Open AccessLipodystrophies in Children
Author(s) -
Francesca Mainieri,
Francesco Chiarelli
Publication year - 2022
Publication title -
hormone research in paediatrics
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.816
H-Index - 89
eISSN - 1663-2826
pISSN - 1663-2818
DOI - 10.1159/000522620
Subject(s) - lipodystrophy , dyslipidemia , medicine , insulin resistance , lipoatrophy , steatosis , etiology , adipose tissue , diabetes mellitus , endocrinology , antiretroviral therapy , human immunodeficiency virus (hiv) , immunology , viral load
Background: Lipodystrophy includes a wide group of diseases characterized by reduction, absence or altered distribution of adipose tissue. Lipodystrophies are classified into generalized or partial, according to the fat distribution, and congenital or acquired, considering the aetiology. Summary: Impaired glucose and lipid metabolism is typically present, thus severe insulin resistance, diabetes mellitus, dyslipidemia and hepatic steatosis are frequent complications. Because of the rarity and the diversification of lipodystrophies, diagnosis might be challenging, typically for partial forms that cannot be easily recognized, leading to progression of the several metabolic abnormalities associated. First management of lipodystrophy is diet and lifestyle changes, followed by the treatment of metabolic complications. Replacement therapy with metreleptin, currently available in the United States and Europe, has shown improvement of metabolic profile in a great number of patients with lipodystrophy. Key messages: The purpose of this review is to describe the phenotypic characteristics of all the known lypodistrophic types and to present specific steps for obtaining early diagnosis and assessing the best treatment of lipodystrophy.