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Insulin-resistant diabetes mellitus at puberty

MedGen UID:
373411
•Concept ID:
C1837792
•
Disease or Syndrome; Finding
HPO: HP:0000877

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVInsulin-resistant diabetes mellitus at puberty

Conditions with this feature

Congenital generalized lipodystrophy type 1
MedGen UID:
318592
•Concept ID:
C1720862
•
Disease or Syndrome
Berardinelli-Seip congenital lipodystrophy (BSCL) is usually diagnosed at birth or soon thereafter. Because of the absence of functional adipocytes, lipid is stored in other tissues, including muscle and liver. Affected individuals develop insulin resistance and approximately 25%-35% develop diabetes mellitus between ages 15 and 20 years. Hepatomegaly secondary to hepatic steatosis and skeletal muscle hypertrophy occur in all affected individuals. Hypertrophic cardiomyopathy is reported in 20%-25% of affected individuals and is a significant cause of morbidity from cardiac failure and early mortality.
See: Condition Record
Congenital generalized lipodystrophy type 2
MedGen UID:
318593
•Concept ID:
C1720863
•
Congenital Abnormality
Berardinelli-Seip congenital lipodystrophy (BSCL) is usually diagnosed at birth or soon thereafter. Because of the absence of functional adipocytes, lipid is stored in other tissues, including muscle and liver. Affected individuals develop insulin resistance and approximately 25%-35% develop diabetes mellitus between ages 15 and 20 years. Hepatomegaly secondary to hepatic steatosis and skeletal muscle hypertrophy occur in all affected individuals. Hypertrophic cardiomyopathy is reported in 20%-25% of affected individuals and is a significant cause of morbidity from cardiac failure and early mortality.
See: Condition Record
Congenital generalized lipodystrophy type 1
Congenital generalized lipodystrophy type 2

Professional guidelines

PubMed

Cardiovascular and Metabolic Complications - Diagnosis and Management in Obese Children.
Vikram NK
Indian J Pediatr 2018 Jul;85(7):535-545. Epub 2017 Dec 8 doi: 10.1007/s12098-017-2504-0. PMID: 29218646
Diagnosis and treatment of late-onset hypogonadism: systematic review and meta-analysis of TRT outcomes.
Corona G, Rastrelli G, Maggi M
Best Pract Res Clin Endocrinol Metab 2013 Aug;27(4):557-79. Epub 2013 Jul 5 doi: 10.1016/j.beem.2013.05.002. PMID: 24054931
Consensus statement on diagnosis and clinical management of Klinefelter syndrome.
Radicioni AF, Ferlin A, Balercia G, Pasquali D, Vignozzi L, Maggi M, Foresta C, Lenzi A
J Endocrinol Invest 2010 Dec;33(11):839-50. doi: 10.1007/BF03350351. PMID: 21293172

Recent clinical studies

Etiology

Stress and Diabetes Mellitus: Pathogenetic Mechanisms and Clinical Outcome.
Ingrosso DMF, Primavera M, Samvelyan S, Tagi VM, Chiarelli F
Horm Res Paediatr 2023;96(1):34-43. Epub 2022 Feb 4 doi: 10.1159/000522431. PMID: 35124671
Spectrum of Phenotypes and Causes of Type 2 Diabetes in Children.
Shah AS, Nadeau KJ, Dabelea D, Redondo MJ
Annu Rev Med 2022 Jan 27;73:501-515. doi: 10.1146/annurev-med-042120-012033. PMID: 35084995Free PMC Article
Hyperglycemia During Pregnancy and Long-Term Offspring Outcomes.
Bianco ME, Josefson JL
Curr Diab Rep 2019 Nov 21;19(12):143. doi: 10.1007/s11892-019-1267-6. PMID: 31754898Free PMC Article
Body composition and insulin resistance in children.
Maffeis C, Morandi A
Eur J Clin Nutr 2018 Sep;72(9):1239-1245. Epub 2018 Sep 5 doi: 10.1038/s41430-018-0239-2. PMID: 30185840
Insulin Resistance of Puberty.
Kelsey MM, Zeitler PS
Curr Diab Rep 2016 Jul;16(7):64. doi: 10.1007/s11892-016-0751-5. PMID: 27179965

Diagnosis

Body composition and insulin resistance in children.
Maffeis C, Morandi A
Eur J Clin Nutr 2018 Sep;72(9):1239-1245. Epub 2018 Sep 5 doi: 10.1038/s41430-018-0239-2. PMID: 30185840
Hypogonadotropic Hypogonadism in Men With Diabesity.
Dhindsa S, Ghanim H, Batra M, Dandona P
Diabetes Care 2018 Jul;41(7):1516-1525. doi: 10.2337/dc17-2510. PMID: 29934480Free PMC Article
Insulin Resistance of Puberty.
Kelsey MM, Zeitler PS
Curr Diab Rep 2016 Jul;16(7):64. doi: 10.1007/s11892-016-0751-5. PMID: 27179965
Rabson-Mendenhall syndrome.
Gupta J, Daniel JM, Vasudevan V
J Indian Soc Pedod Prev Dent 2012 Jul-Sep;30(3):279-82. doi: 10.4103/0970-4388.105026. PMID: 23263437
Lipodystrophies.
Garg A
Am J Med 2000 Feb;108(2):143-52. doi: 10.1016/s0002-9343(99)00414-3. PMID: 11126308

Therapy

The link between obesity and puberty: what is new?
Huang A, Roth CL
Curr Opin Pediatr 2021 Aug 1;33(4):449-457. doi: 10.1097/MOP.0000000000001035. PMID: 34173790
Insulin resistant diabetes mellitus in SHORT syndrome: case report and literature review.
Masunaga Y, Fujisawa Y, Muramatsu M, Ono H, Inoue T, Fukami M, Kagami M, Saitsu H, Ogata T
Endocr J 2021 Jan 28;68(1):111-117. Epub 2020 Sep 3 doi: 10.1507/endocrj.EJ20-0291. PMID: 32879144
Pediatric Type 2 Diabetes: Not a Mini Version of Adult Type 2 Diabetes.
Savic Hitt TA, Katz LEL
Endocrinol Metab Clin North Am 2020 Dec;49(4):679-693. Epub 2020 Oct 14 doi: 10.1016/j.ecl.2020.08.003. PMID: 33153674Free PMC Article
Puberty and ovarian function in girls with type 1 diabetes mellitus.
Codner E, Cassorla F
Horm Res 2009 Jan;71(1):12-21. Epub 2008 Nov 27 doi: 10.1159/000173737. PMID: 19039232
Childhood and adolescent diabetes.
Dunger DB, Regan FM, Acerini CL
Endocr Dev 2005;9:107-120. doi: 10.1159/000085761. PMID: 15879693

Prognosis

The Role of Kisspeptin in the Pathogenesis of Pregnancy Complications: A Narrative Review.
Szydełko-Gorzkowicz M, Poniedziałek-Czajkowska E, Mierzyński R, Sotowski M, Leszczyńska-Gorzelak B
Int J Mol Sci 2022 Jun 14;23(12) doi: 10.3390/ijms23126611. PMID: 35743054Free PMC Article
Spectrum of Phenotypes and Causes of Type 2 Diabetes in Children.
Shah AS, Nadeau KJ, Dabelea D, Redondo MJ
Annu Rev Med 2022 Jan 27;73:501-515. doi: 10.1146/annurev-med-042120-012033. PMID: 35084995Free PMC Article
Glucose tolerance and insulin sensitivity markers in children and adolescents with excess weight.
Kostopoulou E, Tikka M, Rojas Gil AP, Partsalaki I, Spiliotis BE
Eur Rev Med Pharmacol Sci 2021 Oct;25(19):5986-5992. doi: 10.26355/eurrev_202110_26876. PMID: 34661258
Sex Hormone-Binding Globulin in Children and Adolescents.
Aydın B, Winters SJ
J Clin Res Pediatr Endocrinol 2016 Mar 5;8(1):1-12. Epub 2015 Jan 18 doi: 10.4274/jcrpe.2764. PMID: 26761949Free PMC Article
Growth and body composition in type 1 diabetes mellitus.
Dunger D, Ahmed L, Ong K
Horm Res 2002;58 Suppl 1:66-71. doi: 10.1159/000064762. PMID: 12373017

Clinical prediction guides

Spectrum of Phenotypes and Causes of Type 2 Diabetes in Children.
Shah AS, Nadeau KJ, Dabelea D, Redondo MJ
Annu Rev Med 2022 Jan 27;73:501-515. doi: 10.1146/annurev-med-042120-012033. PMID: 35084995Free PMC Article
Type 2 diabetes in prepubertal children.
Astudillo M, Tosur M, Castillo B, Rafaey A, Siller AF, Nieto J, Sisley S, McKay S, Nella AA, Balasubramanyam A, Bacha F, Redondo MJ
Pediatr Diabetes 2021 Nov;22(7):946-950. Epub 2021 Sep 2 doi: 10.1111/pedi.13254. PMID: 34363430
Hyperglycemia During Pregnancy and Long-Term Offspring Outcomes.
Bianco ME, Josefson JL
Curr Diab Rep 2019 Nov 21;19(12):143. doi: 10.1007/s11892-019-1267-6. PMID: 31754898Free PMC Article
Hypogonadotropic Hypogonadism in Men With Diabesity.
Dhindsa S, Ghanim H, Batra M, Dandona P
Diabetes Care 2018 Jul;41(7):1516-1525. doi: 10.2337/dc17-2510. PMID: 29934480Free PMC Article
Puberty and ovarian function in girls with type 1 diabetes mellitus.
Codner E, Cassorla F
Horm Res 2009 Jan;71(1):12-21. Epub 2008 Nov 27 doi: 10.1159/000173737. PMID: 19039232

Recent systematic reviews

IGF-1 and IGF-2 as Molecules Linked to Causes and Consequences of Obesity from Fetal Life to Adulthood: A Systematic Review.
Szydlowska-Gladysz J, Gorecka AE, Stepien J, Rysz I, Ben-Skowronek I
Int J Mol Sci 2024 Apr 2;25(7) doi: 10.3390/ijms25073966. PMID: 38612776Free PMC Article
Clinical Spectrum of LMNA-Associated Type 2 Familial Partial Lipodystrophy: A Systematic Review.
Fernandez-Pombo A, Diaz-Lopez EJ, Castro AI, Sanchez-Iglesias S, Cobelo-Gomez S, Prado-Moraña T, Araujo-Vilar D
Cells 2023 Feb 24;12(5) doi: 10.3390/cells12050725. PMID: 36899861Free PMC Article
Diagnosis and treatment of late-onset hypogonadism: systematic review and meta-analysis of TRT outcomes.
Corona G, Rastrelli G, Maggi M
Best Pract Res Clin Endocrinol Metab 2013 Aug;27(4):557-79. Epub 2013 Jul 5 doi: 10.1016/j.beem.2013.05.002. PMID: 24054931

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