scholarly journals Increased Fecal Calprotectin Is Associated with Worse Gastrointestinal Symptoms and Quality of Life Scores in Children with Cystic Fibrosis

2020 ◽  
Vol 9 (12) ◽  
pp. 4080
Author(s):  
Fabien Beaufils ◽  
Emmanuel Mas ◽  
Marie Mittaine ◽  
Martin Addra ◽  
Michael Fayon ◽  
...  

In cystic fibrosis (CF), cystic fibrosis transmembrane regulator (CFTR) dysfunction leads to digestive disorders that promote intestinal inflammation and dysbiosis enhancing gastrointestinal symptoms. In pancreatic insufficiency CF patients, both intestinal inflammation and dysbiosis, are associated with an increase in the fecal calprotectin (FC) level. However, associations between the FC level, gastrointestinal symptoms, and quality of life (QoL) remain poorly studied. We aimed to assess such associations in pancreatic insufficiency CF children. The FC level was measured in pancreatic insufficiency CF children’s stool samples. Children and their parents completed two questionnaires: The Gastrointestinal Symptoms Scales 3.0-PedsQLTM and the Quality of Life Pediatric Inventory 4.0-PedsQLTM. Lower scores indicated worse symptomatology or QoL. Thirty-seven CF children were included. A FC level above 250 µg/g was associated with worse gastrointestinal symptoms and QoL scores. The FC level was inversely correlated with several gastrointestinal scores assessed by children (i.e., Total, “Heart Burn Reflux”, “Nausea and Vomiting”, and “Gas and Bloating”). Several QoL scores were correlated with gastrointestinal scores. The FC level was weakly associated with clinical parameters. Some gastrointestinal and QoL scores were related to disease severity associated parameters. In CF, the FC level, biomarker previously related to intestinal inflammation and dysbiosis, was associated with worse digestive symptoms and QoL scores.

Author(s):  
Bu’Hussain Hayee ◽  
Kerry-Lee Watson ◽  
Emma Howard ◽  
Penelope Hutchings ◽  
Sanchika Campbell ◽  
...  

Author(s):  
Nicola Shaw ◽  
Sarah Collins ◽  
Thomas Smith ◽  
Anna McCulloch ◽  
Ian Ketchell ◽  
...  

Cystic fibrosis is a life-limiting, inherited, multi-organ disease which affects many systems of the body. Until recently, treatments were only able to ameliorate symptoms, but the introduction of precision medications which modulate the underlying defect in the cystic fibrosis transmembrane conductance regulator (CFTR) gene has changed this. Notably improvements in nutrition and lung function, reduced use of antibiotics and reduced occupation rates for hospital beds have been seen. This article summarises the discussion of a group of healthcare professionals from different specialties and an expert patient, representing their personal views and experience of treating patients who are using CFTR modulators. The discussion was sponsored by an unrestricted grant from Chiesi Limited (Manchester, UK).


2021 ◽  
Vol 11 (1) ◽  
Author(s):  
L. Quénéhervé ◽  
D. Drui ◽  
J. Blin ◽  
M. Péré ◽  
E. Coron ◽  
...  

AbstractGastrointestinal symptoms are frequent in acute adrenal insufficiency. Although digestive symptoms can significantly reduce quality of life, they are rarely described in patients with treated chronic adrenal insufficiency (CAI). We aimed to characterize digestive symptoms in CAI patients. We used the section pertaining functional bowel disorders of the Rome IV questionnaire. A questionnaire was published on the website of the non-profit patient association “Adrenals” (NPPA of CAI patients) for five months. Information on demographics, characteristics of adrenal insufficiency, digestive symptoms and quality of life was collected. The relatives of CAI patients served as a control group. We analyzed responses of 33 control subjects and 119 patients (68 primary adrenal insufficiency (PAI), 30 secondary adrenal insufficiency (SAI) and 21 congenital adrenal hyperplasia (CAH)). Abdominal pain at least once a week over the past 3 months was reported by 40%, 47% and 33% of patients with PAI, SAI and CAH respectively versus 15% for the controls (p = 0.01). Symptoms were consistent with the Rome IV criteria for irritable bowel syndrome in 27%, 33% and 33% of patients respectively versus 6% for the controls (p < 0.0001). Quality of life was described as poor or very poor in 35%, 57% and 24% of patients respectively versus 5% for the controls (p < 0.0001). In conclusion, digestive symptoms are frequent and incapacitating in CAI patients and similar to symptoms of irritable bowel syndrome in 30% of CAI patients. Assessment and management of digestive symptoms should be considered a priority for physicians treating patients with CAI.


2021 ◽  
pp. 136749352110129
Author(s):  
Alice S Schamong ◽  
Hannah Liebermann-Jordanidis ◽  
Konrad Brockmeier ◽  
Elisabeth Sticker ◽  
Elke Kalbe

Congenital heart disease (CHD) is a major global health problem. Until recently, the siblings of this group did not receive much attention. This review, conducted from November 2019 to October 2020, aims to summarize knowledge about psychosocial well-being and quality of life (QoL), associated factors, and interventions for siblings of children with CHD. Systematic searches were conducted in PubMed, PsycINFO, PsycARTICLES, Web of Science via EBSCOhost, and CENTRAL. Twelve articles were included. Results showed that psychosocial well-being was impaired in 14% to 40% of siblings. Negative impact of illness was highest for CHD siblings compared to siblings of children with cancer, cystic fibrosis, or diabetes. QoL was impaired in up to one-third. Siblings of children with CHD and cancer rated their QoL lower than those of siblings of children with cystic fibrosis or type-1 diabetes. Associated factors were sibling age, gender, socioeconomic status, miscarriage, previous sibling death, visibility of illness, and severity of condition. Only one of two interventions focused on siblings of CHD children. Although data are scarce and inhomogeneous, it indicates that siblings of CHD children suffer from lower psychosocial well-being and QoL than siblings of children with other chronic conditions. Interventions to improve their situation should be developed.


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