Idiopathic hypoparathyroidism with intracranial calcifications and dominant skin manifestations.

Presented here is an unusual case of a 41-year-old man with idiopathic hypoparathyroidism strongly connected with dermatological, ophthalmological and neurological disorders. Since the age of 4 he had been treated ineffectively for mycosis resulting in complete baldness and atrophic nail plate changes. At the age of 35 he was diagnosed with idiopathic hypoparathyroidism. He underwent surgery twice due to bilateral cataract. CT scans of the head demonstrated numerous symmetrically located calcifications in both frontal lobes, subcortical nuclei, the paraventricular region, brain fornix, and both cerebellar hemispheres. The neuropsychological examination demonstrated the occurrence of psychosensory disorders under the form of hallucinations accompanied by a sense of fear and anxiety. It seems essential to investigate the parathyroid gland for any sign of pathology, especially amongst patients with severe dermatological and/or neuropsychological symptoms. Early diagnosis and treatment of patients with hypoparathyroidism may prevent the development of many serious complications or at least result in marked improvement of neurological manifestations.

Erythromelalgia--a thrombotic complication in chronic myeloproliferative disorders.

Erythromelalgia is a very specific, thrombotic syndrome related with thrombocythemia that may occur during the course of chronic myeloproliferative disorders (MPD), especially polycythemia vera (PV) and essential thrombocythemia (ET). This poorly understood clinical syndrome is characterized by red, congested distal extremities and painful burning sensations, usually confined to the ball of the foot and one or more toes or fingers. If left untreated, it may progress towards acrocyanosis and even peripheral gangrene. Sometimes, it may precede the diagnosis of MPD by months or years. The pathophysiological aspects of erythromelalgia as well as its differentiation with erythermalgia have been reviewed in this study.

Myeloproliferative disorders--neurological complications.

Primary myeloproliferative disorders (MPD) are often associated with hemostasis abnormalities, which may cause many thrombotic or hemorrhagic complications during the course of the disease. Clinical consequences following abnormal hemostatic conditions include various neurological manifestations. It is extremely difficult to predict and evaluate the risk and chance, that MPD patients will develop neurological symptoms. The up-to-date background of pathological thrombocytosis, as well as the neurological aspects of abnormal hemostasis during the course of myeloproliferative disorders have been reviewed in this study.

The effects of anagrelide on human megakaryocytopoiesis.

Anagrelide, an inhibitor of platelet aggregation, decreases the number of platelets in normal subjects and in patients with myeloproliferative disorders. We describe studies aimed at discovering the general mechanism(s) by which anagrelide acts. We examined three hypotheses: (1) anagrelide shortens platelet survival, (2) anagrelide inhibits the proliferation of megakaryocytic-committed progenitor cells (CFU-M), and (3) anagrelide inhibits maturation of megakaryocytes. We observed that anagrelide did not shorten platelet survival. Proliferation of CFU-M in vivo was not affected by anagrelide, although high concentrations of anagrelide inhibited CFU-M in vitro. In-vivo and in-vitro anagrelide altered the maturation of megakaryocytes, causing a decrease in their size and changing other morphometric features. We conclude that anagrelide decreases the number of platelets primarily by interfering with the maturation of megakaryocytes.

[Autologous blood-derived stem cell transplantation in the treatment of hematopoietic malignant diseases (Part II)]. / Autologiczne krwiopochodne przeszczepy komórek macierzystych w leczeniu chorób rozrostowych ukladu krwiotwórczego (Czesc II).

Autologous transplantation of circulating stem cells is potentially capable of creating a chance of normal hematopoietic reconstitution in the patients in which both allogeneic and autologous bone marrow transplantation was impossible. The authors have reviewed the possibilities for collection of adequate and sufficient numbers of peripheral blood stem cells as well as up-to-date results of their autografting especially in relation to hematopoietic malignant diseases. The advantages resulting from autologous transplantation of circulating stem cells depends on: 1. The lack of risk of GvH--disease. 2. early and rapid hematopoietic and lymphoid recovery, 3. probably, the low risk of graft contamination by tumor cells in early remission.

[Autologous blood-derived stem cell transplantation in the treatment of proliferative disorders of the hematopoietic system (I)]. / Autologiczne krwiopochodne przeszczepy komórek macierzystych w leczeniu chorób rozrostowych ukladu krwiotwórczego (Czesc I).

Peripheral blood can be an alternative source of hematopoietic stem cells which after autografting are capable of sustaining or completely recovering of lymphopoiesis without the necessity of bone marrow harvesting. Theoretical assumptions conditioning the clinical application of circulating stem cells autotransplantation have been described. The results of experimental studies performed in animals and humans have allowed for closer characterization of these cells. However, the physiological significance of their presence in the peripheral blood still remains unknown.

Ultrastructural aspects of leukaemic cells in acute myeloblastic leukaemia.

Ultrastructural aspects of leukaemic cells in a case of myeloblastic leukaemia are described. Changes concerning of mitochondrial ultrastructure, but primarily the presence of vast numbers of fibrillar structures in the cytoplasm of the examined cells were observed. These structures were spreading through their gradual widening to cover almost the whole cell cytoplasm, damaging its organellae and internal architecture. The nature of the observed changes remains unknown.

Histochemical studies of some myocardial oxido-reductive enzymes after experimental beta-adrenergic blockade with propranolol.

Histochemical studies of some myocardial oxido--reductive enzymes after a beta--adrenergic blockade with propranolol have been carried out. Succinate, isocitrate and glucose-6-phosphate dehydrogenases did not indicate any changes in activity, whereas the changes in reaction intensities concerning NADH and NADPH tetrazole reductases and glutamate dehydrogenase have rather a transitory and reversible character. Only lactate dehydrogenase showed an increase in the enzymatic activity which speaks for an increase in the glycolysis process in the heart muscle. In the light of our own presented research results we assume that the experimental beta--adrenergic blockade of the heart muscle in rats does not evoke more important enzymatic changes which are noticeable in histochemical microscope examination.

Some histochemical observations on the myocardial metabolism in experimental conditions. Part I.

In the paper the author is concerned with the histochemical estimation of the metabolic adaptation of the heart muscle of albino rats during an early experimental alloxan diabetes. It has been found that the state of experimentally produced insulin deficiency directly influences metabolism of the heart muscle and the changes observed in the histochemical reactions prove this. An increase in the intensity of histochemical reactions concerns the PAS-positive reaction and the reactions to the NADH and NADPH tetrazole reductase activities. Alkaline phosphatase shows a decrease in the enzymatic activity, whose nature is transitional and reversible with regard to cytochrome oxidase and ATP-ase. The histochemical picture of metabolic changes depends on the duration time of experimental diabetes.

Some histochemical observations on the myocardial metabolism in experimental conditions. Part II.

A histochemical evaluation of the activity of chosen oxido-reductive enzymes of the cardiac muscle connected with the citric acid cycle, glycolysis and pentose shunt in the early experimental alloxan diabetes period in white rats has been carried out. Dehydrogenases: succinate, isocitrate and glucose-6-phosphate indicated a decrease in the enzymatic activity while the lactate dehydrogenase activity did not undergo any change. An increase in the intensity of the histoenzymatic reaction only concerned glutamate dehydrogenase. The presented histoenzymatic evidence of changes in the enzymatic activities may speak for the possibility of an existence of a direct diabetes influence on the cardiac muscle metabolism.

Electronmicroscopic cytochemical studies on lysosomes and peroxisomes of a rat cardiac muscle in the experimental alloxan diabetes.

The combined electronmicroscopic and cytochemical investigations of lysosomes and peroxisomes of a rat cardiac muscle were carried out in various duration time of the alloxan diabetes. The analysis of cytochemical reactions with DAB, confirming the existence of peroxisomes in rat heart muscle revealed that the state of experimental alloxan diabetes did not at all affect either cytochemical or morphological image of peroxisomes. In comparison with the control group, in all 3 experimental groups cytochemical reaction to acid phosphatase markedly pointed to the increase in number and size of electron-dense bodies (lysosomes) with positive reaction. This state may most probably result both from local and general organism disturbances in metabolism, induced by particular experimental conditions.

Histomorphological and ultrastructural studies of a rat myocardium in experimental conditions.

A histomorphological and ultrastructural analysis of the adaptation reaction of the rat heart muscle during an early experimental alloxan diabetes has been carried out. Changes were observed in the orthomorphology of the ultrastructure which appeared as vesicular intercalated discs within the fasciae adherentes regions, as changes in the mitochondria, cell nucleus, myofibrills and in the sarcoplasmic reticulum with a simultaneous increase in the number of lipid bodies. The described alterations are dependent on the duration time of the experiment and on the disturbances in the entire and local system cellular metabolism.

The activity of some dehydrogenases and NADH and NADPH tetrazole reductases in the seminiferous epithelium of reserpinized white rats.

Investigations on the effect of reserpine on the histochemical reactions to some dehydrogenases (succinic, lactic, glucose-6-phosphate dehydrogenase) as well as to NADH and NADPH tetrazole reductases in the seminiferous epithelium of white rats have been performed. A weakening of the dehydrogenase activities has been mainly observed in the young cells of seminal epithelium i, e. in spermatogone and spermatocytes. The changes in histoenzymatical reactions caused by reserpine may be the evidence of the inhibition of oxidation-reduction processes in the studied reproductive cells.

The effect of hypotensive drugs (Serpasil) on the histochemical reactions in the small intestine of white rats.

The influence of Serpasil (Ciba) on histological activities of dehydrogenases and reductases as well as on PAS-positive substances in the small intestine of white rats has been studied. It has been demonstrated that a decrease in the succinic, lactic and glucose-6-phosphate dehydrogenase activities as well as in the NADH and NADPH tetrazole reductase activities occurs in the rat's small intestine epithelial cells after the administration of Serpasil. However, a considerable intensification in the reactions to PAS-positive substances in the mucous cells and an increase in the amount of these cells has been observed.