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Non-Hepatic Hyperammonaemia: an Important, Potentially Reversible Cause of Encephalopathy
Postgrad Med J 2001;77:717–722 717 Postgrad Med J: first published as 10.1136/pmj.77.913.717 on 1 November 2001. Downloaded from CASE REPORTS Non-hepatic hyperammonaemia: an important, potentially reversible cause of encephalopathy N D Hawkes, G A O Thomas, A Jurewicz, O M Williams, C E M Hillier, I N F McQueen, G Shortland Abstract Case reports The clinical syndrome of encephalopathy CASE 1 is most often encountered in the context of A 20 year old man was admitted to a local hos- decompensated liver disease and the diag- pital with two days of inappropriate behaviour, nosis is usually clear cut. Non-hepatic clumsiness, drowsiness, memory loss, slurred causes of encephalopathy are rarer and speech, and abdominal discomfort. Since the tend to present to a wide range of medical age of 2 years he had suVered recurrent rectal specialties with variable and episodic bleeding and investigation had revealed haem- symptoms. Delay can result in the devel- orrhoids. Bleeding from his rectum had contin- opment of potentially life threatening ued over the years but had been worse recently. complications, such as seizures and coma. On examination he was confused. His Early recognition is vital. A history of Glasgow coma scale score (GCS) was 15/15. similar episodes or clinical risk factors Neurological and general examination was nor- and early assessment of blood ammonia mal, with no stigmata of chronic liver disease. levels help establish the diagnosis. In Investigations showed a leucocytosis (leuco- × 9 addition to adequate supportive care, cyte count 22 10 /l) and serum bilirubin level investigation of the underlying cause of of 32 µmol/l. -
Pouchitis in Pediatric Ulcerative Colitis: a Multicenter Study on Behalf
Digestive and Liver Disease 51 (2019) 1551–1556 Contents lists available at ScienceDirect Digestive and Liver Disease jou rnal homepage: www.elsevier.com/locate/dld Alimentary Tract Pouchitis in pediatric ulcerative colitis: A multicenter study on behalf of Italian Society of Pediatric Gastroenterology, Hepatology and Nutrition a b b c Valeria Dipasquale , Girolamo Mattioli , Serena Arrigo , Matteo Bramuzzo , d e e e Caterina Strisciuglio , Simona Faraci , Erminia Francesca Romeo , Anna Chiara Contini , f g h i a,∗ Marco Ventimiglia , Giovanna Zuin , Enrico Felici , Patrizia Alvisi , Claudio Romano a Pediatric Gastroenterology and Cystic Fibrosis Unit, University of Messina, Messina, Italy b Pediatric Surgery Unit, Giannina Research Institute and Children Hospital, Genova, Italy c Pediatric Department, Gastroenterology, Digestive Endoscopy and Nutrition Unit, Institute for Maternal and Child Health, IRCCS “Burlo Garofalo”, Trieste, Italy d Department of Woman, Child and General and Specialistic Surgery, University of Campania “Luigi Vanvitelli”, Naples, Italy e Digestive Endoscopy and Surgery Unit, Bambino Gesù Children’s Hospital IRCCS, Rome, Italy f Inflammatory Bowel Disease Unit, “Villa Sofia-Cervello” Hospital, Palermo, Italy g Pediatric Department, University of Milano Bicocca, FMBBM, San Gerardo Hospital, Monza, Italy h Unit of Pediatrics and “Umberto Bosio” Center for Digestive Diseases, The Children Hospital, AON SS Antonio e Biagio e Cesare Arrigo, Alessandria, Italy i Pediatric Gastroenterology Unit, Maggiore Hospital, Bologna, Italy a r t i c l e i n f o a b s t r a c t Article history: Background: Data on the epidemiology and risk factors for pouchitis following restorative Received 27 April 2019 proctocolectomy and ileal pouch-anal anastomosis (IPAA) in pediatric patients with ulcerative colitis Accepted 27 June 2019 (UC) are scarce. -
Diagnosis and Management of Primary Biliary Cholangitis Ticle
REVIEW ArtICLE 1 see related editorial on page x Diagnosis and Management of Primary Biliary Cholangitis TICLE R Zobair M. Younossi, MD, MPH, FACG, AGAF, FAASLD1, David Bernstein, MD, FAASLD, FACG, AGAF, FACP2, Mitchell L. Shifman, MD3, Paul Kwo, MD4, W. Ray Kim, MD5, Kris V. Kowdley, MD6 and Ira M. Jacobson, MD7 Primary biliary cholangitis (PBC) is a chronic, cholestatic, autoimmune disease with a variable progressive course. PBC can cause debilitating symptoms including fatigue and pruritus and, if left untreated, is associated with a high risk of cirrhosis and related complications, liver failure, and death. Recent changes to the PBC landscape include a REVIEW A name change, updated guidelines for diagnosis and treatment as well as new treatment options that have recently become available. Practicing clinicians face many unanswered questions when managing PBC. To assist these healthcare providers in managing patients with PBC, the American College of Gastroenterology (ACG) Institute for Clinical Research & Education, in collaboration with the Chronic Liver Disease Foundation (CLDF), organized a panel of experts to evaluate and summarize the most current and relevant peer-reviewed literature regarding PBC. This, combined with the extensive experience and clinical expertise of this expert panel, led to the formation of this clinical guidance on the diagnosis and management of PBC. Am J Gastroenterol https://doi.org/10.1038/s41395-018-0390-3 INTRODUCTION addition, diagnosis and treatment guidelines are changing and a Primary biliary cholangitis (PBC) is a chronic, cholestatic, auto- number of guidelines have been updated [4, 5]. immune disease with a progressive course that may extend over Because of important changes in the PBC landscape, and a num- many decades. -
Second European Evidence-Based Consensus on the Diagnosis and Management of Ulcerative Colitis: Special Situations
CROHNS-00649; No of Pages 33 Journal of Crohn's and Colitis (2012) xx, xxx–xxx Available online at www.sciencedirect.com SPECIAL ARTICLE Second European evidence-based consensus on the diagnosis and management of ulcerative colitis: Special situations Gert Van Assche⁎,1,2, Axel Dignass⁎⁎,2, Bernd Bokemeyer1, Silvio Danese1, Paolo Gionchetti1, Gabriele Moser1, Laurent Beaugerie1, Fernando Gomollón1, Winfried Häuser1, Klaus Herrlinger1, Bas Oldenburg1, Julian Panes1, Francisco Portela1, Gerhard Rogler1, Jürgen Stein1, Herbert Tilg1, Simon Travis1, James O. Lindsay1 Received 30 August 2012; accepted 3 September 2012 KEYWORDS Ulcerative colitis; Anaemia; Pouchitis; Colorectal cancer surveillance; Psychosomatic; Extraintestinal manifestations Contents 8. Pouchitis ............................................................ 0 8.1. General ......................................................... 0 8.1.1. Symptoms ................................................... 0 ⁎ Correspondence to: G. Van Assche, Division of Gastroenterology, Department of Medicine, Mt. Sinai Hospital and University Health Network, University of Toronto and University of Leuven, 600 University Avenue, Toronto, ON, Canada M5G 1X5. ⁎⁎ Correspondence to: A. Dignass, Department of Medicine 1, Agaplesion Markus Hospital, Wilhelm-Epstein-Str. 4, D-60431 Frankfurt/Main, Germany. E-mail addresses: [email protected] (G. Van Assche), [email protected] (A. Dignass). 1 On behalf of ECCO. 2 G.V.A. and A.D. acted as convenors of the consensus and contributed equally to this paper. 1873-9946/$ - see front matter © 2012 European Crohn's and Colitis Organisation. Published by Elsevier B.V. All rights reserved. http://dx.doi.org/10.1016/j.crohns.2012.09.005 Please cite this article as: Van Assche G, et al, Second European evidence-based consensus on the diagnosis and management of ulcerative colitis: Special situations, Journal of Crohn's and Colitis (2012), http://dx.doi.org/10.1016/j.crohns.2012.09.005 2 G. -
Calprotectin Could Be a Potential Biomarker for Acute Appendicitis Peter C
Ambe et al. J Transl Med (2016) 14:107 DOI 10.1186/s12967-016-0863-3 Journal of Translational Medicine RESEARCH Open Access Calprotectin could be a potential biomarker for acute appendicitis Peter C. Ambe1*, Daniel Gödde2, Lars Bönicke1, Marios Papadakis1, Stephan Störkel2 and Hubert Zirngibl1 Abstract Background: Acute appendicitis is a common cause for a visit to the emergency department and appendectomy represents the most common emergency procedure in surgery. The rate of negative appendectomy however has remained high despite modern diagnostic apparatus. Therefore, there is need for a better preoperative screening of patients with suspected appendicitis. Calprotectin represents a predominant protein in the cytosol of neutrophil granulocytes and has been extensively investigated with regard to bowel pathologies. This study investigates the expression of calprotectin in the lumen of the vermiform appendix of patients undergoing appendectomy for sus- pected appendicitis. Methods: Appendix specimens from patients undergoing emergency appendectomy for suspected acute appendi- citis were examined. Acute appendicitis was confirmed on histopathology. The qualitative expression of calprotectin in the vermiform appendix specimens was analyzed using specific calprotectin antibodies. Results: Vermiform appendix specimens from 52 patients (22 female and 30 male) including 11 with uncomplicated and 41 with complicated appendicitis were analyzed. Strong immunostainings were achieved with calprotectin antibody in the lumen of all specimens irrespective of the extent of appendicitis. Immunostaining was negative in the uninflamed appendix. Conclusions: High calprotectin activity could be demonstrated within the lumen of vermiform appendix specimens following appendectomy for acute appendicitis. The high luminal accumulation of calprotectin-carrying cells could be interpreted as an invitation to study the expression of calprotectin in stool as a new diagnostic aid in patients with suspected appendicitis. -
Clinical and Pathological Aspects of Inflammatory Bowel Disease
Inflammatory Bowel Diseases: B.R. Bistrian; J.A. Walker-Smith (eds), Nestlé Nutrition Workshop Series Clinical & Performance Programme, Vol. 2, pp. 83–92, Nestec Ltd.; Vevey/S. Karger AG, Basel, © 1999. Clinical and Pathological Aspects of Inflammatory Bowel Disease Ph. Marteau Gastroenterology Department, European Hospital Georges Pompidou, Paris, France The term “inflammatory bowel disease” applies to bowel diseases of unknown etiology characterized by chronic and often relapsing inflammation. They include ulcerative colitis, Crohn’s disease, indeterminate colitis, pouchitis, and micro- scopic colitides. Although these diseases share a number of epidemiological, pathological, and clinical features, they differ sufficiently to be classified as dis- tinct entities. The term “indeterminate colitis” is used for colitides which do not present enough criteria to be classified as ulcerative colitis or Crohn’s disease. Ulcerative Colitis Pathology Ulcerative colitis is a mucosal disease, which always affects the rectum and often also involves a variable contiguous proximal segment of colonic mucosa [1]. The lesions are continuous, and their upper limit is sharply demarcated from the normal mucosa above. They are limited to the rectum in about 25% of the patients (proctitis); reach the sigmoid colon in another 25% (proctosigmoiditis); spread to the splenic flexure in another 25% (left-sided colitis), and affect the whole colon in about 15% (pancolitis). The small intestine is usually normal but may be occasionally involved by superficial inflammation (“backwash ileitis”) in some patients with pancolitis. Macroscopic lesions can be evaluated during endoscopic examination [2]. Active lesions consist of edema, erythema, lack of the normal vascular pattern, bleeding, exudation of mucus or pus, and ulceration (Table 1). -
Inside Liver Disease
Inside autoimmune liver disease 40 Nursing made Incredibly Easy! January/February 2019 www.NursingMadeIncrediblyEasy.com Copyright © 2019 Wolters Kluwer Health, Inc. All rights reserved. 1.5 ANCC CONTACT HOURS An overactive immune system can target any body tissue and cause damage. In AILD, the liver and bile ducts are under attack. By Richard L. Pullen, Jr., EdD, MSN, RN, CMSRN, and Patricia Francis-Johnson, DNP, RN Autoimmune liver disease (AILD)—primary biliary cholangitis (PBC), primary sclerosing cholangitis (PSC), and autoimmune hepatitis (AIH)—is comprised of three distinct pathologic processes in which a person’s immune system doesn’t recognize certain hepatic and biliary structures as belonging to the body. The im- mune system becomes overactive and targets healthy tissue, leading to inflammation, cirrho- sis, and end-stage liver disease (ESLD). Some patients may require liver transplantation as a lifesaving measure. This article presents the pathophysiology, epidemiology, risk factors, signs and symptoms, treatment strategies, and nursing care of patients with AILD. Primary biliary cholangitis PBC is a progressive inflammatory autoimmune disease that’s manifested by a destruction of the small intrahepatic bile ducts. Pathophysiology Destruction of the bile ducts leads to cholestasis, which causes an impairment in the flow and retention of bile—a substance produced by the liver and stored in the gallbladder that flows through the bile ducts and into the small intes- tine to digest lipids. Bile is comprised of bile salts, cholesterol, and bilirubin—a waste product from damaged red blood cells that accumulates in the blood because of cholestasis in PBC. Approximately 95% of patients with PBC pos- sess the antimitochondrial antibody (AMA) and a PBC-specific antinuclear antibody (ANA) that has a MEGIJA / CANSTOCK rim-like staining pattern. -
Pouchitis After Ileal Pouch-Anal Anastomosis for Ulcerative Colitis Occurs with Increased Frequency Cholangitis
234 Gut 1996; 38: 234-239 Pouchitis after ileal pouch-anal anastomosis for ulcerative colitis occurs with increased frequency in patients with associated primary sclerosing Gut: first published as 10.1136/gut.38.2.234 on 1 February 1996. Downloaded from cholangitis C Penna, R Dozois, W Tremaine, W Sandborn, N LaRusso, C Schleck, D Ilstrup Abstract of concomitant primary sclerosing cholangitis Primary sclerosing cholangitis (PSC), (PSC), a chronic cholestatic syndrome of present in 5% of patients with ulcerative unknown cause characterised by fibrosing colitis, may be associated with pouchitis obliteration of the bile ducts, seems to be a after ileal pouch-anal anastomosis. The significant risk factor for the development of cumulative frequency of pouchitis in pouchitis.7 patients with and without PSC who To further explore the association between underwent ileal pouch-anal anastomosis PSC and pouchitis, the aims ofthis study were: for ulcerative colitis was determined. A (a) to determine if PSC represents an indepen- total of 1097 patients who had an ileal dent risk factor for pouchitis; (b) to compare pouch-anal anastomosis for ulcerative clinical, endoscopic, and pathological findings colitis, 54 with associated PSC, were of pouchitis in a subset of patients without studied. Pouchitis was defined by clinical PSC; and (c) to search for correlations criteria in all patients and by clinical, between the risk of pouchitis and status of endoscopic, and histological criteria in liver disease. 83% of PSC patients and 85% of their matched controls. PSC was defined by clinical, radiological, and pathological Methods findings. One or more episodes of pouchitis occurred in 32% of patients Patients without PSC and 63% of patients with Between January 1981 and April 1993, 1097 PSC. -
Primary Biliary Cholangitis: 2018 Practice Guidance from the American Association for the Study of Liver Diseases 1 2 3 4 5 Keith D
| PRACTICE GUIDANCE HEPATOLOGY, VOL. 0, NO. 0, 2018 Primary Biliary Cholangitis: 2018 Practice Guidance from the American Association for the Study of Liver Diseases 1 2 3 4 5 Keith D. Lindor, Christopher L. Bowlus, James Boyer, Cynthia Levy, and Marlyn Mayo Intended for use by health care providers, this guid- Preamble ance identifies preferred approaches to the diagnostic This American Association for the Study of and therapeutic aspects of care for patients with PBC. Liver Diseases (AASLD) 2018 Practice Guidance As with clinical practice guidelines, it provides gen- on Primary Biliary Cholangitis (PBC) is an update eral guidance to optimize the care of the majority of of the PBC guidelines published in 2009. The 2018 patients and should not replace clinical judgment for updated guidance on PBC includes updates on etiol- a unique patient. ogy and diagnosis, role of imaging, clinical manifesta- The major changes from the last guideline to this tions, and treatment of PBC since 2009. The AASLD guidance include information about obeticholic acid 2018 PBC Guidance provides a data-supported (OCA) and the adaptation of the guidance format. approach to screening, diagnosis, and clinical man- agement of patients with PBC. It differs from more recent AASLD practice guidelines, which are sup- Etiology of Primary Biliary ported by systematic reviews and a multidisciplinary panel of experts that rates the quality (level) of the Cholangitis evidence and the strength of each recommendation Primary Biliary Cholangitis (PBC) is considered using the Grading of Recommendations Assessment, an autoimmune disease because of its hallmark sero- Development, and Evaluation system. In contrast, this logic signature, antimitochondrial antibody (AMA), (1-4) guidance was developed by consensus of an expert and specific bile duct pathology. -
Hepatic and Pancreatic Disorders Brian A
CHAPTER 22 Hepatic and Pancreatic Disorders Brian A. Hemstreet, PharmD, BCPS LEARNING OBJECTIVES KEY TERMS AND DEFINITIONS After completing this chapter, you should be able to Ascites — abnormal accumulation of fl uid in the abdominal cavity. This 1. Defi ne the following: is a common complication of cirrhosis. ● Hepatitis Cirrhosis — a chronic liver disease ● Cirrhosis that is a result of longstanding or repeated damage to the liver. Scar ● Ascites tissue replaces tissue resulting in ● Encephalopathy many complications related to loss of ● Jaundice normal liver function. Cirrhosis is often ● Esophageal varices referred to as end stage liver disease. ● Portal hypertension Hepatic encephalopathy ● Pancreatitis (HE) — dysfunction of the brain and nervous system that occurs in ● Malabsorption patients with cirrhosis. This disorder is ● Pseudocyst thought to be due to the presence of 2. Recall common causes and complications of chronic liver disease waste products in the blood stream, such as ammonia, that are normally 3. Review the role and mechanism of common drug treatments for cirrhosis detoxifi ed by the liver. 4. Review adverse effects and drug interactions for medications used in the treatment Hepatitis — hepatitis means of chronic liver disease infl ammation of the liver and may be caused by a variety of diseases, 5. Identify key patient counseling points for medications used to treat complications toxins, and drugs. Hepatitis may by PART of chronic liver disease acute or chronic and patients may 6. Describe the anatomy and normal physiology of the liver and pancreas exhibit symptoms, such as abdominal pain, jaundice, or nausea. Hepatitis 7 7. Recognize common medications used in the management of acute pancreatitis may also be severe enough to require 8. -
Mechanisms, Diagnosis and Management of Hepatic Encephalopathy Ravi Prakash and Kevin D
REVIEWS Mechanisms, diagnosis and management of hepatic encephalopathy Ravi Prakash and Kevin D. Mullen Abstract | Hepatic encephalopathy (HE) is a serious neuropsychiatric complication of both acute and chronic liver disease. Symptoms of HE can include confusion, disorientation and poor coordination. A general consensus exists that the synergistic effects of excess ammonia and inflammation cause astrocyte swelling and cerebral edema; however, the precise molecular mechanisms that lead to these morphological changes in the brain are unclear. Cerebral edema occurs to some degree in all patients with HE, regardless of its grade, and could underlie the pathogenesis of this disorder. The different grades of HE can be diagnosed by a number of investigations, including neuropsychometric tests (such as the psychometric hepatic encephalopathy score), brain imaging and clinical scales (such as the West Haven criteria). HE is best managed by excluding other possible causes of encephalopathy alongside identifying and the precipitating cause, and confirming the diagnosis by a positive response to empiric treatment. Empiric therapy for HE is largely based on the principle of reducing the production and absorption of ammonia in the gut through administration of pharmacological agents such as rifaximin and lactulose, which are approved by the FDA for the treatment of HE. Prakash, R. & Mullen, K. D. Nat. Rev. Gastroenterol. Hepatol. 7, 515–525 (2010); published online 10 August 2010; doi:10.1038/nrgastro.2010.116 Introduction Continuing Medical Education online Hepatic encephalopathy (HE) is a serious neuro This activity has been planned and implemented in accordance psychiatric complication of both acute and chronic with the Essential Areas and policies of the Accreditation Council liver disease.1 This disease encompasses a broad range for Continuing Medical Education through the joint sponsorship of Medscape, LLC and Nature Publishing Group. -
The Microbiome As a Therapy in Pouchitis and Ulcerative Colitis
Preprints (www.preprints.org) | NOT PEER-REVIEWED | Posted: 1 March 2021 doi:10.20944/preprints202103.0022.v1 The microbiome as a therapy in pouchitis and ulcerative colitis Jonathan P Segal1, Jean-Frédéric LeBlanc2, Lucia Maria de Campos Braz2,3, Ailsa L Hart2,3 1. Department of Gastroenterology, The Hillingdon Hospital, Uxbridge, United Kingdom 2. Inflammatory Bowel Disease Unit, St. Mark’s Hospital, Harrow, United Kingdom 3. Faculty of Medicine, Department of Metabolism, Digestion and Reproduction, Imperial College, London, United Kingdom Jonathan P Segal (1,2) BSc (Hons) MBChB, PhD Orcid ID : 0000-0002-9668-0316 Jean-Frédéric LeBlanc MD, FRCPC Orcid ID : 0000-0002-8477-6337 Lucia Maria de Campos Braz Bsc 0000-0002-9572-7717 Ailsa L Hart BA(Hons), BMBCh, FRCP, PhD (1,2) Orcid ID: 0000-0002-7141-6076 Author for Correspondence: Jonathan Segal Hillingdon Hospital Pield Heath Road UB8 3NN [email protected] Main text word count: 4751 References: 68 Tables and figures: 0:2 Disclosures: none of the authors have any relevant disclosures. Guarantor of the Article: Ailsa Hart Author Contributions: JPS, JFL, LMB conducted the literature review and prepared the manuscript. AH provided supervision of the manuscript and gave critical revisions. All authors have agreed on the final version. © 2021 by the author(s). Distributed under a Creative Commons CC BY license. Preprints (www.preprints.org) | NOT PEER-REVIEWED | Posted: 1 March 2021 doi:10.20944/preprints202103.0022.v1 Key Words: Ulcerative colitis, Ileoanal pouch Inflammatory bowel disease, Microbiota Abstract: The gut microbiome is important in the homeostasis of gut health and has pivotal roles in digestion, immune regulation, and metabolic processes.