Abdominal pain in adults with cystic fibrosis

Nash, Edward F.; Ohri, Chandra; Stephenson, Anne L.; Durie, Peter R.

doi:10.1097/meg.0000000000000011

Cited by 10 publications

(6 citation statements)

References 45 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…Constipation is common in pwCF and is thought to result from altered intestinal fluid composition caused by a defect in the CFTR gene 99 . Constipation is common in those with CF and can result in poor appetite, altered bowel patterns, overflow incontinence, and abdominal pain/distention 109,110 . In severe cases of constipation, DIOS can occur, especially in cases in which there is inadequate treatment of EPI with PERT, with subsequent fat malabsorption occurring as a result.…”

Section: Pathophysiology Of the Cf Gi Tractmentioning

confidence: 99%

Cystic fibrosis and fat malabsorption: Pathophysiology of the cystic fibrosis gastrointestinal tract and the impact of highly effective CFTR modulator therapy

McDonald,

Reid,

Pohl

et al. 2024

Nut in Clin Prac

View full text Add to dashboard Cite

Cystic fibrosis (CF) is a progressive, genetic, multi‐organ disease affecting the respiratory, digestive, endocrine, and reproductive systems. CF can affect any aspect of the gastrointestinal (GI) tract, including the esophagus, stomach, small intestine, colon, pancreas, liver, and gall bladder. GI pathophysiology associated with CF results from CF membrane conductance regulator (CFTR) dysfunction. The majority of people with CF (pwCF) experience exocrine pancreatic insufficiency resulting in malabsorption of nutrients and malnutrition. Additionally, other factors can cause or worsen fat malabsorption, including the potential for short gut syndrome with a history of meconium ileus, hepatobiliary diseases, and disrupted intraluminal factors, such as inadequate bile salts, abnormal pH, intestinal microbiome changes, and small intestinal bacterial overgrowth. Signs and symptoms associated with fat malabsorption, such as abdominal pain, bloating, malodorous flatus, gastroesophageal reflux, nausea, anorexia, steatorrhea, constipation, and distal intestinal obstruction syndrome, are seen in pwCF despite the use of pancreatic enzyme replacement therapy. Given the association of poor nutrition status with lung function decline and increased mortality, aggressive nutrition support is essential in CF care to optimize growth in children and to achieve and maintain a healthy body mass index in adults. The introduction of highly effective CFTR modulator therapy and other advances in CF care have profoundly changed the course of CF management. However, GI symptoms in some pwCF may persist. The use of current knowledge of the pathophysiology of the CF GI tract as well as appropriate, individualized management of GI symptoms continue to be integral components of care for pwCF.

show abstract

Section: Pathophysiology Of the Cf Gi Tractmentioning

confidence: 99%

Cystic fibrosis and fat malabsorption: Pathophysiology of the cystic fibrosis gastrointestinal tract and the impact of highly effective CFTR modulator therapy

McDonald,

Reid,

Pohl

et al. 2024

Nut in Clin Prac

View full text Add to dashboard Cite

show abstract

“…1). Eine akute Obstruktionssymptomatik wie beim distalen intestinalen Obstruktionssyndrom (DIOS) verursacht häufig abdominelle Schmerzen [18,22]. Die erschwerte Schmerzkontrolle bei CF könnte zudem durch eine generelle Senkung der Schmerzschwellen durch rezidivierende Ausschüttung proinflammatorischer Zytokine bedingt sein [32].…”

Section: Diskussionunclassified

Zystische Fibrose und Schmerzen – ein unterschätztes Problem

et al. 2022

View full text Add to dashboard Cite

Zusammenfassung Hintergrund Zystische Fibrose (CF) ist eine der häufigsten vererbbaren Stoffwechselerkrankungen in Deutschland. Sie betrifft verschiedene Organsysteme und führt häufig zu Schmerzen, die meist unterschätzt werden. Im deutschsprachigen Raum existieren bis dato keinerlei Leitlinien zu diesem relevanten Thema. Methoden Es erfolgte eine systematische Literaturrecherche in PubMed sowie der deutschsprachigen Leitlinien und Fachliteratur zum Thema CF und Schmerzen. Als relevant wurden Arbeiten gewertet, die Daten zu CF, zu Schmerzen oder deren Behandlung bei der CF enthielten. Insgesamt fanden sich 1690 Artikel, von denen 41 Arbeiten unsererseits aufgenommen wurden. Ergebnisse Die Recherche ergab, dass Schmerzen unterschiedlichster Art aufgrund der diversen Organbeteiligungen weit verbreitet sind. Zur Diagnostik und Therapie von Schmerzen bei CF existieren aber keine deutschsprachigen Leitlinien oder Konsenspapiere. In europäischen und amerikanischen Studien spiegelt sich die klinische Relevanz und Bandbreite des Themas wider, Daten zur Effektivität der Schmerztherapie enthalten diese jedoch nicht. Neben einer klassischen Schmerzmedikation mit Analgetika setzen viele Patienten auch auf alternative Heilversuche wie physikalische Therapien, Akupunktur oder Tetrahydrocannabinol (THC). Angesichts der multiplen Organdysfunktionen ist der Einsatz von Analgetika mit besonderen Risiken verbunden. Diskussion Eine Schmerztherapie ist bei Patienten mit zystischer Fibrose und Multiorganbeteiligung häufig erforderlich und muss der Progression der Erkrankung angepasst werden. Kontraindikationen und Nebenwirkungen der Analgetika sollten individuell ermittelt werden. Die Aufnahme der Behandlung von Schmerzen in deutschsprachige CF-Leitlinien ist dringend erforderlich.

show abstract

“…Pain is a common event in CF [213, 214]. Among several CDKs, roscovitine inhibits CDK5, a kinase activated by the binding of one of regulatory subunits p35 or p39, and their respective proteolytic fragments, p25 or 29.…”

Section: Roscovitine and Cystic Fibrosismentioning

confidence: 99%

Modulating Innate and Adaptive Immunity by (R)-Roscovitine: Potential Therapeutic Opportunity in Cystic Fibrosis

et al. 2016

View full text Add to dashboard Cite

(R)-Roscovitine, a pharmacological inhibitor of kinases, is currently in phase II clinical trial as a drug candidate for the treatment of cancers, Cushing's disease and rheumatoid arthritis. We here review the data that support the investigation of (R)-roscovitine as a potential therapeutic agent for the treatment of cystic fibrosis (CF). (R)-Roscovitine displays four independent properties that may favorably combine against CF: (1) it partially protects F508del-CFTR from proteolytic degradation and favors its trafficking to the plasma membrane; (2) by increasing membrane targeting of the TRPC6 ion channel, it rescues acidification in phagolysosomes of CF alveolar macrophages (which show abnormally high pH) and consequently restores their bactericidal activity; (3) its effects on neutrophils (induction of apoptosis), eosinophils (inhibition of degranulation/induction of apoptosis) and lymphocytes (modification of the Th17/Treg balance in favor of the differentiation of anti-inflammatory lymphocytes and reduced production of various interleukins, notably IL-17A) contribute to the resolution of inflammation and restoration of innate immunity, and (4) roscovitine displays analgesic properties in animal pain models. The fact that (R)-roscovitine has undergone extensive preclinical safety/pharmacology studies, and phase I and II clinical trials in cancer patients, encourages its repurposing as a CF drug candidate.

show abstract

Abdominal pain in adults with cystic fibrosis

Cited by 10 publications

References 45 publications

Cystic fibrosis and fat malabsorption: Pathophysiology of the cystic fibrosis gastrointestinal tract and the impact of highly effective CFTR modulator therapy

Cystic fibrosis and fat malabsorption: Pathophysiology of the cystic fibrosis gastrointestinal tract and the impact of highly effective CFTR modulator therapy

Zystische Fibrose und Schmerzen – ein unterschätztes Problem

Modulating Innate and Adaptive Immunity by (R)-Roscovitine: Potential Therapeutic Opportunity in Cystic Fibrosis

Contact Info

Product

Resources

About