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Purpose/Aim of the study: Acquired partial lipodystrophy (APL) is a rare disease characterized by selective loss of adipose tissue. In this study, we aimed to present a subset of patients with APL, who developed severe metabolic abnormalities, from our national lipodystrophy registry., Materials and Methods: Severe metabolic abnormalities were defined as: poorly controlled diabetes (HbA1c above 7% despite treatment with insulin more than 1 unit/kg/day combined with oral antidiabetics), severe hypertriglyceridemia (triglycerides above 500 mg/dL despite treatment with lipid-lowering drugs), episodes of acute pancreatitis, or severe hepatic involvement (biopsy-proven non-alcoholic steatohepatitis (NASH))., Results: Among 140 patients with all forms of lipodystrophy (28 with APL), we identified 6 APL patients with severe metabolic abnormalities. The geometric mean for age was 37 years (range: 27-50 years; 4 females and 2 males). Five patients had poorly controlled diabetes despite treatment with high-dose insulin combined with oral antidiabetics. Severe hypertriglyceridemia developed in five patients, of those three experienced episodes of acute pancreatitis. Although all six patients had hepatic steatosis at various levels on imaging studies, NASH was proven in two patients on liver biopsy. Our data suggested that APL patients with severe metabolic abnormalities had a more advanced fat loss and longer disease duration., Conclusions: We suggest that these patients represent a potential subgroup of APL who may benefit from metreleptin or investigational therapies as standard treatment strategies fail to achieve a good metabolic control.

Background: Gestational diabetes mellitus (GDM) is a prediabetic state that is known to increase the risk of cardiovascular diseases. We have investigated coronary flow velocity reserve (CFVR) and epicardial fat thickness (EFT), and left ventricular diastolic function in patients with a history of previous GDM (p-GDM)., Methods: Ninety-three women with GDM history and 95 healthy women without GDM history were recruited. We used transthoracic Doppler echocardiography to assess CFVR, EFT, and left ventricular diastolic function. Insulin resistance of each subject was assessed with homeostasis model assessment insulin resistance (HOMA-IR). Hemoglobin A1c and high-sensitivity C-reactive protein (hsCRP) were also measured in all patients., Results: CFVR values were significantly lower (2.34 ± 0.39 versus 2.80 ± 0.24, p < 0.001) and EFT values were significantly higher in patients with p-GDM than the control group (5.5 ± 1.3 versus 4.3 ± 1.1, p < 0.001). E/E' ratio (7.21 ± 1.77 versus 6.53 ± 1.38, p = 0.003), hemoglobin A1c (5.2 ± 0.4 and 5.0 ± 0.3, p = 0.001), HOMA-IR (2.8 ± 1.4 versus 1.7 ± 0.9, p = 0.04), and hsCRP levels were significantly higher in the p-GDM group than the control group. Multivariate analysis revealed that gestational diabetes history is independently associated with CFVR., Conclusion: Women with a GDM history may be at more risk regarding coronary microvascular dysfunction compared to the healthy ones.

This study was designed to compare first-trimester maternal serum biochemical markers of aneuploidy and fetal nuchal translucency in pregnancies complicated by gestational diabetes mellitus and those of a control group. The study included 60 gestational diabetic and 60 control women who attended the first-trimester combined screening program for Down syndrome between 11 and 14 gestational weeks with complete follow-up data and delivered in our institution. Maternal serum free β-human chorionic gonadotropin, pregnancy-associated plasma protein-A and fetal nuchal translucency were investigated. The combined risks, double test risks and age risks were calculated by PRISCA software version 4.0. Comparison of the results between the two groups yielded no significant differences in serum levels of free β-human chorionic gonadotropin and fetal nuchal translucency. However, women who developed gestational diabetes mellitus had significantly lower pregnancy-associated plasma protein-A. And also, the combined risks and double test risks calculated by PRISCA software were statistically higher in gestational diabetic women than normal pregnant women. These results suggest that differences can be seen between diabetic and healthy pregnant women in first-trimester maternal serum biochemical markers of aneuploidy.