Diagnosing constipation in patients with cystic fibrosis applying ESPGHAN criteria

Stefano, Marina Abib; Sandy, Natascha Silva; Zagoya, Carlos; Duckstein, Franziska; Ribeiro, Antonio Fernando; Mainz, Jochen G.; Lomazi, Elizete Aparecida

doi:10.1016/j.jcf.2021.08.021

Cited by 10 publications

(8 citation statements)

References 25 publications

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“…The survey by Stefano et al published in this journal [7] is a welcome addition to these studies. It showed that 43 out of 105 (41%) CF patients between 6 and 21 years of age had, according to the ESPGHAN criteria, symptoms of constipation, with relief of these problems after the initiation of laxatives, which, as pointed out above, is an essential component of these criteria.…”

Section: Journal Of Cystic Fibrosismentioning

confidence: 93%

“…The higher point prevalence in this study might be due to the age of the patients included, as opposed to the 0-18 year group in the article by van der Doef [5] , as it is known that, although constipation can be present in this younger age group, it is more rare [2] . The article by Stefano [7] also once again highlights the differences between constipation in CF and non-CF patients, with the vast majority (86%) of constipated CF patients having a normal frequency of bowel movements (i.e. daily or each other day), while pain or discomfort when passing stools is infrequent, although abdominal pain in general is frequent.…”

Section: Journal Of Cystic Fibrosismentioning

confidence: 99%

See 1 more Smart Citation

Constipation and cystic fibrosis. Slow movement

Doef

Houwen

2022

Journal of Cystic Fibrosis

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Section: Journal Of Cystic Fibrosismentioning

confidence: 93%

Section: Journal Of Cystic Fibrosismentioning

confidence: 99%

Constipation and cystic fibrosis. Slow movement

Doef

Houwen

2022

Journal of Cystic Fibrosis

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“…Basic nutritional care includes a high-calorie, high-fat diet associated with PERT, and fat-soluble vitamins supplementation. Morbidities like pulmonary infections, gastrointestinal complication (gastro-esophageal reflux, constipation, distal intestinal obstructive syndrome) can decrease appetite and determine inadequate nutrient intake [ 31 , 32 , 33 ].…”

Section: Clinical Considerations Of Exocrine Pancreatic Insufficiencymentioning

confidence: 99%

Current and Future Therapeutic Approaches of Exocrine Pancreatic Insufficiency in Children with Cystic Fibrosis in the Era of Personalized Medicine

et al. 2023

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This review presents current updates of pancreatic enzyme replacement therapy in children with cystic fibrosis based on literature published in the last decade and some special considerations regarding pancreatic enzyme replacement therapy in the era of new therapies, such as cystic fibrosis transmembrane conductance regulator modulator therapies. Few articles evaluate the efficacy of pancreatic enzyme replacement therapy in the pediatric population, and most studies also included children and adults with cystic fibrosis. Approximately 85% of cystic fibrosis patients have exocrine pancreatic insufficiency and need pancreatic enzyme replacement therapy. Fecal elastase is the most commonly used diagnostic test for exocrine pancreatic insufficiency, although this value can fluctuate over time. While it is used as a diagnostic test, it cannot be used for monitoring the effectiveness of pancreatic enzyme replacement therapy and for adjusting doses. Pancreatic enzyme replacement therapy, the actual treatment for exocrine pancreatic insufficiency, is essential in children with cystic fibrosis to prevent malabsorption and malnutrition and needs to be urgently initiated. This therapy presents many considerations for physicians, patients, and their families, including types and timing of administration, dose monitoring, and therapy failures. Based on clinical trials, pancreatic enzyme replacement therapy is considered effective and well-tolerated in children with cystic fibrosis. An important key point in cystic fibrosis treatment is the recent hypothesis that cystic fibrosis transmembrane conductance regulator modulators could improve pancreatic function, further studies being essential. Pancreatic enzyme replacement therapy is addressed a complication of the disease (exocrine pancreatic insufficiency), while modulators target the defective cystic fibrosis transmembrane conductance regulator protein. Exocrine pancreatic insufficiency in cystic fibrosis remains an active area of research in this era of cystic fibrosis transmembrane conductance regulator modulator therapies. This new therapy could represent an example of personalized medicine in cystic fibrosis patients, with each class of modulators being addressed to patients with specific genetic mutations.

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“…Impaired fluid secretion in CF newborns contributes to meconium ileus, characterized by viscid and thick meconium that obstructs the terminal ileum and leads to small bowel dilation proximal to the obstruction [ 86 , 87 ]. CF patients experience chronic constipation and intestinal obstructive episodes, which are generally managed with laxatives [ 88 ].…”

Section: Mitochondria Rich (Mr) Cells/ionocytesmentioning

confidence: 99%