Hungarian Immunology

[Antiphospholipid syndrome - focused on the childhood form]

KÁLOVICS Tamás, PONYI Andrea, BENSE Tamás, MÜLLER Judit, DANKÓ Katalin, FEKETE György, CONSTANTIN Tamás

JANUARY 30, 2006

Hungarian Immunology - 2006;5(01)

[Antiphospholipid syndrome is an autoimmune disorder characterized by recurrent thromboembolic events with concurrent presence of antiphospholipid antibodies in the sera. The morbidity and mortality of the syndrome is defined by the clinical manifestations: deep vein thrombosis, cerebrovascular events, myocardial infarct, pulmonary embolism, recurrent pregnancy losses and prematurity. The authors reviewed the pathogenesis, the clinical course and the treatment of the antiphospholipid syndrome focused on the childhood form.]

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[Regional cues and phenotypic responses during the ontogeny and postnatal development of splenic vasculatur]

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[Among structured peripheral lymphoid tissues in man and rodents, the spleen demonstrates the most extensive flexibility in functional activities, which is coupled with considerable tissue architecture adjustments during ontogeny and immune reactions. The sequential conversion of a primary lymphohemopoietic tissue into a major peripheral lymphoid organ (while participating in the post-myeloid period of primary B-lymphopoiesis and retaining the potential for myelopoiesis) is accompanied with the ordered segregation involving various non-hemopoietic components of architecture, including the endothelia of blood vessels. In this report we will survey the functional and structural aspects of heterogeneous endothelial cells lining the various splenic vascular beds, comprising the complex circulatory network of the spleen. These features will be correlated with the characteristics of those regulatory mechanisms that have recently been demonstrated to be responsible for the establishment and maintenance of the endothelial divergence during splenic vascular development, including crucial transcription factors, morphogenic regulatory ligands and receptors of the tumor necrosis factor/lymphotoxin (TNF/LT) family and others. The influence of these regulatory elements in mice appears to be highly restricted in terms of regional involvement of the vasculature, with cellular alterations of the marginal sinus representing the most frequently affected region. This complexity highlights the importance of this tissue region in both the formation of splenic vasculature and a possible source for white pulp stromal elements as well as its function as a major gateway for lymphocyte traffic.]

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[Numerous hypotheses have emerged to solve the problem and the pathomechanism of autoimmunity so far. Different factors are suspected, from viruses to neuroendocrine elements, to be a pathogen in the etiology of autoimmune diseases. Most of the theories are based on the assumption that something happens to the immune system and it leads to an autoimmune reaction against the proper self. This paper indicates that, at least in certain cases, the immune system reacts properly against the altered cells; therefore the cause of the autoimmunity lies in the other improper functioning of the body. Experimental data show that La autoantigen plays a role not only as a diagnostic marker but it may participate in the pathomechanism of certain autoimmune diseases as well. Mutations in the exon 7 of La gene have such consequences that could lead to the formation of autoimmune reactions detected.]

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[Detection of rare phospholipid/co-factor antibodies in lupus patients]

TARR Tünde, KISS Emese, BÓTYIK Balázs, TUMPEK Judit, SOLTÉSZ Pál, ZEHER Margit, SZEGEDI Gyula, LAKOS Gabriella

[OBJECTIVE - Was to detect the rare phospholipid/ co-factor autoantibodies in lupus patients. PATIENTS AND METHODS - In the present study, besides anti-cardiolipin and anti-β2-glycoprotein I, antibodies directed against phosphatidil-serine, protrombin and annexinV were measured by commercial ELISA kits in 85 randomly selected lupus patients, 14 of whom met the criteria of antiphospholipid syndrome. Corralations were determined between the presence and concentration of rare antiphospholipids and those included in the diagnostic criteria of antiphospholid syndrome, as well as with clinical thrombotic manifestations. RESULTS - Anti-cardiolipin IgG was positive in 14 patients, aCL IgM in eight, anti-β2GPI IgG in four and IgM in five patients. Lupus anticoagulant was detected in nine cases. Seven patients were positive for anti-phosphatidilserine IgG, nine for aPS IgM, anti-protrombin IgG was positive in nine cases. Antiprotrombin IgM and anti-annexinV were negative in all patients. Correlation was found between antiphosphatidilserine and anti-cardiolipin antibodies. The frequency and the concentration of rare antiphospholipid/ co-factor antibodies were higher in patients with secondray antiphospholipid syndrome. The presence of such rare antiphospholipid antibodies cumulated in patients with antiphospholipid syndrome. Their presence increased the frequency of thrombotic events in the entire study population, furthermore in those positive for lupus anticoagulant or anti-cardiolipin. CONCLUSIONS - The rare anti-phospholipid/cofactor antibodies were found in 12% of an unselected cohort of lupus patients. Their presence was more frequent in patients with secondary antiphospholipid syndrome, and further increased the risk of thrombotic complications.]

Lege Artis Medicinae

[ANTICOAGULATION IN OBSTETRICS]

DOMJÁN Gyula, GADÓ Klára

[The risk of thrombosis is increased about 5 to10 times during pregnancy and in the puerperium. Beside the classic risk factors, this is also due to special obstetrical causes. Delivery, especially Cesarean section further increases susceptibility to thrombosis. Prophylactic or therapeutic anticoagulant treatment can significantly reduce maternal and fetal morbidity and mortality. Just like in the non-pregnant state, subcutaneous low molecular weight heparin or intravenous or subcutaneous unfractionated heparin is recommended in pregnancy if anticoagulation is indicated. Warfarin is contraindicated in the first trimester because of its teratogenicity and also in the third trimester because of its long-lasting effect. Heparin does not cross the placenta, but its long-term administration may cause several side effects. Dosage, starting time and duration of the treatment depend on the measure of the risk of thrombosis. In certain cases (such as antiphospholipid syndrome) anticoagulant therapy is supplemented by low-dose acetylsalicylic acid. The date of delivery can be electively planned to minimalize bleeding and thrombotic complications. Vaginal delivery is preferred because of its lower risk of bleeding compared to Cesarean section. Intensity of anticoagulant therapy in the peripartum period should depend on the risk of thrombosis. The third phase of delivery should be actively driven by giving oxytocin to avoid bleeding complications. Since anticoagulant therapy is often continued during breast-feeding, it is important to know that neither warfarin, nor heparin is secreted in milk. When planning the treatment, each case requires individual consideration based on the type and number of risk factors, gestation time and, importantly, compliance of the patient.]

Clinical Neuroscience

Neuroscience highlights: Main cell types underlying memory and spatial navigation

KRABOTH Zoltán, KÁLMÁN Bernadette

Interest in the hippocampal formation and its role in navigation and memory arose in the second part of the 20th century, at least in part due to the curious case of Henry G. Molaison, who underwent brain surgery for intractable epilepsy. The temporal association observed between the removal of his entorhinal cortex along with a significant part of hippocampus and the developing severe memory deficit inspired scientists to focus on these regions. The subsequent discovery of the so-called place cells in the hippocampus launched the description of many other functional cell types and neuronal networks throughout the Papez-circuit that has a key role in memory processes and spatial information coding (speed, head direction, border, grid, object-vector etc). Each of these cell types has its own unique characteristics, and together they form the so-called “Brain GPS”. The aim of this short survey is to highlight for practicing neurologists the types of cells and neuronal networks that represent the anatomical substrates and physiological correlates of pathological entities affecting the limbic system, especially in the temporal lobe. For that purpose, we survey early discoveries along with the most relevant neuroscience observations from the recent literature. By this brief survey, we highlight main cell types in the hippocampal formation, and describe their roles in spatial navigation and memory processes. In recent decades, an array of new and functionally unique neuron types has been recognized in the hippocampal formation, but likely more remain to be discovered. For a better understanding of the heterogeneous presentations of neurological disorders affecting this anatomical region, insights into the constantly evolving neuroscience behind may be helpful. The public health consequences of diseases that affect memory and spatial navigation are high, and grow as the population ages, prompting scientist to focus on further exploring this brain region.