Distal Intestinal Obstruction Syndrome and Constipation Following Lung Transplantation, Incidence and Nutritional Consequences

Distal intestinal obstruction syndrome (DIOS) is a debilitating condition which is unique to Cystic Fibrosis (CF). Constipation is increasingly common in CF patients as a result of the altered fluid composition of the intestine caused by the CF transmembrane conductance regulator gene defect. The incidence of both DIOS and constipation is increased in CF-patients post lung transplant1-2.
Source: The Journal of Heart and Lung Transplantation - Category: Transplant Surgery Authors: Tags: 740 Source Type: research

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A 5-year-old girl with a family history of cystic fibrosis presented with constipation, poor weight gain, and a BMI below the third percentile. Three sweat chloride tests were indeterminate, aCFTR analysis panel was negative, and whole-genome sequence analysis revealed 1 cystic fibrosis –causing mutation. What would you do next?
Source: JAMA - Journal of the American Medical Association - Category: General Medicine Source Type: research
Publication date: Available online 24 January 2019Source: The Lancet Respiratory MedicineAuthor(s): John J McNamara, Susanna A McColley, Gautham Marigowda, Fang Liu, Simon Tian, Caroline A Owen, David Stiles, Chonghua Li, David Waltz, Linda T Wang, Gregory S SawickiSummaryBackgroundThe efficacy, safety, and tolerability of lumacaftor and ivacaftor are established in patients aged 6 years and older with cystic fibrosis, homozygous for the F508del-CFTR mutation. We assessed the safety, pharmacokinetics, pharmacodynamics, and efficacy of lumacaftor and ivacaftor in children aged 2–5 years.MethodsIn this multicentre, pha...
Source: The Lancet Respiratory Medicine - Category: Respiratory Medicine Source Type: research
AbstractPlecanatide, a uroguanylin analog, activates the guanylate cyclase C receptors in the epithelial lining of the gastrointestinal tract in a pH-dependent fashion initiating (1) the conversion of intracellular guanosine triphosphate to cyclic guanosine monophosphate, which increases the activity of the cystic fibrosis transmembrane conductance regulator to increase chloride and bicarbonate secretion into the intestinal lumen and (2) a decrease in activity of the sodium-hydrogen ion exchanger. The resulting ionic shifts cause an increase in lumenal fluid to facilitate digestion. Plecanatide has been approved by the FDA...
Source: Drug Safety - Category: Drugs & Pharmacology Source Type: research
The pooled population prevalence of irritable bowel syndrome (IBS) has been estimated at 11.2%.1 Until the turn of the century, treatment options with convincing evidence of effectiveness were lacking.2 Since then, new therapeutic options have emerged, including intestinal secretagogues for the treatment of IBS with constipation (IBS-C), which accounts for approximately 40% of patients with IBS.3 Secretagogues are agents that increase luminal water content through the activation of chloride secretion by the chloride channel type-2 (lubiprostone), through activation of the guanylate cyclase receptor and the cystic fibrosis ...
Source: Gastroenterology - Category: Gastroenterology Authors: Tags: Editorial Source Type: research
Abstract Gastrointestinal dysfunction in Cystic Fibrosis (CF) is a prominent source of pain among patients with CF. Linaclotide, a Guanylate Cyclase C (GCC) receptor agonist, is an FDA-approved drug prescribed for chronic constipation, but has not been widely utilized in CF, as the cystic fibrosis transmembrane conductance regulator (CFTR) is the main mechanism of action. However, anecdotal clinical evidence suggests that linaclotide may be effective for treating some gastrointestinal symptoms in CF. The goal of this study was to determine the effectiveness and mechanism of linaclotide in treating CF gastrointesti...
Source: American Journal of Physiology. Gastrointestinal and Liver Physiology - Category: Physiology Authors: Tags: Am J Physiol Gastrointest Liver Physiol Source Type: research
Publication date: July 2018Source: The Lancet Respiratory Medicine, Volume 6, Issue 7Author(s): Margaret Rosenfeld, Claire E Wainwright, Mark Higgins, Linda T Wang, Charlotte McKee, Daniel Campbell, Simon Tian, Jennifer Schneider, Steve Cunningham, Jane C Davies, Margaret Rosenfeld, Claire E. Wainwright, Mark Higgins, Linda T. Wang, Charlotte McKee, Daniel Campbell, Simon Tian, Jennifer Schneider, Steve Cunningham, Jane C. DaviesSummaryBackgroundIvacaftor is generally safe and effective in patients aged 2 years and older who have cystic fibrosis and specific CFTR mutations. We assessed its use in children aged 12 to
Source: The Lancet Respiratory Medicine - Category: Respiratory Medicine Source Type: research
A 14-year-old girl with cystic fibrosis (CF), mild lung disease, and pancreatic insufficiency presented with crampy lower abdominal pain, exacerbated by eating and movement and associated with anorexia, weight loss, bloating, and constipation. She denied fever, vomiting, diarrhea, and menstrual and urinary symptoms. Physical examination was significant for tenderness in the bilateral lower abdominal quadrants. Laboratory results were significant for normal hepatic functions and pancreatic enzymes.
Source: The Journal of Pediatrics - Category: Pediatrics Authors: Tags: Insights and Images Source Type: research
Abstract BACKGROUND: Cystic fibrosis (CF) is the most common, life-limiting, genetically inherited disease. It affects multiple organs, particularly the respiratory system. However, gastrointestinal problems such as constipation and distal intestinal obstruction syndrome (DIOS) are also important and well-recognised complications in CF. They share similar symptoms e.g. bloating, abdominal pain, but are distinct conditions. Constipation occurs when there is gradual faecal impaction of the colon, but DIOS occurs when there is an accumulation of faeces and sticky mucus, forming a mass in the distal part of the small ...
Source: Cochrane Database of Systematic Reviews - Category: General Medicine Authors: Tags: Cochrane Database Syst Rev Source Type: research
This study is registered with ClinicalTrials.gov, number NCT02725567. Findings Children aged 12 to <24 months were enrolled between Aug 25, 2016, and Nov 1, 2017. Seven children were enrolled in part A, of whom five received 50 mg and two received 75 mg ivacaftor. All completed treatment. Of 19 children enrolled in part B, including one from part A, all received 50 mg ivacaftor and 18 completed treatment (one withdrew because of difficulty with blood draws). All children received at least one dose of ivacaftor. Pharmacokinetics indicated exposure was similar to that in children aged 2 to <6 years and adults. No child...
Source: The Lancet Respiratory Medicine - Category: Respiratory Medicine Source Type: research
Source: Gastroenterology - Category: Gastroenterology Authors: Tags: AGA Abstracts Source Type: research
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