[A case of rare inhibitory coagulopathy - acquired hemophilia A].

Inhibitory coagulopathy is a rare variant of hemorrhagic syndrome. Acquired hemophilia A is caused by the formation of inhibitors (antibodies) to Factor VIII of the blood coagulation system leading to impaired activation of the key stage of blood clotting (factor X) and development of hemorrhagic syndrome of different severity. Acquired hemophilia A is a rare disease with an incidence of 1.38-1.48 per 1 million population per year. We report a case off severe idiopathic acquired hemophilia A in a 53 year-old woman manifest as skin hemorrhages, subcutaneous and intramuscular hematomas. Hemostatic therapy described in the article resulted in the elimination of hemorrhagic syndrome and complete remission. This case represents a rare disease the knowledge of which can be useful for preventing the development of debilitating complications, and sometimes saving the patient's life.

[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.

[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.

[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.

[Novel possibilities of antithrombotic therapy in patients with chronic heart failure].

Modern approaches to prevention of venous thromboembolic complications in patients with chronic heart failure are analyzed in this review which contains results of large studies of low molecular weight heparins. In MEDENOX trial the use of enoxaparin in medical patients was associated with 63% reduction of risk of thrombosis. The authors own experience showed that 2 weeks of therapy with enoxaparin in patients with chronic stage IIB-III heart failure caused significant lowering of soluble fibrin-monomer complexes, fibrinogen, and index of turbo-dynamic potential. These changes evidenced for decreased intravascular blood coagulation. Thus enoxaparin can be effectively used for prevention of thrombosis and thromboembolism in patients with chronic heart failure. Novel antithrombotic agents fondaparinux, idraparinux, ximelagatran, recombinant thrombomodulin are perspective medications for prevention of venous thromboses and embolism in medical patients.

[Clinical manifestations of APS-nephropathy in primary antiphospholipid syndrome]. / Klinicheskie proiavleniia nefropatii, sviazannoi s antifosfolipidnym sindromom, pri pervichnom antifosfolipidnom sindrome.

AIM: To elicit clinical features of nephropathy associated with antiphospholipid syndrome (APSN) in patients with primary antiphospholipid syndrome (PAPS). MATERIAL AND METHODS: The analysis of clinical characteristics and course of APSN has covered 24 patients with PAPS (16 females and 8 males, mean age 34.3 years). Renal damage was represented by arterial hypertension (AH), urinary syndrome, functional decline. All the patients were tested for anticardiolipin antibodies and/or lupus anticoagulant. Renal biopsy was made in 7 patients. RESULTS: PAPS patients developed renal affection in the onset of APS or within the first 5 years of its course. In the majority of patients APSN combined with abnormalities of CNS, heart and skin. Arterial/arteriolar thromboses prevailed. APSN manifested with: AH (n = 23, severe AH in 11), abnormal renal filtration (n = 17, creatinine rise in 8), urinary syndrome with proteinuria (n = 23, in 14 with hematuria). The following clinical variants of APSN were proposed: urinary syndrome with AH (n = 16; 67%), acute nephritic syndrome (n = 7; 29%), nephrotic syndrome (n = 1). Morphological studies of biopsies from APSN patients have revealed sclerotic changes, thrombotic microangiopathy, nonspecific alterations in the glomeruli. CONCLUSION: APSN is a variant of microvascular renal affection caused by thrombotic processes in intra-organ microcirculation. It is an early clinical marker of APS. Clinically, APSN manifests with vascular renal affection, the earliest symptom being inhibition of glomerular filtration. Clinical combinations of the symptoms allow to distinguish variant of APSN suggesting the existence of acute and chronic APSN. Combination of APSN with affection of the CNS, heart and skin points to a special PAPS subtype characterized by generalized ischemic damage to the organs as a result of intraorganic arterial and/or arteriolar thromboses.

[Effect of fragmin, a low-molecular heparin, on hemostasis in patients with alimentary-constitutional obesity]. / Vliianie nizkomolekuliarnogo geparina fragmina na gemostaz u bol'nykh alimentarno-konstitutsional'nym ozhireniem.

The authors conducted comparative study of the hemostasis indices before and in the immediate period after operation for formation of a small stomach in 31 patients with stage III-IV alimentary-constitutional obesity, in 50 patients without obesity who underwent operation on the stomach, and in 15 volunteers. Nonspecific prevention of thrombogenesis was performed in all patients with normal weight and those with obesity. Patients with obesity were given in addition specific thrombogenesis prevention with nonfractionated heparin (group I) and fractionated low-molecular heparin-fragmin produced by KABI (groups II and III). Study of the parameters of the hemostasis system in the postoperative period showed nonspecific prevention of thrombogenesis to be sufficient to reduce the risk of pulmonary artery phlebothrombosis and embolism in most patients without obesity. In patients with obesity the probability of phlebothrombosis and thromboembolism is much higher, in view of which they must be given heparin in addition to nonspecific prevention. Fragmin, as an agent for thrombosis prevention, has certain advantages over nonfractionated heparin and should be used more frequently in clinical practice in patients of the risk group, e. g. with pathological obesity, in a dose no less than 100 U/kg. With the use of fragmin laboratory control before each injection is not needed. Administration of nonfractionated and fractionated heparins must be combined with bandaging of the lower limbs and other measures of nonspecific prevention of thrombogenesis.

[The Kearns-Sayre syndrome]. / Sindrom Kirnsa--Seira.

The paper describes Kearns-Sayre's syndrome, a rare hereditary neuromuscular disease, in a patient aged 17 years. The clinical picture of the disease had a classical triad: external ophthalmoplegia, pigmentary retinopathy, and cardiac conduction disturbances. This triad was supplemented with other polymorphous symptoms characteristic of the syndrome, such as moderate myopathic syndrome, hemeralopia, physical infantilism, hypogonadism, pyramidal syndrome. Bifascicular block in the His-Purkinje system was accompanied by mitral prolapse. The problems of early diagnosis of the syndrome and choice of adequate therapeutical methods are discussed.

[The indices of the hemostatic system in atopic dermatitis patients during treatment by hyperbaric oxygenation]. / Izuchenie pokazatelei sistemy gemostaza u bol'nykh atopicheskim dermatitom v protsesse lecheniia giperbaricheskoi oksigenatziei.

Studies of the blood coagulation processes in the patients with atopic dermatitis have revealed an increase in the activities of the blood coagulation system in the acute phase of the disease, the degree of these changes depending on the severity and activity of the pathologic process. Hyperbaric oxygenation, an effective treatment modality for atopic dermatitis, normalized some of the altered parameters of the hemostasis system.