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Use of arteriovenous fistula in children with bleeding disorders

Abstract

Haemophilia and associated bleeding disorders are chronic conditions that require easy, accessible, and reliable venous access for treatment. Peripheral venous access is traditionally the first option considered for prophylaxis. The initial introduction of prophylaxis to a child is during the first two years of life, and peripheral access can be problematic. Central venous catheters (CVC), for example port-a-caths, are widely used among this group; however, these devices require surgical insertion and are not without their own complications. Data were collected on venous access methods used at the paediatric centre at the Evelina London Children’s Hospital, where 242 children are registered at the comprehensive care centre, 48 of whom have a severe bleeding disorder. Of these 48, 27 have a CVC currently (PICC n=1, Port-a-Cath n=25, Hickman line n=1) and 3 have an arteriovenous fistula (AVF). Patient 1 is a 12 year-old boy with severe haemophilia A and an intracranial haemorrhage at the age of 9 months. He remains on prophylaxis and had an AVF created 5 years ago following repeated port-a-cath infections and poor venous access. Patient 2 is a 7 year-old boy with severe haemophilia B and an inhibitor, who has also had repeated port-a-cath infections. An AVF was constructed 2 years ago. Patient 3 is a 12 year-old girl with type III von Willebrand disease and an inhibitor. Due to need for regular factor treatment in the context of poor venous access, an AVF was formed. We have had a 100% success rate with all three AVFs at a follow-up period of 8-69 months. Our experience suggests AVF is a viable option of venous access in patients with haemophilia and other bleeding disorders, especially so for children with repeated CVC infections or poor peripheral venous access. However, this is not a straightforward option and further evidence on long-term use based on multicentre research will be beneficial in managing AVF in this group of patients.

Open access
The use of tranexamic acid in reducing bleeding complications

Abstract

Tranexamic acid inhibits fibrinolysis by competitively blocking the lysine binding sites of plasminogen, inhibiting binding between fibrin and plasminogen, and activation of plasminogen. It also competitively inhibits tissue plasminogen activator and inhibits plasmininduced platelet activation. The synthesis of tranexamic acid was first reported in 1962. It is used to treat or prevent excessive blood loss from trauma, surgery, and in various medical conditions including haemophilia and heavy menstrual bleeding. As a medicine affecting coagulation, it is listed in the World Health Organization’s List of Essential Medicines. Tranexamic acid remains a versatile and inexpensive agent with potential benefit in health services from the lowest to highest income countries. This pharmacy review focuses on the evidence base to support the use of tranexamic acid in acquired and inherited bleeding disorders to reduce bleeding complications.

Open access
Youth perspectives on genetic inheritance, carrier status and disclosure

Abstract

Knowledge about genetic inheritance as a concept in children and young people with bleeding disorders is synonymous, in many ways, with other inherited genetic conditions. Children and young people have a more physiological understanding of inheritance, but may hold mistaken and inaccurate beliefs in understanding basic genetics. There are complex ethical and social problems in the genetic testing of youngsters with bleeding disorders to establish carrier status. Current guideline recommendations indicate circumstances where clear psychosocial and medical benefits can be demonstrated. However, children and young people have a reduced capacity to understand the tests and their implications, and in many cases family communication may impact the extent of disclosure of genetic risk factors. This paper explores the genetics of inherited bleeding disorders, including basic knowledge of the concept of inheritance and reproductive risks. Carrier status in children and young people will be considered, drawing on legal rulings that may shed light on best practice in establishing carrier status based on genetic testing. Communication patterns within families around inherited bleeding disorders and the complicated process of disclosure will also be discussed.

Open access
Changes in the levels of kynurenic acid and selected proinflammatory cytokines after pharmacological treatment and electroconvulsive therapy (ECT) in patients with depressive disorder

Abstract

The aim of the present study was to compare the concentrations of KYNA, 3-OH-KYN and the cytokines TNF-α and IL-6 in patients with depression vs. healthy controls as well as in patients with depression treated pharmacologically vs. those treated using ECT. We also evaluated the relationship between the concentrations of KYNA, 3-OH-KYN and the cytokines TNF-α and IL-6 and clinical improvement measured on the MADRS scale in patients treated pharmacologically and those treated with ECT.

Subjects and methods: The study group comprised 29 patients aged 28 to 60 years with a diagnosis of a major depressive episode. Eleven of the patients received pharmacological treatment and 18 were treated with ECT.

Patients were assayed for serum levels of KYNA and the cytokines IL-6 and TNF-α. Clinical improvement was measured on the MADRS depression rating scale and the clinical global impression (CGI) scale.

Results: Significant differences were found in KYNA levels between depressive patients and healthy controls. Pharmacological treatment significantly contributed to the increase in KYNA levels and ECT – to the increase in TNF-α levels in depressive patients.

Conclusions:

  1. Depressive patients have significantly lower concentrations of KYNA than healthy individuals
  2. Depressive patients who have undergone pharmacological treatment have significantly higher KYNA concentrations than before treatment.
  3. Depressive patients who have undergone ECT treatment have significantly lower TNF-α concentrations than before treatment.
  4. High pre-treatment levels of IL-6 are associated with a lower MADRS improvement index in pharmacologically treated patients with depression.

Open access
Is it time for baclofen to be included in the official recommendations concerning the treatment of alcoholism?

Abstract

Alcohol dependence and its treatment is not an exactly resolved problem. Based on the EZOP [Epidemiology of Mental Disorders and Accessibility of Mental Health Care] survey, which included a regular analysis of the incidence of mental disorders in the population of adult Polish citizens, we were able to estimate that the problem of alcohol abuse in any period of life affects even 10.9% of the population aged 18-64 years, and those addicted represent 2.2% of the country’s population. The typical symptoms of alcohol dependence according to ICD-10, include alcohol craving, impaired ability to control alcohol consumption, withdrawal symptoms which appear when a heavy drinker stops drinking, alternating alcohol tolerance, growing neglect of other areas of life, and persistent alcohol intake despite clear evidence of its destructive effect on life. At the moment, the primary method of alcoholism treatment is psychotherapy. It aims to change the patient’s habits, behaviours, relationships, or the way of thinking. It seems that psychotherapy is irreplaceable in the treatment of alcoholism, but for many years now attempts have been made to increase the effectiveness of alcoholism treatment with pharmacological agents. In this article we will try to provide a description of medications which help patients sustain abstinence in alcoholism therapy with particular emphasis on baclofen.

Open access
Oral health in female patients with eating disorders

Abstract

The study aimed to evaluate oral health in women with eating disorders. The clinical study covered 30 patients aged 14-36 years suffering from diagnosed eating disorders and treated in closed psychiatric institutions. The control group comprised 30 healthy women at the mean age corresponding to that of the patient group. No relationships were confirmed between eating disorders and the intensity of dental caries. Eating disorders contribute to increased loss of dental hard tissues. In women suffering from eating disorders non-specific lesions in oral cavity are more common than in healthy women.

Open access
The psychometric properties of the Disease Perception Questionnaire

Abstract

This article describes the psychometric properties of the Disease Perception Questionnaire (Kwestionariusz Obrazu Choroby, KOCh, in Polish). An original version of KOCh consisting of 99 items was used to survey a group of 161 subjects aged 25 to 85 years. On the basis of factor analysis, four factors (scales) were identified which pointed to the subjects’ different perceptions of their disease: (1) Disease as a Threat, (2) Disease as a Weakness, (3) Disease as a Task, and (4) Disease as a Benefit. Items with the lowest factor loadings and the lowest factor specificity indexes were rejected. The factors identified explained 38% of the total variance. The final version of the KOCh questionnaire consisted of 57 items. The coefficients of reliability for the individual factors were Cronbach’s alpha = 0.94 for Disease as a Threat, Cronbach’s alpha = 0.86 for Disease as a Weakness, Cronbach’s alpha = 0.84 for Disease as a Task, and Cronbach’s alpha = 0.85 for Disease as a Benefit

Open access
A report from XIX Schizophrenia Forum Conference in Warsaw

Abstract

On 10-11th March in Warsaw, in the Conference Center Muranow, there was held a scientific conference “Schizophrenia-Forum”, organized for the nineteenth time. In this edition, particular attention was paid to educational aspects, by virtue of the fact that among the participants of the Conference there was a large number of young physicians specializing in psychiatry.

The formula of the two-day Conference based on the experts’ debates in a particular way affected the participants, so the conference was very interesting, it enabled the exchange of experiences and reflections on the problems of schizophrenia. The conference was attended by leading scientists and clinicians, for example: Marek Jarema, Janusz Rybakowski, Andrzej Czernikiewicz, Jerzy Samochowiec, Dominika Dudek and others, which is why it allowed to enrich the knowledge of the diagnosis and treatment of psychotic disorders and good presentation of mature clinical thinking. The organizer of the Conference was the Foundation III Department of Psychiatry, “Syntonia” and the guest of honor was Dr. Peter Falkai - Head of the Department of Psychiatry and Psychotherapy Ludwig Maximilian University in Munich. The lecture by Professor Falkaia entitled Schizophrenia treatment guidelines, at a plenary lecture, was presented in English, translated simultaneously, and was divided into parts: the characteristics of schizophrenia, guidelines for pharmacotherapy of schizophrenia, pharmacotherapy of schizophrenia - states of acute pharmacotherapy of schizophrenia - long-term treatment, side effects of long-term pharmacotherapy and conclusions. After a two-day Conference the hugely popular workshop sessions took place. They were addressed to physicians during specialization. All workshop sessions were very popular, as well as the entire conference, which became a forum for the exchange of information, ideas and experiences, sometimes intriguing, inspiring and innovative but never blank. At the end of the Conference the certificates were distributed and there was the opportunity for guests - visiting the permanent exhibition “1000 years of history of Polish Jews,” Polin, located at the Conference Centre Muranow. XIX Conference on Schizophrenia Forum 2016 was an interesting event cognitively and it maintained a high level of scientific and substantive jurisdiction important events in Polish psychiatry.

Open access
Social competence and the need for approval and life satisfaction of women with excess body weight and women of normal weight- preliminary report

Abstract

The purpose of the present study is to examine the life satisfaction levels in two groups of women - with excess body weight and normal weight and the relationship between life satisfaction and the level of social competences The study involved 70 women (35 with excess body weight and 35 with normal weight). The Life Satisfaction Questionnaire, Social Competence Questionnaire and Social Approval Questionnaire were used in the study. The study revealed no significant differences in terms of overall life satisfaction among women with excess weight and normal weight and a number of other relationships between the variables (like the importance of the need for social approval for the assessment of life satisfaction in those who have excess body weight).

Open access
Value preferences in individuals with low and high self-esteem

Abstract

The basic purpose of this article is to compare how people with low and high self-esteem rated particular values. Additionally, the authors look at gender differences concerning the attitudes toward certain values.

The study involved 268 individuals aged 19-24 (M= 21.71, SD= 1.54). The participants were surveyed using the Rosenberg Self-Esteem Scale (SES) and the Rokeach Value Survey (RVS). The results were considered significant at p<0.05. The findings demonstrated that people with lower self-esteem had higher opinion of values like Conformity, Pleasure, and Comfortable Life as well as some Instrumental Values (Self-Control and Politeness), when compared against the individuals with higher self-esteem. On the other hand, they were found to value Courage. There were no differences concerning the way individuals with high and low self-esteem rated Social Recognition, A Sense of Accomplishment and Self-Respect.

Also, the authors noticed that men were more likely to appreciate Hedonistic and Intellectual values, while women attached greater significance to Relational values. There were no gender differences concerning the rating of Aesthetic and Subjective values.

Open access