Lipodystrophy for the Diabetologist-What to Look For.
AGL
APL
Acanthosis nigricans
Acquired generalized lipodystrophy
Acquired partial lipodystrophy
CGL
Congenital generalized lipodystrophy
FPLD
Familial partial lipodystrophy
Hypertriglyceridemia
Journal
Current diabetes reports
ISSN: 1539-0829
Titre abrégé: Curr Diab Rep
Pays: United States
ID NLM: 101093791
Informations de publication
Date de publication:
09 2022
09 2022
Historique:
accepted:
03
06
2022
pubmed:
14
7
2022
medline:
10
8
2022
entrez:
13
7
2022
Statut:
ppublish
Résumé
Genetic or acquired lipodystrophies are characterized by selective loss of body fat along with predisposition towards metabolic complications of insulin resistance, such as diabetes mellitus, hypertriglyceridemia, hepatic steatosis, polycystic ovarian syndrome, and acanthosis nigricans. In this review, we discuss the various subtypes and when to suspect and how to diagnose lipodystrophy. The four major subtypes are autosomal recessive, congenital generalized lipodystrophy (CGL); acquired generalized lipodystrophy (AGL), mostly an autoimmune disorder; autosomal dominant or recessive familial partial lipodystrophy (FPLD); and acquired partial lipodystrophy (APL), an autoimmune disorder. Diagnosis of lipodystrophy is mainly based upon physical examination findings of loss of body fat and can be supported by body composition analysis by skinfold measurements, dual-energy x-ray absorptiometry, and whole-body magnetic resonance imaging. Confirmatory genetic testing is helpful in the proband and at-risk family members with suspected genetic lipodystrophies. The treatment is directed towards the specific comorbidities and metabolic complications, and there is no treatment to reverse body fat loss. Metreleptin should be considered as the first-line therapy for metabolic complications in patients with generalized lipodystrophy and for prevention of comorbidities in children. Metformin and insulin therapy are the best options for treating hyperglycemia and fibrates and/or fish oil for hypertriglyceridemia. Lipodystrophy should be suspected in lean and muscular subjects presenting with diabetes mellitus, hypertriglyceridemia, non-alcoholic fatty liver disease, polycystic ovarian syndrome, or amenorrhea. Diabetologists should be aware of lipodystrophies and consider genetic varieties as an important subtype of monogenic diabetes.
Identifiants
pubmed: 35821558
doi: 10.1007/s11892-022-01485-w
pii: 10.1007/s11892-022-01485-w
pmc: PMC10704567
mid: NIHMS1850376
doi:
Types de publication
Journal Article
Review
Research Support, Non-U.S. Gov't
Research Support, N.I.H., Extramural
Langues
eng
Sous-ensembles de citation
IM
Pagination
461-470Subventions
Organisme : NIDDK NIH HHS
ID : R01 DK105448
Pays : United States
Informations de copyright
© 2022. This is a U.S. Government work and not under copyright protection in the US; foreign copyright protection may apply.
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