ArticleThe American journal of clinical nutrition2012
Genetic modifiers of nutritional status in cystic fibrosis.
Article in The American journal of clinical nutrition, 2012. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 15 papers.
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Who cites it
15 citing papers in PubMed, 42 citations in OpenAlex.
- Impact of Genetics on Endocrine Manifestations of Cystic Fibrosis.Endocrine practice : official journal of the American College of Endocrinology and the American Association of Clinical Endocrinologists · 2026Review
- Nasal Epithelium Transcriptomics Predict Clinical Response to Elexacaftor/Tezacaftor/Ivacaftor.American journal of respiratory cell and molecular biology · 2024Article
- Genetic modifiers of body mass index in individuals with cystic fibrosis.American journal of human genetics · 2024Article
- Assessment of Cystic Fibrosis Distribution Based on Air Pollution by Geographical Information System (GIS).Tanaffos · 2022Article
- Standards of Nutritional Care for Patients with Cystic Fibrosis: A Methodological Primer and AGREE II Analysis of Guidelines.Children (Basel, Switzerland) · 2021Article
- Genetic Modifying Factors of Cystic Fibrosis Phenotype: A Challenge for Modern Medicine.Journal of clinical medicine · 2021Review
- Tensin 1 (TNS1) is a modifier gene for low body mass index (BMI) in homozygous [F508del]CFTR patients.Physiological reports · 2021Article
- Sources of Variation in Sweat Chloride Measurements in Cystic Fibrosis.American journal of respiratory and critical care medicine · 2016Article
- Prevalence of meconium ileus marks the severity of mutations of the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) gene.Genetics in medicine : official journal of the American College of Medical Genetics · 2016Article
- Nutritional advances in patients with respiratory diseases.European respiratory review : an official journal of the European Respiratory Society · 2015Review
- Diabetes-related mortality in adults with cystic fibrosis. Role of genotype and sex.American journal of respiratory and critical care medicine · 2015Article
- Cystic fibrosis genetics: from molecular understanding to clinical application.Nature reviews. Genetics · 2015Review
- Protein is an important but undervalued macronutrient in the nutritional care of patients with cystic fibrosis.Current opinion in clinical nutrition and metabolic care · 2014Review
- Disease-modifying genes and monogenic disorders: experience in cystic fibrosis.The application of clinical genetics · 2014Review
- Pubertal height velocity and associations with prepubertal and adult heights in cystic fibrosis.The Journal of pediatrics · 2013Article
Corrections and comments
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Authors and funding
5 authors at 1 institution in 1 country.
Funding
Abstract
backgroundImproved nutrition early in life is associated with better pulmonary function for patients with cystic fibrosis (CF). However, nutritional status is poorly correlated with the CFTR genotype.
objectiveWe investigated the extent to which modifier genes influence nutrition in children with CF.
designBMI data were longitudinally collected from the CF Twin-Sibling Study and Cystic Fibrosis Foundation Patient Registry for twins and siblings from 2000 to 2010. A nutritional phenotype was derived for 1124 subjects by calculating the average BMI z score from 5-10 y of age (BMI-z(5to10)). The genetic contribution to the variation in BMI-z(5to10) (ie, heritability) was estimated by comparing the similarity of the phenotype in monozygous twins to that in dizygous twins and siblings. Linkage analysis identified potential modifier-gene loci.
resultsThe median BMI-z(5to10) was -0.07 (range: -3.89 to 2.30), which corresponded to the 47th CDC percentile. BMI-z(5to10) was negatively correlated with pancreatic insufficiency, history of meconium ileus, and female sex but positively correlated with later birth cohorts and lung function. Monozygous twins showed greater concordance for BMI-z(5to10) than did dizygous twins and siblings; heritability estimates from same-sex twin-only analyses ranged from 0.54 to 0.82. For 1010 subjects with pancreatic insufficiency, genome-wide significant linkage was identified on chromosomes 1p36.1 [log of odds (LOD): 5.3] and 5q14 (LOD: 5.1). These loci explained ≥16% and ≥15%, respectively, of the BMI variance.
conclusionsThe analysis of twins and siblings with CF indicates a prominent role for genes other than CFTR to BMI variation. Specifically, regions on chromosomes 1 and 5 appear to harbor genetic modifiers of substantial effect.
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