Lipodystrophic syndromes: From diagnosis to treatment.
Ann Endocrinol (Paris)
; 81(1): 51-60, 2020 Feb.
Article
en En
| MEDLINE
| ID: mdl-31982105
ABSTRACT
Lipodystrophic syndromes are acquired or genetic rare diseases, characterised by a generalised or partial lack of adipose tissue leading to metabolic alterations linked to strong insulin resistance. They encompass a variety of clinical entities due to primary defects in adipose differentiation, in the structure and/or regulation of the adipocyte lipid droplet, or due to immune-inflammatory aggressions, chromatin deregulations and/or mitochondrial dysfunctions affecting adipose tissue. 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 counselling. Early lifestyle and dietary measures focusing on regular physical activity and avoiding excess energy intake are crucial. They are accompanied by multidisciplinary follow-up adapted to each clinical form. In case of hyperglycemia, antidiabetic medications, with metformin as a first-line therapy in adults, are used in addition to lifestyle and dietary modifications. 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 syndrome. Metreleptin therapy indications, prescription and monitoring were recently defined in France, representing a major improvement in patient care.
Palabras clave
Texto completo:
1
Bases de datos:
MEDLINE
Asunto principal:
Lipodistrofia
Tipo de estudio:
Diagnostic_studies
Límite:
Adult
/
Humans
Idioma:
En
Revista:
Ann Endocrinol (Paris)
Año:
2020
Tipo del documento:
Article
País de afiliación:
Francia