Presence Of Atypical Forms Research Articles

Clinical, Immunological and Molecular-Genetic Description of Symptoms in Children with Autoimmune Polyglandular Syndrome Type 1: Description of a Case Series

Clinical polymorphism of autoimmune polyglandular syndrome type 1, the presence of atypical forms and a long latent period of the target organ disease determine the difficulties in diagnosing this disease, which leads to late detection of life-threatening conditions and inadequate treatment of such patients. The article presents the results from a retrospective analysis of 7 clinical cases of autoimmune polyglandular syndrome type 1 in children hospitalized between 2006 and 2016. The clinical manifestations of the syndrome included chronic candidiasis (7 patients), hypoparathyroidism (6), chronic adrenal insufficiency (3), and autoimmune hepatitis (3). The atypical clinical signs of the disease (annular erythema, hypoplasia of tooth enamel, sialadenitis) were revealed. All children had high ( 6 norms) antibody titers to interferon , 4 — antibodies to interferon 2. In all cases of chronic adrenal insufficiency and autoimmune hepatitis, the corresponding antibodies were determined in high ( 2 norms) titers. Clinical polymorphism, prolonged monosymptomatic course, prolonged latent period between the debuts of the syndrome manifestations require modern immunological methods of diagnostics and monitoring of the residual function of the target organ for the timely initiation of therapy and prevention of crisis states.

Labour Relations in Central and Eastern Europe

The aims of this report are threefold. The first is to map how much information can be obtained on the labour markets in Central and Eastern Europe (CEE) in order to proceed with analyzing the labour market policies and the growth models in the region. Secondly, while some comparative data can be obtained through international databases, these are often incomplete or not fully up to date. We therefore select a sample of countries (Bulgaria, the Czech Republic, Estonia, Poland, Slovakia and Slovenia) to represent the broad geographic diversity of the region and for these we obtained data that is more detailed and more up to date compared to what is available from existing international databases. Thirdly, we organize some of this information into brief country profiles and a common profile for the region. This provides a handy, up-to-date summary of the state of collective bargaining and other aspects of labour relations and their legal context in these countries and allows us to posit some hypotheses for further research. Why are we interested specifically in the countries in Central and Eastern Europe? These countries seem to be in a “low innovation, low wage equilibrium” (Independent Evaluation Group. 2007). In addition, we might see in a new light the application of some key normative concepts featuring prominently in EU policy debates such as flexicurity and transitional labour markets (TLM). The task here is therefore to open up the black box of labour markets in the new member states by researching labour relations in the region. Overall, CEE countries defy an easy stereotype or model with regard to the labour relations and their wider context. While collective labour relations are quite liberalized, individual labour relations are not, but there is also relatively low presence of atypical forms of labour relations as known from EU-15. However, it can be hypothesized that the role of self-employed and older workers might be to provide flexibility for the labour market. There also seems potential for bifurcation of unemployed, but also parents into “insiders” and “outsiders” – social insurance vs long-term social assistance/paternity benefits. This is worth further investigation.

Immunocytochemical study of the GH cells in the anterior pituitary gland of human fetus II. Anencephalic fetus.

In order to elucidate the effects of hypothalamic regulation on the morphology of GH cells, light and electron microscopic immunocytochemical examinations were carried out comparing GH cells in the anterior pituitary gland of anencephalic fetus with those of normal fetuses. Three types of GH cells were identified in the anterior pituitary gland of anencephalic fetus as well as in the normal fetus. Type-I is a small, round cell containing a few small secretory granules. Type-III is a large, polygonal cell with numerous large secretory granules. Type-II is a polygonal cell with medium-sized secretory granules. The Type-II GH cell was predominant in both anencephalic and normal fetuses. The most striking difference between anencephalic and normal fetuses was the presence of atypical forms of the Type II cell. These were polygonal cells containing secretory granules, which were either immunopositive or immunonegative to anti-human GH (anti-hGH) serum. Furthermore, two other types of GH cells were identified. The somatomammotroph (SM cell) contained GH and PRL in different granules within the same cell. Also, a different type of the GH cell was noted containing two varieties of secretory granules; one was immunolabeled only with anti-hGH and the other was not immunolabeled to either anti-hGH or anti-human PRL (anti-hPRL). From these results, we suggest that an absence of hypothalamic regulation in the anencehpalic does not seriously modify GH cell morphology but induces an altered GH storage pattern in some of the cells.

Prolonged isolated thrombocytopenia after hematopoietic stem cell transplantation: morphologic correlation.

Prolonged isolated thrombocytopenia, defined as recovery of other cell counts with continuous dependence on platelet transfusions for greater than 90 days after hematopoietic stem cell transplantation (HSCT), develops in approximately 5% of patients who undergo HSCT. Although the clinical conditions associated with prolonged isolated thrombocytopenia have been studied, a systematic review of bone marrow biopsies has not been performed and the pathophysiologic basis has not been defined. We reviewed all HSCT at one center from 1990 to 1995 (n = 454) and found 12 cases that met criteria for prolonged isolated thrombocytopenia (incidence = 12/454 or 3%). Bone marrow core biopsies from 12 patients with prolonged isolated thrombocytopenia were reviewed to determine cellularity, numbers of megakaryocytes, the presence of atypical forms, and clusters of megakaryocytes. These marrow megakaryocyte counts were compared to age and disease matched controls, and 11 normal donors. Patients (aged 1-56 years, mean 32 years) who underwent HSCT (four sibling HLA-identical, five autologous bone marrow, three autologous peripheral stem cell) with prolonged isolated thrombocytopenia had a statistically significant lower absolute megakaryocyte count in bone marrow biopsies performed before transplantation and more than 30 days after transplantation compared to control patients (aged 4 months to 50 years, mean 31 years) who underwent HSCT (four sibling HLA-identical, four autologous bone marrow, four autologous peripheral stem cell) for similar conditions. No apparent differences were seen in size of megakaryocytes, nuclear-cytoplasmic ratios, or clustering of megakaryocytes. Overall marrow cellularities were similar in the three groups. These findings suggest that decreased differentiation of megakaryocytes from stem cells, rather than ineffective platelet production or peripheral destruction of platelets, causes prolonged isolated thrombocytopenia in HSCT patients. Low megakaryocyte counts prior to HSCT may be a useful prognostic indicator, as this feature was associated with the development of prolonged isolated thrombocytopenia.