Clinical features of generalized lipodystrophy in Turkey: A cohort analysis

Aim

To describe the Turkish generalized lipodystrophy (GL) cohort with the frequency of each complication and the death rate during the period of the follow-up.

Methods

This study reports on 72 patients with GL (47 families) registered at different centres in Turkey that cover all regions of the country. The mean ± SD follow-up was 86 ± 78 months.

Results

The Kaplan–Meier estimate of the median time to diagnosis of diabetes and/or prediabetes was 16 years. Hyperglycaemia was not controlled in 37 of 45 patients (82.2%) with diabetes. Hypertriglyceridaemia developed in 65 patients (90.3%). The Kaplan–Meier estimate of the median time to diagnosis of hypertriglyceridaemia was 14 years. Hypertriglyceridaemia was severe (≥ 500 mg/dl) in 38 patients (52.8%). Seven (9.7%) patients suffered from pancreatitis. The Kaplan–Meier estimate of the median time to diagnosis of hepatic steatosis was 15 years. Liver disease progressed to cirrhosis in nine patients (12.5%). Liver disease was more severe in congenital lipodystrophy type 2 (CGL2). Proteinuric chronic kidney disease (CKD) developed in 32 patients (44.4%) and cardiac disease in 23 patients (31.9%). Kaplan–Meier estimates of the median time to diagnosis of CKD and cardiac disease were 25 and 45 years, respectively. Females appeared to have a more severe metabolic disease, with an earlier onset of metabolic abnormalities. Ten patients died during the follow-up period. Causes of death were end-stage renal disease, sepsis (because of recurrent intestinal perforations, coronavirus disease, diabetic foot infection and following coronary artery bypass graft surgery), myocardial infarction, heart failure because of dilated cardiomyopathy, stroke, liver complications and angiosarcoma.

Conclusions

Standard treatment approaches have only a limited impact and do not prevent the development of severe metabolic abnormalities and early onset of organ complications in GL.     

Effect of Er:YAG laser on removal of fibre posts from root canal

The aim of this study was to evaluate the efficiency of Er:YAG laser in translucent fibre post removal. 60 human single-rooted anterior teeth were divided into three groups (n = 20) according to post diameters (Exacto1-E1, Exacto2-E2, Exacto3-E3) and subdivided according to laser application. Fibre posts were cemented to endodontically treated teeth, and the specimens were sliced with a thickness of 1.0 ± 0.2 mm. Er:YAG laser was applied and the push out bond strength test was performed. Two-way analysis of variance was used to perform the statistical analysis. Push out bond strengths at apical area were significantly lower in all test groups (p < 0.05). Laser application caused increase in all radicular thirds, but it was significant for E1 and E3 groups. Laser application to the post-dentine interface enhances the connection between post and dentine.     

Surgical treatment of recurrent subvalvular discrete membrane and left ventricular outflow tract stenosis in an adult patient with a history of congenital cardiac surgery

Left ventricular outflow stenosis can develop at the supravalvular, valvular, and subvalvular levels. Resection of strictures at the diffuse subvalvular level is very difficult. In such pathologies, Konno-Rastan procedure provides very successful solutions as an anterior aortoventriculoplasty method. In this article, we performed anterior aortaventriculoplasty surgical treatment for tunnel type left ventricular outflow tract stenosis, recurrent subvalvular discrete membrane, and aortic regurgitation in an adult patient with a history of partial atrioventricular septal defect repair and subvalvular discrete membrane resection operation in early childhood. The Konno-Rastan procedure, which we applied to the redo case, which is rarely used in adult patients and rarely seen in the literature, is shared.     

Synthesis of flurbiprofen thiadiazole urea derivatives and assessment of biological activities and molecular docking studies

Totally 15 novel flurbiprofen urea derivatives were synthesized bearing the thiadiazole ring. Their inhibition effects on tyrosinase were determined. 3c was found to be the strongest inhibitor with the IC₅₀ value of 68.0 μM against tyrosinase. The enzyme inhibition types of the synthesized compounds were determined by examining the kinetic parameters. The inhibition type of 3c was determined as uncompetitive and the K_i value was calculated as 36.3 μM. Moreover, their cytotoxic effects on hepatocellular carcinoma (HepG2), colorectal carcinoma (HT-29), and melanoma (B16F10) cell lines were evaluated. According to the cytotoxicity results, 3l (IC₅₀ = 14.11 μM) showed the highest cytotoxicity on the HT-29 cells, while 3o (IC₅₀ = 4.22 μM) exhibited the strongest cytotoxic effect on HepG2 cell lines. Also, 3j (IC₅₀ = 7.55 μM strongly affected B16F10. The effects of synthesized compounds on the healthy cell line were evaluated on the CCD-986Sk cell line. Molecular modelling studies have indicated the potential binding interactions of the uncompetitive inhibitor 3c with the enzyme-substrate complex.