Bilateral diffuse uveal melanocytic proliferation with good clinical response to plasmapheresis and treatment of the primary tumor.

PURPOSE: To report successful treatment of bilateral diffuse uveal melanocytic proliferation. METHODS: Case report of a patient with clinical diagnosis of bilateral diffuse uveal melanocytic proliferation, imaged with fluorescein angiography and spectral domain optical coherence tomography, and with follow-up data after treatment with plasmapheresis, radiation, and chemotherapy. RESULTS: A 67-year-old white man presented with a history of bilateral rapidly declining vision. The ophthalmoscopic examination, fluorescein angiography, and optical coherence tomography showed in both eyes an exudative retinal detachment and subretinal lesions highly suggestive for bilateral diffuse uveal melanocytic proliferation. Further investigation demonstrated a large cell lung carcinoma. Subsequently, the patient was treated with plasmapheresis, radiation, and chemotherapy. In a few weeks, the exudative retinal detachments resolved with gain of vision, and the retina remained flat during the 5-month follow-up. CONCLUSION: Plasmapheresis and treatment of the primary tumor are valuable treatment options for visually impaired patients with bilateral diffuse uveal melanocytic proliferation.

Generalized alterations in the biomechanical properties of large veins in non-thrombotic thrombophilic young patients.

AIM: In young, post-thrombotic patients, venous distensibility is decreased not only in the affected lower limb, but also in the contralateral limb and in the jugular vein when compared to age-matched control subjects. In the present study, we investigated venous wall mechanical properties in young, asymptomatic thrombophilic patients. METHODS: Eleven young (24+/-0.4 years) control subjects and 9 age-matched patients (21.1+/-1.8 years) with proven thrombophilic molecular defects, but without any signs or history of previous deep vein thrombosis, were compared. Anterolateral and mediolateral diameters of the common femoral, axillary and internal jugular veins were measured by ultrasonography in situ. Pressure alterations were induced by altering body positions and by pressure-controlled Valsalva tests. Distensibility was calculated from diameter and pressure changes. RESULTS: In thrombophilic patients, resting diameter of both the common femoral and of internal jugular veins at low transmural pressure was larger than those for the control subjects. Distensibility, however, was significantly less when high pressures were applied. Alterations in diameter of the axillary vein were minimal. CONCLUSION: Our measurements suggest that there are generalized changes in venous mechanical properties in thrombophilic patients even before the appearance of thrombotic processes. These biomechanical alterations of the venous wall and/or surrounding connective tissue are similar to those found in connection with aging and in post-thrombotic patients. The pathological mechanisms behind these processes are unknown.

Fibrinolysis in a lipid environment: modulation through release of free fatty acids.

BACKGROUND: Thrombolysis is conventionally regarded as dissolution of the fibrin matrix of thrombi by plasmin, but the structure of clots in vivo includes additional constituents (proteins, phospholipids) that modulate their solubilization. OBJECTIVE: We examined the presence of free fatty acids in thrombi and their effects on distinct stages of fibrinolysis (plasminogen activation, plasmin activity). METHODS AND RESULTS: Using the fluorescent probe acrylodated intestinal fatty acid-binding protein, variable quantities (up to millimolar concentrations) of free fatty acids were demonstrated in surgically removed human thrombi. Oleic acid at relevant concentrations reversibly inhibits more than 90% of the amidolytic activity of plasmin on a synthetic substrate (Spectrozyme PL), but only partially inhibits its fibrinolytic activity measured using turbidimetry. Chromogenic assays detecting the generated plasmin activity show that plasminogen activation by tissue-type plasminogen activator (t-PA) is completely blocked by oleic acid in the fluid phase, but is accelerated on a fibrin matrix. A recombinant derivative of t-PA (reteplase) develops higher fibrin specificity in the presence of oleic acid, because both the inhibition of plasminogen activation in free solution and its enhancement on fibrin template are stronger than with wild-type t-PA. CONCLUSION: Through the stimulation of plasminogen activation on a fibrin template and the inhibition of plasminogen activators and plasmin in the fluid phase, free fatty acids confine the action of fibrinolytic proteases to the site of clotting, where they partially oppose the thrombolytic barrier function of phospholipids.

Thrombophilia and venous thromboembolism. International consensus statement. Guidelines according to scientific evidence.

Thrombophilia is the term now used to describe predisposition to increased risk of venous and occasionally arterial thromboembolism due to hematological abnormalities. It can be a multifactorial disorder where congenital defects of anticoagulant or procoagulant factors may be combined with acquired hematological abnormalities. It should be considered in patients with a documented unexplained thrombotic episode or a positive family history. The aim of this document is to provide guidelines for investigation and management of patients with thrombophilia in the presence or absence of venous thromboembolism (VTE).

[Anatomical and extra-anatomical reconstructions in aorto-iliac occlusive diseases]. / Anatomikus és extraanatomikus rekonstrukciók az arteria iliaca rendszer occlusiv betegségeiben.

PURPOSE: In the case of the aorto-iliac arteries occlusion there are two different operative reconstructive possibilities. As an anatomical reconstruction the open desobliteration of the iliaca arteries, the retrograde half-closed desobliteration or the aorto/ilio-femoral bypasses can be mentioned. As an extraanatomical solution ilio/femoro-femoral crossover bypass can be implanted. We compared the results of these two types of operations. PROCEDURE: Between 1 January 1998 and 31 December 1999 at the Cardiovascular Surgical Department of the Semmelweis University Budapest 239 primary, reconstructive operations were done because of the iliac arteries occlusion. We made 175 anatomical and 64 extraanatomical operations. We controlled the results of this operation in this retrospective study. RESULTS: Since the operation 12 patients died, most of them because of cardial reasons. Hundred sixty seven survival patients could be involved in the study. There was no significant difference regarding the age and the Fontaine stadium of the patients in the two groups. Two-third of the patients underwent anatomical, the others extraanatomical operations. The patency rate in the anatomical group was 92.8%, with the extraanatomical patients 90%. In the view of the postoperative function after the anatomical reconstruction the claudication distance was longer. There were two limb amputations, one of them because of distal progression of the atherosclerosis, in the other case the reason was reocclusion of the graft. DISCUSSION: In this study we examined patients who underwent an operation about 3 years ago. The preoperative stadium of the two non-selected groups was similar. The postoperative patency rate was quite similar, but the postoperative function after the anatomical reconstruction was significantly better. In our opinion the results of the two different types of the reconstruction used by proper indication were the similarly satisfactory.

[Thrombolytic therapy as pretreatment before vascular operations and radiologic interventions]. / Ersebészeti és intervencionális radiológiai beavatkozások elókészítése trombolízis alkalmazásával.

The authors describe their 60 cases of thrombolysis with steptokinase (SK). Thrombolysis was required in 55 patients because of arterial, while in 5 patients because of venous side thrombosis. The 73% of the patients with arterial occlusion where thrombolysis was applied belonged Fountain stage IV, while 27% to Fountain stage III. Graft occlusion occurred in 60% of all cases and the acute or subacute thrombosis of the native vessels required thrombolysis in 40%. The thrombolysis alone was sufficient in 26 patients, while it was completed with PTA in 9, with PTA and implantation of stent in 1 and with vascular surgical procedure in 10 cases. Reconstruction surgery was the final solution in 4 patients, for whom the thrombolysis was inadequate. Amputation was unavoidable in 8 cases. The thrombolysis therapy was successful in 77% in our experience. The high number of hemorrhagic complications was due to the bleeding of puncture hole. It needed surgical suture in 10 cases.

[Dyslipoproteinemia and chronic kidney failure]. / Dyslipoproteinémie a chronické selhání ledvin.

Lipoprotein abnormalities are a regular part of metabolic changes associated with chronic renal failure. The character of dyslipoproteinaemia changes with the severity of disorders of renal functions, from initial deviations in the composition and distribution of circulating lipoprotein particles (a decline of glomerular filtration to 0.7-0.8 ml/s) to differently expressed changes of plasma lipid concentrations in terminal renal failure. The basis of the pathogenetic mechanism of these lipid abnormalities is the negative effect of the uraemic environment on the formation and catabolism of triglyceride-risk lipoproteins and on the function of the reverse cholesterol transport. An important part is also played by the modification of lipoprotein particles by oxidation and glycation. To a different extent also the nutritional status is manifested via the unfavourable composition of dietary fats, reduced effectiveness of antioxidant factors and in some instances also carnitine deficiency. Haemodialysis treatment and in particular peritoneal dialysis modify these lipid abnormalities. From the quantitative aspect renal dyslipoproteinaemia is not very striking, despite this its quantitative changes are important. It may have a negative impact on the progression of renal disease by its participation in the development of glomerular sclerosis and tubulointerstitial fibrosis. As one of the important risk factors it participates also in the acceleration of atherosclerosis in patients with chronic renal failure and in their much higher cardiovascular mortality as compared with the general population. These factors justify efforts to influence uraemic dyslipoproteinaemia. Fibrates or statins are indicated in conjunction with the supporting effect of diet and modification of the dialysis regimen. In tables and figures some results assembled by the authors are presented obtained in a group of dialyzed patients (characteristic of the lipid profile under basal conditions on fasting and after an oral lipid load and experience with influencing dysliopoproteinaemia by fenofibrates and atorvastatin).