Metreleptin Treatment in Patients with Non-HIV Associated Lipodystrophy

Lipodystrophies are a heterogeneous group of disorders characterized by congenital or acquired loss of adipose tissue. Recently, metreleptin, a recombinant human leptin analog, has been approved for the treatment of patients with generalized lipodystrophy. Leptin is an adipokine which has a fundamental role in glucose and lipid homeostasis. Metreleptin treatment has been demonstrated to improve metabolic abnormalities such as hyperglycemia, hypertriglyceridemia, increased hepatic fat content and elevated liver enzymes alanine transaminase and aspartate transaminase in patients with generalized lipodystrophy, and to correct hyperphagia that likely occurs as a result of leptin deficiency. Limited data has also suggested that metreleptin treatment might be beneficial on metabolic abnormalities in patients with partial lipodystrophy. This review focuses on potential benefits of metreleptin in various forms of non-HIV associated lipodystrophy. Safety issues have been discussed. Recent patent submissions have also been reviewed

Complications of lipodystrophy syndromes

Lipodystrophy syndromes are rare complex multisystem disorders caused by generalized or partial lack of adipose tissue. Adipose tissue dysfunction in lipodystrophy is associated with leptin deficiency. Lipodystrophy leads to severe metabolic problems. These abnormalities include, but are not limited to, insulin-resistant diabetes, severe hypertriglyceridemia, and lipid accumulation in ectopic organs such as the liver, and are associated with end-organ complications. Metabolic abnormalities can be present at the time of diagnosis or may develop over time as the disease progresses. In addition to metabolic abnormalities, subtype-specific presentations due to underlying molecular etiology in genetic forms and autoimmunity in acquired forms contribute to severe morbidity in lipodystrophy. (c) 2021 Elsevier Masson SAS. All rights reserved

Role of thrombin activatable fibrinolysis inhibitor in endocrine and cardiovascular disorders

Thrombin activatable fibrinolysis inhibitor (TAFI) is a zymogene that potently inhibits fibrinolysis. It is synthesized by the liver and has an inhibitor role in fibrinolysis through the removal of the carboxy-terminal lysine and arginine residues from partially degraded fibrin polymers. Besides, TAFI has a suppressor effect on conversion of inactive plasminogen to plasmin. Alterations in TAFI pathway have been reported in many conditions. Few data are present in the literature regarding the relationship between TAFI and endocrine disorders. The results from few studies in patients with cardiovascular disease are conflicting. Understanding the role of TAFI in the pathogenesis of endocrine and cardiovascular disorders may hold promise for improving management of these diseases. This paper includes a review of the studies and patents concerned with TAFI pathway and endocrine and cardiovascular problems. © 2008 Bentham Science Publishers Ltd

An overview of lipodystrophy and the role of the complement system

The complement system is a major component of innate immunity playing essential roles in the destruction of pathogens, the clearance of apoptotic cells and immune complexes, the enhancement of phagocytosis, inflammation, and the modulation of adaptive immune responses. During the last decades, numerous studies have shown that the complement system has key functions in the biology of certain tissues. For example, complement contributes to normal brain and embryonic development and to the homeostasis of lipid metabolism. However, the complement system is subjected to the effective balance between activation-inactivation to maintain complement homeostasis and to prevent self-injury to cells or tissues. When this control is disrupted, serious pathologies eventually develop, such as C3 glomerulopathy, autoimmune conditions and infections. Another heterogeneous group of ultra-rare diseases in which complement abnormalities have been described are the lipodystrophy syndromes. These diseases are characterized by the loss of adipose tissue throughout the entire body or partially. Complement over-activation has been reported in most of the patients with acquired partial lipodystrophy (also called Barraquer-Simons Syndrome) and in some cases of the generalized variety of the disease (Lawrence Syndrome). Even so, the mechanism through which the complement system induces adipose tissue abnormalities remains unclear. This review focuses on describing the link between the complement system and certain forms of lipodystrophy. In addition, we present an overview regarding the clinical presentation, differential diagnosis, classification, and management of patients with lipodystrophy associated with complement abnormalities

Mean Platelet Volume in Women with Gestational Diabetes

Purpose: Mean platelet volume (MPV) is an indicator of platelet activation which plays an important role in the pathogenesis of atherosclerosis. The purpose of this study was to compare MPV values in patients with previous gestational diabetes (pGDM), who have different degrees of carbohydrate intolerance with that in women without pGDM; and to retrospectively analyze MPV in these women during pregnancy