Generalized lipoatrophy syndromes.

Ekaterina Sorkina, Valentina Chichkova

Journal: Presse medicale (Paris, France : 1983) 2021;50(3):104075

PMID: 34562560

Full Text on Elsevier Science View on PubMed

Abstract

Generalized lipodystrophy (GL) syndromes are a group of rare heterogenous disorders, characterized by total subcutaneous fat loss. The frequency of GL is currently assessed as approximately 0,23 cases per million of the population, in Europe - as 0,96 cases per million of the population. They can be congenital (CGL) or acquired (AGL) depending on the etiology and the time of the onset of fat loss. Both CGL and AGL are often associated with different metabolic complications, such as hypertriglyceridemia, insulin resistance and lipoatrophic diabetes mellitus, metabolically associated FLD, arterial hypertension, proteinuria, reproductive system disorders. In this review we aimed to summarize the information on all forms of generalized lipodystrophy, especially the ones of genetic etiology, their clinical manifestations and complications, the perspectives for diagnostics, treatment and further research.

Address: Endocrinology Research Centre, 11, ulitsa Dmitriya Ulianova, Moscow 117036, Russia. Electronic address: [email protected].; Endocrinology Research Centre, 11, ulitsa Dmitriya Ulianova, Moscow 117036, Russia.

DOI: 10.1016/j.lpm.2021.104075

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MeSH Terms: Acyltransferases, Age of Onset, Caveolin 1, DNA-Binding Proteins, Diabetes Mellitus, Lipoatrophic, Diagnosis, Differential, G Protein-Coupled Inwardly-Rectifying Potassium Channels, GTP-Binding Protein gamma Subunits, Genital Diseases, Humans, Hypertension, Hypertriglyceridemia, Insulin Resistance, Lamin Type A, Lipodystrophy, Lipodystrophy, Congenital Generalized, Mandible, Membrane Proteins, Metalloendopeptidases, Mutation, Progeria, Proteinuria, RNA Polymerase III, RNA-Binding Proteins, Syndrome, Werner Syndrome Helicase