Abstract
Generalized liopdystrophy is an extremely rare disorder characterized by marked loss of adipose tissue with reduced triglyceride storage capacity leading to a severe form of metabolic syndrome such as, hypertriglycerides, insulin resistance, type 2 diabetes mellitus, and hepatic steatosis. Left ventricular hypertrophy (LVH) is a condition where the muscled wall of the heart’s left pumping chamber becomes thicker. LVH is a common condition that is a characteristic of lipodystrophy. For this experiment, researchers performed a cardiac magnetic resonance imaging. This was the first time a study was done on patients with generalized lipodystrophy. They studied six patients with generalized lipodystrophy and 6 healthy control patients that…show more content… Generalized lipodystrophy can be inherited or acquired. Patients who are affected by the disorder are leptin deficient and are severely hyperphagic. They have no adipose tissue to store excess energy and they consequently develop severe forms of metabolic syndrome with insulin resistance, type 2 diabetes mellitus, hypertriglyceridemia, and non-alcoholic fatty liver disease. A popular hypothesis that goes along with Generalized lipodystrophy is that LV hypertrophy could be an extreme clinical example of lipotoxic cardiomyopathy, excessive myocyte accumulation of triglyceride, leading to adverse hypertrophic signaling. In order to determine if there was a relationship between myocardial hypertrophy and triglyceride content in patients with generalized lipodystrophy, researchers used magnetic resonance imaging (MRI) and localized proton magnetic resonance spectroscopy…show more content… There were two major findings from this study. First researchers found myocardial triglyceride content is higher in patients’ hypertrophied cardiomyocites, which was consistent with the lipotoxicity hypothesis. Second, they found pericardial fat to be present in all patients, representing an undescribed depot of adipocytes preserved in generalized lipodystrophy. There was a 3-fold elevation in myocardial triglyceride content in the patients. This showed that the heart is another steatotic target organ in this disease. This means that the heart is susceptible to infiltration of cells with fat associated with the disturbance of metabolism. It was also observed that patients with congenital generalized lipodystrohy, the widespread failure of adipogenesis causes both leptin deficiency and reduced adipocyte pool to store triglyceride. Then patients with acquired generalized lipodystrophy were observed to have loss of adipose tissue caused by an unknown mechanism. Patients lacked central neural action of leptin, which suppresses appetite. They also lacked peripheral anti-steatotic action of leptin which upregulates fatty acid oxidation of non-adipose tissue. It was also interesting that there was a presence of pericardial fat with generalized lipodystrophy. Although, there were some limitations to this study. One limitation was that patients studied did not regularly
