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Biomedical subjects

J Dusek

Publications and source records attributed to J Dusek.

At least 19 recordsLinked to original sources

Dextrocardia and Marfan's syndrome.

It is described the occurrence of dextrocardia together with the congenital cyanotic heart disease in 20 year old man included in the fruste forme of the Marfan's syndrome. The diagnosis was made by the physical examination with the evidence of the arachnodactyly by the metacarpal indices and confirmed by autopsy with the following results: dextrocardia, large atrial septal defect, common ventricle, atresia of the pulmonary artery with the collateral lung perfusion from the descending aorta. There were found neither ocular manifestations, nor unambiguous manifestations of the aortic lesions. The ultrastructural examinations showed only greater accumulation of the PAS positive substances. Dextrocardia as the cardiovascular manifestation of the Marfan's syndrome has not yet been reported in the available literature.

Adult

[Present possibilities of prenatal diagnosis of Fabry's disease].

Fabry's disease (angiokeratoma corporis diffusum universale) is an inborn error of metabolism, which is based on a shortage in the cumulation of glycosphingolipids in endothelial and epithelial cells of glomeruli, vascular endothelia and in ganglion cells with subsequent severe organ damage. Heredity is X-linked recessive. In the submitted paper the authors present a case-history of a family where three men were affected and carriership was confirmed in five women. During the first pregnancy one of them a successful prenatal diagnosis was made from cultivated amniotic fluid cells. Chromosomal examination revealed female sex and the levels of the alpha-galactosidase A were established in cultivated cells. A healthy girl was delivered.

Adult

[Changes in portal circulation in experimental prehepatic portal hypertension].

In a long-term experiment on dogs the lumen of the portal vein was reduced to one fifth by insertion of a metal spiral. The thus created disorder of the portal circulation was succeeded by the development of abundant hepatofugal collaterals and the development of mild portal overpressure without clinical manifestations. The altered portal circulation was followed up by dynamic hepatobiliary scintigraphy for a period up to 12 months. It was revealed that the curve of isotope movement through the liver was altered in a way suggesting slower perfusion of the liver by portal blood. This observation resembles those in patients with prehapatic portal hypertension and their confirmation. The investigation is documented by morphological, X-ray and isotope methods.

Animals

Demonstration of the nucleolar organizer region by silver staining (AgNOR method) in research and in histopathological practice.

The silver-staining method for the detection of nucleolar organizer regions (AgNOR) was tested on a large material and the AgNORs evaluated in normal and pathological tissues, especially in various benign and malignant neoplasms. Pathological lesions were represented by dysplasias and in situ carcinoma of the uterine cervix, dysplasias and various histological forms of breast carcinoma, carcinomas of the lung, of urinary bladder, of prostate, various neoplasms of the gastrointestinal tract, trophoblastic disease, naevocellular naevi and melanomas of the skin, myeloproliferative diseases and haematological malignancies, and also by several cases of xenotransplanted human neoplasms in athymic nude mice. The AgNORs were evaluated using several procedures. Marked AgNOR heterogeneity in individual malignant tumours was found to be a common feature suggesting the presence of cellular subpopulations or clones with different proliferative activity and metabolic properties. Close correlation between the proliferative behaviour and the quantitative and qualitative change of AgNORs was found in most obviously malignant neoplasms. The method, however, could not recognize neoplastic cells as such and is therefore not a specific oncological marker. This limits the diagnostic contribution of the silver staining method in diagnostic pathology, where it does not exceed the value of routinely stained slides of good quality. In spite of these limitations, the AgNOR staining method seems to represent a valuable contribution in the study of some aspects of the proliferative lesions, such as their proliferative and metabolic activity, hormonal dependence etc.

Animals

[The status of pathology in Czechoslovakia in 1988].

Personnel of 112 departments of pathology and 28 autopsy stations comprises 380.74 physicians, 30.63 academic non-physicians 761.43 medical technologists, 22.04 others with secondary school qualification, 267.45 autopsy aids and 205.18 others. In 1988 they performed 40,678 autopsies (53.47 per cent rate of hospital deaths), produced 548,854 necroptic slides, 1,902,707 bioptic slides, 559,709 cytologic slides, 46,955 semi-thin sections, 75,888 histochemical and immunochemical slides (total without necroptic slides represented 82.49 per cent). One physician could be calculated as related to 106.83 autopsies and 8,226.01 slides, one medical technologist to 4,118.41 slides. The proportion of physicians to other personnel was 1 to 3.38. Data from both national republics were quoted.

Czechoslovakia

[The future of pathology in Czechoslovakia].

Further development of Czechoslovak pathology depends on the worldwide speed and direction of progress in pathology, on economical and political progress, and on transformation of the Czechoslovak health care system. Changing orientation of pathology in advanced countries predicts an increased importance of working qualities in the whole scope of pathologist. Intrinsic factors controlling progress in pathology are varied and can be partly influenced. Participation of pathology in the new system is to be actively prepared by mutual information and cooperation.

Czechoslovakia

[Several physiologic aspects of the reactions of foals in the first three days after weaning].

Changes have been observed in nine foals, six months of age, after weaning for the time of three days, namely in the intake of drinking water, hematocrit, sedimentation of erythrocytes and three minerals Na+, K+ and Cl-. Foals respond to weaning immediately, lowering significantly their consumption of drinking water and increasing high significance of Na+ and Cl-. Changes of level K+, hematocrit and sedimentation don't show any significant variations. The results reached are to be considered as an introductory information for further inquiry on the more extensive methodical basis.

Animals

Bone marrow necrosis intravitally recognized in four cases of blastic leukaemia.

Bone marrow necrosis (BMN) is a rare intravitally recognized finding in acute leukaemia with an uncertain clinical significance. The clinical events in 4 patients with AML, ALL, AMoL and blastic transformation of CGL in whom bone marrow cytology and histology revealed BMN are reviewed. One patient with BMN at clinical presentation of AML entered complete, long lasting remission with marrow restoration after the standard DAT therapy. In the three remaining patients survival after BMN diagnosis was 6, 11, and 14 weeks. Clinical, haematological, histological and marrow scanning findings and their significance for early diagnosis and means to asses the extent and evaluation of BMN will be discussed. In contrast to the most earlier reports, BMN does not appear to confer a poor prognosis in all patients with blastic leukaemia.

Acute Disease

The experimental contribution to the study of the portal circulation by means of dynamic hepatobiliary scintigraphy.

Report is given on a group of dogs the portal vein of which was narrowed to 1/5 by putting on a metal spiral. A disorder of the circulation of the portal blood induced in this way was followed by opening of the effective hepatofugal collaterals and forming of slight portal hypertension without clinical manifestation. The changed portal circulation was studied by means of dynamic hepatobiliary scintigraphy within 12 months. It was found out that the curve of progression of the isotope through the liver had been changed in a way which corresponded to slowing of perfusion of the liver by the portal blood. It is analogous to observation of patients with PPH and its verification. The study is documented by morphological, X-ray and isotopic methods.

Animals

[Myelodysplastic syndromes].

Myelodysplastic syndromes (MDS) are a group of heterogenous disorders of blood and of the haematopoeitic bone marrow which have been recently in the center of interest of both clinical haematologists and pathologists. The FAB classification is based on cytology; for histological assessment, a special histopathological classification appears to be more appropriate. The main histological findings in MDS and some ultrastructural changes are shown in 138 bone marrow biopsies from 81 patients with MDS. Histological examination brings some findings that cannot be obtained by cytology. Optimal samples well processed can give valuable information without need of special staining methods. The latter can complement the basic information with some additional data or compensate for some technical defects of the slides.

Bone Marrow

[Diagnosis of lung injury due to amiodarone--contribution of bronchoalveolar lavage].

Based on the authors' own observation of a patient with pulmonary affection during amiodarone treatment, they demonstrate contemporary possibilities of early diagnosis of this potentially serious condition. They emphasize the importance of bronchoalveolar lavage, a non-invasive diagnostic method which provides information on the conditions in the peripheral portions of the lungs. The authors give an account of data in the literature on the pathogenesis of side effects of amiodarone in the lungs and draw attention to the need of systematic pneumological check-up examinations during treatment. If the drug is discontinued in time, all side-effects may recede spontaneously.

Amiodarone

The histologic and electronmicroscopic study of melanoblastoma after argon-laser therapy.

The left eye was enucleated for secondary detachment in a 50-year-old patient, with choroid melanoblastoma treated with argon-laser photocoagulation six months before. Histological examination of the enucleated eyeball showed pigmented melanoblastoma with some focal fibrosis in the area of the previous laser treatment. There were no signs of an actual tumour cell damage on electron microscopy. The fibrosis found at the side of laser treatment was very likely to result from the local therapeutical tumour destruction which, however, was not sufficient to influence substantially its further growth.

Argon

[Vascular changes in essential hypertension after treatment with enalapril].

A group of patients with essential hypertension and healthy subjects from the same work place were investigated for a period of four weeks and were subjected, in addition to a basic clinical medical examination, to an ergometric examination ECHO cardiography, occlusion plethysmography, and concurrently serum levels of adrenaline (A), noradrenaline (NA), renin, cyclic adenosine monophosphate (cAMP) beta receptors before and after four weeks treatment with enalapril were assessed. The results are consistent with available data from the literature where administration of inhibitors of the angiotensin converting enzyme (ACE) caused a reduction of the peripheral vascular resistance and increased blood flow assessed by means of occlusion plethysmography. Similar results were recorded after captopril administration for a one year period, as reported by Agabiti-Rose (1). Statistically significant differences were detected between hypertonics given placebo and those given enalapril for four weeks, as regards blood flow at rest and peripheral vascular resistance at rest. There was also a significant difference when hypertonic and normotonic subjects were compared. The results are presented in tables and figures.

Adult

[Relation between echocardiographic indicators and beta 2-adrenergic receptors in essential hypertension during treatment with enalapril].

A group of 22 men with essential hypertension stage I and II according to WHO, mean age 29.1 +/- 1.4 years, was divided at random into two sub-groups. Eleven patients were treated for four weeks with enalapril (E), 10 mg/day, 11 patients were given placebo (P), also for a period of four weeks. The patients were subjected to echocardiographic examination before and after four-week therapy with enalapril or placebo. In patients treated with E a significant reduction of the left ventricular wall and the interventricular septum was observed. There was also a highly significant decline of the weight of the left ventricle and the index of left ventricular mass. Enalapril led to a decline of the specific binding capacity and affinity of beta 2-adrenergic lymphocyte receptors. In the group given P no differences were found during the examination repeated after four weeks. By means of multidimensional linear regression estimates a close relationship was revealed between the difference in the weight of the left ventricle, the difference of the index of left ventricular mass and the difference of specific binding capacity and affinity of beta 2-adrenergic lymphocyte receptors.

Adult