dc.creator | Bagias C., Xiarchou A., Bargiota A., Tigas S. | en |
dc.date.accessioned | 2023-01-31T07:35:05Z | |
dc.date.available | 2023-01-31T07:35:05Z | |
dc.date.issued | 2020 | |
dc.identifier | 10.2147/DMSO.S206053 | |
dc.identifier.issn | 11787007 | |
dc.identifier.uri | http://hdl.handle.net/11615/71039 | |
dc.description.abstract | Lipodystrophies are a heterogeneous group of congenital or acquired disorders, characterized by partial or generalized loss of adipose tissue. Familial partial lipodystrophy (FPLD) presents with genetic and phenotypic variability with insulin resistance, hypertrigly-ceridemia and hepatic steatosis being the cardinal metabolic features. The severity of the metabolic derangements is in proportion with the degree of lipoatrophy. The underpinning pathogenetic mechanism is the limited capacity of adipose tissue to store lipids leading to lipotoxicity, low-grade inflammation, altered adipokine secretion and ectopic fat tissue accumulation. Advances in molecular genetics have led to the discovery of new genes and improved our knowledge of the regulation of adipose tissue biology. Diagnosis relies predominantly on clinical findings, such as abnormal fat tissue topography and signs of insulin resistance and is confirmed by genetic analysis. In addition to anthropometry and conventional imaging, new techniques such as color-coded imaging of fat depots allow more accurate assessment of the regional fat distribution and differentiation of lipodystrophic syndromes from common metabolic syndrome phenotype. The treatment of patients with lipodystrophy has proven to be challenging. The use of a human leptin analogue, metreleptin, has recently been approved in the management of FPLD with evidence suggesting improved metabolic profile, satiety, reproductive function and self-perception. Preliminary data on the use of glucagon-like peptide 1 receptor agonists (GLP1 Ras) and sodium-glucose co-transporter 2 (SGLT2) inhibitors in cases of FPLD have shown promising results with reduction in total insulin requirements and improvement in glycemic control. Finally, investigational trials for new therapeutic agents in the management of FPLD are underway. © 2020 Bagias et al. | en |
dc.language.iso | en | en |
dc.source | Diabetes, Metabolic Syndrome and Obesity: Targets and Therapy | en |
dc.source.uri | https://www.scopus.com/inward/record.uri?eid=2-s2.0-85084350102&doi=10.2147%2fDMSO.S206053&partnerID=40&md5=47103c53f6c77ac43a62c14081a8d5cf | |
dc.subject | 2,4 thiazolidinedione derivative | en |
dc.subject | adipocytokine | en |
dc.subject | anorexigenic agent | en |
dc.subject | evinacumab | en |
dc.subject | fat droplet | en |
dc.subject | gemcabene | en |
dc.subject | glucagon like peptide 1 receptor agonist | en |
dc.subject | insulin | en |
dc.subject | metreleptin | en |
dc.subject | peroxisome proliferator activated receptor gamma | en |
dc.subject | phosphatidylinositol 3 kinase | en |
dc.subject | protein kinase B | en |
dc.subject | sodium glucose cotransporter 2 inhibitor | en |
dc.subject | adipocyte | en |
dc.subject | adipogenesis | en |
dc.subject | adipose tissue | en |
dc.subject | ADRA2A gene | en |
dc.subject | AKT2 gene | en |
dc.subject | anthropometry | en |
dc.subject | Article | en |
dc.subject | BLM gene | en |
dc.subject | body fat distribution | en |
dc.subject | CAV1 gene | en |
dc.subject | CIDEC gene | en |
dc.subject | clinical feature | en |
dc.subject | cytokine release | en |
dc.subject | diagnostic imaging | en |
dc.subject | diet therapy | en |
dc.subject | differential diagnosis | en |
dc.subject | disease severity | en |
dc.subject | DNA repair | en |
dc.subject | esthetic surgery | en |
dc.subject | familial partial lipodystrophy | en |
dc.subject | fatty liver | en |
dc.subject | gene | en |
dc.subject | gene mutation | en |
dc.subject | genetic analysis | en |
dc.subject | genetic variability | en |
dc.subject | glycemic control | en |
dc.subject | human | en |
dc.subject | hypertriglyceridemia | en |
dc.subject | insulin resistance | en |
dc.subject | insulin signaling | en |
dc.subject | lifestyle modification | en |
dc.subject | LIPE gene | en |
dc.subject | lipolysis | en |
dc.subject | lipotoxicity | en |
dc.subject | LMNA gene | en |
dc.subject | metabolic disorder | en |
dc.subject | metabolic syndrome X | en |
dc.subject | molecular genetics | en |
dc.subject | molecular pathology | en |
dc.subject | PCYT1A gene | en |
dc.subject | phenotype | en |
dc.subject | PIK3R1 gene | en |
dc.subject | PLIN1 gene | en |
dc.subject | POLD1 gene | en |
dc.subject | PPARg gene | en |
dc.subject | PSMB8 gene | en |
dc.subject | Roux-en-Y gastric bypass | en |
dc.subject | WRN gene | en |
dc.subject | Dove Medical Press Ltd. | en |
dc.title | Familial partial lipodystrophy (FPLD): Recent insights | en |
dc.type | journalArticle | en |