Update of literature from cystic fibrosis registries 2012–2015. Part 6: Epidemiology, nutrition and complications

Salvatore, Donatello; Buzzetti, Roberto; Mastella, G

doi:10.1002/ppul.23611

Cited by 20 publications

(15 citation statements)

References 42 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…The frequency of hepatic involvement observed in this study (62.3%) was greater than that described by Salvatore et al (5.7% in children) [ 28 ]. In a Brazilian study, hepatobiliary disease was diagnosed in 16.4% of the patients, and it occurred as an initial manifestation of CF in 55.6% of these cases [ 29 ].…”

Section: Discussioncontrasting

confidence: 78%

Cystic Fibrosis: Clinical Phenotypes in Children and Adolescents

Santos

Nogueira

et al. 2018

Pediatr Gastroenterol Hepatol Nutr

View full text Add to dashboard Cite

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) (p=0.01). The age of diagnosis was reduced following implementation of neonatal screening protocols for CF (6.0 months before vs. 3.0 months after, p=0.02).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.

show abstract

Section: Discussioncontrasting

confidence: 78%

Cystic Fibrosis: Clinical Phenotypes in Children and Adolescents

Santos

Nogueira

et al. 2018

Pediatr Gastroenterol Hepatol Nutr

View full text Add to dashboard Cite

show abstract

“…Cystic fibrosis (CF) represents the most common inherited disease in Caucasians and an epidemiological study performed in 2016 even predicts an increase of about 50% in the overall number of patients diagnosed for this life-threatening disease by the year 2025 in Western European countries (1,2). The pathophysiology of CF is based on defined mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene, which cause dysfunction of the CFTR chloride channel and, subsequently, an impaired epithelial chloride and bicarbonate transport resulting in viscid mucus production (3,4).…”

Section: Introductionmentioning

confidence: 99%

ILC2 Lung-Homing in Cystic Fibrosis Patients: Functional Involvement of CCR6 and Impact on Respiratory Failure

et al. 2020

View full text Add to dashboard Cite

Cystic fibrosis patients suffer from a progressive, often fatal lung disease, which is based on a complex interplay between chronic infections, locally accumulating immune cells and pulmonary tissue remodeling. Although group-2 innate lymphoid cells (ILC2s) act as crucial initiators of lung inflammation, our understanding of their involvement in the pathogenesis of cystic fibrosis remains incomplete. Here we report a marked decrease of circulating CCR6 + ILC2s in the blood of cystic fibrosis patients, which significantly correlated with high disease severity and advanced pulmonary failure, strongly implicating increased ILC2 homing from the peripheral blood to the chronically inflamed lung tissue in cystic fibrosis patients. On a functional level, the CCR6 ligand CCL20 was identified as potent promoter of lung-directed ILC2 migration upon inflammatory conditions in vitro and in vivo using a new humanized mouse model with light-sheet fluorescence microscopic visualization of lung-accumulated human ILC2s. In the lung, blood-derived human ILC2s were able to augment local eosinophil and neutrophil accumulation and induced a marked upregulation of pulmonary type-VI collagen expression. Studies in primary human lung fibroblasts additionally revealed ILC2-derived IL-4 and IL-13 as important mediators of this type-VI collagen-inducing effect. Taken together, the here acquired results suggest that pathologically increased CCL20 levels in cystic fibrosis airways induce CCR6-mediated lung homing of circulating human ILC2s. Subsequent ILC2 activation then triggers local production of type-VI collagen and might thereby drive extracellular matrix remodeling potentially influencing pulmonary tissue destruction in cystic fibrosis patients. Thus, modulating the lung homing capacity of circulating ILC2s and their local effector functions opens new therapeutic avenues for cystic fibrosis treatment.

show abstract

“…Based on registry data from Can-ada, the United States, and the United Kingdom, the estimated median age of survival is 40 years; the projected trend shows that those born in 2000 will have a median age of Ն50 y. 2 With aging, the development of secondary musculoskeletal complications, for example, skeletal muscle dysfunction, has become an important concern in this population. Many factors, such as loss of muscle mass, poor nutritional status, systemic inflammation, corticosteroid use, and low physical activity levels, may contribute to the development of muscle dysfunction in adults with cystic fibrosis.…”

Section: Introductionmentioning

confidence: 99%

Functional Tests of Leg Muscle Strength and Power in Adults With Cystic Fibrosis

et al. 2018

View full text Add to dashboard Cite

BACKGROUND: Muscle weakness is an important systemic consequence in adults with cystic fibrosis, but it can be challenging to evaluate clinically. This study examined the validity of lowerextremity functional tests to assess quadriceps muscle strength and muscle power. METHODS: The subjects underwent 4 functional tests: 30-s sit-to-stand test, stair-climb power test, vertical jump height, and triple hop distance. Quadriceps muscle strength and power were tested by using a dynamometer (the accepted standard). Quadriceps strength was measured from 5 maximum voluntary isometric contractions to obtain peak torque. Quadriceps power was evaluated from the peak power and peak velocity attained during isotonic contractions of the quadriceps at a preset load of 20% of the peak torque. Pearson correlations were used to determine associations between functional tests and accepted measures of quadriceps strength and power. RESULTS: Fifteen adults with cystic fibrosis (9 males; mean ؎ SD age, 32 ؎ 13 y; mean ؎ SD FEV 1 % predicted, 73 ؎ 19) completed the study. The stair-climb power test had the strongest correlations with peak torque (r ‫؍‬ 0.84, P < .001) and power (r ‫؍‬ 0.65, P ‫؍‬ .009). Vertical jump height was moderately correlated with quadriceps strength (r ‫؍‬ 0.62, P ‫؍‬ .014) and quadriceps peak power (r ‫؍‬ 0.51, P ‫؍‬ .048). Similarly, triple hop distance had moderate correlations with quadriceps strength (r ‫؍‬ 0.78, P ‫؍‬ .001) and peak power (r ‫؍‬ 0.57, P ‫؍‬ .026). The sit-to-stand test was only associated with quadriceps strength (r ‫؍‬ 0.55, P ‫؍‬ .034). CONCLUSIONS: Functional tests can be applied clinically to measure leg muscle strength and power, with the stair-climb power test having the strongest associations with the standard measures. The utility of using functional tests to evaluate longitudinal changes in muscle function and its association with clinical outcomes should be examined in cystic fibrosis.

show abstract

Update of literature from cystic fibrosis registries 2012–2015. Part 6: Epidemiology, nutrition and complications

Cited by 20 publications

References 42 publications

Cystic Fibrosis: Clinical Phenotypes in Children and Adolescents

Cystic Fibrosis: Clinical Phenotypes in Children and Adolescents

ILC2 Lung-Homing in Cystic Fibrosis Patients: Functional Involvement of CCR6 and Impact on Respiratory Failure

Functional Tests of Leg Muscle Strength and Power in Adults With Cystic Fibrosis

Contact Info

Product

Resources

About