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Acquired generalized lipodystrophy

MedGen UID:
543499
•Concept ID:
C0271693
•
Disease or Syndrome
Synonyms: Acquired Generalized Lipodystrophy; acquired generalized lipodystrophy; Acquired lipoatrophic diabetes; acquired lipoatrophic diabetes; Acquired lipodystrophic diabetes; Acquired total lipoatrophy; Lawrence syndrome; Lawrence-Seip syndrome
SNOMED CT: Acquired total lipoatrophy (86907008); Acquired generalized lipodystrophy (86907008); Acquired lipodystrophic diabetes (86907008)
Modes of inheritance:
Not genetically inherited
MedGen UID:
988794
•Concept ID:
CN307044
•
Finding
Source: Orphanet
clinical entity without genetic inheritance.
See: This record
Not genetically inherited (Orphanet)
 
Monarch Initiative: MONDO:0019193
Orphanet: ORPHA79086

Definition

A rare lipodystrophic syndrome characterized by loss of adipose tissue, and is a syndrome of insulin resistance that leads to increased cardiovascular risk. Acquired generalized lipodystrophy is related to a selective loss of subcutaneous adipose tissue occurring exclusively at the extremities (face, legs, arms, palms and sometimes soles). [from ORDO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVAcquired generalized lipodystrophy
Follow this link to review classifications for Acquired generalized lipodystrophy in Orphanet.

Professional guidelines

PubMed

Diagnosis and treatment of lipodystrophy: a step-by-step approach.
Araújo-Vilar D, Santini F
J Endocrinol Invest 2019 Jan;42(1):61-73. Epub 2018 Apr 27 doi: 10.1007/s40618-018-0887-z. PMID: 29704234Free PMC Article
Long-term effectiveness and safety of metreleptin in the treatment of patients with generalized lipodystrophy.
Brown RJ, Oral EA, Cochran E, Araújo-Vilar D, Savage DB, Long A, Fine G, Salinardi T, Gorden P
Endocrine 2018 Jun;60(3):479-489. Epub 2018 Apr 12 doi: 10.1007/s12020-018-1589-1. PMID: 29644599Free PMC Article
Clinical Features and Management of Non-HIV-Related Lipodystrophy in Children: A Systematic Review.
Gupta N, Asi N, Farah W, Almasri J, Barrionuevo P, Alsawas M, Wang Z, Haymond MW, Brown RJ, Murad MH
J Clin Endocrinol Metab 2017 Feb 1;102(2):363-374. doi: 10.1210/jc.2016-2271. PMID: 27967300Free PMC Article

Recent clinical studies

Etiology

Lipodystrophy for the Diabetologist-What to Look For.
Patni N, Garg A
Curr Diab Rep 2022 Sep;22(9):461-470. Epub 2022 Jul 11 doi: 10.1007/s11892-022-01485-w. PMID: 35821558Free PMC Article
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
Lymphoma in acquired generalized lipodystrophy.
Brown RJ, Chan JL, Jaffe ES, Cochran E, DePaoli AM, Gautier JF, Goujard C, Vigouroux C, Gorden P
Leuk Lymphoma 2016;57(1):45-50. Epub 2015 May 12 doi: 10.3109/10428194.2015.1040015. PMID: 25864863Free PMC Article
Cardiomyopathy in congenital and acquired generalized lipodystrophy: a clinical assessment.
Lupsa BC, Sachdev V, Lungu AO, Rosing DR, Gorden P
Medicine (Baltimore) 2010 Jul;89(4):245-250. doi: 10.1097/MD.0b013e3181e9442f. PMID: 20616664Free PMC Article
Generalized lipodystrophy, congenital and acquired (lipoatrophy).
Seip M, Trygstad O
Acta Paediatr Suppl 1996 Jun;413:2-28. doi: 10.1111/j.1651-2227.1996.tb14262.x. PMID: 8783769

Diagnosis

Lipodystrophy for the Diabetologist-What to Look For.
Patni N, Garg A
Curr Diab Rep 2022 Sep;22(9):461-470. Epub 2022 Jul 11 doi: 10.1007/s11892-022-01485-w. PMID: 35821558Free PMC Article
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
Diagnosis and treatment of lipodystrophy: a step-by-step approach.
Araújo-Vilar D, Santini F
J Endocrinol Invest 2019 Jan;42(1):61-73. Epub 2018 Apr 27 doi: 10.1007/s40618-018-0887-z. PMID: 29704234Free PMC Article
Leptin in congenital and HIV-associated lipodystrophy.
Tsoukas MA, Farr OM, Mantzoros CS
Metabolism 2015 Jan;64(1):47-59. Epub 2014 Aug 12 doi: 10.1016/j.metabol.2014.07.017. PMID: 25267014
Lipodystrophies.
Garg A
Am J Med 2000 Feb;108(2):143-52. doi: 10.1016/s0002-9343(99)00414-3. PMID: 11126308

Therapy

Lipodystrophy for the Diabetologist-What to Look For.
Patni N, Garg A
Curr Diab Rep 2022 Sep;22(9):461-470. Epub 2022 Jul 11 doi: 10.1007/s11892-022-01485-w. PMID: 35821558Free PMC Article
Leptin induces TNFα-dependent inflammation in acquired generalized lipodystrophy and combined Crohn's disease.
Ziegler JF, Böttcher C, Letizia M, Yerinde C, Wu H, Freise I, Rodriguez-Sillke Y, Stoyanova AK, Kreis ME, Asbach P, Kunkel D, Priller J, Anagnostopoulos I, Kühl AA, Miehle K, Stumvoll M, Tran F, Fredrich B, Forster M, Franke A, Bojarski C, Glauben R, Löscher BS, Siegmund B, Weidinger C
Nat Commun 2019 Dec 10;10(1):5629. doi: 10.1038/s41467-019-13559-7. PMID: 31822667Free PMC Article
Diagnosis and treatment of lipodystrophy: a step-by-step approach.
Araújo-Vilar D, Santini F
J Endocrinol Invest 2019 Jan;42(1):61-73. Epub 2018 Apr 27 doi: 10.1007/s40618-018-0887-z. PMID: 29704234Free PMC Article
Lymphoma in acquired generalized lipodystrophy.
Brown RJ, Chan JL, Jaffe ES, Cochran E, DePaoli AM, Gautier JF, Goujard C, Vigouroux C, Gorden P
Leuk Lymphoma 2016;57(1):45-50. Epub 2015 May 12 doi: 10.3109/10428194.2015.1040015. PMID: 25864863Free PMC Article
Cardiomyopathy in congenital and acquired generalized lipodystrophy: a clinical assessment.
Lupsa BC, Sachdev V, Lungu AO, Rosing DR, Gorden P
Medicine (Baltimore) 2010 Jul;89(4):245-250. doi: 10.1097/MD.0b013e3181e9442f. PMID: 20616664Free PMC Article

Prognosis

Lymphoma in acquired generalized lipodystrophy.
Brown RJ, Chan JL, Jaffe ES, Cochran E, DePaoli AM, Gautier JF, Goujard C, Vigouroux C, Gorden P
Leuk Lymphoma 2016;57(1):45-50. Epub 2015 May 12 doi: 10.3109/10428194.2015.1040015. PMID: 25864863Free PMC Article
A Report of Three Cases With Acquired Generalized Lipodystrophy With Distinct Autoimmune Conditions Treated With Metreleptin.
Lebastchi J, Ajluni N, Neidert A, Oral EA
J Clin Endocrinol Metab 2015 Nov;100(11):3967-70. Epub 2015 Sep 21 doi: 10.1210/jc.2015-2589. PMID: 26390101Free PMC Article
A Novel Syndrome of Generalized Lipodystrophy Associated With Pilocytic Astrocytoma.
Patni N, Alves C, von Schnurbein J, Wabitsch M, Tannin G, Rakheja D, Garg A
J Clin Endocrinol Metab 2015 Oct;100(10):3603-6. Epub 2015 Aug 7 doi: 10.1210/jc.2015-2476. PMID: 26252356Free PMC Article
Type 1 diabetes associated with acquired generalized lipodystrophy and insulin resistance: the effect of long-term leptin therapy.
Park JY, Chong AY, Cochran EK, Kleiner DE, Haller MJ, Schatz DA, Gorden P
J Clin Endocrinol Metab 2008 Jan;93(1):26-31. Epub 2007 Oct 16 doi: 10.1210/jc.2007-1856. PMID: 17940115Free PMC Article
Generalized lipodystrophy, congenital and acquired (lipoatrophy).
Seip M, Trygstad O
Acta Paediatr Suppl 1996 Jun;413:2-28. doi: 10.1111/j.1651-2227.1996.tb14262.x. PMID: 8783769

Clinical prediction guides

Autoantibodies Against Perilipin 1 as a Cause of Acquired Generalized Lipodystrophy.
Corvillo F, Aparicio V, López-Lera A, Garrido S, Araújo-Vilar D, de Miguel MP, López-Trascasa M
Front Immunol 2018;9:2142. Epub 2018 Sep 19 doi: 10.3389/fimmu.2018.02142. PMID: 30283460Free PMC Article
Clinical Features and Management of Non-HIV-Related Lipodystrophy in Children: A Systematic Review.
Gupta N, Asi N, Farah W, Almasri J, Barrionuevo P, Alsawas M, Wang Z, Haymond MW, Brown RJ, Murad MH
J Clin Endocrinol Metab 2017 Feb 1;102(2):363-374. doi: 10.1210/jc.2016-2271. PMID: 27967300Free PMC Article
Leptin in congenital and HIV-associated lipodystrophy.
Tsoukas MA, Farr OM, Mantzoros CS
Metabolism 2015 Jan;64(1):47-59. Epub 2014 Aug 12 doi: 10.1016/j.metabol.2014.07.017. PMID: 25267014
The liver diseases of lipodystrophy: the long-term effect of leptin treatment.
Safar Zadeh E, Lungu AO, Cochran EK, Brown RJ, Ghany MG, Heller T, Kleiner DE, Gorden P
J Hepatol 2013 Jul;59(1):131-7. Epub 2013 Feb 21 doi: 10.1016/j.jhep.2013.02.007. PMID: 23439261Free PMC Article
Generalized lipodystrophy, congenital and acquired (lipoatrophy).
Seip M, Trygstad O
Acta Paediatr Suppl 1996 Jun;413:2-28. doi: 10.1111/j.1651-2227.1996.tb14262.x. PMID: 8783769

Recent systematic reviews

Clinical Features and Management of Non-HIV-Related Lipodystrophy in Children: A Systematic Review.
Gupta N, Asi N, Farah W, Almasri J, Barrionuevo P, Alsawas M, Wang Z, Haymond MW, Brown RJ, Murad MH
J Clin Endocrinol Metab 2017 Feb 1;102(2):363-374. doi: 10.1210/jc.2016-2271. PMID: 27967300Free PMC Article

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