Lipodystrophies in Children

Abstract

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.

Insulin resistance relates to DKA severity and affects insulin requirement in children with type 1 diabetes at onset

“…It is well known that insulin levels during a fasting state and after an oral glucose load differs significantly according to insulin sensitivity, having subjects with insulin resistance (e.g., obese children or subject with lipodystrophies) significantly higher values. 17,25 Similarly, it is well known that values of glucose/insulin ratio in a fasting state significantly and inversely reflects insulin sensitivity. 26 Although a strong correlation is documented between pH values and IIR, GIR and GIR/IIR ratio suggesting a strong relationship between insulin sensitivity state and severity of DKA, these indexes represent surrogate markers of insulin sensitivity thus further multicenter studies are needed to confirm our results.…”

“…Taken together, these results suggest a progressive worsening of insulin sensitivity state ranging from mild to severe DKA state. It is well known that insulin levels during a fasting state and after an oral glucose load differs significantly according to insulin sensitivity, having subjects with insulin resistance (e.g., obese children or subject with lipodystrophies) significantly higher values 17,25 . Similarly, it is well known that values of glucose/insulin ratio in a fasting state significantly and inversely reflects insulin sensitivity 26 .…”