Cystic Fibrosis (CF) is a complex disorder that requires multidisciplinary expertise for effective management. The GALAXY study estimated the prevalence of constipation to be about 25% among People with Cystic Fibrosis (PwCF), identifying it as one of the common gastrointestinal (GI) symptoms within this patient population. Quality of Life (QoL) assessments uncovered high patient dissatisfaction, highlighting the imperative need for enhanced treatment strategies. Similarly, Distal Intestinal Obstruction Syndrome (DIOS) is a unique condition exclusive to PwCF that, if left undiagnosed, can lead to considerable morbidity and mortality. Given the broad spectrum of differential diagnoses for abdominal pain, including constipation and DIOS, it is paramount for healthcare providers to possess a clear understanding of these conditions. This paper aims to delineate various differentials for abdominal pain while elucidating the pathogenesis, diagnostic criteria, and treatment options for managing constipation and DIOS in PwCF.
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http://dx.doi.org/10.1002/ppul.27104 | DOI Listing |
Pediatr Pulmonol
December 2024
Department of Pharmacy, University of Utah Health, Salt Lake City, Utah, USA.
Introduction: Cystic Fibrosis (CF) is a multisystem autosomal recessive disease characterized by thick, sticky mucus which causes lung disease, pancreatic insufficiency, and many other manifestations. Constipation is a common complication in CF and few advances in treatment have been made until recent years. Linaclotide is a treatment approved for chronic idiopathic constipation and irritable bowel syndrome with constipation.
View Article and Find Full Text PDFHeadache
September 2024
Pharmacy Department, Hospital de la Santa Creu i Sant Pau, Barcelona, Spain.
Objective: To evaluate, in patients with chronic migraine (CM) in real-world conditions, the persistence, effectiveness, and tolerability of erenumab, fremanezumab, and galcanezumab anti-calcitonin gene-related peptide (anti-CGRP) monoclonal antibodies (mAbs) and the persistence and effects of switching.
Background: Anti-CGRP mAbs represent a novel therapeutic approach to the management of CM; however, real-world data on persistence, effectiveness, and tolerability, especially after switching, are scarce.
Methods: This was a retrospective observational cohort study including all patients with CM treated with erenumab, fremanezumab, and/or galcanezumab in a tertiary hospital between January 2019 and December 2022.
Pediatr Pulmonol
September 2024
Department of Pediatrics, Division of Pediatric Gastroenterology, Hepatology and Nutrition, Cardinal Glennon Children's Medical Center, Saint Louis University School of Medicine, St. Louis, Missouri, USA.
Frontline Gastroenterol
May 2024
Department of Respiratory and Sleep Medicine, Monash Children's Hospital, Melbourne, Victoria, Australia.
Objective: Constipation and distal intestinal obstruction syndrome (DIOS) are common gastrointestinal manifestations of cystic fibrosis (CF). The primary aim was to describe the characteristics of constipation and DIOS hospitalisations in a paediatric and adult CF service over a 12-year period. The secondary aims were to determine the proportion of constipation and DIOS presentations which met the European Society for Paediatric Gastroenterology, Hepatology and Nutrition (ESPGHAN) CF Working Group definitions and to describe management strategies of both conditions.
View Article and Find Full Text PDFRSC Med Chem
November 2023
Department of Pediatrics, University of California, San Francisco San Francisco CA USA
A loss of prosecretory Cl channel CFTR activity in the intestine is considered as the key cause of gastrointestinal problems in cystic fibrosis (CF): meconium ileus, distal intestinal obstruction syndrome (DIOS) and constipation. Since CFTR modulators have minimal effects on gastrointestinal symptoms, there is an unmet need for novel treatments for CF-associated gastrointestinal disorders. Meconium ileus and DIOS mainly affect the ileum (distal small intestine).
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