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Partial lipodystrophy: Clinical presentation and treatment.
- Source :
-
Annales d'endocrinologie [Ann Endocrinol (Paris)] 2024 Jun; Vol. 85 (3), pp. 197-200. Date of Electronic Publication: 2024 Jun 12. - Publication Year :
- 2024
-
Abstract
- Lipodystrophic syndromes are acquired or genetic rare diseases, characterized by a generalized or partial lack of adipose tissue leading to metabolic alterations linked to strong insulin resistance. They are probably underdiagnosed, especially for partial forms. They are characterized by a lack of adipose tissue or a lack of adipose development leading to metabolic disorders associated with often severe insulin resistance, hypertriglyceridemia and hepatic steatosis. In partial forms of lipodystrophy, these mechanisms are aggravated by excess visceral adipose tissue and/or subcutaneous adipose tissue in the upper part of the body. Diagnosis is based on clinical examination, pathological context and comorbidities, and on results of metabolic investigations and genetic analyses, which together determine management and genetic counseling. Early lifestyle and dietary measures focusing on regular physical activity, and balanced diet avoiding excess energy intake are crucial. They are accompanied by multidisciplinary follow-up adapted to each clinical form. When standard treatments have failed to control metabolic disorders, the orphan drug metreleptin, an analog of leptin, can be effective in certain forms of lipodystrophy syndromes.<br /> (Copyright © 2024 Elsevier Masson SAS. All rights reserved.)
- Subjects :
- Humans
Insulin Resistance
Lipodystrophy, Familial Partial therapy
Lipodystrophy, Familial Partial diagnosis
Lipodystrophy, Familial Partial genetics
Lipodystrophy, Familial Partial complications
Adipose Tissue pathology
Leptin therapeutic use
Leptin analogs & derivatives
Life Style
Lipodystrophy therapy
Lipodystrophy diagnosis
Lipodystrophy etiology
Lipodystrophy genetics
Subjects
Details
- Language :
- English
- ISSN :
- 2213-3941
- Volume :
- 85
- Issue :
- 3
- Database :
- MEDLINE
- Journal :
- Annales d'endocrinologie
- Publication Type :
- Academic Journal
- Accession number :
- 38871513
- Full Text :
- https://doi.org/10.1016/j.ando.2024.05.015