JOURNAL OF THE CZECH PEDIATRIC SOCIETY AND THE SLOVAK PEDIATRIC SOCIETY

Čes-slov Pediat 2024, 79(4):221-225 | DOI: 10.55095/CSPediatrie2024/036

Clinical presentation and management of childhood lipodystrophic syndromes

Jan Lebl1, Francesco Chiarelli2
1 Pediatrická klinika, 2. lékařská fakulta, Univerzita Karlova a Fakultní nemocnice v Motole, Praha
2 Department of Paediatrics, University of Chieti, Chieti, Italy

Lipodystrophy syndromes represent a heterogeneous group of rare diseases characterized by various degree of adipose tissue loss that results in severe metabolic complications, including hypertriglyceridemia, steatohepatitis and particularly insulin resistance. These complications may increase the mortality rate. Adipokine deficiency, and especially leptin deficiency, is due to fat

tissue loss. Therapy for lipodystrophy primarily consists of a conventional approach that involves standard treatments of metabolic abnormalities. Recently, recombinant leptin.(metreleptin) administration has been introduced to compensate for leptin deficiency. This therapy markedly improves the overall patients' status and metabolic complications and was approved for the treatment of both

generalized and partial lipodystrophy.

Keywords: lipodystrophy, leptin, metreleptin, insulin resistance, metabolic complications, therapy

Accepted: May 31, 2024; Published: August 1, 2024  Show citation

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Lebl J, Chiarelli F. Clinical presentation and management of childhood lipodystrophic syndromes. Ces-slov Pediat. 2024;79(4):221-225. doi: 10.55095/CSPediatrie2024/036.
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