[Hemoglobin Haná or alpha 2 beta 2 63 (E7) His-Asn: a new unstable hemoglobin variant with a paradoxically different clinical manifestations in smokers and non-smokers in the same family].

V Divoký, M Luhový, M Divoká, R Melichárková, Pospísilová, K Indrák

Vnitr Lek 1997, 43(5):267-272

The authors describe the identification and the clinical manifestations of a new structural variant of haemoglobin found in three subjects from two generations of a Moravian family. It is manifested by mild haemolytic anaemia with Heinz bodies in the proband and a slightly elevated value of methaemoglobin. The sequential analysis of the beta-globin gene provided evidence that the cause is mutation CAT-AAT in codon 63 which leads to the exchange of distal histidine /E7/ for asparagine. The ratio of beta X:beta A is 38:62. The mother with the same mutation is asymptomatic. The relative amount of beta X:beta A mRNA transcripts in mother and daughter are...

[A fully implantable port system for venous access. Personal experience with 91 patients].

P Zák, M Tauchman, V Maisnar, K Podzimek, S Mirová, J Voglová, P Dulícek, S Filip, L Chrobák

Vnitr Lek 1997, 43(5):273-278

A fully implantable venous port system with a cannula inserted into the central venous system was implanted to 91 patients. The system was used for the administration of different types of intravenous preparations, incl. transfusions of erythrocyte mass and parenteral nutrition. With in the framework of autologous transplantation via the port system haematopoietic progenitor cells from the peripheral blood stream were administered. Part of the introduced systems was used for monitoring of the central venous overpressure and for collecting blood samples. The system was introduced on an average for 289 days, with a median of 245 days, range 82-872 days....

[External quality control of hematologic tests in the Czech Republic 1994-1996].

J Horák, M Budina

Vnitr Lek 1997, 43(5):279-284

In 1994 in the Czech Republic external quality evaluation of haemograms was started. Quality was evaluated by sending control samples to laboratories and by comparing the results of their assessment with the correct value. A permitted range to results was assessed: for platelets 24%, for leucocyte 20% and for erythrocytes, haemoglobin, haematocrit and the mean corpuscular volume (MCV) 10%. The relative error of blood cell count was expressed by means of a coefficient of variance. The error declined during the period of evaluation, during the last cycle it was 4% for erythrocytes 8% for leucocytes and 10% for platelets. Assessment of haemoglobin was...

[Clinical characteristics of mycosis fungoides and Sézary syndrome].

L Krc, J Dobesová, M Bucek, J Dusek, J Macák, D Galuszková, O Jurysek

Vnitr Lek 1997, 43(5):285-289

Mycosis fungoides and Sézary syndrome are typical representatives of malignant lymphomas of the T-series affecting the skin. Both affections have a varied clinical picture, a different spectrum of histological and laboratory findings and lack a uniform prognosis. In the submitted review the authors discuss in more detail all mentioned aspects of the disease and draw attention to new therapeutic possibilities.

[Diagnosis of mycotic infections in oncology patients using the polymerase chain reaction].

J Vorlícek, J Mayer, A Kovarík, J Cíhalová, V Kubálek, D Dvoráková

Vnitr Lek 1997, 43(5):290-297

Diagnosis of mycotic infections is despite the immense effort devoted to this problem still very inaccurate. A new and promising method is the polymerase chain reaction, PCR, which theoretically can detect a single cell. In their original study the authors decided to develop a new method for the detection of fungi by PCR and to compare this examination with post-mortem findings. Thus it was possible to determine sufficiently reliably the sensitivity and specificity of the method. For the detection of fungi the authors selected the sequence coding for a small subunit of ribosomal RNA (18S rDNA). The method is able to detect the amount of DNA from some...

[Clinical findings in individuals with the factor V Leiden mutation].

M Matýsková, Z Vorlová, I Hrachovinová, A Buliková, J Zavrelová, M Slechtová

Vnitr Lek 1997, 43(5):298-301

Leiden mutation of the coagulation factor V is the most frequent known congenital risk factor of thrombophilia. The authors examined a group of 440 subjects with thrombosis in the case- or family-history. The mutation was found in 146. In 94 thrombotic manifestations were recorded in the case-history, five women were examined because of repeated abortions. 52.74% carriers of FVL had venous thrombosis of the lower extremities and pelvis in their case-history, 19.18% had pulmonary embolism in the case-history. In 27.40% during the initial manifestations of thrombosis no other risk factor of thrombosis was detected. In 10.27% the first thrombosis developed...

[Treatment of MALT lymphoma of the digestive tract].

J Hudecek, M Paceková, R Michalová, P Szépe, M Adamkov, R Hyrdel, L Plank, P Kubisz

Vnitr Lek 1997, 43(5):302-306

Mucosa-associated lymphoid tissue (MALT) is an autonomous part of the immune system with a specific function, histological structure and circulation of cells. As a result of malignant transformation of this tissue maltomas develop, peripheral extranodal non-Hodgkin lymphomas of B-origin more than two-thirds of which are found in the digestive system. The authors investigated in a retrospective study the effectiveness of different types of treatment of maltomas of the digestive tract with a low and high grade of malignity in 16 patients. It appears that splenectomy is not necessary in these patients and that the effectiveness of primary chemotherapy...

[Vascular intercellular adhesive molecule-1 (VCAM-1)--a new indicator of activity in systemic lupus erythematosus?].

V Scudla, P Horák, L Faltýnek, Z Pospísil, M Budíková, Z Hermanová

Vnitr Lek 1997, 43(5):307-311

The objective of the submitted study was to evaluate the role of the serum level of the soluble form of the vascular adhesive molecule-1(VCAM-1) in systemic lupus erythematosus (SLE) and to evaluate the possible relation to selected clinical and laboratory indicators of activity of the disease and cytokine serum levels. The analyzed group comprised 20 women, median age 37 years (range 18-65 years). Elevated VACM-1 serum levels were detected in 18 subjects (90%). Statistical analysis (Pearson's test, p < 0.05) revealed a significant relationship between serum levels of VCAM-1 and the index of clinical activity of SLE evaluated by the ECLAM system...

[IgD plasmacytoma--observations in 5 patients].

F Matěja, M Tichý, M Zvarová, J Bucek

Vnitr Lek 1997, 43(5):312-316

The authors describe five cases of plasmocytoma IgD--two men aged 37 and 49 years and three women aged 52, 62 and 80 years. In four instances light lambda chains were involved, in one instance kappa chains. In all patients there was a massive finding of Bence Jones protein in urine. The diagnosis was assessed after bone marrow puncture, immunochemical examination of serum and urine and X-ray of the skeleton. In zone electrophoresis of serum a marked gradient was found only in three patients. In two instances, where the disease was considered at first chronic as glomerulonephritis in the final stage of renal failure, renal biopsy helped with the diagnosis....

[Heparin cofactor II and thrombophilic conditions].

J Hudecek, E Haľáková, J Ivanková, P Kubisz

Vnitr Lek 1997, 43(5):317-319

Heparin co-factor II is in addition to antithrombin III another heparin dependent thrombin inhibitor. This glycoprotein blocks thrombin action not only in haemostasis but also in its other effects. Congenital deficiency of heparin co-factor II was found equally frequently among asymptomatic subjects and patients with thrombotic complications. Although its deficiency probably is not a serious risk factor of thromboembolic disease, it can promote its development in a significant way. The authors describe the case of a child with thrombotic complications and a congenital deficiency of heparin co-factor II.

[Therapeutic possibilities in non-Hodgkin's lymphoma].

P Klener

Vnitr Lek 1997, 43(5):320-325

The author presents a review of optimal therapeutic procedures in the treatment of non-Hodgkin lymphomas (NHL) with a low and high grade malignity. He emphasizes that the prerequisite of successful treatment is detailed assessment of the type of NHL, the extent of the disease and prognostic signs. Therefore a brief account of diagnostic procedures is presented, incl.molecular biological diagnosis.

[Nucleoli in leukemic cells (overview and personal results)].

K Smetana, H Subrtová, I Jirásková, H Klamová, L Rosa

Vnitr Lek 1997, 43(5):326-331

The investigation of nucleoli is a simple but useful complementary approach for the evaluation of individual leukemic cells in addition to other hematological laboratory procedures. Since main nucleolar types represent the morphological expression of nucleolar biosynthetic activities, i.e. RNA transcription, the incidence of functionally dominant nucleolar types in leukemic cells reflects the state of these cells which is related to that transcription. Similarly, the investigation of AgNORs in individual leukemic cells may provide a complementary useful information on their proliferation activity.

[New findings in the diagnosis of minimal residual disease in acute leukemia with emphasis on flow cytometry].

D Vosková, L Váleková, P Kubisz

Vnitr Lek 1997, 43(5):332-336

The authors present a review of contemporary findings of investigations of minimal residual disease in patients with acute leukaemia. They present brief information on different monitoring techniques and on their practical application. Attention is paid above all to multiparameter flow cytometry as this method is used in their laboratory work.

[Activation of blood coagulation in oncology patients].

M Penka

Vnitr Lek 1997, 43(5):337-339

Thromboembolic complications are a very important part of neoplastic diseases. In these complications specific processes participate which are the result of the action of substances produced by the tumour or they are formed as a consequences of the reaction to the neoplastic disease, its complications or treatment. A special role in this respect is played by the tissue factor and cancer procoagulant which are very important procoagulant proteins. Post-mortem evaluation reveals thromboembolic manifestations in as many as 50% of all oncological patients. Considerable attention is paid to the prevention of thromboembolic episodes or their progression...

[The role of the atrium and optimal values of AV intervals in sequentially paced patients].

J Kvasnicka, M Měsťan, M Tauchman, Z Tusl, V Rozsíval, J Jandík, I Gajdosová

Vnitr Lek 1997, 43(5):340-344

In 1995, 2249 dual chamber pacemakers were implanted in the Czech Republic. These pacemakers make it possible to set an optimal AV delay between the atrial and ventricular impulse. Although the optimization of the AV interval has its well defined physiologic advantages, it does not seem to be necessary in otherwise healthy individuals with a good atrial and ventricular function. In these patients the default value, usually about 170 ms, is acceptable. However, AV interval optimization--i.e. finding the interval at which the atrial contribution to ventricular filling is maximal--should be done in all patients with left ventricular dysfunction, indicated...

[High-dose therapy of multiple myeloma with autologous hematopoietic stem cell transplantation and subsequent maintenance therapy with interferon alfa versus interferon alfa and dexamethasone--a randomized clinical study "4W"].

I Mareschová

Vnitr Lek 1997, 43(5):345