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PANCREATIC AGENESIS 3(PAGEN3)

MedGen UID:
1056427
•Concept ID:
CN378680
•
Disease or Syndrome
Synonym: PAGEN3
 
Gene (location): ZNF808 (19q13.41)
 
OMIM®: 620991

Definition

Pancreatic agenesis-3 (PAGEN3) is an autosomal recessive disorder characterized by neonatal diabetes and exocrine pancreatic insufficiency (De Franco et al., 2023). For a general phenotypic description and discussion of genetic heterogeneity of pancreatic agenesis, see PAGEN1 (260370). [from OMIM]

Professional guidelines

PubMed

Clinical Management of Hypertriglyceridemia in the Prevention of Cardiovascular Disease and Pancreatitis.
Hernandez P, Passi N, Modarressi T, Kulkarni V, Soni M, Burke F, Bajaj A, Soffer D
Curr Atheroscler Rep 2021 Sep 13;23(11):72. doi: 10.1007/s11883-021-00962-z. PMID: 34515873Free PMC Article
AGA Clinical Practice Update on Functional Gastrointestinal Symptoms in Patients With Inflammatory Bowel Disease: Expert Review.
Colombel JF, Shin A, Gibson PR
Clin Gastroenterol Hepatol 2019 Feb;17(3):380-390.e1. Epub 2018 Aug 9 doi: 10.1016/j.cgh.2018.08.001. PMID: 30099108Free PMC Article
Orlistat, a new lipase inhibitor for the management of obesity.
Heck AM, Yanovski JA, Calis KA
Pharmacotherapy 2000 Mar;20(3):270-9. doi: 10.1592/phco.20.4.270.34882. PMID: 10730683Free PMC Article

Recent clinical studies

Etiology

Clinical Management of Hypertriglyceridemia in the Prevention of Cardiovascular Disease and Pancreatitis.
Hernandez P, Passi N, Modarressi T, Kulkarni V, Soni M, Burke F, Bajaj A, Soffer D
Curr Atheroscler Rep 2021 Sep 13;23(11):72. doi: 10.1007/s11883-021-00962-z. PMID: 34515873Free PMC Article
Chronic pancreatitis.
Kleeff J, Whitcomb DC, Shimosegawa T, Esposito I, Lerch MM, Gress T, Mayerle J, Drewes AM, Rebours V, Akisik F, Muñoz JED, Neoptolemos JP
Nat Rev Dis Primers 2017 Sep 7;3:17060. doi: 10.1038/nrdp.2017.60. PMID: 28880010
Towards reducing the risk of new onset diabetes after pancreatitis.
Jivanji CJ, Soo DH, Petrov MS
Minerva Gastroenterol Dietol 2017 Sep;63(3):270-284. Epub 2017 Jan 12 doi: 10.23736/S1121-421X.16.02365-5. PMID: 28079345
Pancreas Divisum.
Taj MA, Qureshi S, Ghazanfar S, Siddiqui AR, Niaz SK, Quraishy MS, Shahid M
J Coll Physicians Surg Pak 2016 Feb;26(2):96-9. PMID: 26876393
Stent-guided sphincterotomy.
Siegel JH, Cohen SA, Kasmin FE, Veerappan A
Gastrointest Endosc 1994 Sep-Oct;40(5):567-72. doi: 10.1016/s0016-5107(94)70254-3. PMID: 7988820

Diagnosis

Complications of lipodystrophy syndromes.
Akinci G, Celik M, Akinci B
Presse Med 2021 Nov;50(3):104085. Epub 2021 Oct 30 doi: 10.1016/j.lpm.2021.104085. PMID: 34728268
Chronic pancreatitis.
Kleeff J, Whitcomb DC, Shimosegawa T, Esposito I, Lerch MM, Gress T, Mayerle J, Drewes AM, Rebours V, Akisik F, Muñoz JED, Neoptolemos JP
Nat Rev Dis Primers 2017 Sep 7;3:17060. doi: 10.1038/nrdp.2017.60. PMID: 28880010
Pancreas Divisum.
Taj MA, Qureshi S, Ghazanfar S, Siddiqui AR, Niaz SK, Quraishy MS, Shahid M
J Coll Physicians Surg Pak 2016 Feb;26(2):96-9. PMID: 26876393
Isoniazid-induced recurrent pancreatitis.
Mattioni S, Zamy M, Mechai F, Raynaud JJ, Chabrol A, Aflalo V, Biour M, Bouchaud O
JOP 2012 May 10;13(3):314-6. PMID: 22572141
MR cholangiopancreatography.
Fulcher AS, Turner MA
Radiol Clin North Am 2002 Dec;40(6):1363-76. doi: 10.1016/s0033-8389(02)00046-5. PMID: 12479716

Therapy

Atypical Hemolytic Uremic Syndrome.
Dixon BP, Gruppo RA
Pediatr Clin North Am 2018 Jun;65(3):509-525. doi: 10.1016/j.pcl.2018.02.003. PMID: 29803280
Hyperinsulinism in the Neonate.
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
Maturity-onset diabetes of the young (MODY): an update.
Anık A, Çatlı G, Abacı A, Böber E
J Pediatr Endocrinol Metab 2015 Mar;28(3-4):251-63. doi: 10.1515/jpem-2014-0384. PMID: 25581748
Orlistat, a new lipase inhibitor for the management of obesity.
Heck AM, Yanovski JA, Calis KA
Pharmacotherapy 2000 Mar;20(3):270-9. doi: 10.1592/phco.20.4.270.34882. PMID: 10730683Free PMC Article
Stent-guided sphincterotomy.
Siegel JH, Cohen SA, Kasmin FE, Veerappan A
Gastrointest Endosc 1994 Sep-Oct;40(5):567-72. doi: 10.1016/s0016-5107(94)70254-3. PMID: 7988820

Prognosis

Implications of Postpancreatectomy Hypophosphatemia.
Wong P, White M, Tozzi F, Warner SG, Woo Y, Singh G, Fong Y, Melstrom L
Am Surg 2021 Jan;87(1):61-67. Epub 2020 Sep 13 doi: 10.1177/0003134820949517. PMID: 32924538
Surgical approach to a left-sided gallbladder.
Pereira R, Perera M, Roberts M, Avramovic J
BMJ Case Rep 2019 Aug 15;12(8) doi: 10.1136/bcr-2019-230681. PMID: 31420438Free PMC Article
Atypical Hemolytic Uremic Syndrome.
Dixon BP, Gruppo RA
Pediatr Clin North Am 2018 Jun;65(3):509-525. doi: 10.1016/j.pcl.2018.02.003. PMID: 29803280
Pancreas Divisum.
Taj MA, Qureshi S, Ghazanfar S, Siddiqui AR, Niaz SK, Quraishy MS, Shahid M
J Coll Physicians Surg Pak 2016 Feb;26(2):96-9. PMID: 26876393
Pancreatic panniculitis.
Rongioletti F, Caputo V
G Ital Dermatol Venereol 2013 Aug;148(4):419-25. PMID: 23900163

Clinical prediction guides

Surgical approach to a left-sided gallbladder.
Pereira R, Perera M, Roberts M, Avramovic J
BMJ Case Rep 2019 Aug 15;12(8) doi: 10.1136/bcr-2019-230681. PMID: 31420438Free PMC Article
Towards reducing the risk of new onset diabetes after pancreatitis.
Jivanji CJ, Soo DH, Petrov MS
Minerva Gastroenterol Dietol 2017 Sep;63(3):270-284. Epub 2017 Jan 12 doi: 10.23736/S1121-421X.16.02365-5. PMID: 28079345
HIFU Tissue Ablation: Concept and Devices.
Ter Haar G
Adv Exp Med Biol 2016;880:3-20. doi: 10.1007/978-3-319-22536-4_1. PMID: 26486329
Isoniazid-induced recurrent pancreatitis.
Mattioni S, Zamy M, Mechai F, Raynaud JJ, Chabrol A, Aflalo V, Biour M, Bouchaud O
JOP 2012 May 10;13(3):314-6. PMID: 22572141
Postburn pancreatitis.
Ryan CM, Sheridan RL, Schoenfeld DA, Warshaw AL, Tompkins RG
Ann Surg 1995 Aug;222(2):163-70. doi: 10.1097/00000658-199508000-00008. PMID: 7543741Free PMC Article

Recent systematic reviews

Natural history of Wolcott-Rallison syndrome: A systematic review and follow-up study.
Aldrian D, Bochdansky C, Kavallar AM, Mayerhofer C, Deeb A, Habeb A, Romera Rabasa A, Khadilkar A, Uçar A, Knoppke B, Zafeiriou D, Lang-Muritano M, Miqdady M, Judmaier S, McLin V, Furdela V, Müller T, Vogel GF
Liver Int 2024 Mar;44(3):811-822. Epub 2024 Jan 17 doi: 10.1111/liv.15834. PMID: 38230874
Parathyroidectomy for adults with primary hyperparathyroidism.
Pappachan JM, Lahart IM, Viswanath AK, Borumandi F, Sodi R, Metzendorf MI, Bongaerts B
Cochrane Database Syst Rev 2023 Mar 8;3(3):CD013035. doi: 10.1002/14651858.CD013035.pub2. PMID: 36883976Free PMC Article
The Clinical Characteristics and Gene Mutations of Maturity-Onset Diabetes of the Young Type 5 in Sixty-One Patients.
Ge S, Yang M, Cui Y, Wu J, Xu L, Dong J, Liao L
Front Endocrinol (Lausanne) 2022;13:911526. Epub 2022 Jun 30 doi: 10.3389/fendo.2022.911526. PMID: 35846334Free PMC Article
Non-coding RNAs underlying the pathophysiological links between type 2 diabetes and pancreatic cancer: A systematic review.
Dehghanian F, Azhir Z, Khalilian S, Grüning B
J Diabetes Investig 2022 Mar;13(3):405-428. Epub 2021 Dec 14 doi: 10.1111/jdi.13727. PMID: 34859606Free PMC Article
Premorbid Obesity and Mortality in Patients With Pancreatic Cancer: A Systematic Review and Meta-analysis.
Majumder K, Gupta A, Arora N, Singh PP, Singh S
Clin Gastroenterol Hepatol 2016 Mar;14(3):355-368.e; quiz e32. Epub 2015 Oct 14 doi: 10.1016/j.cgh.2015.09.036. PMID: 26460214Free PMC Article

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