[Hyponatremia in chronic heart failure].

Hyponatremia - lowering of sodium in blood serum below 135 mmol/l - is a frequent disorder of electrolyte metabolism in patients with chronic heart failure (CHF). Hyponatremia is a risk factor of elevated mortality, repetitive hospitalizations, and worsening of kidney function in patients with decompensation of CHF. In this review we present pathophysiology of hyponatremia and propose therapeutic approach to correction of this disorder.

[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.

[Successful treatment of massive thrombosis of the vena cava inferior with nephrotic syndrome and chronic bilateral pulmonary artery thromboembolism in a patient with genetic thrombophilia].

A case is reported of a 23-year-old male patient who developed, after severe blunt injury of the lumbar region, massive thrombosis of the vena cava inferior (VCI), both renal veins, bilateral pulmonary artery thromboembolism (PATE), nephrotic syndrome (NS). In spite of anticoagulant therapy, the condition of the patient progressively aggravated for 1.5 year: thrombosis involved the ileac and femoral arteries on the right, thrombus floated in the right atrium with PATE recurrent episodes, pulmonary hypertension reached 120 mm Hg with formation of decompensated cor pulmnonale, proteinuria and hypoalbuminemia deteriorated, anasarca edema developed Multigenic thrombophilia was diagnosed (1 homozygous and 5 heterozygous mutations). A radical one-stage operation was successful: thromboectomy from the VCI, right ileac and left renal veins, thrombendarterectomy from the pulmonary arteries, suture of the interatrial septum defect, installation of cava-filter After the operation pulmonary pressure lowered to 40-45 mm Hg, right heart volume normalized, immunosuppressive therapy with prednisolone and cyclosporine led to nephropathy remission. The discussion covers mechanisms and factors (including genetic) of thrombosis progression, correlations between intravascular thrombosis, NS and chronic glomerulonephritis (possible NS development due to bilateral thrombosis of the renal veins and nephropathy role in thrombosis progression), approaches to conservative and surgical treatment of such patients. Global experience in conduction of pulmonary thrombendarterectomy and thrombectomy from VCI is reviewed (one-stage operations were not described earlier).

[Comparative characteristics of hemostasis system in patients with endstage renal disease admitted for urgent and elective hemodialysis].

AIM: To evaluate parameters of hemostasis system in patients with end-stage renal disease (ESRD) with consideration of elective or urgent start of dialysis treatment. MATERIAL AND METHODS: A total of 47 patients with ESRD entered the study. They were divided into two groups depending on urgent (group 1) or elective (group 2) start of hemodialysis. Group 1 consisted of 31 patients (13 female, 18 male) aged 18-86 years, group 2 - of 16 patients (9 female, 7 male) aged 36-79 years. The patients were comparable by ESRD causes. Clinical and laboratory findings were compared: activated partial thromboplastin time, prothrombin time, levels of fibrinogen, soluble complexes fibrin-monomers (SCFM). RESULTS: Azotemia, hyperkalemia and anemia were close to similar. Group 1 patients had more severe alterations of nutrition status and fat metabolism, marked hyperhydration and hypervolemia, arterial hypertension, more frequent neurological and infectious complications, symptoms of enteritis. Thrombotic complications developed in 51.5%, thromboses of the vascular access in 45% in group 1 vs group 2 which demonstrated only one type of thrombotic complications - thromboses of primary arteriovenous fistula (in 1 patient, 6.25%). Hemorrhagic complications were absent in group 2, in group 1 these developed 5 times less frequently than thromboses. Platelet count was significantly less (p = 0.001) in group 1 than in group 2. Hyperfibrinogenemia occurred in about 65% patients of group 1 and in 46% in group 2. SCFM levels were elevated in both groups, but in group 1 these levels were by 50% higher than in group 2 (p = 0.005). This evidences for stronger activation of intravascular coagulation in patients on urgent hemodialysis. CONCLUSION: ESRD patients admitted for urgent hemodialysis had more severe uremic syndrome with stronger activation of blood coagulation than patients admitted for elective hemodialysis. Frequency of thrombosis in patients admitted for urgent hemodialysis was 8.3 times higher than in patients admitted for elective hemodialysis.

[Endothelial dysfunction in patients with systemic lupus erythematosus].

It is currently shown that endothelial function study is a rather informative way of assessing the cardiovascular risk. Many investigations have shown the high rate of endothelial dysfunction (ED) in systemic lupus erythematosus (SLE); however, clinical data on ED in patients with SLE are scarce; which seems to be generated by difficulties in the diagnosis of this condition and to necessitate the modification of the existing diagnostic methods. The signs of ED were detected in 40% of the patients. Its development was associated with the activity of SLE, the presence of Raynaud's syndrome, elevated systolic blood pressure, plasma fibrinogen levels, and the larger daily dose ofprednisolone. The method proposed by the authors to study endothelialfunction from the diagnostic capacities on the basis of volumetric compression oscillometry is comparable with the standard ultrasound method, which allows its clinical use to evaluate the endothelial state.

[Clinicomorphological characteristics of renal disorders in patients with genetic thrombophilia].

AIM: To characterize the course and clinicomorphological features of chronic glomerulonephritis (CGN) in patients with genetic thrombophilia. MATERIAL AND METHODS: A clinical picture and evidence on renal biopsy from 25 patients (12 females, mean age 32 +/- 12 years and 13 males, mean age 36 +/- 8.8 years) admitted to hospital with diagnosis of chronic glomerulonephritis were analysed. Mean duration of renal problem to the moment of biopsy was 37.6 +/- 39 months. Renal end point was stable rise of Scr > 1.4 mg/dl for 6 months. Polymerase chain reaction defined polymorphisms of the genes MTHFR C677T; PTG G20210A; FV Leiden G1691A; FGB G455A; ITGB3 T176C L33P; PAI-1 4G/5G 675. RESULTS: Mutation in one gene was detected in 24% patients, a multigenic form of thrombophilia--in 76% patients. Morphologically, all the patients' renal tissue had the signs of thrombotic microangiopathy (TMA), 8 patients had a combination of acute and chronic TMA. TMA was the only histological sign of nephropathy in 3 (13%) patients, the rest patients showed TMA combination with different morphological variants of CGN. Sclerotic alterations were most severe in combined carriage of the alleles 4G PAI-1 and T MTHFR. A correlation was found between the renal end point and number of mutant alleles (r = 0.6, p < 0.05), the presence of allele 4G (r = 0.46, p = 0.05) and interstitial sclerosis (r = 0.5, p = 0.05). CONCLUSION: Hereditary thrombophilia promotes induction of nephrosclerosis leading to activation of intraglomerular blood clotting which contributes to CGN progression. Patients with genetic thrombophilia may develop acute TMA as the only variant of renal damage.

[Characteristics of the kidney lesion in a patient with Sneddon's syndrome].

This case report draws attention to renal damage in a young patient with Sneddon's syndrome, analyses a course of nephropathy and methods of its diagnosis, shows efficacy of anticoagulant therapy, demonstrates possible development of generalized affection of the microcirculatory bed with involvement not only of the skin and brain vessels suggesting that Sneddon's syndrome is a systemic ischemic pathology the manifestations of which in many cases mask polyorganic impairment.

[Multifocal electroretinography in ophtalmological symptoms in patients with antiphospholipid syndrome].

The paper describes the first experience in using multifocal electroretinography (mfERG) in patients with antiphospholipid syndrome (APS). In the latter, thrombogenesis is mosaic in most cases so the techniques providing a spatial imaging of the pathological process is of interest in the examination of patients with this disease. These techniques include mfERG. The quadrant mfERG analysis that reveals pathological changes in relation to the vascular involvement area is of the greatest informative value in patients with APS-associated retinal vascular occlusions.

[Types of lupus nephritis exacerbations: prognostic significance].

AIM: To characterize the course of lupus nephritis (LN) in terms of demographic indices (sex, age of renal disease onset), the presence of antiphospholipid syndrome (APS) and to ascertain a prognostic role of the disease exacerbations. MATERIAL AND METHODS: A total of 121 LN patients were followed up from 1997 to 2004 (mean duration of the follow-up 5.6 +/- 6.4 years). A LN course was characterized by the presence of a complete or partial remission, exacerbation of the disease, repeated hospitalisations. Two types of exacerbations were considered: proteinuric, running with progressive proteinuria and normal renal function (type 1); functional, running with elevation of blood creatinine (type 2). RESULTS: Exacerbations were observed in one third of the examinees, 70% of them ran with renal dysfunction. Exacerbations occurred more frequently in males than in females (50 vs 27%, respectively; p = 0.08) and in patients with early onset of LN (at the age of 40 years and younger, 80 vs 60%, respectively; p < 0.05). Exacerbations of type 2 occurred in males, in patients with early onset of renal damage and in APS association. It is shown that LN exacerbations, their incidence and type (a functional type) have a negative influence on renal survival of the patients. CONCLUSION: Identification of groups of LN patients at high risk of exacerbations and unfavourable prognostic role of exacerbations dictates the necessity of due immunosuppressive therapy for maintenance of remission.

[Lupus nephritis as a factor of atherosclerosis risk in patients with systemic lupus erythematosus].

AIM: To investigate early atherosclerosis (AS) risk factors in patients with systemic lupus erythematosus (SLE) in respect to the presence of lupus nephritis (LN) and antiphospholipid (APL) antibodies. MATERIAL AND METHODS: We analysed case histories of 137 SLE patients observed in E.M. Tareev clinic from 1970 to 2006. AS manifestations were studied by echocardiography, ultrasonic dopplerography of the peripheral vessels, x-ray methods. AS was considered early if it arose at the age under 55 years. Patients with chronic renal failure were not included in the study. RESULTS: AS development was seen in 54 (45%) patients, early symptoms appeared at the age of 25-68 years (mean 54 +/- 10 years). In 37 (31%) patients AS symptoms manifested at the age under 55 years. Significant factors of early AS risk were age, hyperlipidemia, arterial hypertension, menopause for women, APL antibodies circulation, stage IV chronic disease of the kidneys, hyperuricemia, higher blood creatinine, mean dose of prednisolone over 15 mg/day, frequent elevation of the level of C-reactive protein. A direct correlation between lupus nephritis or nephrotic syndrome (NS) and early AS was not found. In LN hyperlipidemia occurred more often (p = 0.055), lipids level and NS were not related during its remission. LN patients developed AS more frequently, had lower complement concentration in the end of the study, were treated with prednisolone more intensively than patients free of renal disease (p < 0.05). CONCLUSION: Early AS develops in more than one third of SLE patients. Main risk factors of early AS are conventional ones and APL antibodies, persistence of chronic inflammation, decreased glomerular filtration rate, prednisolone therapy. LN influence on the process of atherogenesis in SLE may be mediated and caused by high rate of other risk factors.

[Clinicomorphological features of nephropathy in primary and secondary antiphospholipid syndrome].

AIM: To investigate specific features of extrarenal manifestations of antiphospholipid syndrome (APS) in patients with APS-associated nephropathy (APSN) in primary APS and lupus nephritis (LN) with secondary APS; to compare clinicomorphological signs of APSN in primary and secondary APS. MATERIAL AND METHODS: We examined 44 APSN patients with primary APS and 90 patients with LN: 57 with secondary APS, 33 with antiphospholipid antibodies (APA) without history of thrombosis. In addition to clinical and immunological examination, detection of serological APS markers, morphological examination of renal tissue and ultrasound dopplerography (USDG) of the renal vessels were made (in some patients) for assessment of the condition of intrarenal vascular bed. RESULTS: In patients with primary APS, renal disorder and secondary APS in LN frequency of arterial thrombosis doubles that of venous ones. Renal disorder irrespective of a clinical APS form (primary, secondary) combines with affection of the CNS, heart and skin (livedo). This correlates with frequency of arterial thrombosis. In patients with primary APSN rate of arterial hypertension (AH), especially severe, and renal dysfunction is higher than in LN with APS while this group is characterized by more severe proteinuria, microhematuria and higher incidence of nephrotic syndrome. A direct correlation exists between the incidence of arterial thrombosis and severity of AH, between AH and renal ischemia by USDG. Morphologically, glomerulosclerosis, marked arteriolosclerosis and diffuse interstitial sclerosis occur more often in patients with primary APSN compared to LN patients with APS. CONCLUSION: In primary and secondary (in SLE) APS combination of APSN with impairment of the CNS, heart and skin, correlation of its basic clinical manifestations with arterial thrombosis allow us to single out a special clinical variant of APS manifesting with generalized ischemic lesions of the organs as a result of arterial/arteriolar thrombosis. Irrespective of its nature, APSN has common characteristic features--combination of AH, persistent renal dysfunction and transitory hypercreatininemia--correlating with development of arterial thrombosis; therefore, this pathology can be considered as a variant of thrombotic vascular lesion of the kidneys.

[Ischemic disease of the kidneys and combined chronic nephropaties: clinical and prognostic features].

AIM: To characterize clinical features and course of ischemic renal disease (IRD) combined with other chronic nephropathies. MATERIAL AND METHODS: We examined 102 patients with IRD treated in the E. M. Tareev clinic of the I. M. Sechenov Moscow Medical Academy in 2001-2006. We made a general clinical examination, clinical and biochemical blood tests. Glomerular filtration rate (GFR) was calculated according to Cockroft-Gault formula. Arterial hypertension was assessed according to ESH. RESULTS: IRD associated with other chronic nephropathies was detected in 35 (34.3%) patients. Association of atherosclerotic stenosis of the renal arteries (ASRA) with diabetic, urate nephropathies was diagnosed in 9 and 8 patients, respectively; chronic pyelonephritis--in 7 cases, chronic glomerulonephritis--in 4 patients. The age of IRD patients associated with other chronic renal diseases was younger than of those with isolated IRD. Systolic arterial pressure was significantly higher in patients with isolated IRD, body mass index and total cholesterol--in the groups of IRD association with other chronic nephropathies. Aggravation of renal failure in patients with CRD association with other chronic nephropathies was provoked by ACE inhibitors and blockers of angiotensin II receptors. Revascularization of the kidneys in IRD associated with other chronic nephropathies leads to significant lowering of systolic blood pressure and stabilization of creatininemia. CONCLUSION: Development of IRD is possible in the presence of other chronic nephropathies. Diagnosis of IRD arising in patients suffering from other chronic renal diseases often requires radical change of therapeutic policy, but renal revascularization is not contraindicated.

[Clinical and morphological characteristics of lupus nephritis in systemic lupus erythematosus with antiphospholipid syndrome].

AIM: To ascertain clinical and morphological features of lupus nephritis (LN) in systemic lupus erythematosus (SLE) associated with antiphospholipid syndrome (APS). MATERIAL AND METHODS: Immunological markers of SLE and APS, clinical picture, urine indices were examined in 138 patients with SLE, APS and renal dysfunction. RESULTS: LN associated with APS is characterized with marked arterial hypertension, such patients had arterial thromboses more frequently than patients with isolated LN. Patients with anticardiolipin antibodies have arteriolosclerosis, in APS - diffuse interstitial sclerosis. CONCLUSION: Renal impairment in SLE may run not only with LN but also with thrombotic microangiopathy modifying clinical symptoms and course of the disease.

[Clinical significance of myocardial lesion in antiphospholipid syndrome].

Antiphospholipin syndrome (APLS) is one of the most frequent reasons for arterial and venous thromboses. Primary and secondary APLS can lead to thrombotic process in coronary arteries. The biggest diagnostic difficulties appear in cases of small coronary vasal involvement leading to diffuse myocardial lesion. Perfusion myocardial scintigraphy (MS) allows specification of the character of myocardial changes. Revealing of myocardial changes by means of MS makes it possible to start timely anticoagulative therapy, which significantly improves prognosis and life quality.