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Endocrine pancreas disorder

MedGen UID:: 124407
•Concept ID:: C0271633
•: Disease or Syndrome

Synonym:	Endocrine pancreas disease
SNOMED CT:	Disorder of endocrine pancreas (17346000); Disorder of pancreatic islets (17346000); Disorder of islets of Langerhans (17346000)

Monarch Initiative:	MONDO:0001933

Definition

A non-neoplastic or neoplastic disorder that affects the endocrine pancreatic tissue (islets of Langerhans). Representative examples include diabetes mellitus and neuroendocrine neoplasms. [from NCI]

Term Hierarchy

GTR
MeSH

CClinical test, RResearch test, OOMIM, GGeneReviews, VClinVar

CROGVEndocrine pancreas disorder

Pathological Conditions, Signs and Symptoms
- Pathological process
  - Disease
    - Disorder by Site
      - Disorder of endocrine system
        Endocrine pancreas disorder
        Non-Neoplastic Endocrine Pancreas Disorder
        Diabetes mellitus
        Childhood Diabetes Mellitus
        Complication due to diabetes mellitus
        Diabetes mellitus type 1
        Diabetes mellitus without complication
        Gestational diabetes
        Latent autoimmune diabetes in adults
        Leprechaunism syndrome
        Lipoatrophic diabetes
        Maturity onset diabetes mellitus in young
        Mitochondrial Diabetes
        Monogenic diabetes
        New Onset Diabetes After Transplant
        Pre-Gestational Diabetes
        Prediabetes syndrome
        Type 2 diabetes mellitus
        Familial hyperinsulinism
        Adult-onset non-insulinoma persistent hyperinsulinemic hypoglycemia
        Atypical Fanconi syndrome-neonatal hyperinsulinism syndrome
        Exercise-induced hyperinsulinism
        Hyperinsulinemic hypoglycemia, familial, 1
        Hyperinsulinemic hypoglycemia, familial, 2
        Hyperinsulinemic hypoglycemia, familial, 4
        Hyperinsulinism due to glucokinase deficiency
        Hyperinsulinism due to INSR deficiency
        Hyperinsulinism-hyperammonemia syndrome
        Nesidioblastosis
        Hyperinsulinism in the Infant of a Diabetic Mother
        Insulin resistance - type A
        KATP-Associated Hyperinsulinism
        Diffuse KATP-Associated Hyperinsulinism
        Focal KATP-Associated Hyperinsulinism
        Monogenic Hyperinsulinism
        Glucokinase-Associated Hyperinsulinism
        Hepatocyte Nuclear Factor 1-Alpha Hyperinsulinism
        Hepatocyte Nuclear Factor 4-Alpha Hyperinsulinism
        Mitochondrial Hydroxyacyl-Coenzyme A Dehydrogenase Hyperinsulinism
        Mitochondrial Uncoupling Protein 2 Hyperinsulinism
        Obesity-Associated Insulin Resistance
        Perinatal-Stress Induced Hyperinsulinism
        Transient Hyperinsulinism
        Pancreatic endocrine tumor
        Glucagonoma syndrome
        Malignant Pancreatic Glucagonoma
        Metastatic Pancreatic Neuroendocrine Neoplasm
        Advanced Pancreatic Neuroendocrine Neoplasm
        Metastatic Pancreatic Neuroendocrine Carcinoma
        Metastatic Pancreatic Neuroendocrine Tumor
        Neuroendocrine pancreatic tumor
        Functional pancreatic neuroendocrine tumor
        Non-functional pancreatic neuroendocrine tumor
        Pancreatic delta cell neuroendocrine tumor
        Pancreatic gastrin-producing neuroendocrine tumor
        Pancreatic insulinoma
        Pancreatic Neuroendocrine Tumor by AJCC v8 Stage
        Pancreatic neuroendocrine tumor G1
        Pancreatic Neuroendocrine Tumor G2
        Pancreatic Neuroendocrine Tumor G3
        Pancreatic vasoactive intestinal peptide producing tumor
        Pancreatic endocrine carcinoma
        Gastrin-producing neuroendocrine tumor
        Pancreatic large cell neuroendocrine carcinoma
        Pancreatic small cell neuroendocrine carcinoma
        Recurrent Pancreatic Neuroendocrine Carcinoma
        Refractory Pancreatic Neuroendocrine Carcinoma
        Somatostatinoma
        Unresectable Pancreatic Neuroendocrine Carcinoma
        VIPoma
        Pancreatic islet cell adenoma
        Multiple pancreatic beta-cell adenomas
        Unresectable Pancreatic Neuroendocrine Neoplasm

Professional guidelines

PubMed

Acute Pancreatitis: Diagnosis and Treatment.

Szatmary P, Grammatikopoulos T, Cai W, Huang W, Mukherjee R, Halloran C, Beyer G, Sutton R
Drugs 2022 Aug;82(12):1251-1276. Epub 2022 Sep 8 doi: 10.1007/s40265-022-01766-4. PMID: 36074322 Free PMC Article

ACG Clinical Guideline: Chronic Pancreatitis.

Gardner TB, Adler DG, Forsmark CE, Sauer BG, Taylor JR, Whitcomb DC
Am J Gastroenterol 2020 Mar;115(3):322-339. doi: 10.14309/ajg.0000000000000535. PMID: 32022720

ESPEN guideline on clinical nutrition in acute and chronic pancreatitis.

Arvanitakis M, Ockenga J, Bezmarevic M, Gianotti L, Krznarić Ž, Lobo DN, Löser C, Madl C, Meier R, Phillips M, Rasmussen HH, Van Hooft JE, Bischoff SC
Clin Nutr 2020 Mar;39(3):612-631. Epub 2020 Jan 22 doi: 10.1016/j.clnu.2020.01.004. PMID: 32008871

See all (376)

Recent clinical studies

Etiology

The Current Status of Allogenic Islet Cell Transplantation.

Czarnecka Z, Dadheech N, Razavy H, Pawlick R, Shapiro AMJ
Cells 2023 Oct 10;12(20) doi: 10.3390/cells12202423. PMID: 37887267 Free PMC Article

Epigenetic regulation of insulin action and secretion - role in the pathogenesis of type 2 diabetes.

Ling C
J Intern Med 2020 Aug;288(2):158-167. Epub 2020 May 3 doi: 10.1111/joim.13049. PMID: 32363639

Type 1 diabetes mellitus.

Katsarou A, Gudbjörnsdottir S, Rawshani A, Dabelea D, Bonifacio E, Anderson BJ, Jacobsen LM, Schatz DA, Lernmark Å
Nat Rev Dis Primers 2017 Mar 30;3:17016. doi: 10.1038/nrdp.2017.16. PMID: 28358037

Autoantibodies in type 1 diabetes.

Taplin CE, Barker JM
Autoimmunity 2008 Feb;41(1):11-8. doi: 10.1080/08916930701619169. PMID: 18176860

Mechanisms linking obesity to insulin resistance and type 2 diabetes.

Kahn SE, Hull RL, Utzschneider KM
Nature 2006 Dec 14;444(7121):840-6. doi: 10.1038/nature05482. PMID: 17167471

See all (3059)

Diagnosis

Pathogenesis of Type 1 Diabetes: Established Facts and New Insights.

Zajec A, Trebušak Podkrajšek K, Tesovnik T, Šket R, Čugalj Kern B, Jenko Bizjan B, Šmigoc Schweiger D, Battelino T, Kovač J
Genes (Basel) 2022 Apr 16;13(4) doi: 10.3390/genes13040706. PMID: 35456512 Free PMC Article

Autoantibody-Negative Type 1 Diabetes: A Neglected Subtype.

Patel SK, Ma CS, Fourlanos S, Greenfield JR
Trends Endocrinol Metab 2021 May;32(5):295-305. Epub 2021 Mar 9 doi: 10.1016/j.tem.2021.02.001. PMID: 33712367

Organisation of the human pancreas in health and in diabetes.

Atkinson MA, Campbell-Thompson M, Kusmartseva I, Kaestner KH
Diabetologia 2020 Oct;63(10):1966-1973. Epub 2020 Sep 7 doi: 10.1007/s00125-020-05203-7. PMID: 32894306 Free PMC Article

Type 1 diabetes mellitus.

Katsarou A, Gudbjörnsdottir S, Rawshani A, Dabelea D, Bonifacio E, Anderson BJ, Jacobsen LM, Schatz DA, Lernmark Å
Nat Rev Dis Primers 2017 Mar 30;3:17016. doi: 10.1038/nrdp.2017.16. PMID: 28358037

Type 1 diabetes.

Atkinson MA, Eisenbarth GS, Michels AW
Lancet 2014 Jan 4;383(9911):69-82. Epub 2013 Jul 26 doi: 10.1016/S0140-6736(13)60591-7. PMID: 23890997 Free PMC Article

See all (2561)

Therapy

Subcutaneous device-free islet transplantation.

Zhou X, Xu Z, You Y, Yang W, Feng B, Yang Y, Li F, Chen J, Gao H
Front Immunol 2023;14:1287182. Epub 2023 Oct 18 doi: 10.3389/fimmu.2023.1287182. PMID: 37965322 Free PMC Article

Current practices in islet cell autotransplantation.

Chen ME, Desai CS
Expert Rev Endocrinol Metab 2023 Sep-Nov;18(5):419-425. Epub 2023 Sep 7 doi: 10.1080/17446651.2023.2256407. PMID: 37680038

Type 1 diabetes mellitus.

Katsarou A, Gudbjörnsdottir S, Rawshani A, Dabelea D, Bonifacio E, Anderson BJ, Jacobsen LM, Schatz DA, Lernmark Å
Nat Rev Dis Primers 2017 Mar 30;3:17016. doi: 10.1038/nrdp.2017.16. PMID: 28358037

The Role of Oxidative Stress and Hypoxia in Pancreatic Beta-Cell Dysfunction in Diabetes Mellitus.

Gerber PA, Rutter GA
Antioxid Redox Signal 2017 Apr 1;26(10):501-518. Epub 2016 Jun 30 doi: 10.1089/ars.2016.6755. PMID: 27225690 Free PMC Article

Type 1 diabetes.

Atkinson MA, Eisenbarth GS, Michels AW
Lancet 2014 Jan 4;383(9911):69-82. Epub 2013 Jul 26 doi: 10.1016/S0140-6736(13)60591-7. PMID: 23890997 Free PMC Article

See all (1818)

Prognosis

Pathogenesis of Type 1 Diabetes: Established Facts and New Insights.

Patch-Seq Links Single-Cell Transcriptomes to Human Islet Dysfunction in Diabetes.

Camunas-Soler J, Dai XQ, Hang Y, Bautista A, Lyon J, Suzuki K, Kim SK, Quake SR, MacDonald PE
Cell Metab 2020 May 5;31(5):1017-1031.e4. Epub 2020 Apr 16 doi: 10.1016/j.cmet.2020.04.005. PMID: 32302527 Free PMC Article

Type 1 diabetes mellitus.

Katsarou A, Gudbjörnsdottir S, Rawshani A, Dabelea D, Bonifacio E, Anderson BJ, Jacobsen LM, Schatz DA, Lernmark Å
Nat Rev Dis Primers 2017 Mar 30;3:17016. doi: 10.1038/nrdp.2017.16. PMID: 28358037

Type 1 diabetes.

Atkinson MA, Eisenbarth GS, Michels AW
Lancet 2014 Jan 4;383(9911):69-82. Epub 2013 Jul 26 doi: 10.1016/S0140-6736(13)60591-7. PMID: 23890997 Free PMC Article

Autoantibodies in type 1 diabetes.

Taplin CE, Barker JM
Autoimmunity 2008 Feb;41(1):11-8. doi: 10.1080/08916930701619169. PMID: 18176860

See all (1659)

Clinical prediction guides

Pathogenesis of Type 1 Diabetes: Established Facts and New Insights.

Epigenetic regulation of insulin action and secretion - role in the pathogenesis of type 2 diabetes.

Ling C
J Intern Med 2020 Aug;288(2):158-167. Epub 2020 May 3 doi: 10.1111/joim.13049. PMID: 32363639

Patch-Seq Links Single-Cell Transcriptomes to Human Islet Dysfunction in Diabetes.

Type 1 diabetes.

Atkinson MA, Eisenbarth GS, Michels AW
Lancet 2014 Jan 4;383(9911):69-82. Epub 2013 Jul 26 doi: 10.1016/S0140-6736(13)60591-7. PMID: 23890997 Free PMC Article

Autoantibodies in type 1 diabetes.

Taplin CE, Barker JM
Autoimmunity 2008 Feb;41(1):11-8. doi: 10.1080/08916930701619169. PMID: 18176860

See all (1992)

Recent systematic reviews

Appraising the causal role of smoking in multiple diseases: A systematic review and meta-analysis of Mendelian randomization studies.

Larsson SC, Burgess S
EBioMedicine 2022 Aug;82:104154. Epub 2022 Jul 8 doi: 10.1016/j.ebiom.2022.104154. PMID: 35816897 Free PMC Article

Safety and Efficacy of Teplizumab for Treatment of Type One Diabetes Mellitus: A Systematic Review and Meta-Analysis.

Nourelden AZ, Elshanbary AA, El-Sherif L, Benmelouka AY, Rohim HI, Helmy SK, Sayed MK, Ismail A, Ali AS, Ragab KM, Zaazouee MS
Endocr Metab Immune Disord Drug Targets 2021;21(10):1895-1904. doi: 10.2174/1871530320999201209222921. PMID: 33302842

Coronaviruses and Endocrine System: A Systematic Review on Evidence and Shadows.

Parolin M, Parisotto M, Zanchetta F, Sartorato P, De Menis E
Endocr Metab Immune Disord Drug Targets 2021;21(7):1242-1251. doi: 10.2174/1871530320666200905123332. PMID: 32888287

Metabolic dysfunction in polycystic ovary syndrome: Pathogenic role of androgen excess and potential therapeutic strategies.

Sanchez-Garrido MA, Tena-Sempere M
Mol Metab 2020 May;35:100937. Epub 2020 Feb 5 doi: 10.1016/j.molmet.2020.01.001. PMID: 32244180 Free PMC Article

Cystic pancreatic neuroendocrine tumors (cPNETs): a systematic review and meta-analysis of case series.

Hurtado-Pardo L, Cienfuegos JA, Ruiz-Canela M, Panadero P, Benito A, Hernández Lizoain JL
Rev Esp Enferm Dig 2017 Nov;109(11):778-787. doi: 10.17235/reed.2017.5044/2017. PMID: 29072081

See all (34)

MedGen

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Endocrine pancreas disorder

Definition

Term Hierarchy

Professional guidelines

PubMed

Recent clinical studies

Etiology

Diagnosis

Therapy

Prognosis

Clinical prediction guides

Recent systematic reviews

Supplemental Content

Table of contents

Clinical resources

Practice guidelines

Consumer resources

Reviews

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