[Acquired von Willebrand's disease in the course of severe primary hypothyroidism in a patient with autoimmune polyglandular syndrome type 3]. / Zespól nabytego niedoboru czynnika von Willebranda w przebiegu glebokiej pierwotnej niedoczynnosci tarczycy u chorej z autoimmunologicznym zespolem niedoczynnosci wielogruczolowej typu III.

The case of a 20-year old female, who had been followed because of von Willebrand disease (vWD) was presented in this paper . She had a past history of menorrhagia and bleeding after dental procedures and the activity of von Willebrand factor (vWF) was decreased. Because of suggestive clinical features, the workup for hypothyroidism was performed and the patient was found to have severe hypothyroidism due to Hashimoto thyroiditis. After the institution of replacement therapy with levothyroxine, von Willebrand factor activity returned to normal range and symptoms of von Willebrand disease disappeared. Based on these findings, the diagnosis of acquired von Willebrand syndrome (AvWS) due to hypothyroidism was made. The development of myasthenia led to the final diagnosis of autoimmune polyglandular syndrome type 3 (APS) with myasthenia gravis and vitiligo.

Serum selenium concentration in patients with Wilson's disease.

BACKGROUND/AIMS: Wilson's disease is a genetically determined disorder of copper metabolism in the liver. Due to the toxic accumulation of this trace element, body organs are damaged by free radical generation, lipid peroxidase and inhibition of synthesis of some proteins. Behavior of anti-oxidative factors in Wilson's disease has not been completely evaluated yet. The aim of the study was to assess blood serum concentrations of selenium in patients with Wilson's disease. METHODOLOGY: Twenty-five patients with Wilson's disease and 30 healthy volunteers, constituting a control group were included in the study. The patients were in good clinical condition. In all the subjects blood serum concentrations of selenium were tested using the atomic absorption spectroscopy, hydride generation method. RESULTS: Selenium concentrations in the blood serum of the patients and healthy controls did not show statistical differences between both groups. Correlations between selenium concentrations and biochemical parameters: activity of alanine and aspartate aminotransferase, alkaline phosphatase, gamma-glutamyltranspeptidase, concentration of bilirubin, albumin and gamma globulin, international normalized prothrombin index as well as serum copper, ceruloplasmine and 24-h urine copper excretion were assessed. Statistically significant correlation was found only between selenium concentration and aspartate aminotransferase activity. No statistically significant differences between selenium concentrations in the serum of patients with different forms of Wilson's disease were found. CONCLUSIONS: On the basis of the results obtained in the study it can be assumed that in treated patients with Wilson's disease the antioxidant status measured as serum selenium concentration is comparable to healthy controls.

[Multi-reasons, severe megaloblastic anaemia--description of two cases]. / Wieloprzyczynowa ciezka niedokrwistosc megaloblastyczna--opis dwóch przypadków.

We report two cases of young patients (44 and 26 years old) with severe macrocytic, megaloblastic anaemia in course of Addison-Biermer's disease. In addition we found also other reasons of vitamin B12 deficiency. The one of them was chronic autoimmune thyroiditis and the second was, surely, Helicobacter pylori infection. The presented cases confirm the importance of spreading the diagnosis with antithyroid antibodies detection and Helicobacter pylori test in all patients with Addison-Biermer's diseaese.

[Portal vein thrombosis in patients with thrombophilia--own observations]. / Zakrzepica ukladu zyly wrotnej u chorych z trombofilia-- obserwacje wlasne.

Portal vein thrombosis is one of the main prehepatic causes of portal hypertension. The most frequent causes of thrombosis in this localization, apart from hepatic cirrhosis, are the following: acute inflammatory diseases and abdominal cancers, traumas, proliferative diseases of the hematopoietic system. In recent years attention was given to disorders in hemostasis, such as thrombophilia, in the course of which thrombosis development is particularly common. The authors present 10 patients after an incident of portal vein thrombosis, in which primary hepatic pathology was excluded and tests directed at thrombophilia were performed. In seven patients abnormalities in the examined parameters were found, and what is more, in two cases they had a complex character and involved more than one parameter. In five patients hyperhomocysteinemia was found. Among them, in two patients there was also a decreased protein S activity and in one of them there was also APC-resistance. In the next two patients there were abnormalities in one of the examined parameters - APC-resistance. Hyperhomocysteinemia was found in all patients with idiopathic thrombosis, and in one of them there were concurrent changes in protein S activity and APC-resistance. In patients with the history of portal vein thrombosis diagnostics of thrombophilia should be performed.

[Negative D-dimers and exclusion of venous thromboembolism--own experience]. / Ujemny dimer D a wykluczenie zylnej choroby zakrzepowo-zatorowej--doswiadczenia wlasne.

The assessment of D-dimer concentration has become essential step during diagnostic algorithm of venous thromboembolism (VTE). This test characterizes high sensitivity but limited specificity. Negative D-dimer with high probability excludes VTE. The aim of this study was to assess the percentage of patients treated in Department of Internal Medicine, Endocrinology and Haemostatic Disorders, Medical University of Gdanisk, who in spite of clinical signs of VTE showed normal D-dimer level. Between 2000 and 2004 in our department 57 cases with recent deep vein thrombosis (DVT) were diagnosed, in 2 cases with co-existence of pulmonary embolism (PE). The D-dimer concentration was assessed in patients' plasma with the use of immunoturbidometry. Between 57 cases with VTE, 7 patients (12%) showed normal D-dimer level (<500 microg/ml). This group consisted of 4 men and 3 women, aged from 40 to 82 years (the mean age of 58 years). In all 7 cases DVT was diagnosed, in 2 patients with concomitent PE. The final diagnosis was confirmed by compression ultrasonography and pulmonary scintigraphy. Our analysis underlines the observation that occurrence of VTE and negative d-dimer concentration is possible and may probably be related to methodological limitations. However, the lack of increase of D-dimer could also be caused by fibrinolysis alteration.