DOCSLIB.ORG

Zollinger-Ellison Syndrome and Acid Hypersecretion (Ogg 2009)

Total Page：16

File Type：pdf, Size：1020Kb

Download full-text PDF Read full-text
Zollinger-Ellison Syndrome and Acid Hypersecretion (Ogg 2009) ZollingerZollinger--EllisonEllison SyndromeSyndrome andand AcidAcid HypersecretionHypersecretion CoreCore CurriculumCurriculum ConferenceConference SeptemberSeptember 3,3, 20092009 GarrettGarrett OggOgg ZollingerZollinger--EllisonEllison SyndromeSyndrome First described by Robert Zollinger and Edwin Ellison in the Annals of Surgery, October 1955. Presented 2 cases with jejunal ulcers demonstrating marked gastric hypersecretion and hyperacidity. Refractory to surgical therapy necessitating total gastrectomy. In 1968, McGuigan and Trudeau showed elevated gastrin levels in patients with ZES. ZollingerZollinger--EllisonEllison SyndromeSyndrome ClassicClassic TriadTriad ofof ZES:ZES: SevereSevere pepticpeptic ulcerulcer diseasedisease GastricGastric acidacid hypersecretionhypersecretion NonbetaNonbeta cellcell gastringastrin producingproducing tumortumor ofof pancreaspancreas GastrinGastrin PhysiologyPhysiology GastrinGastrin PhysiologyPhysiology StimulantsStimulants ofof Gastrin:Gastrin: Luminal amino acids Elevated gastric pH GastrinGastrin PhysiologyPhysiology GastrinGastrin stimulatesstimulates fundicfundic enterochromafinenterochromafin likelike (ECL)(ECL) cellscells toto secretesecrete histamine.histamine. HistamineHistamine actsacts onon parietalparietal cellscells toto releaserelease HH+.. Acidic Gastric pH Negative Feedback to Gastrin D‐cell CGRP Enteric Nerves Somatostatin G‐cell X Gastrin CGNP=Calcitonin gene‐related peptide PathologyPathology Gastrinomas are derived from multipotential stem cells of endodermal origin. Like other neuroendocrine tumors, typically stain positive for chromogranins, neuron specific enolase, and synaptophysins. Expanded glandular compartment do to excess parietal cells PathologyPathology Most gastrinomas occur in the pancreas and duodenum in “Gastrinoma Triangle”. Duodenum (50-70%) Often multiple, < 2cm, and less malignant st nd More than 90% in 1st or 2nd portion Pancreas (25%) Solitary, >2 cm, and more malignant Lymph node adjacent to the pancreas (5%) GastrinomaGastrinoma TriangleTriangle EpidemiologyEpidemiology 0.10.1 toto 33 patientspatients perper millionmillion MeanMean ageage atat timetime ofof diagnosisdiagnosis isis 4141 yrs.yrs. 1.5:11.5:1 toto 2:12:1 -- Male:FemaleMale:Female SporadicSporadic 78%,78%, MENMEN--II 22%22% H.H. pyloripylori (+)(+) –– 1010--50%50% LocalizedLocalized diseasedisease –– 70%70% MeanMean delaydelay ofof diagnosisdiagnosis –– 5.25.2 yrs.yrs. Roy P, Venzon DJ, Shojamanesh H, et al. Medicine 2000; 79:379. Jensen et al. Lippincott Williams and Wilkins;2001:291 Berna MJ; Hoffmann KM; et al. Medicine. 2006 Nov;85(6):295-330. PresentationPresentation Data from Roy, PK, Venzon, DJ, Shojamenesh, H, et al, Medicine (Baltimore) 2000; 79:379 PresentationPresentation ClinicalClinical featuresfeatures suspicioussuspicious forfor ZESZES Postbulbar duodenal ulcer Multiple duodenal or jejunal ulcers PUD with chronic diarrhea PUD refractory to medical therapy History of PUD and nephrolithiasis Recurrent PUD in absence of H.pylori or NSAIDS Family history of PUD and hypercalcemia Feldman: Sleisenger & Fordtran's GI and Liver Disease, 8th ed. DiagnosisDiagnosis FastingFasting serumserum gastringastrin concentrationconcentration SecretinSecretin stimulationstimulation testtest GastricGastric acidacid secretionsecretion studiesstudies FastingFasting SerumSerum GastrinGastrin UpperUpper limitlimit ofof normalnormal isis 110110 pg/mlpg/ml GastrinGastrin ofof >> 10001000 inin settingsetting ofof gastricgastric pHpH ofof lessless thanthan 55 isis highlyhighly specificspecific forfor ZES.ZES. 2/32/3 havehave gastringastrin levelslevels 150150--10001000 pg/mlpg/ml FalseFalse positivepositive withwith PPIPPI’’ss -- mustmust bebe offoff moremore thanthan oneone week.week. ChronicChronic atrophicatrophic gastritisgastritis oror severesevere H.H. pyloripylori cancan givegive falsefalse positivepositive SecretinSecretin StimulationStimulation TestTest UsefulUseful forfor confirmationconfirmation ofof ZESZES inin patientspatients withwith indeterminateindeterminate gastringastrin levelslevels SecretinSecretin stimulatesstimulates gastringastrin releaserelease fromfrom gastrinomasgastrinomas SecretinSecretin inhibitsinhibits normalnormal GG--cellscells SecretinSecretin StimulationStimulation TestTest SecretinSecretin 0.40.4 µµg/kgg/kg IVIV overover 11 minuteminute MeasureMeasure baselinebaseline gastringastrin twicetwice andand thenthen 2,2, 5,5, 10,10, 15,15, andand 2020 minutesminutes postpost infusioninfusion TraditionallyTraditionally -- positivepositive ifif gastringastrin increasesincreases byby 200pg/mL200pg/mL oror moremore Sens 83%, Spec 100% UsingUsing aa cutcut offoff ofof 120pg/mL120pg/mL increasesincreases Sens 94%, Spec 100% (1) PeakPeak atat aboutabout 55--1010 minutesminutes (1) Berna et al. Medicine (Baltimore) 2006;85,331 SecretinSecretin StimulationStimulation TestTest OtherOther TestsTests ChromograninChromogranin AA GeneralGeneral markermarker forfor neuroendocrineneuroendocrine tumorstumors LevelLevel correlatescorrelates withwith tumortumor volumevolume LessLess sensitivesensitive andand specificspecific thanthan secretin,secretin, butbut cancan bebe usedused forfor confirmationconfirmation EndoscopicEndoscopic FindingsFindings EndoscopicEndoscopic FindingsFindings DifferentialDifferential DiagnosisDiagnosis ofof HypergastrenemiaHypergastrenemia Acid-suppressive Massive small bowel medications resection Chronic atrophic gastritis Ovarian cancer Diabetes mellitus Pernicious Anemia Foregut carcinoid Pheochromocytoma (histamine) Renal insufficiency Gastrin cell Retained gastric antrum hyperplasia/hyperfunction Rheumatoid arthritis Gastric outlet obstruction Systemic mastocytosis H. pylori infection Vitiligo Idiopathic ZE Increased intracranial pressure TumorTumor LocalizationLocalization TwoTwo mainmain modalitiesmodalities areare octreotideoctreotide scanscan andand EUSEUS >90%>90% ofof tumorstumors areare identifiedidentified ifif bothboth modalitiesmodalities areare usedused Alternatives:Alternatives: HelicHelicaall CT,CT, MRI,MRI, angiography,angiography, arterialarterial stimulation,stimulation, venousvenous sampling,sampling, andand laparotomylaparotomy TumorTumor LocalizationLocalization Gastrinoma ZESZES AlgorithmAlgorithm PrognosisPrognosis ofof ZESZES MostMost importantimportant factorfactor isis presencepresence oror absenceabsence ofof liverliver metastasismetastasis Patients with liver metastases had a 10-year survival of only 30 percent compared to a 15-year survival of 83 percent in those without liver metastases LowerLower curecure ratesrates withwith MENMEN II CushingCushing’’ss syndromesyndrome fromfrom ectopicectopic ACTHACTH releaserelease byby gastrinomagastrinoma associatedassociated withwith aggressiveaggressive diseasedisease ManagementManagement ZES Confirmed PPI Tumor Evaluation No Liver Metastases Liver Metastases Men I Status Evaluation Octreotide If response, surgical If no response, consider adding resection, RFA, or Positive Negative Chemotherapy or interferon chemoembolization Tumor > 2 cm Tumor < 2 cm Exploratory Laparotomy Consider Exploratory Follow Laparotomy Tumor Status (+) (-) Resection Parietal Cell Vagotomy MedicalMedical ManagementManagement Goal:Goal: LimitLimit complicationscomplications ofof diseasedisease ProtonProton pumppump inhibitorsinhibitors OmeprazOmeprazoolele 6060 mgmg QDQD –– BIDBID (or(or itsits equivalent)equivalent) isis sufficientsufficient inin 95%95% ofof patientspatients EsomeprazoleEsomeprazole 120120 mgmg QDQD--BIDBID LansoprazoleLansoprazole 4545 mgmg QDQD--BIDBID RabeprazoleRabeprazole 6060 mgmg QDQD--BIDBID PantoprazolePantoprazole 120120 mgmg QDQD--BIDBID MedicalMedical ManagementManagement HistamineHistamine 22 receptorreceptor antagonistsantagonists (also(also effective)effective) Require higher dosing Cimetidine – 3.6 g/day Ranitidine - 1.2 g/day Famotidine – 0.25 g/day MENMEN--11 patientspatients seemseem toto bebe moremore resistantresistant toto medicalmedical treatmenttreatment SurgicalSurgical ManagementManagement AcidAcid reducingreducing surgerysurgery suchsuch asas gastrectomygastrectomy andand vagotomyvagotomy areare rarerare sincesince thethe introductionintroduction ofof PPIPPI’’s.s. ConsiderConsider curativecurative surgerysurgery ifif tumortumor sizesize isis lessless thanthan 22 cm.cm. MetastaticMetastatic DiseaseDisease TumorsTumors spreadspread toto liverliver first,first, thenthen bonebone (spine(spine andand sacrum)sacrum) TreatmentTreatment optionsoptions Octreotide can decrease fasting serum gastrin levels Hepatic lobectomy in the absence of bilobar disease Hepatic arterial embolization Radiofrequency ablation, cyroablation Liver transplant (investigational) Chemotherapy – response rate 10-40% MENMEN II (Wermer(Wermer’’ss Syndrome)Syndrome) PrimaryPrimary hyperparathyroidismhyperparathyroidism PituitaryPituitary adenomasadenomas PancreaticPancreatic isletislet cell/gastrointestinalcell/gastrointestinal adenomasadenomas (ZE,(ZE, insulinomas,insulinomas, nonnon--functioningfunctioning pancreaticpancreatic tumors)tumors) MKSAPMKSAP 1414 AA 3333--yearyear--oldold womanwoman hashas aa 33--weekweek historyhistory ofof burningburning epigastricepigastric pain,pain, nausea,nausea, intermittentintermittent vomitingvomiting ofof partiallypartially digesteddigested food,food, andand earlyearly satiety.satiety. TheThe painpain improvesimproves slightlyslightly withwith antacids.antacids.
Load more

Recommended publications
  • Calcium-Sensing Receptor Is a Physiologic Multimodal Chemosensor Regulating Gastric G-Cell Growth and Gastrin Secretion
    Calcium-sensing receptor is a physiologic multimodal chemosensor regulating gastric G-cell growth and gastrin secretion Jianying Fenga, Clark D. Petersena, David H. Coyb, Jian-Kang Jiangc, Craig J. Thomasc, Martin R. Pollakd, and Stephen A. Wanka,1 aDigestive Diseases Branch, National Institute of Diabetes and Digestive and Kidney Diseases, National Institutes of Health, Bethesda, MD 20892-1804; bPeptide Research Laboratories, Department of Medicine, Tulane Health Sciences Center, New Orleans, LA 70112-2699; cNational Institutes of Health Chemical Genomics Center, National Human Genome Research Institute, National Institutes of Health, Bethesda, MD 20892; and dDivision of Nephrology, Beth Israel Deaconess Medical Center, Boston, MA 02215 Edited* by Martha Vaughan, National Heart, Lung, and Blood Institute, Bethesda, MD, and approved September 1, 2010 (received for review June 25, 2010) The calcium-sensing receptor (CaR) is the major sensor and The sensing of extracellular Ca2+ and the regulation of Ca2+ regulator of extracellular Ca2+, whose activity is allosterically reg- homestasis has been largely attributed to the calcium-sensing ulated by amino acids and pH. Recently, CaR has been identified in receptor (CaR), a member of the C family of G protein-coupled the stomach and intestinal tract, where it has been proposed to receptors (GPCR). Consistent with this function, CaR is ex- 2+ function in a non-Ca2+ homeostatic capacity. Luminal nutrients, pressed on extracellular Ca -regulating cells such as para- such as Ca2+ and amino acids, have been recognized for decades thyroid, thyroid parafollicular, renal tubular, and bone cells (6). as potent stimulants for gastrin and acid secretion, although the The CaR has a large NH2 terminal extracellular domain molecular basis for their recognition remains unknown.
    [Show full text]
  • Endocrine Tumors of the Pancreas
    Friday, November 4, 2005 8:30 - 10:30 a. m. Pancreatic Tumors, Session 2 Chairman: R. Jensen, Bethesda, MD, USA 9:00 - 9:30 a. m. Working Group Session Pathology and Genetics Group leaders: J.–Y. Scoazec, Lyon, France Questions to be answered: 12 Medicine and Clinical Pathology Group leader: K. Öberg, Uppsala, Sweden Questions to be answered: 17 Surgery Group leader: B. Niederle, Vienna, Austria Questions to be answered: 11 Imaging Group leaders: S. Pauwels, Brussels, Belgium; D.J. Kwekkeboom, Rotterdam, The Netherlands Questions to be answered: 4 Color Codes Pathology and Genetics Medicine and Clinical Pathology Surgery Imaging ENETS Guidelines Neuroendocrinology 2004;80:394–424 Endocrine Tumors of the Pancreas - gastrinoma Epidemiology The incidence of clinically detected tumours has been reported to be 4-12 per million inhabitants, which is much lower than what is reported from autopsy series (about 1%) (5,13). Clinicopathological staging (12, 14, 15) Well-differentiated tumours are the large majority of which the two largest fractions are insulinomas (about 40% of cases) and non-functioning tumours (30-35%). When confined to the pancreas, non-angioinvasive, <2 cm in size, with <2 mitoses per 10 high power field (HPF) and <2% Ki-67 proliferation index are classified as of benign behaviour (WHO group 1) and, with the notable exception of insulinomas, are non-functioning. Tumours confined to the pancreas but > 2 cm in size, with angioinvasion and /or perineural space invasion, or >2mitoses >2cm in size, >2 mitoses per 20 HPF or >2% Ki-67 proliferation index, either non-functioning or functioning (gastrinoma, insulinoma, glucagonoma, somastatinoma or with ectopic syndromes, such as Cushing’s syndrome (ectopic ACTH syndrome), hypercaliemia (PTHrpoma) or acromegaly (GHRHoma)) still belong to the (WHO group 1) but are classified as tumours with uncertain behaviour.
    [Show full text]
  • Zollinger-Ellison Syndrome. Case Report
    https://doi.org/10.15446/cr.v5n1.71686 ZOLLINGER-ELLISON SYNDROME. CASE REPORT Keywords: Gastrinoma; Zollinger-Ellison Sydrome; Multiple Endocrine Neoplasia Type 1. Palabras clave: Gastrinoma; Síndrome de Zollinger-Ellison; Neoplasia endocrina múltiple tipo 1. Juan Felipe Rivillas-Reyes Juan Leonel Castro-Avendaño Universidad Nacional de Colombia - Sede Bogotá - Facultad de Medicina - Programa de Medicina - Bogotá D.C. - Colombia. Héctor Fabián Martínez-Muñoz Universidad Nacional de Colombia - Sede Bogotá - Facultad de Medicina - Departamento de Cirugía - Bogotá D.C. - Colombia. Corresponding author Juan Felipe Rivillas-Reyes. Facultad de Medicina, Universidad Nacional de Colombia. Bogotá D.C. Colombia. Email: [email protected]. Received: 13/04/2018 Accepted: 20/11/2018 zollinger-ellison syndrome ABSTRACT RESUMEN 29 Introduction: The Zollinger-Ellison syndrome Introducción. El síndrome de Zollinger-Elli- (ZES) is a pathology caused by a neuroendo- son (SZE) es una patología producida por un crine tumor, usually located in the pancreas tumor neuroendocrino habitualmente localiza- or the duodenum, which is characterized by do a nivel duodenal o pancreático, el cual pro- elevated levels of gastrin, resulting in an ex- duce niveles elevados de gastrina, derivando cessive production of gastric acid. en hipersecreción de ácido gástrico. Case presentation: A 42-year-old female Presentación del caso. Paciente femenino patient with a history of longstanding peptic de 42 años con antecedente de enfermedad ulcer disease, who consulted due to persistent ulceropéptica de larga data, quién consulta por epigastric pain, melena and signs of peritoneal epigastralgia persistente y deposiciones meléni- irritation. Perforated peptic ulcer was suspect- cas y presenta signos de irritación peritoneal. Se ed, requiring emergency surgical intervention.
    [Show full text]
  • Overview of Gastrointestinal Function
    Overview of Gastrointestinal Function George N. DeMartino, Ph.D. Department of Physiology University of Texas Southwestern Medical Center Dallas, TX 75390 The gastrointestinal system Functions of the gastrointestinal system • Digestion • Absorption • Secretion • Motility • Immune surveillance and tolerance GI-OP-13 Histology of the GI tract Blood or Lumenal Serosal Side or Mucosal Side Structure of a villus Villus Lamina propria Movement of substances across the epithelial layer Tight junctions X Lumen Blood Apical membrane Basolateral membrane X X transcellular X X paracellular GI-OP-19 Histology of the GI tract Blood or Lumenal Serosal Side or Mucosal Side Motility in the gastrointestinal system Propulsion net movement by peristalsis Mixing for digestion and absorption Separation sphincters Storage decreased pressure GI-OP-42 Intercellular signaling in the gastrointestinal system • Neural • Hormonal • Paracrine GI-OP-10 Neural control of the GI system • Extrinsic nervous system autonomic central nervous system • Intrinsic (enteric) nervous system entirely with the GI system GI-OP-14 The extrinsic nervous system The intrinsic nervous system forms complete functional circuits Sensory neurons Interneurons Motor neurons (excitatory and inhibitory) Parasympathetic nerves regulate functions of the intrinsic nervous system Y Reflex control of gastrointestinal functions Vago-vagal Afferent reflex Salivary Glands Composition of Saliva O Proteins α−amylase lactoferrin lipase RNase lysozyme et al mucus O Electrolyte solution water Na+ , K + - HCO3
    [Show full text]
  • Cellular Localization of Gastric Inhibitory Polypeptide in the Duodenum and Jejunum
    Gut: first published as 10.1136/gut.14.4.284 on 1 April 1973. Downloaded from Gut, 1973, 14, 284-288 Cellular localization of gastric inhibitory polypeptide in the duodenum and jejunum JULIA M. POLAK, S. R. BLOOM', MARION KUZIO, J. C. BROWN, AND A. G. E. PEARSE From the Department ofHistochemistry, Royal Postgraduate Medical School, Hammersmith Hospital, London, and the Department ofPhysiology, University ofBritish Columbia, Vancouver, Canada SUMMARY Indirect immunofluorescence studies using an antiserum to purified porcine gastric inhibitory polypeptide indicate, in the gastrointestinal tract of dog and man, that this polypeptide is present in cells situated predominantly in the mid-zone of the glands in the duodenum and, to a lesser extent, in the jejunum. Absolute correlation of the gastric inhibitory polypeptide cell with one or other of the known endocrine-like cells identified by electron microscopy awaits confirmation by electron immunocytochemistry. It is here identified as an endocrine polypeptide cell of the APUD series and, provisionally, as the D, cell. While the hormonal status of a given polypeptide depends ultimately on physiological experiments the present results strengthen the view that gastric inhibitory polypeptide is indeed a hormone. In 1969 Brown, Pederson, Jorpes, and Mutt described We report here the results of immunofluorescence an enterogastrone, extractable from porcine intestine, studies on the localization of GIP in human and http://gut.bmj.com/ which strongly inhibited gastric acid secretion. The canine intestine. purification of an apparently similar enterogastrone was reported in the same year by Lucien, Itoh, Sun, Material and Methods Meyer, Carlton, and Schally. The first of these poly- peptides, named gastric inhibitory polypeptide Operative samples of duodenal and jejunal mucosa (GIP) by Brown, Mutt, and Pederson (1970), was from seven human subjects were studied.
    [Show full text]
  • Acute Diarrhea in Adults WENDY BARR, MD, MPH, MSCE, and ANDREW SMITH, MD Lawrence Family Medicine Residency, Lawrence, Massachusetts
    Acute Diarrhea in Adults WENDY BARR, MD, MPH, MSCE, and ANDREW SMITH, MD Lawrence Family Medicine Residency, Lawrence, Massachusetts Acute diarrhea in adults is a common problem encountered by family physicians. The most common etiology is viral gastroenteritis, a self-limited disease. Increases in travel, comorbidities, and foodborne illness lead to more bacteria- related cases of acute diarrhea. A history and physical examination evaluating for risk factors and signs of inflammatory diarrhea and/or severe dehydration can direct any needed testing and treatment. Most patients do not require labora- tory workup, and routine stool cultures are not recommended. Treatment focuses on preventing and treating dehydra- tion. Diagnostic investigation should be reserved for patients with severe dehydration or illness, persistent fever, bloody stool, or immunosuppression, and for cases of suspected nosocomial infection or outbreak. Oral rehydration therapy with early refeeding is the preferred treatment for dehydration. Antimotility agents should be avoided in patients with bloody diarrhea, but loperamide/simethicone may improve symptoms in patients with watery diarrhea. Probiotic use may shorten the duration of illness. When used appropriately, antibiotics are effective in the treatment of shigellosis, campylobacteriosis, Clostridium difficile,traveler’s diarrhea, and protozoal infections. Prevention of acute diarrhea is promoted through adequate hand washing, safe food preparation, access to clean water, and vaccinations. (Am Fam Physician. 2014;89(3):180-189. Copyright © 2014 American Academy of Family Physicians.) CME This clinical content cute diarrhea is defined as stool with compares noninflammatory and inflamma- conforms to AAFP criteria increased water content, volume, or tory acute infectious diarrhea.7,8 for continuing medical education (CME).
    [Show full text]
  • Gastrinoma: a Small Tumor with Big Symptoms
    Case Report ISSN: 2574 -1241 DOI: 10.26717/BJSTR.2020.31.005159 Gastrinoma: A Small Tumor with Big Symptoms Hira Irfan1, Ahmed Imran Siddiqi1,2*, Waqas Shafiq1 and Umal Azmat1 1Department of Endocrinology, Shaukat Khanum memorial cancer hospital and research center, Lahore, Pakistan 2Department of Medicine Jersey General Hospital, Channel Islands UK *Corresponding author: Ahmed Imran Siddiqi, Department of Endocrinology, Shaukat Khanum memorial cancer hospital and research center, Lahore, Pakistan; Department of Medicine Jersey General Hospital, Channel Islands UK ARTICLE INFO ABSTRACT Received: November 04, 2020 Gastrinoma is rare gastrin secreting tumor and can be challenging to diagnose in the Published: November 11, 2020 absence of typical clinical features. Surgical resection remains the mainstay of treatment and can be curative, but the clinical presentation can compromise complete resection especially if the disease has already metastasized. We present here a 77-year-old lady Citation: Hira Irfan, Ahmed Imran Siddiqi, with abdominal pain and secretory diarrhea. Gastrinoma was diagnosed in a stepwise approach of investigations and then surgically removed. Following surgery, the patient Small Tumor with Big Symptoms. Biomed got prescribed octreotide for complete resolution of symptoms. Conservative treatment JWaqas Sci &Shafiq, Tech Umal Res Azmat.31(5)-2020. Gastrinoma: BJSTR. A is only recommended for patients unsuitable for surgery, with widespread metastasis and MS.ID.005159. on patients’ own choice. Abbreviations: NET: Neuroendocrine Tumors; ZES: Zollinger-Ellison Syndrome; MEN-1: Multiple Endocrine Neoplasia Type-1; SSTRs: Somatostatin Receptors; PTH: Parathyroid Hormone Introduction for a Gastrinoma (5). We report here a case of a 77-year-old lady Gastrinomas are functional neuroendocrine tumors (NET) diagnosed with a Gastrinoma.
    [Show full text]
  • A Review of Normal Function and Role of Gastrin in Zollinger-Ellison
    When Gastrin Goes Awry: A Review of Normal Function and Role of Gastrin in Zollinger-Ellison Syndrome Leigha LaTourette1, Janel Cross2, Barbara Brabetz2 Department of Plant and Animal Science1, Department of Natural Sciences and Mathematics2 Introduction Gastrin: Normal Mode of Action Gastrin’s Role in Zollinger-Ellison Syndrome MEN 1 & ZES Gastrin is a digestive hormone that acts on the MEN1 is an inheritable disease that causes over neuroendocrine and parietal cells of the secretion of hormones via tumors on the gastrointestinal tract to ultimately secrete gastric endocrine glands throughout the body.4 Around a acid. Gastrin secretion begins even before food 30% of these tumors are found to be malignant. is consumed, as the mere anticipation of a meal ZES is commonly associated with the MEN1 due can lead to a series of events ending in the to the presence of tumors found in the stomach creation of the gastric acid needed to breakdown and small intestine.4 These gastrointestinal foods. Not only does Gastrin support the tumors caused by MEN 1 give the person a digestion of foods, but it also stimulates growth, higher likelihood of contracting ZES, as the secretion, blood flow, and acts as a defense probability of having pancreatic or duodenal mechanism against bacteria in the tumors is increased.4 gastrointestinal system. When the production of Gastrin becomes overt, major consequences like that of Zollinger Ellison Syndrome (ZES). ZES is an extremely rare disease occurring in people between the ages of 30-60.4 ZES is related to the uncontrolled secretion of gastrin due to the presence of certain pancreatic or duodenal tumors.
    [Show full text]
  • Reciprocal Regulation of Antral Gastrin and Somatostatin Gene Expression by Omeprazole-Induced Achlorhydria
    Reciprocal regulation of antral gastrin and somatostatin gene expression by omeprazole-induced achlorhydria. S J Brand, D Stone J Clin Invest. 1988;82(3):1059-1066. https://doi.org/10.1172/JCI113662. Research Article Gastric acid exerts a feedback inhibition on the secretion of gastrin from antral G cells. This study examines whether gastrin gene expression is also regulated by changes in gastric pH. Achlorhydria was induced in rats by the gastric H+/K+ ATPase inhibitor, omeprazole (100 mumol/kg). This resulted in fourfold increases in both serum gastrin (within 2 h) and gastrin mRNA levels (after 24 h). Antral somatostatin D cells probably act as chemoreceptors for gastric acid to mediate a paracrine inhibition on gastrin secretion from adjacent G cells. Omeprazole-induced achlorhydria reduced D-cell activity as shown by a threefold decrease in antral somatostatin mRNA levels that began after 24 h. Exogenous administration of the somatostatin analogue SMS 201-995 (10 micrograms/kg) prevented both the hypergastrinemia and the increase in gastrin mRNA levels caused by omeprazole-induced achlorhydria. Exogenous somatostatin, however, did not influence the decrease in antral somatostatin mRNA levels seen with achlorhydria. These data, therefore, support the hypothesis that antral D cells act as chemoreceptors for changes in gastric pH, and modulates somatostatin secretion and synthesis to mediate a paracrine inhibition on gastrin gene expression in adjacent G cells. Find the latest version: https://jci.me/113662/pdf Reciprocal Regulation of Antral Gastrin and Somatostatin Gene Expression by Omeprazole-induced Achlorhydria Stephen J. Brand and Deborah Stone Departments ofMedicine, Harvard Medical School and Massachusetts General Hospital, Gastrointestinal Unit, Boston, Massachusetts Abstract Substantial evidence supports the hypothesis that gastric acid inhibits gastrin secretion through somatostatin released Gastric acid exerts a feedback inhibition on the secretion of from antral D cells (9, 10).
    [Show full text]
  • Benign Oesophageal Stricture and Chronic Diarrhoea As Atypical
    Open Access Case Report DOI: 10.7759/cureus.16593 Benign Oesophageal Stricture and Chronic Diarrhoea As Atypical Presenting Symptoms of an Advanced Metastatic Pancreatic Gastrinoma: A Case Report and Review of Literature Muhammad Hafiz Kamarul Bahrin 1 , Raoof Hagag 2 , Abuobeida Ali 3 , Seifeldin Yahia 4 , Richard Armstrong 5 1. Gastroenterology, United Lincolnshire Hospitals Trust, Boston, GBR 2. Acute Medicine, United Lincolnshire Hospitals National Health Service (NHS) Trust, Boston, GBR 3. Gastroenterology, Peterborough City Hospital, Peterborough, GBR 4. Diabetes and Endocrinology, United Lincolnshire Hospitals National Health Service (NHS) Trust, Boston, GBR 5. Gastroenterology, United Lincolnshire Hospitals National Health Service (NHS) Trust, Boston, GBR Corresponding author: Muhammad Hafiz Kamarul Bahrin, [email protected] Abstract Gastrinoma or otherwise known as Zollinger-Ellison syndrome is characterised by hypersecretion of gastrin and gastric acid leading to the formation of recurrent atypical ulcers along the upper gastrointestinal tract. It is extremely difficult to diagnose during an acute presentation both due to its rarity and its lack of pathognomonic symptoms. Its symptoms range from mild to severe to life-threatening and often get mistaken for a different condition such as viral gastroenteritis as seen in our case report. The most common symptoms of gastrinoma include abdominal pain, dyspepsia and chronic diarrhoea. It rarely presents as a benign oesophageal stricture with some case series reporting the frequency to be as low as 0.4%. Our literature review of 9 random case reports on gastrinoma/Zollinger-Ellison syndrome selected from Pubmed Central reviewed the frequency of its presenting symptoms and investigation modalities involved throughout its diagnostic process. In summary, it agrees with the findings postulated by Jensen’s series.
    [Show full text]
  • GASTRIC CANCER STRUCTURED REPORTING PROTOCOL (2Nd Edition, 2020)
    GASTRIC CANCER STRUCTURED REPORTING PROTOCOL (2nd Edition, 2020) Incorporating the: International Collaboration on Cancer Reporting (ICCR) Carcinoma of the Stomach Dataset www.ICCR-Cancer.org Core Document versions: • ICCR dataset: Carcinoma of the Stomach 1st edition • AJCC Cancer Staging Manual 8th edition • Digestive System Tumours, World Health Organization Classification of Tumours, 5th Edition, Volume 1, 2019 ii Gastric Cancer Structured Reporting Protocol 2nd edition ISBN: 978-1-76081-425-0 Publications number (SHPN): (CI) 200282 Online copyright © RCPA 2020 This work (Protocol) is copyright. You may download, display, print and reproduce the Protocol for your personal, non-commercial use or use within your organisation subject to the following terms and conditions: 1. The Protocol may not be copied, reproduced, communicated or displayed, in whole or in part, for profit or commercial gain. 2. Any copy, reproduction or communication must include this RCPA copyright notice in full. 3. With the exception of Chapter 6 - the checklist, no changes may be made to the wording of the Protocol including any Standards, Guidelines, commentary, tables or diagrams. Excerpts from the Protocol may be used in support of the checklist. References and acknowledgments must be maintained in any reproduction or copy in full or part of the Protocol. 4. In regard to Chapter 6 of the Protocol - the checklist: o The wording of the Standards may not be altered in any way and must be included as part of the checklist. o Guidelines are optional and those which are deemed not applicable may be removed. o Numbering of Standards and Guidelines must be retained in the checklist, but can be reduced in size, moved to the end of the checklist item or greyed out or other means to minimise the visual impact.
    [Show full text]
  • HISTOLOGICAL TYPING of ENDOCRINE TUMOURS INTERNATIONAL HISTOLOGICAL CLASSIFICATION of TUMOURS No
    HISTOLOGICAL TYPING OF ENDOCRINE TUMOURS INTERNATIONAL HISTOLOGICAL CLASSIFICATION OF TUMOURS No. 23 HISTOLOGICAL TYPING OF ENDOCRINE TUMOURS E. D. WILLIAMS Head, WHO Collaboratmg Centre for the Histologica/ Classificatton of Endocrine Tumours. · Department of Pathology, The Welsh National School of Medicine, Cardiff. Wales, Umted Kingdom in collaboration with R. E. SIEBENMANN L. H. SOBIN lnstitute of Pathology, Pathologist, Stadtspital Triemli, World Hea/th Orgamzation, Zurich, Switzerland Geneva, Switzer/and and pathologists in 13 countries WORLD HEALTH ORGANIZATION GENEVA 1980 ISBN 92 4 176023 O © World Health Organization 1980 Pubhcations ofthe World Health Orgamzation enjoy copyright protection m accordance with the provisions of Protocol2 of the Universal Copyright Convention. For rights of reproduction or translation of WHO publicatwns, m part or in tofo, application should be made to the Office of Publications, World Health Orgamzatwn, Geneva, Sw1tzerland. The World Health Organi­ zation welcomes such applications. The designatwns employed and the presentatwn of the material in this publicatwn do not 1mply the expression of any opinion whatsoever on the part of the Duector-General of the World Health Organization concerning the legal status of any country, territory, city or area or of 1ts authorities, or concerning the delimitation of its front1ers or boundanes. The mention of specific compames or of certam manufacturers' products does not imply that they are endorsed or recommended by the W orld Health Organizatwn in preference to others of a similar nature that are not mentioned. Errors and omJsswns excepted, the names of proprietary products are distingmshed by imt1al capitalletters. Authors alone are responsible for v1ews expressed in th1s publication.
    [Show full text]