Hereditary peripheral neuropathy, known asCharcot Marie Tooth disease (CMT) and with an incidence of 1:2500 -1:10 000, is the most common hereditary neuromuscular disorder. Type CMT 1A is the most common form of CMT refering to the group of primary demyelinitateing motor and sensory peripheral neuropathies. CMT phenotype is clinically characterized by chronic slowly progressive distal muscle weakness and atrophy with hypo or areflexia and mild to moderate acral sensory loss. The lower limbs are predominantly affected. The aims of this study were to describe the first and most common signs of CMT1A during the first decade of life, to characterize their progression, and evaluate the sensitivity of CMTNS (Charcot-Marie- Tooth neuropath scale) for CMT1A young children. Sixteen children aged 3 to 10 years with genetically proven CMT 1A were examined. All patients were clinically examined, underwent electrophysiological examination, and were scored by CMTNS. Eight were followed for up to two years. Our data shows that CMT 1A in children under the age of 10 years causes only a mild disability. Initial signs of CMT 1A were difficulty in heel walking (15/16, 93%) and lower limb hypo or areflexia ( 13/16, 81%). The test of heel walking can be easily used as a screening test for hereditary neuropathies in pediatrics....

This thesis: "The current status of pediatrics nurse field and its impacts on practice" deals with actual problem and it means education of pediatrics nurses. There were set two targets: 1. Changes in pediatrics nurses field in time. 2. Detections of problems in education of pediatrics nurses that are caused by new changes in the study program. These targets were specified by four research points and three hypothesis yet. Implementation of research was carried by a combination of qualitative and quantitative research methods. Five head nurses participated to the research and then one hundred nurses from children's unit in hospital as well. Our research was divided in three parts. In the first there were made interviews with head nurses. We used semi - structured form. In second phase we spread our written survey in pediatrics nurses who are working in children's unit. It was created an online survey mainly for students of medical disciplines for comparison. In last phase there were analyzed documents of work load of nurses from children's unit. Results show that in practice there we are able to see many problems. Pediatrics nurses who completed their education in high school grow old. New ones are missed and it means that like pediatrics nurses work general nurses as well. In our survey we found out that nurses with different length of practice are the same opinion on education. Then that pediatrics and general nurses, who are working in children's unit perceive the problems equally. But nurses with different length of practice are different opinion on actual long of study. Final results of this thesis will be published in professional journal.

Due to the increase of number of ionizing radiation examinations in the pediatric population, it is important to focus on protecting them, as we assume that children have a long life ahead, and therefore the likelihood of a higher risk of ionizing radiation damage, where repeated use of ionizing radiation can cause irreversible changes of the organism. Therefore, the main objective is to evaluate and investigate the level of radiation protection both of professionals and of the general public.

Childhood acute leukaemias are a heterogeneous group of malignant diseases. Based on cell origin, clinical manifestations, and molecular/chromosomal changes, we distinguish two main subtypes: acute myeloid leukaemia and acute lymphoblastic leukaemia. Acute lymphoblastic leukaemia (ALL) is the most frequent form of childhood leukaemia. Acute myeloid leukaemia (AML) is predominantly found in adults, being rarer in childhood. In the Czech Republic, the ALL is in childhood diagnosed approximately five times more often compared to AML. Despite the intensive research, aetiology of leukaemia has not been entirely clarified. So far, we only have knowledge of certain risk factors (ionising radiation, some chemicals and viruses) but in the vast majority of cases the aetiopathogenesis has not yet been made clear. Some of the answers may be provided by studies dealing with the presence of (pre)-leukaemic cells in a material archived prior to the clinical onset of the disease. Such are for example the so-called Guthrie cards, the dried blood samples collected immediately after birth and used in screening of the newborns for metabolic disorders. The better availability of material collected before the diagnosis of a secondary leukaemia (originally meant for the follow-up of the primary malignancy) might help us in better...

This diploma thesis deals with basic stimulation, specifically in intensive care units. Basic stimulation is a holistic method that approaches the patient as a whole. It views patients as individual beings with specific needs that make them different from others. We use basic simulation to enable people with physical or mental disabilities an easier integration in life. When taking care of patients within the framework of basic stimulation, we take their habits, manners of communication as well as experience into consideration. We make use of everything that an individual perceives as pleasant and is used to. When using the elements of basic stimulation we stimulate the individual whom we thus enable to perceive his or her bodily boundaries and surroundings, we facilitate his or her communication with the surrounding world and his or her return to becoming aware of his or her own ?I? ? of who I am actually. The objective of our thesis was to find out whether nurses working at paediatric intensive care units apply methods of basic stimulation in their profession and what attitude they adopt to it. Further, we investigated whether the parents of hospitalised children have information about basic stimulation, and again, we were interested in the attitude parents adopt to this method. To achieve our objectives, we chose both the quantitative method, by means of an anonymous questionnaire that we handed out to nurses at paediatric intensive care units, and the qualitative method, by which we obtained information from the parents of hospitalised children by means of a semi-structured interview. Our respondents were the above nurses from paediatric intensive care units and the parents of hospitalised children. We have found out from our results that despite the fact that basic stimulation is not applied by all nurses we interviewed, it is a widely used method in the care of patients and it brings quality results in care. The nurses adopt, in most cases, a very positive attitude to this method. They find improvement and calming in their patients. Although basic stimulation is a frequently applied method, some parents of hospitalised children have not heard of it. The parents got involved in the care of their children with pleasure, also when elements of basic stimulation was applied. The nurses were very often willing to teach the parents these elements. The parents regarded basic stimulation in nursing as very beneficial and observed its positive effect on their children.

This thesis deals with not only a question of competence of nurses and their children's development, but also in terms of preparation of professional nurses. The nursing skills are necessary competence to perform the nursing profession. Represent this area of knowledge, skills and attitudes that are acquired through education. One of the ways to qualify nurses to the profession, is today a university course. The second method is a specialized education, provided that the candidate meets the conditions for admission and received his professional competence to practice nurses. Determination of the competencies of nurses in the Czech Republic is governed by Law No. 96/2004 Coll. Acquisition and recognition of eligibility for non-medical professions and for activities related to providing health care and amending certain related acts, the last decree on the activities of health workers and other professionals, No. 55/2011 Coll. The thesis is divided into theoretical and practical part. The theoretical cross section contains the history of pediatrics and nursing, selected chapters from health legislation and development of children's ward in České Budějovice Hospital. For the practical part, I chose a qualitative research method of interviewing and pediatric nurses. The research sample consisted of 10 nurses who work or have worked on the inpatient pediatric ward. There were set three goals in this work. The first task was to determine the development of competencies nurses. The second objective was to determine the extent of the authority nurses at present. In the last part of my job I surveyed whether nurses are aware of their general competence. The research showed that their competencies developed. A large share of this development has the technological development in medicine, new insights in treatment and use of modern diagnostic methods and increasing nursing education. Results of this research can serve for professionals in pediatric congresses and publication in professional journals.

The number children and young people with high blood pressure has been increasing. The current lifestyle is one of the contributing factors to the disease. The most adverse is poor quality diet and excessive intake of salt. Further, the energy expenditure is lower, e.g. due lack of physical activities. Many children and young people do not have enough information about the risks of high blood pressure and its prevention. It is critical to identify groups with a risk of hypertension, i.e. children and young people with the so-called ?high normal? blood pressure. At the beginning we set four objectives: to learn the blood pressure measuring techniques suitable for children ? using digital and mercury tonometers, to find 25 ? 30 children with the ?high normal? blood pressure, to instruct nurses, particularly in the primary care, about the correct technique of blood pressure measurement in children and to compare the values of blood pressure measured with mercury and digital tonometers. The research was conducted in basic and secondary schools in České Budějovice and in a pediatric surgery. The researched group consisted of children and young people aged 13 ? 18. To achieve the first objective we measured blood pressure in 613 children with a digital tonometer and to achieve the second objective we measured blood pressure in 50 children both with mercury and digital tonometers. The results have been presented in form of tables. We found out that it was not necessary to examine more children, as we had expected, to get a sample group of 30 children with the ?high normal? blood pressure. Further, we found out that there was no significant difference between the values measured with a mercury tonometer and with a digital tonometer. The results have shown that it is essential to use the correct technique for blood pressure measurement in children. Based on our findings we have developed an educational material for nurses.

:.In our work we wanted to confirm our clinical experience with therapy of biliary and pancreatic duct injuries from the endoscopic retrograde cholangiopancreatography (ERCP) which was done in 267. Children and infants with a variety of biliary tract disorders and traumatic injuries in the area of biliary and pancreatic duct.. Pressure of the bile plays the key role in the therapy of biliary tract injuries Therefore we have measured the pressure in biliary tract and duodenum before and after the sphincterotomy of Oddi sphincter. Thea aim of our study was to confirm the insertion of drainage into the biliary and pancreatic duct in children with injury in this area. Our results showed significant differences between biliary duct pressure and duodenal pressure in the patients before and after sphincterotomy of Oddi sphincter. This results on theoretical basis confirmed, that it is necessary in children after traumatic rupture of biliary duct to provide ERCP and insert a biliary drainage after sphincterotomy. With this procedure the biliary tract injury is healed ad integrum without surgical liver resection. To provide only papilosphincterotomy without biliary drainage is not sufficient. This new miniinvasive procedure plays a fundamental role in the therapy of blunt abdominal injuries in a children and infants...

The Charter of rights of children in hospital (the EACH Charter) is an international document authored by the European Association for Children in Hospital (EACH) in the eighties of the twentieth century. The Charter can be perceived as a response to the fact that a child's stay in hospital - and paediatric hospital as an institution - is strongly influenced and formed by economical, technological and administrative aspects prevailing over the ethical ones. The Charter highlights specific situations where the health care profession - by neglecting children's needs and ignoring and/or tolerating avoidable suffering - becomes unethical. The aim of my thesis is to scrutinize the ethical appeal of the EACH Charter and to explore the Charter's ethical implications for contemporary paediatric practice in the Czech Republic.

In my thesis I deal with ionizing radiation, its origin, its effects and principles. I also deal with radiation protection and its principles, medical exposure and pediatric radiodiagnostics in terms of imaging methods and specifics of radiation protection in paediatrics. And whether all the methods used to reduce radiation exposure in children undergoing ionizing radiation are used. Part of the work is also devoted to the way of communication in this area and awareness, whether it is paid enough attention or this area is neglected in the Czech Republic.