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Nesidioblastosis

MedGen UID:
45047
Concept ID:
C0027773
Disease or Syndrome
Synonym: Islet cell hyperplasia
SNOMED CT: Pancreatic endocrine cell hyperplasia (42681006); Islet cell hyperplasia (42681006); Nesidioblastosis (42681006)
 
HPO: HP:0034346
OMIM®: 256450; 601820

Definition

An abnormality of pancreatic beta cells characterized by a proliferation of abnormal beta cells throughout the entire pancreas, with enlarged islet size and number (hypertrophic islets), increased periductular islets, enlarged beta-cell nuclei and abundant clear cytoplasm. Occasionally beta cells with pleomorphic nuclei, ductuloinsular complexes, and neoformation of islets from ducts are observed. [from HPO]

Conditions with this feature

Hyperinsulinemic hypoglycemia, familial, 2
MedGen UID:
419173
Concept ID:
C2931833
Disease or Syndrome
Congenital hyperinsulinism is a condition that causes individuals to have abnormally high levels of insulin. Insulin is a hormone that helps control levels of blood glucose, also called blood sugar. People with this condition have frequent episodes of low blood glucose (hypoglycemia). In infants and young children, these episodes are characterized by a lack of energy (lethargy), irritability, or difficulty feeding. Repeated episodes of low blood glucose increase the risk for serious complications such as breathing difficulties, seizures, intellectual disability, vision loss, brain damage, and coma.\n\nThe severity of congenital hyperinsulinism varies widely among affected individuals, even among members of the same family. About 60 percent of infants with this condition experience a hypoglycemic episode within the first month of life. Other affected children develop hypoglycemia by early childhood. Unlike typical episodes of hypoglycemia, which occur most often after periods without food (fasting) or after exercising, episodes of hypoglycemia in people with congenital hyperinsulinism can also occur after eating.

Professional guidelines

PubMed

Pattison DA, Hicks RJ
Endocr Relat Cancer 2017 Jun;24(6):R203-R221. Epub 2017 Apr 11 doi: 10.1530/ERC-17-0005. PMID: 28400403
Malik S, Mitchell JE, Steffen K, Engel S, Wiisanen R, Garcia L, Malik SA
Obes Res Clin Pract 2016 Jan-Feb;10(1):1-14. Epub 2015 Oct 27 doi: 10.1016/j.orcp.2015.07.003. PMID: 26522879Free PMC Article
Kaplan EL, Arganini M, Kang SJ
Surg Clin North Am 1987 Apr;67(2):395-410. doi: 10.1016/s0039-6109(16)44191-5. PMID: 3551151

Recent clinical studies

Etiology

Kattamanchi D, Maralusiddappa PGC, Manne Veerabhadraiah K, Mangalgi S
BMJ Case Rep 2024 Sep 12;17(9) doi: 10.1136/bcr-2024-261701. PMID: 39266029
Christ E, Antwi K, Fani M, Wild D
Endocr Relat Cancer 2020 Apr;27(4):R79-R92. doi: 10.1530/ERC-19-0476. PMID: 31951592Free PMC Article
Gilis-Januszewska A, Piątkowski J, Skalniak A, Piwońska-Solska B, Nazim J, Pach D, Przybylik-Mazurek E, Sowa-Staszczak A, Starzyk J, Hubalewska-Dydejczyk A
Endokrynol Pol 2015;66(4):344-54. doi: 10.5603/EP.2015.0044. PMID: 26323472
Dacou-Voutetakis C, Psychou F, Maniati-Christidis M
J Pediatr Endocrinol Metab 1998 Mar;11 Suppl 1:131-41. doi: 10.1515/jpem.1998.11.s1.131. PMID: 9642651
Stanley CA
Pediatr Clin North Am 1997 Apr;44(2):363-74. doi: 10.1016/s0031-3955(05)70481-8. PMID: 9130925

Diagnosis

Rariy CM, Rometo D, Korytkowski M
Curr Diab Rep 2016 Feb;16(2):19. doi: 10.1007/s11892-015-0711-5. PMID: 26868861
Malik S, Mitchell JE, Steffen K, Engel S, Wiisanen R, Garcia L, Malik SA
Obes Res Clin Pract 2016 Jan-Feb;10(1):1-14. Epub 2015 Oct 27 doi: 10.1016/j.orcp.2015.07.003. PMID: 26522879Free PMC Article
Krampitz GW, Norton JA
Curr Probl Surg 2013 Nov;50(11):509-45. doi: 10.1067/j.cpsurg.2013.08.001. PMID: 24206780
Seki S, Ikenoue T, Murakami N, Hokanishi H, Kikuura T, Tokunaga M, Akiyama H, Hayakawa K
Acta Paediatr Jpn 1990 Jun;32(3):308-10. doi: 10.1111/j.1442-200x.1990.tb00829.x. PMID: 2239304
Low LC, Yu EC, Chow OK, Yeung CY, Young RT
Aust Paediatr J 1989 Jun;25(3):174-7. doi: 10.1111/j.1440-1754.1989.tb01446.x. PMID: 2764841

Therapy

Rariy CM, Rometo D, Korytkowski M
Curr Diab Rep 2016 Feb;16(2):19. doi: 10.1007/s11892-015-0711-5. PMID: 26868861
Malik S, Mitchell JE, Steffen K, Engel S, Wiisanen R, Garcia L, Malik SA
Obes Res Clin Pract 2016 Jan-Feb;10(1):1-14. Epub 2015 Oct 27 doi: 10.1016/j.orcp.2015.07.003. PMID: 26522879Free PMC Article
Krampitz GW, Norton JA
Curr Probl Surg 2013 Nov;50(11):509-45. doi: 10.1067/j.cpsurg.2013.08.001. PMID: 24206780
Stanley CA
Pediatr Clin North Am 1997 Apr;44(2):363-74. doi: 10.1016/s0031-3955(05)70481-8. PMID: 9130925
Low LC, Yu EC, Chow OK, Yeung CY, Young RT
Aust Paediatr J 1989 Jun;25(3):174-7. doi: 10.1111/j.1440-1754.1989.tb01446.x. PMID: 2764841

Prognosis

Kattamanchi D, Maralusiddappa PGC, Manne Veerabhadraiah K, Mangalgi S
BMJ Case Rep 2024 Sep 12;17(9) doi: 10.1136/bcr-2024-261701. PMID: 39266029
Dravecka I, Lazurova I
Neoplasma 2014;61(3):252-6. doi: 10.4149/neo_2014_047. PMID: 24645840
Krampitz GW, Norton JA
Curr Probl Surg 2013 Nov;50(11):509-45. doi: 10.1067/j.cpsurg.2013.08.001. PMID: 24206780
Stanley CA
Pediatr Clin North Am 1997 Apr;44(2):363-74. doi: 10.1016/s0031-3955(05)70481-8. PMID: 9130925
Zuppinger K
Prog Pediatr Surg 1983;16:51-61. PMID: 6308714

Clinical prediction guides

Al-Ameer A, Alsomali A, Habib Z
Pediatr Surg Int 2023 Apr 20;39(1):183. doi: 10.1007/s00383-023-05470-6. PMID: 37079145
Sempoux C, Poggi F, Brunelle F, Saudubray JM, Fekete C, Rahier J
Diabete Metab 1995 Dec;21(6):402-7. PMID: 8593920
Seki S, Ikenoue T, Murakami N, Hokanishi H, Kikuura T, Tokunaga M, Akiyama H, Hayakawa K
Acta Paediatr Jpn 1990 Jun;32(3):308-10. doi: 10.1111/j.1442-200x.1990.tb00829.x. PMID: 2239304
Low LC, Yu EC, Chow OK, Yeung CY, Young RT
Aust Paediatr J 1989 Jun;25(3):174-7. doi: 10.1111/j.1440-1754.1989.tb01446.x. PMID: 2764841
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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