Vatier et al. discuss the diagnostic and management challenges of lipodystrophic syndromes, many of which are typically diagnosed at birth, or soon thereafter (e.g. Berardinelli–Seip congenital lipodystrophy)Citation1. Infants with congenital lipodystrophic syndromes often present with a non-specific clinical picture (including generalized lipoatrophy, hepatomegaly, malnutrition and failure to thrive) and abnormal blood tests, including lactic acidosis, hypertriglyceridaemia and hypercholesterolaemiaCitation1.
There is evidence suggesting that several relatively novel adipocytokines (e.g. resistin, visfatin, apelin, chemerin, obestatin, vaspin and omentin-1) play an important role not only in intrauterine fetal growth, but also in the development of subsequent early life and adult diseaseCitation2–5. Future studies should investigate whether or not some of these adipocytokines may facilitate the challenging diagnosis of congenital lipodystrophic syndromes.
The authors correctly report that metreleptin (recombinant human methionyl leptin, a synthetic analogue of the hormone leptin) can be helpful to efficiently control the metabolic complications of congenital generalized lipodystrophyCitation1. This statement is also supported by a recent multi-society practice guidelineCitation6. Metreleptin decreases hyperphagia, frequently leading to weight lossCitation6. Besides metreleptin, other treatments not specific for lipodystrophy may be helpful (e.g. metformin for diabetes and statins/fibrates for hyperlipidaemia)Citation6. Statins in particular play an important role in the management of hyperlipidaemia/dyslipidaemiaCitation7–9 and potentially during gestation, in specific situationsCitation10. Therefore, besides metreleptin, statins may also prove useful in the management of patients with lipodystrophic syndromes.
Transparency
Declaration of funding
This commentary was written independently; no company or institution supported the authors financially or by providing a professional writer.
Declaration of financial/other relationships
No potential conflict of interest was reported by the authors. CMRO peer reviewers on this manuscript have no relevant financial or other relationships to disclose.
Acknowledgements
None reported.
Related Research Data
References
- Vatier C, Vantyghem MC, Storey C, et al. Monogenic forms of lipodystrophic syndromes: diagnosis, detection, and practical management considerations from clinical cases. Curr Med Res Opin. 2019;35:543–552.
- Briana DD, Malamitsi-Puchner A. Intrauterine growth restriction and adult disease: the role of adipocytokines. Eur J Endocrinol. 2009;160:337–347.
- Briana DD, Boutsikou M, Baka S, et al. Perinatal changes of plasma resistin concentrations in pregnancies with normal and restricted fetal growth. Neonatology. 2008;93:153–157.
- Boutsikou T, Briana DD, Boutsikou M, et al. Cord blood chemerin and obestatin levels in large for gestational age infants. J Matern Fetal Neonatal Med. 2013;26:123–126.
- Kafalidis G, Boutsikou T, Briana DD, et al. Adipokines vaspin and omentin-1 are up-regulated in large for gestational age infants at term. Cytokine. 2013;62:70–74.
- Brown RJ, Araujo-Vilar D, Cheung PT, et al. The diagnosis and management of lipodystrophy syndromes: a multi-society practice guideline. J Clin Endocrinol Metab. 2016;101:4500–4511.
- Mikhailidis DP, Athyros VG. Dyslipidaemia in 2013: new statin guidelines and promising novel therapeutics. Nat Rev Cardiol. 2014;11:72–74.
- Florentin M, Liberopoulos EN, Mikhailidis DP, et al. Emerging options in the treatment of dyslipidemias: a bright future? Expert Opin Emerg Drugs. 2011;16:247–270.
- Cicero AF, Colletti A, Bajraktari G, et al. Lipid-lowering nutraceuticals in clinical practice: position paper from an International Lipid Expert Panel. Nutr Rev. 2017;75:731–767.
- Maierean SM, Mikhailidis DP, Toth PP, et al. The potential role of statins in preeclampsia and dyslipidemia during gestation: a narrative review. Expert Opin Investig Drugs. 2018;27:427–435.