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Abnormality of the pancreas

MedGen UID:
892541
Concept ID:
C4025751
Anatomical Abnormality
Synonym: Pancreatic disease
 
HPO: HP:0001732

Definition

An abnormality of the pancreas. [from HPO]

Conditions with this feature

Trichohepatoenteric syndrome 1
MedGen UID:
1644087
Concept ID:
C4551982
Disease or Syndrome
Trichohepatoenteric syndrome (THES), generally considered to be a neonatal enteropathy, is characterized by intractable diarrhea (seen in almost all affected children), woolly hair (seen in all), intrauterine growth restriction, facial dysmorphism, and short stature. Additional findings include poorly characterized immunodeficiency, recurrent infections, skin abnormalities, and liver disease. Mild intellectual disability (ID) is seen in about 50% of affected individuals. Less common findings include congenital heart defects and platelet anomalies. To date 52 affected individuals have been reported.

Professional guidelines

PubMed

Malgras B, Dokmak S, Aussilhou B, Pocard M, Sauvanet A
J Visc Surg 2023 Feb;160(1):39-51. Epub 2023 Jan 24 doi: 10.1016/j.jviscsurg.2023.01.002. PMID: 36702720
Vanbiervliet G, Strijker M, Arvanitakis M, Aelvoet A, Arnelo U, Beyna T, Busch O, Deprez PH, Kunovsky L, Larghi A, Manes G, Moss A, Napoleon B, Nayar M, Pérez-Cuadrado-Robles E, Seewald S, Barthet M, van Hooft JE
Endoscopy 2021 Apr;53(4):429-448. Epub 2021 Mar 16 doi: 10.1055/a-1397-3198. PMID: 33728632
Gutta A, Fogel E, Sherman S
Expert Rev Gastroenterol Hepatol 2019 Nov;13(11):1089-1105. Epub 2019 Nov 8 doi: 10.1080/17474124.2019.1685871. PMID: 31663403Free PMC Article

Recent clinical studies

Etiology

Xourafa G, Korbmacher M, Roden M
Nat Rev Endocrinol 2024 Jan;20(1):27-49. Epub 2023 Oct 16 doi: 10.1038/s41574-023-00898-1. PMID: 37845351
Gutta A, Fogel E, Sherman S
Expert Rev Gastroenterol Hepatol 2019 Nov;13(11):1089-1105. Epub 2019 Nov 8 doi: 10.1080/17474124.2019.1685871. PMID: 31663403Free PMC Article
Addeo P, Locicero A, Bachellier P
J Visc Surg 2019 Oct;156(5):467-468. Epub 2019 Feb 14 doi: 10.1016/j.jviscsurg.2019.02.001. PMID: 30773440
Stram M, Liu S, Singhi AD
Surg Pathol Clin 2016 Dec;9(4):643-659. Epub 2016 Oct 12 doi: 10.1016/j.path.2016.05.008. PMID: 27926364
Pohl JF, Uc A
Curr Opin Gastroenterol 2015 Sep;31(5):380-6. doi: 10.1097/MOG.0000000000000197. PMID: 26181572Free PMC Article

Diagnosis

Normatov I, Sentongo T
Pediatr Ann 2019 Nov 1;48(11):e441-e447. doi: 10.3928/19382359-20191018-01. PMID: 31710363
Gutta A, Fogel E, Sherman S
Expert Rev Gastroenterol Hepatol 2019 Nov;13(11):1089-1105. Epub 2019 Nov 8 doi: 10.1080/17474124.2019.1685871. PMID: 31663403Free PMC Article
Pohl JF, Uc A
Curr Opin Gastroenterol 2015 Sep;31(5):380-6. doi: 10.1097/MOG.0000000000000197. PMID: 26181572Free PMC Article
O'Neill E, Hammond N, Miller FH
Radiol Clin North Am 2014 Jul;52(4):757-77. Epub 2014 Apr 2 doi: 10.1016/j.rcl.2014.02.006. PMID: 24889170
Kamisawa T, Takuma K, Anjiki H, Egawa N, Kurata M, Honda G, Tsuruta K, Sasaki T
Clin Gastroenterol Hepatol 2009 Nov;7(11 Suppl):S84-8. doi: 10.1016/j.cgh.2009.08.024. PMID: 19896105

Therapy

Inamdar S, Cote GA, Yadav D
Gastrointest Endosc Clin N Am 2023 Oct;33(4):789-805. Epub 2023 May 26 doi: 10.1016/j.giec.2023.04.012. PMID: 37709411
Malgras B, Dokmak S, Aussilhou B, Pocard M, Sauvanet A
J Visc Surg 2023 Feb;160(1):39-51. Epub 2023 Jan 24 doi: 10.1016/j.jviscsurg.2023.01.002. PMID: 36702720
Lord K, De León DD
Clin Perinatol 2018 Mar;45(1):61-74. Epub 2017 Dec 6 doi: 10.1016/j.clp.2017.10.007. PMID: 29406007
Pohl JF, Uc A
Curr Opin Gastroenterol 2015 Sep;31(5):380-6. doi: 10.1097/MOG.0000000000000197. PMID: 26181572Free PMC Article
Lim EL, Hollingsworth KG, Aribisala BS, Chen MJ, Mathers JC, Taylor R
Diabetologia 2011 Oct;54(10):2506-14. Epub 2011 Jun 9 doi: 10.1007/s00125-011-2204-7. PMID: 21656330Free PMC Article

Prognosis

Xourafa G, Korbmacher M, Roden M
Nat Rev Endocrinol 2024 Jan;20(1):27-49. Epub 2023 Oct 16 doi: 10.1038/s41574-023-00898-1. PMID: 37845351
Malgras B, Dokmak S, Aussilhou B, Pocard M, Sauvanet A
J Visc Surg 2023 Feb;160(1):39-51. Epub 2023 Jan 24 doi: 10.1016/j.jviscsurg.2023.01.002. PMID: 36702720
Gutta A, Fogel E, Sherman S
Expert Rev Gastroenterol Hepatol 2019 Nov;13(11):1089-1105. Epub 2019 Nov 8 doi: 10.1080/17474124.2019.1685871. PMID: 31663403Free PMC Article
Pohl JF, Uc A
Curr Opin Gastroenterol 2015 Sep;31(5):380-6. doi: 10.1097/MOG.0000000000000197. PMID: 26181572Free PMC Article
DiMagno MJ, Wamsteker EJ
Curr Gastroenterol Rep 2011 Apr;13(2):150-6. doi: 10.1007/s11894-010-0170-8. PMID: 21222060Free PMC Article

Clinical prediction guides

Xourafa G, Korbmacher M, Roden M
Nat Rev Endocrinol 2024 Jan;20(1):27-49. Epub 2023 Oct 16 doi: 10.1038/s41574-023-00898-1. PMID: 37845351
Malgras B, Dokmak S, Aussilhou B, Pocard M, Sauvanet A
J Visc Surg 2023 Feb;160(1):39-51. Epub 2023 Jan 24 doi: 10.1016/j.jviscsurg.2023.01.002. PMID: 36702720
Gutta A, Fogel E, Sherman S
Expert Rev Gastroenterol Hepatol 2019 Nov;13(11):1089-1105. Epub 2019 Nov 8 doi: 10.1080/17474124.2019.1685871. PMID: 31663403Free PMC Article
DiMagno MJ, Wamsteker EJ
Curr Gastroenterol Rep 2011 Apr;13(2):150-6. doi: 10.1007/s11894-010-0170-8. PMID: 21222060Free PMC Article
Geokas MC, Baltaxe HA, Banks PA, Silva J Jr, Frey CF
Ann Intern Med 1985 Jul;103(1):86-100. doi: 10.7326/0003-4819-103-1-86. PMID: 3890660

Recent systematic reviews

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: 32244180Free PMC Article
Hansen CC, Søreide K
Medicine (Baltimore) 2018 Aug;97(35):e12154. doi: 10.1097/MD.0000000000012154. PMID: 30170459Free PMC Article
Vanga RR, Tansel A, Sidiq S, El-Serag HB, Othman MO
Clin Gastroenterol Hepatol 2018 Aug;16(8):1220-1228.e4. Epub 2018 Jan 31 doi: 10.1016/j.cgh.2018.01.027. PMID: 29374614Free PMC Article
Hafezi M, Mayschak B, Probst P, Büchler MW, Hackert T, Mehrabi A
Am J Surg 2017 Sep;214(3):525-537. Epub 2017 Jan 3 doi: 10.1016/j.amjsurg.2016.12.025. PMID: 28110914
Cienfuegos JA, Rotellar F, Salguero J, Benito A, Solórzano JL, Sangro B
Rev Esp Enferm Dig 2016 Aug;108(8):479-84. doi: 10.17235/reed.2016.4474/2016. PMID: 27468966

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