High Serum Retinol and Lung Function in Young Patients With Cystic Fibrosis

Rivas-Crespo, M. Francisco; Jiménez, David González; Quirós, María D. Acuña; Aguirre, Amaia Sojo; González, S. Heredia; Martín, Juan José Díaz; Otero, Jesús M. Garagorri; Almarza, Aurora Lázaro; Bousoño-García, Carlos

doi:10.1097/mpg.0b013e31828b1d90

Cited by 11 publications

(16 citation statements)

References 24 publications

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“…Some data suggested significant positive correlations between vitamin A concentration and FEV1 in children, adolescent and young adults with CF (Aird et al, 2006;Rivas-Crespo et al, 2013). Rivas-Crespo et al established that 90% of CF patients with a serum retinol concentration up to 110 μg/dL had FEV1 ≥ 80% (Rivas-Crespo et al, 2013). However, Woestenenk et al (2015) in their longitudinal study did not find such a relationship in children and adolescents with CF (n = 228).…”

Section: Discussionmentioning

confidence: 70%

“…However, these results are very often ambiguous. Some data suggested significant positive correlations between vitamin A concentration and FEV1 in children, adolescent and young adults with CF (Aird et al, 2006;Rivas-Crespo et al, 2013). Rivas-Crespo et al established that 90% of CF patients with a serum retinol concentration up to 110 μg/dL had FEV1 ≥ 80% (Rivas-Crespo et al, 2013).…”

Section: Discussionmentioning

confidence: 99%

“…In some studies, discussed below, the researchers attempted to analyze the correlation between retinol concentration and different clinical parameters (Aird et al, 2006;Brei et al, 2013;Duggan et al, 1996;Greer et al, 2004;Rivas-Crespo et al, 2013;Woestenenk et al, 2015). However, these results are very often ambiguous.…”

Section: Discussionmentioning

confidence: 99%

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Vitamin A status and its determinants in patients with cystic fibrosis

Sapiejka¹,

Krzyżanowska

Walkowiak

et al. 2017

Acta Sci Pol Technol Aliment

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Background. Routine administration of vitamin A, recommended in CF patients, can help to prevent its deficiency. However, high vitamin A supplementation may lead to its excessive level and possible toxicity. Therefore, the aim of the present study was to assess the status of vitamin A and the determinants of its body resources in CF patients. Material and methods. In 196 CF patients aged from 4 months to 47 years, the following parameters were analysed: nutritional status (standardized body weight and height, serum albumin concentration) and clinical expression of disease (lung function − spirometry; biochemical markers of liver function − ALT, AST, GGT; respiratory tract colonization by Pseudomonas aeruginosa; diabetes; cirrhosis, non-cirrhotic liver disease; exocrine pancreatic function − fecal elastase-1 concentration; blood clotting -INR and vitamin A supplementation). Results. Median vitamin A concentration in the study group was 383.0 ng/ml (1

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Section: Discussionmentioning

confidence: 70%

Section: Discussionmentioning

confidence: 99%

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Vitamin A status and its determinants in patients with cystic fibrosis

Sapiejka¹,

Krzyżanowska

Walkowiak

et al. 2017

Acta Sci Pol Technol Aliment

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“…This relationship was illustrated in a large US cohort study where airway function was found to be independently and directly related to serum vitamin A level (4). A similar correlation was also established in children with respiratory tract infection-associated wheezing and adult patients with obstructive lung diseases such as asthma, chronic obstructive pulmonary disease (COPD), and cystic fibrosis (5)(6)(7)(8). In addition, a number of genome-wide association studies (GWAS) identified a genomic locus in the RA receptor-β (RARB) gene to be highly associated with airway function in healthy cohorts and patients with asthma or COPD (9)(10)(11)(12).…”

Section: Introductionmentioning

confidence: 79%

Retinoic acid signaling is essential for airway smooth muscle homeostasis

Chen¹,

Shao²,

Hinds³

et al. 2018

JCI Insight

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Airway smooth muscle (ASM) is a dynamic and complex tissue involved in regulation of bronchomotor tone, but the molecular events essential for the maintenance of ASM homeostasis are not well understood. Observational and genome-wide association studies in humans have linked airway function to the nutritional status of vitamin A and its bioactive metabolite retinoic acid (RA). Here, we provide evidence that ongoing RA signaling is critical for the regulation of adult ASM phenotype. By using dietary, pharmacologic, and genetic models in mice and humans, we show that (a) RA signaling is active in adult ASM in the normal lung, (b) RA-deficient ASM cells are hypertrophic, hypercontractile, profibrotic, but not hyperproliferative, (c) TGF-β signaling, known to cause ASM hypertrophy and airway fibrosis in human obstructive lung diseases, is hyperactivated in RA-deficient ASM, (d) pharmacologic and genetic inhibition of the TGF-β activity in ASM prevents the development of the aberrant phenotype induced by RA deficiency, and (e) the consequences of transient RA deficiency in ASM are long-lasting. These results indicate that RA signaling actively maintains adult ASM homeostasis, and disruption of RA signaling leads to aberrant ASM phenotypes similar to those seen in human chronic airway diseases such as asthma.

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“…An article recently published in this journal by Woestenenk et al [1] cites a study on young people with cystic fibrosis published by our research group [2].…”

mentioning

confidence: 99%