapulmonary manifestations were frequent and clinically relevant, and should mandate early detection and treatment. Neonatal screening for CF led to earlier diagnosis in patients with pancreatic failure, and therefore, should be adopted universally.
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