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Transient Hyperinsulinism

MedGen UID:
1381549
•Concept ID:
C4331426
•
Disease or Syndrome

Definition

Hyperinsulinism that resolves spontaneously. This is the most common cause of neonatal hypoglycemia. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVTransient Hyperinsulinism

Professional guidelines

PubMed

Increased referrals for congenital hyperinsulinism genetic testing in children with trisomy 21 reflects the high burden of non-genetic risk factors in this group.
Hewat TI, Laver TW, Houghton JAL, Männistö JME, Alvi S, Brearey SP, Cody D, Dastamani A, De Los Santos La Torre M, Murphy N, Rami-Merhar B, Wefers B, Huopio H, Banerjee I, Johnson MB, Flanagan SE
Pediatr Diabetes 2022 Jun;23(4):457-461. Epub 2022 Mar 23 doi: 10.1111/pedi.13333. PMID: 35294086Free PMC Article
Challenges in management of transient hyperinsulinism - a retrospective analysis of 36 severely affected children.
Hoermann H, Roeper M, Salimi Dafsari R, Koestner F, Reinauer C, Mayatepek E, Meissner T, Kummer S
J Pediatr Endocrinol Metab 2021 Jul 27;34(7):867-875. Epub 2021 Apr 16 doi: 10.1515/jpem-2020-0639. PMID: 33860651
Higher C-peptide levels and glucose requirements may identify neonates with transient hyperinsulinism hypoglycemia who will benefit from diazoxide treatment.
Davidov AS, Elkon-Tamir E, Haham A, Shefer G, Weintrob N, Oren A, Lebenthal Y, Mandel D, Eyal O
Eur J Pediatr 2020 Apr;179(4):597-602. Epub 2019 Dec 20 doi: 10.1007/s00431-019-03544-z. PMID: 31863303

Recent clinical studies

Etiology

Delayed-Onset Transient Hyperinsulinism in Infants with Very Low and Extremely Low Birth Weights: A Cohort Study.
Hoermann H, Roeper M, Welters A, Baertling F, Mayatepek E, Meissner T, Kummer S
J Pediatr 2023 Jul;258:113399. Epub 2023 Apr 3 doi: 10.1016/j.jpeds.2023.113399. PMID: 37019330
Transient neonatal hyperinsulinism: early predictors of duration.
Fuchs S, Regev R, Harrington J
J Pediatr Endocrinol Metab 2021 Aug 26;34(8):1041-1044. Epub 2021 Apr 16 doi: 10.1515/jpem-2020-0594. PMID: 33866697
The effect of hypoglycaemia on neurocognitive outcome in children and adolescents with transient or persistent congenital hyperinsulinism.
Muukkonen L, Männistö J, Jääskeläinen J, Hannonen R, Huopio H
Dev Med Child Neurol 2019 Apr;61(4):451-457. Epub 2018 Sep 23 doi: 10.1111/dmcn.14039. PMID: 30246438
Association of maternal nutrition with transient neonatal hyperinsulinism.
Louvigne M, Rouleau S, Caldagues E, Souto I, Montcho Y, Bouvagnet AM, Baud O, Carel JC, Gascoin G, Coutant R
PLoS One 2018;13(5):e0195383. Epub 2018 May 3 doi: 10.1371/journal.pone.0195383. PMID: 29723237Free 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

Diagnosis

Delayed-Onset Transient Hyperinsulinism in Infants with Very Low and Extremely Low Birth Weights: A Cohort Study.
Hoermann H, Roeper M, Welters A, Baertling F, Mayatepek E, Meissner T, Kummer S
J Pediatr 2023 Jul;258:113399. Epub 2023 Apr 3 doi: 10.1016/j.jpeds.2023.113399. PMID: 37019330
Transient neonatal hyperinsulinism: early predictors of duration.
Fuchs S, Regev R, Harrington J
J Pediatr Endocrinol Metab 2021 Aug 26;34(8):1041-1044. Epub 2021 Apr 16 doi: 10.1515/jpem-2020-0594. PMID: 33866697
Association of maternal nutrition with transient neonatal hyperinsulinism.
Louvigne M, Rouleau S, Caldagues E, Souto I, Montcho Y, Bouvagnet AM, Baud O, Carel JC, Gascoin G, Coutant R
PLoS One 2018;13(5):e0195383. Epub 2018 May 3 doi: 10.1371/journal.pone.0195383. PMID: 29723237Free 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
Heterogeneity of persistent hyperinsulinaemic hypoglycaemia. A series of 175 cases.
de Lonlay P, Fournet JC, Touati G, Groos MS, Martin D, Sevin C, Delagne V, Mayaud C, Chigot V, Sempoux C, Brusset MC, Laborde K, Bellane-Chantelot C, Vassault A, Rahier J, Junien C, Brunelle F, Nihoul-Fékété C, Saudubray JM, Robert JJ
Eur J Pediatr 2002 Jan;161(1):37-48. doi: 10.1007/s004310100847. PMID: 11808879

Therapy

Low-dose diazoxide is safe and effective in infants with transient hyperinsulinism.
Malhotra N, Yau D, Cunjamalay A, Gunasekara B, S A, Gilbert C, Morgan K, Dattani M, Dastamani A
Clin Endocrinol (Oxf) 2024 Feb;100(2):132-137. Epub 2023 Dec 7 doi: 10.1111/cen.14987. PMID: 38059644
Challenges in management of transient hyperinsulinism - a retrospective analysis of 36 severely affected children.
Hoermann H, Roeper M, Salimi Dafsari R, Koestner F, Reinauer C, Mayatepek E, Meissner T, Kummer S
J Pediatr Endocrinol Metab 2021 Jul 27;34(7):867-875. Epub 2021 Apr 16 doi: 10.1515/jpem-2020-0639. PMID: 33860651
Higher C-peptide levels and glucose requirements may identify neonates with transient hyperinsulinism hypoglycemia who will benefit from diazoxide treatment.
Davidov AS, Elkon-Tamir E, Haham A, Shefer G, Weintrob N, Oren A, Lebenthal Y, Mandel D, Eyal O
Eur J Pediatr 2020 Apr;179(4):597-602. Epub 2019 Dec 20 doi: 10.1007/s00431-019-03544-z. PMID: 31863303
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
Heterogeneity of persistent hyperinsulinaemic hypoglycaemia. A series of 175 cases.
de Lonlay P, Fournet JC, Touati G, Groos MS, Martin D, Sevin C, Delagne V, Mayaud C, Chigot V, Sempoux C, Brusset MC, Laborde K, Bellane-Chantelot C, Vassault A, Rahier J, Junien C, Brunelle F, Nihoul-Fékété C, Saudubray JM, Robert JJ
Eur J Pediatr 2002 Jan;161(1):37-48. doi: 10.1007/s004310100847. PMID: 11808879

Prognosis

Transient neonatal hyperinsulinism: early predictors of duration.
Fuchs S, Regev R, Harrington J
J Pediatr Endocrinol Metab 2021 Aug 26;34(8):1041-1044. Epub 2021 Apr 16 doi: 10.1515/jpem-2020-0594. PMID: 33866697
Higher C-peptide levels and glucose requirements may identify neonates with transient hyperinsulinism hypoglycemia who will benefit from diazoxide treatment.
Davidov AS, Elkon-Tamir E, Haham A, Shefer G, Weintrob N, Oren A, Lebenthal Y, Mandel D, Eyal O
Eur J Pediatr 2020 Apr;179(4):597-602. Epub 2019 Dec 20 doi: 10.1007/s00431-019-03544-z. PMID: 31863303
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
Free and total insulin-like growth factor (IGF)-I levels decline during fasting: relationships with insulin and IGF-binding protein-1.
Katz LE, DeLeón DD, Zhao H, Jawad AF
J Clin Endocrinol Metab 2002 Jun;87(6):2978-83. doi: 10.1210/jcem.87.6.8601. PMID: 12050283

Clinical prediction guides

Increased referrals for congenital hyperinsulinism genetic testing in children with trisomy 21 reflects the high burden of non-genetic risk factors in this group.
Hewat TI, Laver TW, Houghton JAL, Männistö JME, Alvi S, Brearey SP, Cody D, Dastamani A, De Los Santos La Torre M, Murphy N, Rami-Merhar B, Wefers B, Huopio H, Banerjee I, Johnson MB, Flanagan SE
Pediatr Diabetes 2022 Jun;23(4):457-461. Epub 2022 Mar 23 doi: 10.1111/pedi.13333. PMID: 35294086Free PMC Article
Transient neonatal hyperinsulinism: early predictors of duration.
Fuchs S, Regev R, Harrington J
J Pediatr Endocrinol Metab 2021 Aug 26;34(8):1041-1044. Epub 2021 Apr 16 doi: 10.1515/jpem-2020-0594. PMID: 33866697
Higher C-peptide levels and glucose requirements may identify neonates with transient hyperinsulinism hypoglycemia who will benefit from diazoxide treatment.
Davidov AS, Elkon-Tamir E, Haham A, Shefer G, Weintrob N, Oren A, Lebenthal Y, Mandel D, Eyal O
Eur J Pediatr 2020 Apr;179(4):597-602. Epub 2019 Dec 20 doi: 10.1007/s00431-019-03544-z. PMID: 31863303
Association of maternal nutrition with transient neonatal hyperinsulinism.
Louvigne M, Rouleau S, Caldagues E, Souto I, Montcho Y, Bouvagnet AM, Baud O, Carel JC, Gascoin G, Coutant R
PLoS One 2018;13(5):e0195383. Epub 2018 May 3 doi: 10.1371/journal.pone.0195383. PMID: 29723237Free PMC Article
Fetal adaptations in insulin secretion result from high catecholamines during placental insufficiency.
Limesand SW, Rozance PJ
J Physiol 2017 Aug 1;595(15):5103-5113. Epub 2017 May 26 doi: 10.1113/JP273324. PMID: 28194805Free PMC Article

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