[Alterations of the complement system in patients with catastrophic antiphospholipid syndrome].

AIM: To investigate alterations of the complement system in patients with catastrophic antiphospholipid syndrome (CAPS). SUBJECTS AND METHODS: Four patients (2 men aged 23 and 40 years and 2 women aged 39 and 58 years) diagnosed as having CAPS, including 3 patients with systemic lupus erythematosus and secondary antiphospholipid syndrome (APS) and 1 patient with primary APS, were examined. The activity of the complement components C1-C5 and total hemolytic activity were determined in all the patients at the moment of an acute episode and in 1 patient after treatment. RESULTS: The activity of the studied complement components and total hemolytic complement activity proved to be significantly decreased in all the patients. That of complement components recovered after treatment using fresh frozen plasma. The possibility and mechanisms of complement system activation in the patients with CAPS are discussed. CONCLUSION: The preliminary results obtained by the examination of few cases may lead to the conclusion that the complement system may be involved in the development of CAPS.

[Significance of the factors of hypoxia and endothelial dysfunction in kidney injury in the presence of obesity].

AIM: To define the clinical significance of asymmetric dimethylarginine (ADMA) and that of methylenetetrahydrofolate reductase (MTHFR) gene polymorphism as factors of endothelial dysfunction (ED) in the development of early kidney injury in obese patients. SUBJECTS AND METHODS: The investigation included 86 patients (64 men and 22 women aged 44 +/- 11 years) with abdominal obesity. Along with physical examination, the authors determined albuminuria, calculated glomerular filtration rate (GFR) using the Modification of Diet in Renal Disease (MDRD) formula, estimated insulin resistance markers (fasting plasma insulin and C-peptide concentrations and homeostatic model assessment (HOMA) index), as well as serum ADMA levels by enzyme immunoassay in all the patients. C677T polymorphism in the MTHFR gene was studied by allele-specific polymerase chain reaction and restriction fragment length polymorphism analysis. Kidney injury (chronic kidney disease (CKD)) was diagnosed using the Kidney Disease Outcomes Quality Initiative (KDOQI) criteria. Early vascular remodeling was determined from the increased intima-media thickness (IMT) of the common carotid artery (CCA). RESULTS: CKD was diagnosed in 27(31%) patients. The latter, unlike the patients with CKD, were observed to have more pronounced obesity (body mass index (BMI) 36.8 +/- 8.0 and 32.0 +/- 4.7 kg/m2, respectively (p < 0.001)), waist circumference (119 +/- 18 and 109 +/- 11 cm (p = 0.002)), higher levels of C-peptide (1348 +/- 363 and 1028 +/- 363 pmol/I; p < 0.001), insulin (16.9 +/- 7.3 and 11.7 +/- 5.5 microU/ ml; p < 0.001), and HOMA index (4.3 +/- 1.7 and 2.9 +/- 1.5; p < 0.001). In the patients with Stage IIIa CKD, ADMA concentrations (0.77 +/- 0.19 micromol/l) was higher than in those with Stages I (0.58 +/- 0.11 micromol/l; p = 0.048) and II (0.61 +/- 0.13 micromol/l; p = 0.071). An association between ADMA concentrations, CCA IMT, and estimated GFR was revealed in the patients with CKD. The predictors of an estimated GFR reduction in obesity were elevated serum concentrations of ADMA, uric acid, insulin, and HOMA index. The combination of obstructive sleep apnea syndrome and metabolic syndrome increased the risk of CKD by 2.1-fold (95% confidence interval, 1.06-3.14). Evaluation of the impact of MTHFR gene polymorphism on kidney injury in obesity disclosed that the patients with homozygous carriage of the abnormal T allele of the MTHFR gene had a higher risk for Stages I-IIIa CKD (2.60 with 95% confidence interval, 1.32-3.88), more marked obesity and hyperinsulinemia, and increased serum ADMA concentrations. CONCLUSION: Insulin resistance and ED hold a central position in the pathogenesis of CKD in obese patients. The mechanisms of the atherosclerotic vascular remodeling associated with elevated serum ADMA concentrations are of paramount importance in the progression of early-stage CKD. The homozygous carriage of the abnormal T allele of the MTHFR gene increases the risk of Stages I-IIIa by more than twice.

[Association of obstructive sleep apnea syndrome with the signs of nonalcoholic fatty liver disease and calculated glomerular filtration rate in metabolic syndrome].

AIM: to assess a relationship between obstructive sleep apnea syndrome (OSAS) and the signs of nonalcoholic fatty liver disease (NAFLD) in patients with metabolic syndrome (MS). MATERIAL AND METHODS: Serum total cholesterol, triglycerides, high-density lipoprotein cholesterol concentrations, fasting blood glucose, ASAT, ALAT, gamma-glutamyl transpeptidase (gamma-GT), alkaline phosphatase (AP), total bilirubin, uricemia, and creatininemia were determined in 32 patients (mean age 47.6 +/- 9. 7 years) with MS. The authors also measured fasting blood insulin levels with the further calculation of the homeostatic model assessment (HOMA) index, as well as plasma C-peptide concentration. Abdominal ultrasound study and OSAS diagnosis by the routine scheme were made in all the patients. RESULTS: OSAS was associated with the significant increase in the body mass index (40.5 +/- 9.4 and 31.9 +/- 4.6 kg/m2; p = 0.001), waist circumference (130.0 +/- 22.5 and 107.5 +/- 11.0 cm; p = 0.001), and C-peptide levels (1611.0 +/- 614.0 pmol/1; p = 0.019), and the serum activities of gamma-GT (117.0 +/- 57.0 and 42.01 +/- 14.0 IU/ml; p = 0.05) and AP (80.0 +/- 53.0 and 74.0 +/- 48.0 UI/ml; p = 0.05). The patients OSAS had also a significantly lower glomerular filtration rate (GFR) estimated using the modification of diet in renal disease (MDRD) formula (66.0 +/- 19.0 and 82.0 +/- 21.0 ml/min/1.73 m2; p < 0.05). CONCLUSION: The presence of OSAS in patients with MS is associated with the increase in laboratory signs of NAFLD and the decrease in the GFR calculated by the MDRD formula.

[Myocarditis and fibroplastic endocarditis in Wegener's granulomatosis].

The paper describes an autopsy case of Wegener's granulomatosis with a rare variant of a cardiac lesion--the concurrence of aortic valvular disease (aortic regurgitation), fibroplastic endocarditis, and complete transverse blockade, followed by pacemaker implantation. The direct cause of death in the female patient was multiple organ dysfunction: progressive chronic heart failure, adult respiratory distress syndrome, acute hepatorenal failure, and pancreonecrosis. The data available in the Russian and foreign literature on investigations dealing with cardiac pathology in patients with Wegener's granulomatosis are analyzed.

[Aortal regurgitation and atrioventricular block III in Wegener's granulomatosis].

The article presents a clinical observation of a female patient suffering from Wegener's granulomatosis with a rare variant of cardiac involvement--a combination of an aortal valvular disease (aortal regurgitation) and total atrioventricular blockade--who later underwent pacemaker implantation. The direct cause of the lethal outcome in this patient was destructive pancreatitis. Data from Russian and foreign literature on cardiac pathology in patients with Wegener's granulomatosis are analyzed.