Pierwotna małopłytkowość immunologiczna u dzieci

2018 ◽  
Vol 22 (4) ◽  
Author(s):  
Anna Dusza ◽  
Michał Matysiak

In this article we present current investigation on primary immune thrombocytopenia in children. There are described pathomorphology, clinical symptoms, diagnosis and treatment. We also present current data from literature about genetic tests and latest data on treating options in children. Primary immune thrombocytopenia (ITP) is one of the most frequent hematological disorders in pediatric population. Although the majority of children have a self-limited and short duration of the disease. However, approximately 20-30% of those patients can develop chronic ITP, which can cause significant complications and higher mortality and reduced quality of life. Especially regarding to long-term immunosupression or surgical interventions, such like splenectomy and restrictions on daily activities to avoid trauma. Over the past decades a lot of informations has been reported about pathogenic features of ITP. Nowdays, we know that it is not only caused by increased platet destruction and decreased platet production, but also complex, multifactorial immune dysregulation, like loss of immune tolerance and generation of platelet autoantibodies. In this article we present current investigation on ITP including clinical symptoms, diagnosis, pathomorphology and latests options on treatment in children. We also present current data about genetic biomarker, such as Vanin-1 (VNN-1) which has been suggested as one of predictors of chronic disease and potentially can offer early prognosis estimation.

Author(s):  
Giuseppe Lassandro ◽  
Valentina Palladino ◽  
Giovanni Carlo Del Vecchioa ◽  
Viviana Valeria Palmieri ◽  
Paola Carmela Corallo ◽  
...  

Background and Objective: Immune thrombocytopenia (ITP) is a common bleeding disorder in childhood. The management of ITP in children is controversial, requiring personalized assessment of patients and therapeutic choices. Thrombopoietin receptor agonists (TPO-RAs), eltrombopag and romiplostim, have been shown to be safety and effective for the treatment of pediatric ITP. The aim of our research is defining the role of thrombopoietin receptor agonists in the management of pediatric ITP. Method: This review focuses on the use of TPO-RAs in pediatric ITP, in randomized trials and in clinical routine, highlighting their key role in management of the disease. Results: Eltrombopag and romiplostim appear effective treatment options for children with ITP. Several clinical studies have assessed that the use of TPO-RAs increases platelet count, decreases bleeding symptoms and improves health-related quality of life. Moreover, TPO-RAs are well tolerated with minor side effects. Conclusion: Although TPO-RAs long term efficacy and safety still require further investigations, their use is gradually expanding in clinical practice of children with ITP.


2013 ◽  
Vol 132 (1) ◽  
pp. 24-29 ◽  
Author(s):  
Cristina Santoro ◽  
Francesca Biondo ◽  
Erminia Baldacci ◽  
Maria Stefania De Propris ◽  
Anna Guarini ◽  
...  

2019 ◽  
Vol 1 (1) ◽  
pp. 66-71
Author(s):  
Bogdan Stanescu ◽  
Adriana Cuciureanu

The present article presents the expertise realized by the Department of Environmental Monitoring Pollution Evaluation within the INCD ECOIND, in the evaluation of the quality of urban soils in the municipality of Bucharest and the main big cities in Romania. The current data available at the level of the 27 member states of the European Union show that annually over 100,000 hectares of land are introduced into the urban environment, a direct consequence of the development of cities. There are a number of legislative obstacles to strategic soil protection measures. Moreover, at the level of the local authorities there is a conflict regarding the measures of soil protection in the long term, on the one hand, and, the accelerated economic development in the short term, on the other. European environmental experts consider that the urban development, absolutely necessary for the economic growth, requires an adequate management of the natural resources in order for the development to be done on a sustainable basis, respectively to follow a series of strategic objectives. In our country, at least in the last decade, we find on a large scale the conversion of industrial areas into commercial or residential areas. The footprint of industrial activities can be found even after long periods of time present by identifying the remnant of soil pollution or in those areas known as historically polluted (for example the town of Copsa Mica). The conclusions stemming from the assessment of pollution in urban areas over large areas, in correlation with the potential sources of pollution, underline the need to monitor the quality of soils in the urban environment, but also to apply a performance management in order to protect this natural resource in the long term.


2019 ◽  
Vol 2019 ◽  
pp. 1-9 ◽  
Author(s):  
Marco Daperno ◽  
Alessandro Armuzzi ◽  
Silvio Danese ◽  
Walter Fries ◽  
Giuseppina Liguori ◽  
...  

Background. The lifelong and remitting nature of ulcerative colitis results in considerable disability and a substantial negative impact on quality of life. The major goal of the therapy of ulcerative colitis is considered to be the modification of the course of the disease, so that the patient’s quality of life can be improved while minimising disease-related disability. Although considerable progress in understanding the molecular pathways involved in ulcerative colitis has led to improved treatment options, there is currently no definitive cure for ulcerative colitis, there remain considerable unmet needs in terms of long-term efficacy and safety, and there are many patients who continue to be burdened by physical and psychological symptoms. Defining unmet needs can help to increase the awareness of the shortcomings of current therapeutic management and highlight the need to achieve not only a control of clinical symptoms but also control of mucosal healing, in order to attain the best possible long-term outcomes. Methods. With the aim of providing a better understanding of the unmet needs of patients towards improving overall care, a Delphi process was used to obtain consensus among a group of Italian ulcerative colitis experts. The consensus group met with a major focus of delineating the unmet needs of current treatment strategies and overall management of ulcerative colitis, while also focusing on quality of life and patient care. Results. Three main areas were identified: (i) treatment, (ii) monitoring and risk management, and (iii) patient-related issues. A high level of consensus was reached on all but one of the statements identified. Conclusions. The findings arising from the Delphi process provide valuable insights into the unmet needs in the management of moderate-to-severe ulcerative colitis from the clinician’s perspective, while emphasising the benefits of therapeutic individualization and suggesting areas that need additional study with the aim of optimising the treatment of patients with ulcerative colitis.


2009 ◽  
Vol 1 ◽  
pp. CMT.S1094
Author(s):  
Kim Lawson

Fibromyalgia (FM) is a chronic complex pain disorder that is multidimensional and exhibits heterogeneity requiring a long-term multidisciplinary approach to management. Many of the drugs used in the treatment of FM have been focused to the management of single symptoms; often such drugs fail to demonstrate acceptable efficacy in the majority of the patient population. Pregabalin is an α2-δ ligand that regulates the release and postsynaptic actions of neurotransmitters related to analgesic, anticonvulsant and anxiolytic properties. In randomized, double-blind, placebo-controlled studies, pregabalin has demonstrated an improvement in pain, sleep and fatigue symptoms associated with FM, as well as offering an improvement in parameters related to quality of life. Although the positive outcomes obtained with pregabalin support its use as an option for the management of FM, the efficacy was restricted to a selected patient population outside of the usual care setting. Current data do not allow an explanation where there are any limitations of pregabalin as a treatment of patients with FM, as to whether this is a deficiency of the drug or the process of assessment (e.g. assessment tools of FM, clinical trial design).


2020 ◽  
Vol 2020 ◽  
pp. 1-8
Author(s):  
Céline De Cuyper ◽  
Tristan Pauwels ◽  
Eric Derom ◽  
Michel De Pauw ◽  
Daniël De Wolf ◽  
...  

Background. A patent foramen ovale (PFO) is a rare cause of hypoxemia and clinical symptoms of dyspnea. Due to a right-to-left shunt, desaturated blood enters the systemic circulation in a subset of patients resulting in dyspnea and a subsequent reduction in quality of life (QoL). Percutaneous closure of PFO is the treatment of choice. Objectives. This retrospective multicentre study evaluates short- and long-term results of percutaneous closure of PFO in patients with dyspnea and/or reduced oxygen saturation. Methods. Patients with respiratory symptoms were selected from databases containing all patients percutaneously closed between January 2000 and September 2018. Improvement in dyspnea, oxygenation, and QoL was investigated using pre- and postprocedural lung function parameters and two postprocedural questionnaires (SF-36 and PFSDQ-M). Results. The average follow-up period was 36 [12–43] months, ranging from 0 months to 14 years. Percutaneous closure was successful in 15 of the 16 patients. All patients reported subjective improvement in dyspnea immediately after device deployment, consistent with their improvement in oxygen saturation (from 90 ± 6% to 94 [92–97%] on room air and in upright position) (p<0.05). Both questionnaires also indicated an improvement of dyspnea and QoL after closure. The two early and two late deaths were unrelated to the procedure. Conclusion. PFO-related dyspnea and/or hypoxemia can be treated successfully with a percutaneous intervention with long-lasting benefits on oxygen saturation, dyspnea, and QoL.


2016 ◽  
Vol 88 (11) ◽  
pp. 168-171
Author(s):  
S I Kayukova

The paper outlines a concise review of Russian and foreign literature on the specific features of the course of respiratory tuberculosis in reproductive-aged women. It shows the impact of active tuberculosis and massive chemotherapy on the reproductive system, analyzes clinical symptoms, immediate and long-term consequences, and prognosis of future reproductive function. The timely diagnosis and optimal correction of reproductive disorders in women with respiratory tuberculosis can improve their quality of life, fertile capacities, and birth of a successive healthy offspring


2020 ◽  
Vol 64 (1) ◽  
pp. 28-33
Author(s):  
T. Morgoshiya

When comparing the results of surgical interventions in the modifications of Billroth-I and Billroth-II concerning carcinoma there are no significant differences both in immediate and in long-term results of treatment. At the same time the functional results of interventions and quality of life of patients are better after reconstruction of the digestive tract with the help of gastroduodenal anastomosis. Gastric stump cancer is more often occur after Billroth-II surgery. The main cause of the development of carcinoma in this case is the appearance of atrophic gastritis as a result of denervation of the organ and the transfer of bile to the operated stomach.


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