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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

IUGR: Genetic influences, metabolic problems, environmental associations/triggers, current and future management.
Darendeliler F
Best Pract Res Clin Endocrinol Metab 2019 Jun;33(3):101260. Epub 2019 Jan 22 doi: 10.1016/j.beem.2019.01.001. PMID: 30709755
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

Recent clinical studies

Etiology

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
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
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

Diagnosis

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
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
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

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
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
Childhood obesity and adult morbidities.
Biro FM, Wien M
Am J Clin Nutr 2010 May;91(5):1499S-1505S. Epub 2010 Mar 24 doi: 10.3945/ajcn.2010.28701B. PMID: 20335542Free PMC Article
Treatment of PCOS in adolescence.
Warren-Ulanch J, Arslanian S
Best Pract Res Clin Endocrinol Metab 2006 Jun;20(2):311-30. doi: 10.1016/j.beem.2006.02.002. PMID: 16772160
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

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
Type 2 diabetes mellitus in the pediatric population.
Gaylor AS, Condren ME
Pharmacotherapy 2004 Jul;24(7):871-8. doi: 10.1592/phco.24.9.871.36099. PMID: 15303451
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
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
Estrogen and type 1 diabetes mellitus.
Codner E
Pediatr Endocrinol Rev 2008 Dec;6(2):228-34. PMID: 19202509

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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