Unit:
Κατεύθυνση Σακχαρώδης Διαβήτης και ΠαχυσαρκίαLibrary of the School of Health Sciences
Supervisors info:
Bάϊα Λαμπαδιάρη, Καθηγήτρια, Ιατρική Σχολή, ΕΚΠΑ, Επιβλέπουσα
Κωνσταντίνος Μακρυλάκης, Καθηγητής, Ιατρική Σχολή, ΕΚΠΑ
Αλέξανδρος Κόκκινος, Καθηγητής, Ιατρική Σχολή, ΕΚΠΑ
Original Title:
Λιποδυστροφίες - Σύνδρομα σοβαρής ινσουλινοαντίστασης - Νοσοκομειακή εμπειρία
Translated title:
Lipodystrophies - Syndromes of severe insulin resistance - Hospital experience
Summary:
Lipodystrophies are rare disorders with genetic or acquired etiology and are characterized of
complete or partial loss of adipose tissue. Adipose tissue normally stores excess energy in
the form of triglycerides and releases it in conditions of poverty after proper hormonal
signaling. It also has additional endocrine functions by secreting a large percentage of
bioactive molecules, among which are leptin and adiponectin which have several beneficial
metabolic effects. The loss of adipose tissue exposes the non-adipose tissues to lipotoxicity
resulting in ectopic deposition of fat intrahepatically and intramuscularly and finally quite
severe insulin resistance, to which contribute the low levels of leptin and adiponectin. The
result is the development of metabolic complications such as hepatic steatosis,
hypertriglyceridemia and diabetes mellitus leading to early atherosclerosis and high
cardiovascular risk and ultimately to increased mortality of these patients. Lipodystrophic
syndromes are often underdiagnosed and there are not any diagnostic criteria as guidelines .
Their diagnosis is based on phenotypic characteristics, their metabolic complications and
genetic testing. Their treatment lies in the treatment of their metabolic complications as well
as -with specific indications- in the administration of recombinant leptin which seems to
significantly improve the metabolic profile of patients.
Main subject category:
Health Sciences
Keywords:
Adipose tissue, Insulin resistance, Genetic lipodystrophies, Acquired lipodystrophies, Metreleptin
Number of references:
152
