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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

Metabolic Consequences of Pediatric Obesity: A Review of Pathophysiology, Screening, and Treatment.
Gunaratne N, Deplewski D
Pediatr Ann 2023 Feb;52(2):e62-e67. Epub 2023 Feb 1 doi: 10.3928/19382359-20230102-06. PMID: 36779885
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
Does recombinant insulin-like growth factor I have a role in the treatment of insulin-dependent diabetes mellitus during adolescence?
Dunger DB, Cheetham TD, Holly JM, Matthews DR
Acta Paediatr Suppl 1993 Mar;388:49-52; discussion 53. doi: 10.1111/j.1651-2227.1993.tb12841.x. PMID: 8329831

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
The epidemiology of hidradenitis suppurativa.
Ingram JR
Br J Dermatol 2020 Dec;183(6):990-998. Epub 2020 Sep 3 doi: 10.1111/bjd.19435. PMID: 32880911
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
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
Epidemiology of Uterine Fibroids: From Menarche to Menopause.
Wise LA, Laughlin-Tommaso SK
Clin Obstet Gynecol 2016 Mar;59(1):2-24. doi: 10.1097/GRF.0000000000000164. PMID: 26744813Free PMC Article

Diagnosis

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
Endocrinology and metabolism.
Root AW
Curr Opin Pediatr 2008 Aug;20(4):446-7. doi: 10.1097/MOP.0b013e328305e44b. PMID: 18622202
Lipodystrophies.
Garg A
Am J Med 2000 Feb;108(2):143-52. doi: 10.1016/s0002-9343(99)00414-3. PMID: 11126308
Polycystic ovary syndrome.
Goudas VT, Dumesic DA
Endocrinol Metab Clin North Am 1997 Dec;26(4):893-912. doi: 10.1016/s0889-8529(05)70286-3. PMID: 9429864

Therapy

Endocrinologic Aspects of Hidradenitis Suppurativa.
Karagiannidis I, Nikolakis G, Zouboulis CC
Dermatol Clin 2016 Jan;34(1):45-9. doi: 10.1016/j.det.2015.08.005. PMID: 26617357
Use of metformin in pediatric age.
Brufani C, Fintini D, Nobili V, Patera PI, Cappa M, Brufani M
Pediatr Diabetes 2011 Sep;12(6):580-8. Epub 2011 Mar 3 doi: 10.1111/j.1399-5448.2010.00741.x. PMID: 21366813
The influence of female sex steroids on glucose metabolism and insulin action.
Godsland IF
J Intern Med Suppl 1996;738:1-60. PMID: 9010389
Does recombinant insulin-like growth factor I have a role in the treatment of insulin-dependent diabetes mellitus during adolescence?
Dunger DB, Cheetham TD, Holly JM, Matthews DR
Acta Paediatr Suppl 1993 Mar;388:49-52; discussion 53. doi: 10.1111/j.1651-2227.1993.tb12841.x. PMID: 8329831
Diabetes in puberty.
Dunger DB
Arch Dis Child 1992 May;67(5):569-70. doi: 10.1136/adc.67.5.569. PMID: 1599288Free PMC Article

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
Epidemiology of Uterine Fibroids: From Menarche to Menopause.
Wise LA, Laughlin-Tommaso SK
Clin Obstet Gynecol 2016 Mar;59(1):2-24. doi: 10.1097/GRF.0000000000000164. PMID: 26744813Free PMC Article
The Effects of Maternal Obesity on Neonates, Infants, Children, Adolescents, and Adults.
Hemond J, Robbins RB, Young PC
Clin Obstet Gynecol 2016 Mar;59(1):216-27. doi: 10.1097/GRF.0000000000000179. PMID: 26694497

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
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
Endocrinologic Aspects of Hidradenitis Suppurativa.
Karagiannidis I, Nikolakis G, Zouboulis CC
Dermatol Clin 2016 Jan;34(1):45-9. doi: 10.1016/j.det.2015.08.005. PMID: 26617357
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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