scholarly journals Bicarbonate Transport in Cystic Fibrosis and Pancreatitis

Cells ◽  
2021 ◽  
Vol 11 (1) ◽  
pp. 54
Author(s):  
Dora Angyal ◽  
Marcel J. C. Bijvelds ◽  
Marco J. Bruno ◽  
Maikel P. Peppelenbosch ◽  
Hugo R. de Jonge
Keyword(s):  
Cystic Fibrosis ◽  
Current Knowledge ◽  
Pancreatic Insufficiency ◽  
Anion Channel ◽  
Exocrine Pancreatic Insufficiency ◽  
Scaffolding Protein ◽  
Bicarbonate Transport ◽  
Human Pancreas ◽  
Ductal Cells

CFTR, the cystic fibrosis (CF) gene-encoded epithelial anion channel, has a prominent role in driving chloride, bicarbonate and fluid secretion in the ductal cells of the exocrine pancreas. Whereas severe mutations in CFTR cause fibrosis of the pancreas in utero, CFTR mutants with residual function, or CFTR variants with a normal chloride but defective bicarbonate permeability (CFTRBD), are associated with an enhanced risk of pancreatitis. Recent studies indicate that CFTR function is not only compromised in genetic but also in selected patients with an acquired form of pancreatitis induced by alcohol, bile salts or smoking. In this review, we summarize recent insights into the mechanism and regulation of CFTR-mediated and modulated bicarbonate secretion in the pancreatic duct, including the role of the osmotic stress/chloride sensor WNK1 and the scaffolding protein IRBIT, and current knowledge about the role of CFTR in genetic and acquired forms of pancreatitis. Furthermore, we discuss the perspectives for CFTR modulator therapy in the treatment of exocrine pancreatic insufficiency and pancreatitis and introduce pancreatic organoids as a promising model system to study CFTR function in the human pancreas, its role in the pathology of pancreatitis and its sensitivity to CFTR modulators on a personalized basis.

scholarly journals Role of Fibre in Nutritional Management of Pancreatic Diseases

Nutrients ◽  
2019 ◽  
Vol 11 (9) ◽  
pp. 2219 ◽  
Author(s):  
Emanuela Ribichini ◽  
Serena Stigliano ◽  
Sara Rossi ◽  
Piera Zaccari ◽  
Maria Carlotta Sacchi ◽  
...  
Keyword(s):  
Current Knowledge ◽  
Multiorgan Failure ◽  
Pancreatic Insufficiency ◽  
Pancreatic Enzyme ◽  
Intestinal Barrier ◽  
Fibre Surface ◽  
Exocrine Pancreatic Insufficiency ◽  
Pancreatic Enzymes ◽  
Nutritional Management

The role of fibre intake in the management of patients with pancreatic disease is still controversial. In acute pancreatitis, a prebiotic enriched diet is associated with low rates of pancreatic necrosis infection, hospital stay, systemic inflammatory response syndrome and multiorgan failure. This protective effect seems to be connected with the ability of fibre to stabilise the disturbed intestinal barrier homeostasis and to reduce the infection rate. On the other hand, in patients with exocrine pancreatic insufficiency, a high content fibre diet is associated with an increased wet fecal weight and fecal fat excretion because of the fibre inhibition of pancreatic enzymes. The mechanism by which dietary fibre reduces the pancreatic enzyme activity is still not clear. It seems likely that pancreatic enzymes are absorbed on the fibre surface or entrapped in pectin, a gel-like substance, and are likely inactivated by anti-nutrient compounds present in some foods. The aim of the present review is to highlight the current knowledge on the role of fibre in the nutritional management of patients with pancreatic disorders.

scholarly journals 2 The syndrome of exocrine pancreatic insufficiency – cystic fibrosis or other disease?

2012 ◽  
Vol 11 ◽  
pp. S55
Author(s):  
A. Sobczynska-Tomaszewska ◽  
K. Czerska ◽  
A. Szpecht-Potocka ◽  
D. Popielarz ◽  
K. Wertheim-Tysarowska ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Pancreatic Insufficiency ◽  
Exocrine Pancreatic Insufficiency

scholarly journals The Multifaceted Roles of MicroRNAs in Cystic Fibrosis

Diagnostics ◽  
2020 ◽  
Vol 10 (12) ◽  
pp. 1102
Author(s):  
Fatima Domenica Elisa De Palma ◽  
Valeria Raia ◽  
Guido Kroemer ◽  
Maria Chiara Maiuri
Keyword(s):  
Cystic Fibrosis ◽  
Respiratory Infections ◽  
Current Knowledge ◽  
Pancreatic Insufficiency ◽  
Clinical Manifestations ◽  
Predictive Biomarkers ◽  
Loss Of Function ◽  
Gene Encoding ◽  
Multisystemic Disease

Cystic fibrosis (CF) is a lifelong disorder affecting 1 in 3500 live births worldwide. It is a monogenetic autosomal recessive disease caused by loss-of-function mutations in the gene encoding the chloride channel cystic fibrosis transmembrane conductance regulator (CFTR), the impairment of which leads to ionic disequilibria in exocrine organs. This translates into a chronic multisystemic disease characterized by airway obstruction, respiratory infections, and pancreatic insufficiency as well as hepatobiliary and gastrointestinal dysfunction. Molecular characterization of the mutational heterogeneity of CFTR (affected by more than 2000 variants) improved the understanding and management of CF. However, these CFTR variants are linked to different clinical manifestations and phenotypes, and they affect response to treatments. Expanding evidence suggests that multisystemic disease affects CF pathology via impairing either CFTR or proteins regulated by CFTR. Thus, altering the expression of miRNAs in vivo could constitute an appealing strategy for developing new CF therapies. In this review, we will first describe the pathophysiology and clinical management of CF. Then, we will summarize the current knowledge on altered miRNAs in CF patients, with a focus on the miRNAs involved in the deregulation of CFTR and in the modulation of inflammation. We will highlight recent findings on the potential utility of measuring circulating miRNAs in CF as diagnostic, prognostic, and predictive biomarkers. Finally, we will provide an overview on potential miRNA-based therapeutic approaches.

scholarly journals Efficacy and Safety of a New Formulation of Pancrelipase (Ultrase MT20) in the Treatment of Malabsorption in Exocrine Pancreatic Insufficiency in Cystic Fibrosis

2010 ◽  
Vol 2010 ◽  
pp. 1-7 ◽  
Author(s):  
Michael W. Konstan ◽  
Theodore G. Liou ◽  
Steven D. Strausbaugh ◽  
Richard Ahrens ◽  
Jamshed F. Kanga ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Pancreatic Insufficiency ◽  
Pancreatic Enzyme ◽  
Gastrointestinal Symptoms ◽  
Signs And Symptoms ◽  
Exocrine Pancreatic Insufficiency ◽  
Efficacy And Safety ◽  
Protein Nitrogen ◽  
Enzyme Replacement ◽  
New Formulation

Background. Pancreatic enzyme replacement therapy is the standard of care for treatment of malabsorption in patients with cystic fibrosis (CF) and exocrine pancreatic insufficiency (PI).Aim. To evaluate efficacy and safety of a new formulation of pancrelipase (Ultrase MT20) in patients with CF and PI. Coefficients of fat absorption (CFA%) and nitrogen absorption (CNA%) were the main efficacy parameters. Safety was evaluated by monitoring laboratory analyses, adverse events (AEs), and overall signs and symptoms.Methods. Patients (n=31) were randomized in a crossover design comparing this pancrelipase with placebo during 2 inpatient evaluation periods (6-7 days each). Fat and protein/nitrogen ingestion and excretion were measured from food diaries and 72-hour stool collections. CFA% and CNA% were calculated for each period and compared.Results. Twenty-four patients provided analyzable data. This pancrelipase increased mean CFA% and CNA% (+34.7% and +25.7%, resp.,P<.0001for both), reduced stool frequency, and improved stool consistency compared with placebo. Placebo-treated patients reported more AEs, with gastrointestinal symptoms being the most frequently reported AE.Conclusions. This pancrelipase is a safe and effective treatment for malabsorption associated with exocrine PI in patients with CF.

Detection and follow up of exocrine pancreatic insufficiency in cystic fibrosis: a review

2000 ◽  
Vol 159 (8) ◽  
pp. 563-568 ◽  
Author(s):  
Jasmine Leus ◽  
St&#x000E9;phanie Van Biervliet ◽  
Eddy Robberecht
Keyword(s):  
Cystic Fibrosis ◽  
Pancreatic Insufficiency ◽  
Exocrine Pancreatic Insufficiency

scholarly journals Evidence-based pancreatology 2018 (review of research results on diseases and exocrine pancreatic insufficiency)

2019 ◽  
Vol 43 (2) ◽  
pp. 4-14
Author(s):  
N. B. Gubergrits ◽  
N. V. Byelyayeva ◽  
A. Ye. Klochkov ◽  
G. M. Lukashevich ◽  
P. G. Fomenko ◽  
...  
Keyword(s):  
Enzyme Replacement Therapy ◽  
Replacement Therapy ◽  
Pancreatic Insufficiency ◽  
Bacterial Overgrowth ◽  
Exocrine Pancreatic Insufficiency ◽  
Pancreatic Tumors ◽  
Research Results ◽  
Enzyme Replacement ◽  
Pancreatic Pathology

The article presents a detailed review of the research results in the field of pancreatology published in 2018. Certain parts of the review are devoted to the pathogenesis, diagnostics, course of pancreatitis, its treatment, as well as autoimmune, hereditary pancreatitis, pancreatic pathology in children, as well as treatment. In studying the pathogenesis of pancreatitis, attention is paid to genetic markers of pancreatitis along with a role of bacterial overgrowth syndrome in the small intestine, both in terms of worsening of the course of pancreatitis and the lack of effectiveness of enzyme replacement therapy. The study of the role of alcohol abuse and smoking in the pathogenesis of pancreatic pathology is still in progress. Diagnostics is going on. Endosonography remains the most informative method. A number of studies have been devoted to the studying of exocrine and endocrine pancreatic insufficiency both upon pancreatitis, pancreatic tumors, and in functional dyspepsia and HIV infection. Autoimmune pancreatitis is increasingly being diagnosed, a number of studies are devoted to its diagnostics and treatment. Pancreatic diseases in children develop mainly on the background of genetic predisposition, while functional pancreatic insufficiency occurs in adult patients. The study of the peculiarities of the effect of enzyme replacement therapy continues. The immediate and remote results of the surgical treatment of pancreatic pathology are assessed.

scholarly journals The role of nutrition and pancreatic enzyme replacement therapy in children with cystic fibrosis

2021 ◽  
Vol 4 (2) ◽  
pp. 84-93
Author(s):  
Muzal Kadim ◽  
William Cheng
Keyword(s):  
Cystic Fibrosis ◽  
Nutritional Status ◽  
Enzyme Replacement Therapy ◽  
Replacement Therapy ◽  
Pancreatic Insufficiency ◽  
Pancreatic Enzyme ◽  
Nutritional Intervention ◽  
Enzyme Replacement ◽  
Pancreatic Enzyme Replacement Therapy

Background Cystic fibrosis (CF) is an inherited genetic disorder with high mortality and morbidity. CF is strongly correlated with malnutrition due to higher energy losses, pancreatic insufficiency, chronic inflammation, higher resting energy expenditure, and feeding problems. Malnutrition in CF patients associated with worse survival. Thus, appropriate and prompt nutritional intervention should be addressed to reduced malnutrition in CF patients. Methods The literature search was performed on 9 August 2021 in four major databases such as MEDLINE, EBSCOhost, Cochrane Reviews, and Web of Sciences to find the role of nutrition and pancreatic enzyme replacement therapy in pediatrics population with cystic fibrosis. Recent findings In recent decades, early nutritional management and pancreatic enzyme replacement therapy (PERT) have been shown to improve CF patient’s outcomes. Nutrition should be given in higher calories compared to healthy individuals with close and regular nutritional status monitoring. High protein and fat diets are essential for CF patient’s overall survival. Adequate level of micronutrients should be ensured to avoid morbidity caused by micronutrients deficiency. Regular pancreatic insufficiency screening should be done annually in order to start PERT early.  Further research focusing on body composition, growth chart, protein intake, and PERT are needed to further improve the management of CF patient. Conclusion Nutritional intervention and PERT play an important role in prolonging CF patient survival. Both treatments should be initiated early with nutritional status close monitoring and tailored to each individual. Collaboration with parents and children is critical to warrant that CF patients followed the dietary advice.

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