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Bile acid diarrhea

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https://www.readbyqxmd.com/read/27858369/cerebrotendinous-xanthomatosis-presenting-with-infantile-spasms-and-intellectual-disability
#1
Austin Larson, James D Weisfeld-Adams, Tim A Benke, Penelope E Bonnen
Cerebrotendinous xanthomatosis (CTX) is an inborn error of metabolism leading to progressive multisystem disease. Symptoms often begin in the first decade of life with chronic diarrhea, cataracts, developmental delay, intellectual disability, and cerebellar or pyramidal dysfunction. Later manifestations include tendon xanthomas, polyneuropathy, and abnormal neuroimaging. Pathogenic biallelic variants in CYP27A1 leading to compromised function of sterol 27-hydroxylase result in accumulation of detectable toxic intermediates of bile acid synthesis rendering both genetic and biochemical testing effective diagnostic tools...
November 18, 2016: JIMD Reports
https://www.readbyqxmd.com/read/27856362/colonic-transit-and-bile-acid-synthesis-or-excretion-in-patients-with-irritable-bowel-syndrome-diarrhea-without-bile-acid-malabsorption
#2
Cédric Peleman, Michael Camilleri, Irene Busciglio, Duane Burton, Leslie Donato, Alan R Zinsmeister
: bile acid malabsorption (BAM). BAs are passively absorbed to a different extent along the mammalian colon, so that levels are lower in the feces than in proximal colon. Our aim was to explore associations among total, primary, and secretory BA in stool and colonic transit in patients with irritable bowel syndrome-diarrhea (IBS-D) without overt BAM. METHODS: In a cross-sectional observational study of 116 patients with IBS-D recruited from local communities in Minnesota, we measured total and individual main fecal BA excretion, fecal fat and fecal weight over 48 hrs, fasting serum levels of C4 (surrogate for BA synthesis), and overall colonic transit by scintigraphy (geometric center at 24 hrs and 48 hrs)...
November 14, 2016: Clinical Gastroenterology and Hepatology
https://www.readbyqxmd.com/read/27777930/characterization-of-aqps-in-mouse-rat-and-human-colon-and-their-selective-regulation-by-bile-acids
#3
Jonathan Yde, Stephen Keely, Qi Wu, Johan F Borg, Natalia Lajczak, Aoife O'Dwyer, Peter Dalsgaard, Robert A Fenton, Hanne B Moeller
In normal individuals, the epithelium of the colon absorbs 1.5-2 l of water a day to generate dehydrated feces. However, in the condition of bile acid malabsorption (BAM), an excess of bile acids in the colon results in diarrhea. Several studies have attempted to address the mechanisms contributing to BAM induced by various bile acids. However, none have addressed a potential dysregulation of aquaporin (AQP) water channels, which are responsible for the majority of transcellular water transport in epithelial cells, as a contributing factor to the onset of diarrhea and the pathogenesis of BAM...
2016: Frontiers in Nutrition
https://www.readbyqxmd.com/read/27777888/ursodeoxycholic-acid-in-treatment-of-non-cholestatic-liver-diseases-a-systematic-review
#4
Jillian Reardon, Trana Hussaini, Majid Alsahafi, Vladimir Marquez Azalgara, Siegfried R Erb, Nilufar Partovi, Eric M Yoshida
Aims: To systematically evaluate the literature for evidence to support the use of bile acids in non-cholestatic liver conditions. Methods: Searches were conducted on the databases of Medline (1948-March 31, 2015), Embase (1980-March 31, 2015) and the Cochrane Central Register of Controlled Trials, and on Google and Google Scholar to identify articles describing ursodeoxycholic acid (UDCA) and its derivatives for non-cholestatic hepatic indications. Combinations of the following search terms were used: ursodeoxycholic acid, ursodiol, bile acids and/or salts, non alcoholic fatty liver, non alcoholic steatohepatitis, fatty liver, alcoholic hepatitis, alcohol, liver disease, autoimmune, autoimmune hepatitis, liver transplant, liver graft, transplant rejection, graft rejection, ischemic reperfusion injury, reperfusion injury, hepatitis B, hepatitis C, viral hepatitis, chronic hepatitis, acute hepatitis, transaminases, alanine transaminase, liver enzymes, aspartate aminotransferase, gamma-glutamyl transferase, gamma-glutamyl transpeptidase, bilirubin, alkaline phosphatase...
September 28, 2016: Journal of Clinical and Translational Hepatology
https://www.readbyqxmd.com/read/27684270/the-expanding-role-of-the-bile-acid-receptor-farnesoid-x-in-the-intestine-and-its-potential-clinical-implications
#5
Laurens J Ceulemans, Emilio Canovai, Len Verbeke, Jacques Pirenne, Ricard Farré
Knowledge about the role of farnesoid X receptor (FXR) in the intestine is rapidly expanding. In pre-clinical animal models of inflammatory bowel disease and bile duct ligation, FXR activation has proven to directly target the three pillars of intestinal homeostasis: intestinal permeability, inflammation and bacterial translocation. The protective role of FXR-ligands on this homeostasis has implications for many intestinal pathologies like inflammatory bowel disease, ischemia reperfusion injury, the metabolic syndrome, colon cancer and even diarrhea...
August 10, 2016: Acta Chirurgica Belgica
https://www.readbyqxmd.com/read/27616761/a-systematic-literature-search-and-review-of-sodium-concentrations-of-body-fluids%C3%A2
#6
Elaine M Kaptein, Divya Sreeramoju, John M Kaptein, Matthew J Kaptein
BACKGROUND: Wide ranges of sodium concentrations for different body fluid losses have been noted with minimal substantiating data and variability among sources, leading to use of "cumulative fluid balance" regardless of composition in hospitalized patients. AIMS: To define the sodium concentrations of fluid losses from the body. METHOD: We performed a systematic search and literature review in adult humans using PubMed database. RESULTS: Inclusion criteria were met for 107 full-text articles...
October 2016: Clinical Nephrology
https://www.readbyqxmd.com/read/27609770/chemical-and-molecular-factors-in-irritable-bowel-syndrome-current-knowledge-challenges-and-unanswered-questions
#7
REVIEW
Michael Camilleri, Ibironke Oduyebo, Houssam Halawi
Several chemical and molecular factors in the intestine are reported to be altered and to have a potentially significant role in irritable bowel syndrome (IBS), particularly in IBS with diarrhea. These include bile acids; short-chain fatty acids; mucosal barrier proteins; mast cell products such as histamine, proteases, and tryptase; enteroendocrine cell products; and mucosal mRNAs, proteins, and microRNAs. This article reviews the current knowledge and unanswered questions in the pathobiology of the chemical and molecular factors in IBS...
November 1, 2016: American Journal of Physiology. Gastrointestinal and Liver Physiology
https://www.readbyqxmd.com/read/27570415/mechanisms-of-triglyceride-metabolism-in-patients-with-bile-acid-diarrhea
#8
Nidhi Midhu Sagar, Michael McFarlane, Chuka Nwokolo, Karna Dev Bardhan, Ramesh Pulendran Arasaradnam
Bile acids (BAs) are essential for the absorption of lipids. BA synthesis is inhibited through intestinal farnesoid X receptor (FXR) activity. BA sequestration is known to influence BA metabolism and control serum lipid concentrations. Animal data has demonstrated a regulatory role for the FXR in triglyceride metabolism. FXR inhibits hepatic lipogenesis by inhibiting the expression of sterol regulatory element binding protein 1c via small heterodimer primer activity. Conversely, FXR promotes free fatty acids oxidation by inducing the expression of peroxisome proliferator-activated receptor α...
August 14, 2016: World Journal of Gastroenterology: WJG
https://www.readbyqxmd.com/read/27512828/an-integrated-multi-omic-approach-to-assess-radiation-injury-on-the-host-microbiome-axis
#9
Maryam Goudarzi, Tytus D Mak, Jonathan P Jacobs, Bo-Hyun Moon, Steven J Strawn, Jonathan Braun, David J Brenner, Albert J Fornace, Heng-Hong Li
Medical responders to radiological and nuclear disasters currently lack sufficient high-throughput and minimally invasive biodosimetry tools to assess exposure and injury in the affected populations. For this reason, we have focused on developing robust radiation exposure biomarkers in easily accessible biofluids such as urine, serum and feces. While we have previously reported on urine and serum biomarkers, here we assessed perturbations in the fecal metabolome resulting from exposure to external X radiation in vivo...
September 2016: Radiation Research
https://www.readbyqxmd.com/read/27501174/primary-pancreatic-secretinoma-further-evidence-supporting-secretin-as-a-diarrheogenic-hormone
#10
William Y Chey, Wendy L Frankel, Sashwati Roy, Soma Datta, Chandan K Sen, Mary Dillhoff, Peter Muscarella, Konrad H Soergel, Ronald K Tompkins, Ta-Min Chang, Edward L Bradley, Edwin Christopher Ellison
OBJECTIVES: To document the existence of primary pancreatic secretinoma in patients with watery diarrhea syndrome (WDS) and achlorhydria and establish secretin as a diarrheogenic hormone. BACKGROUND: Vasoactive intestinal peptide (VIP) has been widely accepted as the main mediator of WDS. However, in 1968, Zollinger et al reported 2 female patients with pancreatic neuroendocrine tumors, WDS, and achlorhydria. During surgery on the first, a 24-year-old patient, they noticed distended duodenum filled with fluid and a dilated gallbladder containing dilute bile with high bicarbonate concentration...
August 5, 2016: Annals of Surgery
https://www.readbyqxmd.com/read/27225395/cerebrotendinous-xanthomatosis-a-metabolic-disease-with-different-neurological-signs-two-case-reports
#11
Maria Donata Di Taranto, Monica Gelzo, Carola Giacobbe, Marco Gentile, Gennaro Marotta, Silvia Savastano, Antonio Dello Russo, Giuliana Fortunato, Gaetano Corso
Cerebrotendinous xanthomatosis (CTX) is an autosomal recessive inborn error of bile acids synthesis and lipid accumulation caused by a deficiency of the mitochondrial cytochrome P450 sterol 27-hydroxylase enzyme encoded by CYP27A1. Pathogenic variants in CYP27A1 cause elevated cholestanol levels in the body, which leads to a variable clinical presentation that often includes cataracts, intellectual disability, neurological features, tendon xanthomas, and chronic diarrhea. Herein we describe the cases of two unrelated adult CTX patients...
October 2016: Metabolic Brain Disease
https://www.readbyqxmd.com/read/27158477/irritable-bowel-syndrome-new-insights-into-symptom-mechanisms-and-advances-in-treatment
#12
REVIEW
Robin Spiller
Despite being one of the most common conditions leading to gastroenterological referral, irritable bowel syndrome (IBS) is poorly understood. However, recent years have seen major advances. These include new understanding of the role of both inflammation and altered microbiota as well as the impact of dietary intolerances as illuminated by magnetic resonance imaging (MRI), which has thrown new light on IBS. This article will review new data on how excessive bile acid secretion mediates diarrhea and evidence from post infectious IBS which has shown how gut inflammation can alter gut microbiota and function...
2016: F1000Research
https://www.readbyqxmd.com/read/27060367/interplay-between-bile-acid-metabolism-and-microbiota-in-irritable-bowel-syndrome
#13
M Dior, H Delagrèverie, H Duboc, P Jouet, B Coffin, L Brot, L Humbert, G Trugnan, P Seksik, H Sokol, D Rainteau, J-M Sabate
BACKGROUND: Irritable bowel syndrome (IBS) physiopathology is multifactorial and roles for both microbiota and bile acid (BA) modifications have been proposed. We investigated role of dysbiosis, transit pattern and BA metabolism in IBS. METHODS: Clinical data, serum, and stool samples were collected in 15 healthy subjects (HS), 16 diarrhea-predominant (IBS-D) and 15 constipation-predominant IBS (IBS-C). Fecal microbiota composition was analyzed by real-time PCR...
September 2016: Neurogastroenterology and Motility: the Official Journal of the European Gastrointestinal Motility Society
https://www.readbyqxmd.com/read/27018117/dissecting-molecular-mechanisms-in-bile-acid-diarrhea
#14
COMMENT
Michael Camilleri
It was previously demonstrated that patients with bile acid (BA) diarrhea have low fasting serum levels of the ileal hormone--fibroblast growth factor-19 (FGF-19). In this paper, the authors demonstrate a positive correlation between ileal transcripts of FGF-19 and (75)SeHCAT retention, providing further evidence of FGF-19 deficiency as an explanation for BA diarrhea. Variants in KLB and FGFR4 genes (that determine the functional re-uptake of BA in the portal circulation by hepatocytes) are also demonstrated to be associated with (75)SeHCAT retention, confirming a second potential mechanism for the development of BA diarrhea...
March 2016: American Journal of Gastroenterology
https://www.readbyqxmd.com/read/26975510/bloom-and-bust-intestinal-microbiota-dynamics-in-response-to-hospital-exposures-and-clostridium-difficile-colonization-or-infection
#15
Caroline Vincent, Mark A Miller, Thaddeus J Edens, Sudeep Mehrotra, Ken Dewar, Amee R Manges
BACKGROUND: Clostridium difficile infection (CDI) is the leading infectious cause of nosocomial diarrhea. Hospitalized patients are at increased risk of developing CDI because they are exposed to C. difficile spores through contact with the hospital environment and often receive antibiotics and other medications that can disrupt the integrity of the indigenous intestinal microbiota and impair colonization resistance. Using whole metagenome shotgun sequencing, we examined the diversity and composition of the fecal microbiota in a prospective cohort study of 98 hospitalized patients...
2016: Microbiome
https://www.readbyqxmd.com/read/26939572/glucose-and-lipid-effects-of-the-ileal-apical-sodium-dependent-bile-acid-transporter-inhibitor-gsk2330672-double-blind-randomized-trials-with-type-2-diabetes-subjects-taking-metformin
#16
D J Nunez, X Yao, J Lin, A Walker, P Zuo, L Webster, S Krug-Gourley, M J Zamek-Gliszczynski, D S Gillmor, S L Johnson
AIMS: To investigate the pharmacodynamics, pharmacokinetics and safety/tolerability of blocking reuptake of bile acids using the inhibitor GSK2330672 (GSK672) in patients with type 2 diabetes (T2D). METHODS: Subjects with T2D taking metformin were enrolled in two studies in which they took metformin 850 mg twice daily for 2 weeks prior to and during the randomized treatment periods. In the first crossover study (n = 15), subjects received GSK672 45 mg, escalating to 90 mg, twice daily, or placebo for 7 days...
July 2016: Diabetes, Obesity & Metabolism
https://www.readbyqxmd.com/read/26891261/how-to-diagnose-and-treat-ibd-mimics-in-the-refractory-ibd-patient-who-does-not-have-ibd
#17
Karen A Chachu, Mark T Osterman
Inflammatory bowel disease (IBD) is a chronic inflammatory disease of the gastrointestinal tract and includes both Crohn's disease and ulcerative colitis. Patients with IBD often present with abdominal pain, diarrhea, and rectal bleeding but may also have a wide variety of other symptoms such as weight loss, fever, nausea, vomiting, and possibly obstruction. Given that the presentation of IBD is not specific, the differential diagnosis is broad and encompasses a wide spectrum of diseases, many of which can mimic and/or even coexist with IBD...
May 2016: Inflammatory Bowel Diseases
https://www.readbyqxmd.com/read/26874804/bile-acids-in-drug-induced-liver-injury-key-players-and-surrogate-markers
#18
REVIEW
Heiko S Schadt, Armin Wolf, Francois Pognan, Salah-Dine Chibout, Michael Merz, Gerd A Kullak-Ublick
Bile acid research has gained great momentum since the role of bile acids as key signaling molecules in the enterohepatic circulation was discovered. Their physiological function in regulating their own homeostasis, as well as energy and lipid metabolism make them interesting targets for the pharmaceutical industry in the context of diseases such as bile acid induced diarrhea, bile acid induced cholestasis or nonalcoholic steatohepatitis. Changes in bile acid homeostasis are also linked to various types of drug-induced liver injury (DILI)...
June 2016: Clinics and Research in Hepatology and Gastroenterology
https://www.readbyqxmd.com/read/26856750/characterizing-factors-associated-with-differences-in-fgf19-blood-levels-and-synthesis-in-patients-with-primary-bile-acid-diarrhea
#19
Ian M Johnston, Jonathan D Nolan, Sanjeev S Pattni, Richard N Appleby, Justine H Zhang, Sarah L Kennie, Gaganjit K Madhan, Sina Jameie-Oskooei, Shivani Pathmasrirengam, Jeremy Lin, Albert Hong, Peter H Dixon, Catherine Williamson, Julian R F Walters
OBJECTIVES: Chronic diarrhea caused by primary bile acid diarrhea (PBAD) is a common condition. We have previously shown PBAD is associated with low fasting serum levels of the ileal hormone, fibroblast growth factor 19 (FGF19). FGF19 is a negative regulator of hepatic bile acid synthesis and is stimulated by farnesoid X receptor agonists, which produce symptomatic improvement in PBAD. We aimed to assess possible causes for low serum FGF19 in patients with PBAD. METHODS: Patients with PBAD, defined by reduced (75)Se-labelled homocholic acid taurine (SeHCAT) retention, and idiopathic diarrhea controls had measurements of fasting lipids and fasting/post-prandial FGF19 serum profiles...
March 2016: American Journal of Gastroenterology
https://www.readbyqxmd.com/read/26832436/a-multicenter-prospective-study-to-investigate-the-diagnostic-accuracy-of-the-sehcat-test-in-measuring-bile-acid-malabsorption-research-protocol
#20
Fiona Reid, Janet Peacock, Bola Coker, Viktoria McMillan, Cornelius Lewis, Stephen Keevil, Roy Sherwood, Gill Vivian, Robert Logan, Jennifer Summers
BACKGROUND: Bile acid malabsorption (BAM) is one possible explanation for chronic diarrhea. BAM may be idiopathic, or result from ileal resection or inflammation including Crohn's disease, or may be secondary to other conditions, including cholecystectomy, peptic ulcer surgery, and chronic pancreatitis. No "gold standard" exists for clinical diagnosis of BAM, but response to treatment with a bile acid sequestrant (BAS) is often accepted as confirmation. The SeHCAT (tauroselcholic [selenium-75] acid) test uses a radiolabeled synthetic bile acid and provides a diagnostic test for BAM, but its performance against "trial of treatment" is unknown...
February 1, 2016: JMIR Research Protocols
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