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Intestinal chronic dysmotility

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https://www.readbyqxmd.com/read/29143474/chronic-intestinal-pseudo-obstruction-progress-in-management
#1
REVIEW
G Di Nardo, T B Karunaratne, S Frediani, R De Giorgio
Chronic intestinal pseudo-obstruction (CIPO) is a severe form of gastrointestinal dysmotility (often due to derangement of the innervation/smooth muscle/interstitial cells of Cajal) with recurrent episodes of intestinal subocclusion mimicking a mechanical obstruction. Because of its complexity and heterogeneity, CIPO is often misdiagnosed or remains unrecognized until advanced stages. Management is a critical aspect in CIPO patient care. So far, most prokinetic drugs have not proven efficacy in restoring intestinal propulsion, thus nutritional support, fluid/electrolyte replacement, and antibiotics are the mainstay of treatment...
December 2017: Neurogastroenterology and Motility: the Official Journal of the European Gastrointestinal Motility Society
https://www.readbyqxmd.com/read/28927774/chronic-intestinal-pseudo-obstruction-in-a-child-with-treacher-collins-syndrome
#2
E Giabicani, J Lemale, L Dainese, S Boudjemaa, A Coulomb, P Tounian, B Dubern
BACKGROUND: Treacher Collins syndrome (TCS) mainly presents with severe craniofacial developmental abnormalities characterized by a combination of bilateral downward-slanting palpebral fissures, colobomas of the lower eyelids, hypoplasia of the facial bones, cleft palate, malformation of the external ears, atresia of the external auditory canals, and bilateral conductive hearing loss. It is due to mutations in Treacher Collins syndrome 1 (TCOF1) (5q32-q33.1) and Polymerase RNA 1 polypeptides D and C (POLR1D [13q12...
September 15, 2017: Archives de Pédiatrie: Organe Officiel de la Sociéte Française de Pédiatrie
https://www.readbyqxmd.com/read/28377888/chronic-intestinal-pseudo-obstruction-did-you-search-for-lysosomal-storage-diseases
#3
J Politei, C Durand, A B Schenone, A Torres, J Mukdsi, B L Thurberg
Chronic intestinal pseudo-obstruction results in clinical manifestations that resemble intestinal obstruction but in the absence of any physical obstructive process. Fabry disease is an X-linked lysosomal storage disease characterized by the dysfunction of multiple systems, including significant gastrointestinal involvement. We report the occurrence of chronic intestinal pseudo-obstruction in two unrelated patients with Fabry disease and the possible explanation of a direct relation of these two disorders. In Fabry disease, gastrointestinal symptoms occur in approximately 70% of male patients, but the frequency ranges from 19% to 69% in different series...
June 2017: Molecular Genetics and Metabolism Reports
https://www.readbyqxmd.com/read/28327177/intestinal-t-lymphocyte-homing-is-associated-with-gastric-emptying-and-epithelial-barrier-function-in-critically-ill-a-prospective-observational-study
#4
Christian Greis, Zohal Rasuly, Rolf A Janosi, Lambros Kordelas, Dietrich W Beelen, Tobias Liebregts
BACKGROUND: Impaired gastric emptying is common in critically ill patients. Intestinal dysmotility, a major cause of feed intolerance, may foster infectious complications due to mucosal barrier disruption. However, little is known about gut-directed immune activation, intestinal barrier function and its association with impaired gastric emptying in critically ill patients at ICU admission. METHODS: We conducted a prospective observational study at two tertiary care medical ICUs...
March 22, 2017: Critical Care: the Official Journal of the Critical Care Forum
https://www.readbyqxmd.com/read/28186337/impaired-intestinal-cholecystokinin-secretion-a-fascinating-but-overlooked-link-between-coeliac-disease-and-cholesterol-gallstone-disease
#5
REVIEW
Helen H Wang, Min Liu, Xiaodan Li, Piero Portincasa, David Q-H Wang
BACKGROUND: Coeliac disease is a chronic, small intestinal, immune-mediated enteropathy caused by a permanent intolerance to dietary gluten in genetically predisposed individuals. Clinical studies have found that intestinal cholecystokinin secretion and gallbladder emptying in response to a fatty meal are impaired before coeliac patients start the gluten-free diet (GFD). DESIGN: However, it was never really appreciated whether coeliac disease is associated with gallstones because there were very few studies investigating the mechanism underlying the impact of coeliac disease on the pathogenesis of gallstones...
April 2017: European Journal of Clinical Investigation
https://www.readbyqxmd.com/read/28043906/heterotrimeric-g-stimulatory-protein-%C3%AE-subunit-is-required-for%C3%A2-intestinal-smooth-muscle-contraction-in-mice
#6
Xiaoteng Qin, Shangming Liu, Qiulun Lu, Meng Zhang, Xiuxin Jiang, Sanyuan Hu, Jingxin Li, Cheng Zhang, Jiangang Gao, Min-Sheng Zhu, Robert Feil, Huashun Li, Min Chen, Lee S Weinstein, Yun Zhang, Wencheng Zhang
BACKGROUND & AIMS: The α subunit of the heterotrimeric G stimulatory protein (Gsa), encoded by the guanine nucleotide binding protein, α-stimulating gene (Gnas, in mice), is expressed ubiquitously and mediates receptor-stimulated production of cyclic adenosine monophosphate and activation of the protein kinase A signaling pathway. We investigated the roles of Gsa in vivo in smooth muscle cells of mice. METHODS: We performed studies of mice with Cre recombinase-mediated disruption of Gnas in smooth muscle cells (Gsa(SMKO) and SM22-CreER(T2), induced in adult mice by tamoxifen)...
April 2017: Gastroenterology
https://www.readbyqxmd.com/read/28003719/cirrhotic-patients-have-worse-bowel-preparation-at-screening-colonoscopy-than-chronic-liver-disease-patients-without-cirrhosis
#7
Anika K Anam, Kunal Karia, Arun B Jesudian, Brian P Bosworth
BACKGROUND: Cirrhosis has been shown in small studies to be a predictor of suboptimal bowel preparation at screening colonoscopy. It has yet to be established whether patients with chronic liver disease in the absence of cirrhosis experience equally poor colon cleansing. Intestinal dysmotility related to cirrhosis might impair bowel preparation in this population more than those with chronic liver disease without cirrhosis. OBJECTIVE: This study compared the quality of bowel preparation in cirrhotic and non-cirrhotic patients with chronic liver disease and determined whether this influenced polyp detection rate...
December 2016: Journal of Clinical and Experimental Hepatology
https://www.readbyqxmd.com/read/27683196/chronic-intestinal-pseudo-obstruction-in-children-and-adults-diagnosis-and-therapeutic-options
#8
REVIEW
G Di Nardo, C Di Lorenzo, A Lauro, V Stanghellini, N Thapar, T B Karunaratne, U Volta, R De Giorgio
BACKGROUND: Chronic intestinal pseudo-obstruction (CIPO) represents the most severe form of gastrointestinal dysmotility with debilitating and potentially lethal consequences. Symptoms can be non-specific, and result in this condition being diagnosed incorrectly or too late with consequences for morbidity and even mortality. PURPOSE: The present article aims to provide pediatric and adult gastroenterologists with an up to date review about clinical features, diagnosis and therapeutic options for CIPO...
January 2017: Neurogastroenterology and Motility: the Official Journal of the European Gastrointestinal Motility Society
https://www.readbyqxmd.com/read/27427419/a-novel-mutation-in-nucleoporin-35-causes-murine-degenerative-colonic-smooth-muscle-myopathy
#9
Ian A Parish, Lincon A Stamp, Ayla May D Lorenzo, Suzanne M Fowler, Yovina Sontani, Lisa A Miosge, Debbie R Howard, Christopher C Goodnow, Heather M Young, John B Furness
Chronic intestinal pseudo-obstruction (CIPO) is a rare but life-threatening disease characterized by severe intestinal dysmotility. Histopathologic studies in CIPO patients have identified several different mechanisms that appear to be involved in the dysmotility, including defects in neurons, smooth muscle, or interstitial cells of Cajal. Currently there are few mouse models of the various forms of CIPO. We generated a mouse with a point mutation in the RNA recognition motif of the Nup35 gene, which encodes a component of the nuclear pore complex...
September 2016: American Journal of Pathology
https://www.readbyqxmd.com/read/27273837/chronic-intestinal-pseudo-obstruction-in-a-child-harboring-a-founder-hirschsprung-ret-mutation
#10
Valentina Rossi, Manuela Mosconi, Paolo Nozza, Daniele Murgia, Girolamo Mattioli, Isabella Ceccherini, Alessio Pini Prato
Chronic intestinal pseudo obstruction (CIPO) is a rare clinical entity characterized by symptoms and signs of intestinal obstruction without either recognizable anatomical abnormalities or intestinal aganglionosis. A Chinese female infant presented to our institution with a clinical diagnosis of CIPO. Aganglionosis was ruled out by full thickness colonic and ileal biopsies and by rectal suction biopsies. Unexpectedly, direct sequencing and PCR amplification of RET proto-oncogene from peripheral blood extracted DNA identified a RET R114H mutation...
September 2016: American Journal of Medical Genetics. Part A
https://www.readbyqxmd.com/read/27259308/inhibitory-effects-and-sympathetic-mechanisms-of-distension-in-the-distal-organs-on-small-bowel-motility-and-slow-waves-in-canine
#11
Jun Song, Jieyun Yin, Jiande D Z Chen
Rectal distension (RD) is known to induce intestinal dysmotility. Few studies were performed to compare effects of RD, colon distension (CD) and duodenal distension (DD) on small bowel motility. This study aimed to investigate effects and underlying mechanisms of distensions in these regions on intestinal motility and slow waves. Eight dogs chronically implanted with a duodenal fistula, a proximal colon fistula, and intestinal serosal electrodes were studied in six sessions: control, RD, CD, DD, RD + guanethidine, and CD + guanethidine...
December 2015: Cell Biochemistry and Biophysics
https://www.readbyqxmd.com/read/27222079/patients-with-chronic-kidney-disease-have-abnormal-upper-gastro-intestinal-tract-digestive-function-a-study-of-uremic-enteropathy
#12
Claire J Grant, Laura E Harrison, Caroline L Hoad, Luca Marciani, Penny A Gowland, Christopher W McIntyre
BACKGROUND AND AIM: Chronic kidney disease (CKD) affects gastrointestinal (GI) function and results in numerous adaptive and maladaptive responses. Disruption of the colonic microbiome and its attendant consequences-the loss of gut barrier integrity and increased generation of uremic toxins-has become well-recognized. However, less attention has been paid to characterizing the mechanisms behind dysfunction of the upper GI tract, largely owing to the difficulty of studying small bowel function in vivo...
February 2017: Journal of Gastroenterology and Hepatology
https://www.readbyqxmd.com/read/26886038/-gastrointestinal-causes-of-weight-loss-clinical-presentation-diagnostic-workup-and-therapy
#13
REVIEW
Sophie Fromhold-Treu, Georg Lamprecht
This review describes the gasterointestinal entities, their pathophysiology, clinical presentation, diagnostic workup and therapy that typically involve weight loss as the major presenting symptom. The differentiation of malassimilation into maldigestion and malabsorption is clinically mostly not helpful. Instead primary malasssimilation can be distinguished from secondary due to another disease. Celiac disease, lambliasis, small bowel CD, CVIDS and Whipple's disease result in loss of absorptive surface. Chronic intestinal pseudobstruction leads to weight loss through dysmotility and postprandial pain...
February 2016: Deutsche Medizinische Wochenschrift
https://www.readbyqxmd.com/read/26818425/short-bowel-syndrome-and-intestinal-failure-in-crohn-s-disease
#14
REVIEW
Berkeley N Limketkai, Alyssa M Parian, Neha D Shah, Jean-Frédéric Colombel
Crohn's disease is a chronic and progressive inflammatory disorder of the gastrointestinal tract. Despite the availability of powerful immunosuppressants, many patients with Crohn's disease still require one or more intestinal resections throughout the course of their disease. Multiple resections and a progressive reduction in bowel length can lead to the development of short bowel syndrome, a form of intestinal failure that compromises fluid, electrolyte, and nutrient absorption. The pathophysiology of short bowel syndrome involves a reduction in intestinal surface area, alteration in the enteric hormonal feedback, dysmotility, and related comorbidities...
May 2016: Inflammatory Bowel Diseases
https://www.readbyqxmd.com/read/26661570/comparative-quantitative-assessment-of-global-small-bowel-motility-using-magnetic-resonance-imaging-in-chronic-intestinal-pseudo-obstruction-and-healthy-controls
#15
RANDOMIZED CONTROLLED TRIAL
A Menys, S Butt, A Emmanuel, A A Plumb, A Fikree, C Knowles, D Atkinson, N Zarate, S Halligan, S A Taylor
BACKGROUND: Chronic intestinal pseudo-obstruction (CIPO) is characterized by dilatation of the bowel lumen and abnormal motility. In this study, we aimed to quantify small bowel dysmotility in CIPO using a validated pan-intestinal motility assessment technique based on motion capture magnetic resonance imaging (MRI) compared to normal controls. In addition, we explored if motility responses of CIPO patients to neostigmine challenge differed from healthy volunteers. METHODS: Twenty healthy volunteers (mean age 28, range 22-48) and 11 CIPO patients (mean age 47, range 19-90) underwent MRI enterography to capture global small bowel motility...
March 2016: Neurogastroenterology and Motility: the Official Journal of the European Gastrointestinal Motility Society
https://www.readbyqxmd.com/read/26488457/single-center-adult-chronic-intestinal-failure-cohort-analyzed-according-to-the-espen-endorsed-recommendations-definitions-and-classifications
#16
Christopher Filtenborg Brandt, Siri Tribler, Mark Hvistendahl, Michael Staun, Per Brøbech, Palle Bekker Jeppesen
BACKGROUND/AIMS: The objective of this study was to describe a clinically well-defined, single-center, intestinal failure (IF) cohort based on a template of definitions and classifications endorsed by the European Society for Clinical Nutrition and Metabolism (ESPEN). METHODS: A cross-sectional, retrospective, adult IF cohort, receiving parenteral support (PS), was extracted from the Copenhagen IF database at the tertiary IF center, Copenhagen University Hospital, Rigshospitalet, Denmark...
May 2017: JPEN. Journal of Parenteral and Enteral Nutrition
https://www.readbyqxmd.com/read/26387781/effect-of-neostigmine-on-gastroduodenal-motility-in-patients-with-suspected-gastrointestinal-motility-disorders
#17
G Parthasarathy, K Ravi, M Camilleri, C Andrews, L A Szarka, P A Low, A R Zinsmeister, A E Bharucha
BACKGROUND: Acetylcholinesterase inhibitors (ACIs), e.g., neostigmine, are known to increase upper and lower gastrointestinal (GI) motility and are used to treat acute colonic pseudoobstruction. However, their effects on gastroduodenal motility in humans are poorly understood. Our hypotheses were that, in patients with suspected GI motility disorders, neostigmine increases gastric and small intestinal motor activity, and these effects are greater in patients with cardiovagal neuropathy, reflecting denervation sensitivity...
December 2015: Neurogastroenterology and Motility: the Official Journal of the European Gastrointestinal Motility Society
https://www.readbyqxmd.com/read/26252289/gad-antibodies-as-key-link-between-chronic-intestinal-pseudoobstruction-autonomic-neuropathy-and-limb-stiffness-in-a-nondiabetic-patient-a-care-compliant-case-report-and-review-of-the-literature
#18
REVIEW
Andrea Maier, Vera Mannartz, Hermann Wasmuth, Christian Trautwein, Ulf-Peter Neumann, Joachim Weis, Joachim Grosse, Matthias Fuest, Max-J Hilz, Joerg B Schulz, Christina Haubrich
Chronic intestinal pseudoobstruction (CIP) can be a severe burden and even a life-threatening disorder. Typically, several years of uncertainty are passing before diagnosis. We are reporting the case of a young woman with a decade of severe, progressive gastrointestinal dysmotility. Unusually, she had also developed an autonomic neuropathy, and a stiff limb syndrome.In addition to achalasia and CIP the young woman also developed neuropathic symptoms: orthostatic intolerance, urinary retention, a Horner syndrome, and lower limb stiffness...
August 2015: Medicine (Baltimore)
https://www.readbyqxmd.com/read/26239907/oxalate-nephropathy-in-systemic-sclerosis-case-series-and-review-of-the-literature
#19
REVIEW
Colin B Ligon, Laura K Hummers, Zsuzsanna H McMahan
OBJECTIVE: To increase awareness of oxalate nephropathy as a cause of acute kidney injury (AKI) among systemic sclerosis patients with small intestinal dysmotility and malabsorption, and to prompt consideration of dietary modification and early treatment of predisposing causes of oxalate nephropathy in this population. METHODS: Two cases of biopsy-proven oxalate nephropathy were identified among systemic sclerosis patients in the course of direct clinical care. Subsequently, a retrospective search of the Johns Hopkins Pathology databases identified a third patient with systemic sclerosis who developed oxalate nephropathy...
December 2015: Seminars in Arthritis and Rheumatism
https://www.readbyqxmd.com/read/26194403/the-role-of-mast-cells-in-functional-gi-disorders
#20
REVIEW
Mira M Wouters, Maria Vicario, Javier Santos
Functional gastrointestinal disorders (FGIDs) are characterized by chronic complaints arising from disorganized brain-gut interactions leading to dysmotility and hypersensitivity. The two most prevalent FGIDs, affecting up to 16-26% of worldwide population, are functional dyspepsia and irritable bowel syndrome. Their etiopathogenic mechanisms remain unclear, however, recent observations reveal low-grade mucosal inflammation and immune activation, in association with impaired epithelial barrier function and aberrant neuronal sensitivity...
January 2016: Gut
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