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Diffuse pancreatic islet hyperplasia

MedGen UID:
1616488
•Concept ID:
C4531265
•
Congenital Abnormality
HPO: HP:0031224

Definition

Hyperplasia of the islets of Langerhans with a generalized distribution. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVDiffuse pancreatic islet hyperplasia

Professional guidelines

PubMed

Genotype-histotype-phenotype correlations in hyperinsulinemic hypoglycemia.
Larsen AR, Brusgaard K, Christesen HT, Detlefsen S
Histol Histopathol 2024 Jul;39(7):817-844. Epub 2024 Jan 12 doi: 10.14670/HH-18-709. PMID: 38305063
Differential Morphological Diagnosis of Various Forms of Congenital Hyperinsulinism in Children.
Mitrofanova LB, Perminova AA, Ryzhkova DV, Sukhotskaya AA, Bairov VG, Nikitina IL
Front Endocrinol (Lausanne) 2021;12:710947. Epub 2021 Aug 23 doi: 10.3389/fendo.2021.710947. PMID: 34497584Free PMC Article
Current approach to the management of gastrinoma and insulinoma in adults with multiple endocrine neoplasia type I.
Mignon M, Ruszniewski P, Podevin P, Sabbagh L, Cadiot G, Rigaud D, Bonfils S
World J Surg 1993 Jul-Aug;17(4):489-97. doi: 10.1007/BF01655108. PMID: 8103251

Recent clinical studies

Etiology

Circularity of islets is a distinct marker for the pathological diagnosis of adult non-neoplastic hyperinsulinemic hypoglycemia using surgical specimens.
Nakagawa R, Minamiguchi S, Kataoka TR, Fujikura J, Masui T, Fujimoto M, Yamada Y, Takeuchi Y, Teramoto Y, Ito H, Saka M, Kitamura K, Otsuki S, Nishijima R, Haga H
Diagn Pathol 2023 Oct 20;18(1):115. doi: 10.1186/s13000-023-01403-y. PMID: 37864201Free PMC Article
Biological Assessment of Stevioside and Sucralose as Sucrose Substitutes for Diabetics on STZ-Induced Diabetes in Rats.
Barakat H, Al-Roug K, Algonaiman R, Althwab SA, Alfheeaid HA, Alhomaid RM, Almujaydil MS, Bushnaq T, Ebeid TA
Molecules 2023 Jan 17;28(3) doi: 10.3390/molecules28030940. PMID: 36770608Free PMC Article
Differential Morphological Diagnosis of Various Forms of Congenital Hyperinsulinism in Children.
Mitrofanova LB, Perminova AA, Ryzhkova DV, Sukhotskaya AA, Bairov VG, Nikitina IL
Front Endocrinol (Lausanne) 2021;12:710947. Epub 2021 Aug 23 doi: 10.3389/fendo.2021.710947. PMID: 34497584Free PMC Article
Persistent hyperinsulinaemic hypoglycaemia.
de Lonlay P, Touati G, Robert JJ, Saudubray JM
Semin Neonatol 2002 Feb;7(1):95-100. doi: 10.1053/siny.2001.0090. PMID: 12069542
Clinical features of 52 neonates with hyperinsulinism.
de Lonlay-Debeney P, Poggi-Travert F, Fournet JC, Sempoux C, Dionisi Vici C, Brunelle F, Touati G, Rahier J, Junien C, Nihoul-Fékété C, Robert JJ, Saudubray JM
N Engl J Med 1999 Apr 15;340(15):1169-75. doi: 10.1056/NEJM199904153401505. PMID: 10202168

Diagnosis

Genotype-histotype-phenotype correlations in hyperinsulinemic hypoglycemia.
Larsen AR, Brusgaard K, Christesen HT, Detlefsen S
Histol Histopathol 2024 Jul;39(7):817-844. Epub 2024 Jan 12 doi: 10.14670/HH-18-709. PMID: 38305063
Pancreatic α-cell hyperplasia: facts and myths.
Yu R
J Clin Endocrinol Metab 2014 Mar;99(3):748-56. Epub 2013 Nov 27 doi: 10.1210/jc.2013-2952. PMID: 24285676
Congenital hyperinsulinism.
Arnoux JB, de Lonlay P, Ribeiro MJ, Hussain K, Blankenstein O, Mohnike K, Valayannopoulos V, Robert JJ, Rahier J, Sempoux C, Bellanné C, Verkarre V, Aigrain Y, Jaubert F, Brunelle F, Nihoul-Fékété C
Early Hum Dev 2010 May;86(5):287-94. Epub 2010 Jun 13 doi: 10.1016/j.earlhumdev.2010.05.003. PMID: 20550977
Rare syndromes.
Jabbour SA, Davidovici BB, Wolf R
Clin Dermatol 2006 Jul-Aug;24(4):299-316. doi: 10.1016/j.clindermatol.2006.04.005. PMID: 16828412
Persistent hyperinsulinaemic hypoglycaemia.
de Lonlay P, Touati G, Robert JJ, Saudubray JM
Semin Neonatol 2002 Feb;7(1):95-100. doi: 10.1053/siny.2001.0090. PMID: 12069542

Therapy

Pancreatic α-cell hyperplasia: facts and myths.
Yu R
J Clin Endocrinol Metab 2014 Mar;99(3):748-56. Epub 2013 Nov 27 doi: 10.1210/jc.2013-2952. PMID: 24285676
Severe hypoglycaemia post-gastric bypass requiring partial pancreatectomy: evidence for inappropriate insulin secretion and pancreatic islet hyperplasia.
Patti ME, McMahon G, Mun EC, Bitton A, Holst JJ, Goldsmith J, Hanto DW, Callery M, Arky R, Nose V, Bonner-Weir S, Goldfine AB
Diabetologia 2005 Nov;48(11):2236-40. Epub 2005 Sep 30 doi: 10.1007/s00125-005-1933-x. PMID: 16195867
Pancreatectomy extension in persistent hyperinsulinaemic hypoglycaemia: a new strategy.
Martinez-Ibanez V, Gussinyer M, Toran N, Lloret J, Abad P, Carrascosa A, Boix-Ochoa J
Eur J Pediatr Surg 2002 Aug;12(4):262-6. doi: 10.1055/s-2002-34476. PMID: 12369005
Persistent hyperinsulinaemic hypoglycaemia.
de Lonlay P, Touati G, Robert JJ, Saudubray JM
Semin Neonatol 2002 Feb;7(1):95-100. doi: 10.1053/siny.2001.0090. PMID: 12069542
Insulin-producing islet cell tumors.
Fajans SS, Vinik AI
Endocrinol Metab Clin North Am 1989 Mar;18(1):45-74. PMID: 2537193

Prognosis

Congenital hyperinsulinism.
Arnoux JB, de Lonlay P, Ribeiro MJ, Hussain K, Blankenstein O, Mohnike K, Valayannopoulos V, Robert JJ, Rahier J, Sempoux C, Bellanné C, Verkarre V, Aigrain Y, Jaubert F, Brunelle F, Nihoul-Fékété C
Early Hum Dev 2010 May;86(5):287-94. Epub 2010 Jun 13 doi: 10.1016/j.earlhumdev.2010.05.003. PMID: 20550977
Persistent hyperinsulinaemic hypoglycaemia.
de Lonlay P, Touati G, Robert JJ, Saudubray JM
Semin Neonatol 2002 Feb;7(1):95-100. doi: 10.1053/siny.2001.0090. PMID: 12069542
Current approach to the management of gastrinoma and insulinoma in adults with multiple endocrine neoplasia type I.
Mignon M, Ruszniewski P, Podevin P, Sabbagh L, Cadiot G, Rigaud D, Bonfils S
World J Surg 1993 Jul-Aug;17(4):489-97. doi: 10.1007/BF01655108. PMID: 8103251
Insulin-producing islet cell tumors.
Fajans SS, Vinik AI
Endocrinol Metab Clin North Am 1989 Mar;18(1):45-74. PMID: 2537193
Gastrointestinal/pancreatic hormone concentrations in the portal venous system of nine patients with organic hyperinsulinism.
Glaser B, Valtysson G, Fajans SS, Vinik AI, Cho K, Thompson N
Metabolism 1981 Oct;30(10):1001-10. doi: 10.1016/0026-0495(81)90101-3. PMID: 6116152

Clinical prediction guides

Biological Assessment of Stevioside and Sucralose as Sucrose Substitutes for Diabetics on STZ-Induced Diabetes in Rats.
Barakat H, Al-Roug K, Algonaiman R, Althwab SA, Alfheeaid HA, Alhomaid RM, Almujaydil MS, Bushnaq T, Ebeid TA
Molecules 2023 Jan 17;28(3) doi: 10.3390/molecules28030940. PMID: 36770608Free PMC Article
Adult diffuse nesidioblastosis: genetically or environmentally induced?
Klöppel G, Anlauf M, Raffel A, Perren A, Knoefel WT
Hum Pathol 2008 Jan;39(1):3-8. doi: 10.1016/j.humpath.2007.09.010. PMID: 18070631
p57(KIP2) expression in normal islet cells and in hyperinsulinism of infancy.
Kassem SA, Ariel I, Thornton PS, Hussain K, Smith V, Lindley KJ, Aynsley-Green A, Glaser B
Diabetes 2001 Dec;50(12):2763-9. doi: 10.2337/diabetes.50.12.2763. PMID: 11723059
Current approach to the management of gastrinoma and insulinoma in adults with multiple endocrine neoplasia type I.
Mignon M, Ruszniewski P, Podevin P, Sabbagh L, Cadiot G, Rigaud D, Bonfils S
World J Surg 1993 Jul-Aug;17(4):489-97. doi: 10.1007/BF01655108. PMID: 8103251
Pancreatic nesidioblastosis in adults.
Fong TL, Warner NE, Kumar D
Diabetes Care 1989 Feb;12(2):108-14. doi: 10.2337/diacare.12.2.108. PMID: 2649323

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