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Lipoatrophic diabetes

MedGen UID:
4256
•Concept ID:
C0011859
•
Disease or Syndrome
Synonyms: Diabete, Lipoatrophic; Diabetes Mellitus, Lipoatrophic; Diabetes, Lipoatrophic; Lipoatrophic Diabete; Lipoatrophic Diabetes; Lipoatrophic Diabetes Mellitus
SNOMED CT: Lipodystrophic diabetes (127012008); Lipoatrophic diabetes (127012008)
 
Monarch Initiative: MONDO:0005827

Definition

A rare syndrome characterized by almost complete absence of body fat, accentuated muscularity, insulin-resistant diabetes, hyperlipidemia, hepatomegaly, and hypermetabolism. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVLipoatrophic diabetes
Follow this link to review classifications for Lipoatrophic diabetes in Orphanet.

Professional guidelines

PubMed

Problems in differential diagnosis of diabetes types.
Małecki M, Skupień J
Pol Arch Med Wewn 2008 Jul-Aug;118(7-8):435-40. PMID: 18714740
The thrifty genotype in type 2 diabetes: an unfinished symphony moving to its finale?
Joffe B, Zimmet P
Endocrine 1998 Oct;9(2):139-41. doi: 10.1385/ENDO:9:2:139. PMID: 9867247
Letter: Management of lipoatrophic diabetes.
Upton GV, Corbin A, Mabry CC, Hollingsworth DR
N Engl J Med 1975 Mar 13;292(11):592. doi: 10.1056/NEJM197503132921115. PMID: 1110678

Curated

UK NICE Guideline NG19, Diabetic foot problems: prevention and management, 2019

Recent clinical studies

Etiology

Lipoatrophic diabetes in familial partial lipodystrophy type 2: From insulin resistance to diabetes.
Treiber G, Guilleux A, Huynh K, Bonfanti O, Flaus-Furmaniuk A, Couret D, Mellet N, Bernard C, Le-Moullec N, Doray B, Jéru I, Maiza JC, Domun B, Cogne M, Meilhac O, Vigouroux C, Meikle PJ, Nobécourt E
Diabetes Metab 2023 Mar;49(2):101409. Epub 2022 Nov 15 doi: 10.1016/j.diabet.2022.101409. PMID: 36400409
Generalized lipoatrophy syndromes.
Sorkina E, Chichkova V
Presse Med 2021 Nov;50(3):104075. Epub 2021 Sep 22 doi: 10.1016/j.lpm.2021.104075. PMID: 34562560
Problems in differential diagnosis of diabetes types.
Małecki M, Skupień J
Pol Arch Med Wewn 2008 Jul-Aug;118(7-8):435-40. PMID: 18714740
Diseases of adipose tissue: genetic and acquired lipodystrophies.
Capeau J, Magré J, Lascols O, Caron M, Béréziat V, Vigouroux C, Bastard JP
Biochem Soc Trans 2005 Nov;33(Pt 5):1073-7. doi: 10.1042/BST0331073. PMID: 16246048
Lipoatrophy revisited.
Reitman ML, Arioglu E, Gavrilova O, Taylor SI
Trends Endocrinol Metab 2000 Dec;11(10):410-6. doi: 10.1016/s1043-2760(00)00309-x. PMID: 11091118

Diagnosis

Lipoatrophic diabetes in familial partial lipodystrophy type 2: From insulin resistance to diabetes.
Treiber G, Guilleux A, Huynh K, Bonfanti O, Flaus-Furmaniuk A, Couret D, Mellet N, Bernard C, Le-Moullec N, Doray B, Jéru I, Maiza JC, Domun B, Cogne M, Meilhac O, Vigouroux C, Meikle PJ, Nobécourt E
Diabetes Metab 2023 Mar;49(2):101409. Epub 2022 Nov 15 doi: 10.1016/j.diabet.2022.101409. PMID: 36400409
Generalized lipoatrophy syndromes.
Sorkina E, Chichkova V
Presse Med 2021 Nov;50(3):104075. Epub 2021 Sep 22 doi: 10.1016/j.lpm.2021.104075. PMID: 34562560
Visual Vignette.
Yang AJ, Magill SB
Endocr Pract 2020 Oct;26(10):1230. doi: 10.4158/EP-2019-0592. PMID: 33471725
Lipodystrophies.
Garg A
Am J Med 2000 Feb;108(2):143-52. doi: 10.1016/s0002-9343(99)00414-3. PMID: 11126308
Lipoatrophic diabetes.
Sasaki T, Ono H, Nakajima H, Sugimoto J
J Dermatol 1992 Apr;19(4):246-9. doi: 10.1111/j.1346-8138.1992.tb03217.x. PMID: 1607489

Therapy

Visual Vignette.
Yang AJ, Magill SB
Endocr Pract 2020 Oct;26(10):1230. doi: 10.4158/EP-2019-0592. PMID: 33471725
The multifaceted nature of diabetes mellitus induced by checkpoint inhibitors.
Marchand L, Disse E, Dalle S, Reffet S, Vouillarmet J, Fabien N, Thivolet C, Cugnet-Anceau C
Acta Diabetol 2019 Dec;56(12):1239-1245. Epub 2019 Aug 19 doi: 10.1007/s00592-019-01402-w. PMID: 31423559
Rethinking leptin and insulin action: therapeutic opportunities for diabetes.
Yildiz BO, Haznedaroglu IC
Int J Biochem Cell Biol 2006;38(5-6):820-30. Epub 2005 Oct 3 doi: 10.1016/j.biocel.2005.09.013. PMID: 16236542
Immunologic insulin resistance.
Davidson JK, DeBra DW
Diabetes 1978 Mar;27(3):307-18. doi: 10.2337/diab.27.3.307. PMID: 640236
Hypothesis: hypothalamic dysfunction and lipoatrophic diabetes.
Upton GV, Corbin A
Yale J Biol Med 1973 Oct;46(4):314-23. PMID: 4365697Free PMC Article

Prognosis

Clinical and laboratory features of youth onset type 2 diabetes in Jamaica.
Tulloch-Reid MK, Boyne MS, Smikle MF, Choo-Kang EG, Parkes RH, Wright-Pascoe RA, Barton EN, Wilks RJ, Williams DE
West Indian Med J 2010 Mar;59(2):131-8. PMID: 21275116
Problems in differential diagnosis of diabetes types.
Małecki M, Skupień J
Pol Arch Med Wewn 2008 Jul-Aug;118(7-8):435-40. PMID: 18714740
Syndrome of lipodystrophy, hyperlipidemia, insulin resistance, and diabetes in treated patients with human immunodeficiency virus infection.
Graber AL
Endocr Pract 2001 Nov-Dec;7(6):430-7. doi: 10.4158/EP.7.6.430. PMID: 11747278
Lipodystrophy in patients with juvenile dermatomyositis--evaluation of clinical and metabolic abnormalities.
Huemer C, Kitson H, Malleson PN, Sanderson S, Huemer M, Cabral DA, Chanoine JP, Petty RE
J Rheumatol 2001 Mar;28(3):610-5. PMID: 11296968
Diagnosis, prediction, and natural course of HIV-1 protease-inhibitor-associated lipodystrophy, hyperlipidaemia, and diabetes mellitus: a cohort study.
Carr A, Samaras K, Thorisdottir A, Kaufmann GR, Chisholm DJ, Cooper DA
Lancet 1999 Jun 19;353(9170):2093-9. doi: 10.1016/S0140-6736(98)08468-2. PMID: 10382692

Clinical prediction guides

EPHX1 mutations cause a lipoatrophic diabetes syndrome due to impaired epoxide hydrolysis and increased cellular senescence.
Gautheron J, Morisseau C, Chung WK, Zammouri J, Auclair M, Baujat G, Capel E, Moulin C, Wang Y, Yang J, Hammock BD, Cerame B, Phan F, Fève B, Vigouroux C, Andreelli F, Jeru I
Elife 2021 Aug 3;10 doi: 10.7554/eLife.68445. PMID: 34342583Free PMC Article
Clinical and laboratory features of youth onset type 2 diabetes in Jamaica.
Tulloch-Reid MK, Boyne MS, Smikle MF, Choo-Kang EG, Parkes RH, Wright-Pascoe RA, Barton EN, Wilks RJ, Williams DE
West Indian Med J 2010 Mar;59(2):131-8. PMID: 21275116
Lipoatrophic diabetes and end-stage liver disease secondary to nonalcoholic steatohepatitis with recurrence after liver transplantation.
Cauble MS, Gilroy R, Sorrell MF, Mailliard ME, Sudan DL, Anderson JC, Wisecarver JL, Balakrishnan S, Larsen JL
Transplantation 2001 Apr 15;71(7):892-5. doi: 10.1097/00007890-200104150-00012. PMID: 11349722
Diagnosis, prediction, and natural course of HIV-1 protease-inhibitor-associated lipodystrophy, hyperlipidaemia, and diabetes mellitus: a cohort study.
Carr A, Samaras K, Thorisdottir A, Kaufmann GR, Chisholm DJ, Cooper DA
Lancet 1999 Jun 19;353(9170):2093-9. doi: 10.1016/S0140-6736(98)08468-2. PMID: 10382692
Congenital generalized lipodystrophy and experimental lipoatrophic diabetes in rabbits treated successfully with fenfluramine.
Trygstad O, Foss I
Acta Endocrinol (Copenh) 1977 Jun;85(2):436-48. doi: 10.1530/acta.0.0850436. PMID: 577333

Recent systematic reviews

Growth hormone axis treatments for HIV-associated lipodystrophy: a systematic review of placebo-controlled trials.
Sivakumar T, Mechanic O, Fehmie DA, Paul B
HIV Med 2011 Sep;12(8):453-62. Epub 2011 Jan 25 doi: 10.1111/j.1468-1293.2010.00906.x. PMID: 21265979

Supplemental Content

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    Clinical resources

    Practice guidelines

    • PubMed
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      See practice and clinical guidelines in NCBI Bookshelf. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.

    Curated

    • NICE, 2019
      UK NICE Guideline NG19, Diabetic foot problems: prevention and management, 2019

    Consumer resources

    Reviews

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