Ograniczenie lub pozbawienie władzy rodzicielskiej a uczestnictwo rodziców w procesie diagnostyczno-terapeutycznym podejmowanym wobec małoletniego pacjenta

This paper addresses the issues of parents’ participation in the diagnostic and therapeutic procedures, whose parental authority has been restricted or suspended or deprived of parental authority. The problems of participation in the diagnosis and therapy are discussed taking into account the right to give consent to the provision of health and the right to be informed about the health of the child.     

Sposoby oceny przez rodziców ciepłoty ciała dzieci

Introduction

There are various methods of measuring the temperature, various types of thermometers and places where the temperature can be measured. Currently electronic thermometers are the most widely used, mercury thermometers gradually becoming obsolete. A common point of measurement is the armpit, but in pediatric practice the measurement in the external auditory canal is considered to be the golden standard. Many people also assess temperature by touch, regardless of whether they have a home thermometer or not.The aim of the study was to analyze parental ways to measure body temperature, taking into account the thermometer measurements and approximate measurement.

Materials and methods

The survey was conducted on 206 people (194 mothers, 11 fathers, and 1 grandmother). The mean age of children was 24.2 months (min 2 weeks, max. 17 years, SD 37.7). The questionnaire included questions open and closed, single- and multiple-choice, and were concerning the assessment of the child's body temperature when fever was suspected. They were asked for a measurement method and the interpretation of the other symptoms of the child's fever. The results were statistically analyzed using Statistica version 6.0.

Results

The parents most often measure the temperature only using the electronic thermometer – 143 people (often in urban areas); 42 people measured fever using the mercury thermometer (more often in older children as well as in rural areas); in some cases – with several types of thermometers. Frequently temperature is measured in the armpit (in this way 90/186 people measure the temperature; more often in older children), less frequently – on the forehead (46/186 people, often younger children), rectum (40/186 people; often younger children), in the ear (31/186 people), and occasionally in other places. 115/125 people also measure children's temperature in an “orientation” way, by hand, usually on the head/forehead. For parents, other methods of assessing a child's fever are: the evaluation of the child's behavior, the change of the face expression, eyes, mouth, and a change in breathing.

Conclusions

In our study all parents use thermometers. The most commonly used are electronic thermometers, rarely – mercury thermometers. Frequently temperature is measured in the children's armpit, alternatively on the forehead, in the rectum, and the outer ear. A large number of parents additionally assess the child's body temperature by touch, and sometimes parents pay attention to other symptoms of fever.     

Udział mleka kobiecego w żywieniu noworodków urodzonych przedwcześnie

Aim

The objective of this study was to assess methods of feeding preterm neonates during hospitalization and at NICU discharge.

Material and methods

It was a questionnaire-based observational study on the use of human breast milk in nutrition of preterm infants. The questionnaires were distributed among mothers of preterm neonates on the last day of hospitalization.The material was divided into two groups: children born before 33 weeks of gestation (group 1) and children born between 33 and 37 weeks of gestation (group 2).

Results

During hospitalization, 85% received maternal breast milk – 24% were given breast milk only and 61% were additionally fed with neonatal formula for preterm babies, whereas 15% infants received only the formula. At the day of discharge data on feeding ways were: 59% children received exclusive breast-feeding, 12% mixed-feeding (maternal breast milk combined with formula in a 50:50 ratio), 9% mixed-feeding with formula constituting over 50% of the total supply, and 20% were not breastfed at all. In group 2, during hospitalization, 84% were breastfed and the remaining 16% received formula. At the day of discharge the ratio was in favor of maternal breast milk: 95% of the infants received it. Additional analysis of ways of administering breast milk and formula were made. The results revealed that 19 mothers were breastfeeding, 4 used expressed breast milk, and 28 combined both methods. Additionally 29% children received formula: in 8 of them, i.e. a half of the studied cases, it constituted less than half of daily milk supply, while the remaining subjects received maternal breast milk and formula in a 1:1 ratio.     

Udział epigenetycznych i genetycznych defektów regionu 11p15 w etiologii zespołu Beckwitha i Wiedemanna

Epigenetic processes, including genomic imprinting, are important for normal human growth and development. Beckwith-Wiedemann syndrome (BWS) is a disorder characterized by pre- and postnatal overgrowth in children, abdominal wall defects, macroglossia and a high risk of tumors. BWS is caused by different molecular defects of the 11p15 region containing several imprinted genes. The genes are localized into two domains: the first one is controlled by the imprinting control region ICR1 and the second one is controlled by ICR2. Epigenetic and genetic disturbances observed in BWS include ICR1 hypermethylation, ICR2 hypomethylation, paternal uniparental disomy, deletions, duplications, translocations, inversions in the 11p15 region, and point mutations in the CDKN1C gene. In this work new findings about Beckwith-Wiedemann syndrome etiology and molecular diagnostics are presented.     

Rozsiane aspiracyjne zapalenie oskrzelików w przebiegu achalazji przełyku u 9-letniego chłopca – opis przypadku

Achalasia is a rare disease in children. Diagnostic difficulties arise from nonspecific symptoms, especially in young children, and similarity to other more common clinical entities such as gastroesophageal reflux disease.     

Leczenie biologiczne we wtórnych zespołach hemofagocytarnych. Przegląd piśmiennictwa i opis przypadku zespołu aktywacji makrofagów w przebiegu leczenia etanerceptem układowej postaci młodzieńczego idiopatycznego zapalenia stawów

Macrophage activation syndrome (MAS), a secondary hemophagocytic lymphohistiocytosis (HLH), may be diagnosed in the course of the systemic inflammatory connective tissue diseases, especially systemic onset juvenile idiopathic arthritis (so-JIA). The pathological hyperactivation of the immune system results from a defective cytotoxicity of T lymphocytes and NK cells, leading to organ infiltration by these cells, hypercytokinemia and multiorgan failure. The classic clinical symptoms of this syndrome include fever, hepatosplenomegaly, bleeding. The most typical laboratory findings are hyperferritinemia, hypofibrinogenemia, a decrease of erythrocyte sedimentation rate, cytopenias or sudden drop in blood cells count and hemophagocytosis in the bone marrow or in tissues. The clinical course may be rapid and fatal. In the last decade, the biological agents against inflammatory cytokines – particularly inhibitors of tumor necrosis factor (TNF) and interleukin 1 and 6 (IL-1, IL-6) – are more widely used in the treatment of JIA. In Poland, etanercept and adalimumab are registered for use in children with polyarthritis JIA and tocilizumab (anti-IL-6) in so-JIA. Some casuistic reports on biological therapy, both as a risk factor of MAS and about its high efficiency in the treatment of this disease, have been found in medical literature recently.     

Napiętnowanie społeczne w chorobach przewlekłych

Regardless of diagnosis, chronic disease usually stigmatizes any patient. Social stigmatizing is one of the major risk factors for the quality of an individual's psychosocial functioning. Because of label of disease, patient is often ostracized. Social rejection equals a decline in mood and self-esteem. It also threatens emotional, social and intellectual growth of patients, particularly in developmental age. Limitation of peer contacts and lack of self-esteem may contribute to a loss of motivation. Such a condition often leads to discontinuation of medical recommendations. The stigma of the disease exerts a strong influence on the whole family system. Both patient's parents and siblings struggle with burden, whose negative consequences are reflected in family's and social's relationships as well as daily functioning. This paper discusses the definition, mechanism of the phenomenon, and consequences of social stigma, as well as presents literature review relating to stigmatization in the context of selected chronic diseases and family burden.     

Pseudouchyłek powikłaniem długotrwałego zalegania ciała obcego w przełyku u 5,5-rocznego chłopca

We present a case of a 5.5-year-old boy with complications after long-term retention of a foreign body in the esophagus. The patient presented certain symptoms such as dysphagia, odynophagia, cough and vomiting. The case mentioned above proves that in children with chronic respiratory symptoms (such as cough, stridor) or gastrointestinal ones (dysphagia, odynophagia) the possibility of foreign body ingestion should always be considered as the cause of these complaints.     

Badania kliniczne leku jako przykład eksperymentu medycznego z udziałem małoletniego – analiza wybranych aspektów prawnych

The article presents the analysis of chosen legal aspects connected with the participation of a juvenile in clinical examination of a medicine. What is more, the article discusses an issue of juvenile's consent to participate in medical experiment and providing information that is the condition of consent's validity. The authors pointed out the attention on acceptability of carrying out medical experiment with the participation of the juvenile in case of statutory representative's objection.     

Spożywanie wspólnych posiłków,wsparcie i komunikacja w rodzinie jako predyktory zdrowia subiektywnego i zadowolenia z życia nastolatków

Objective

To analyze relationships between the frequency of family meals, clear communication, support within the family and subjective health and life satisfaction of 11–15-year-olds.

Material and Methods

The study was conducted in 2012 among 319 primary and lower secondary school pupils. Young people filled the anonymous questionnaire about family meals, family relations, health and life satisfaction. Statistical analysis included partial correlations, linear regression analysis and structural modelling.

Results

The vast majority of the young people at least once a week, ate each of the main meals with their parents, 31% daily ate breakfast with their parents, and 46% – lunch and dinner. Frequency of family meals was significantly correlated with clarity of communication and support within the family. Family meals and family relationships positively correlated with subjective health and life satisfaction of young people. Frequency of eating family meals was a predictor of subjective adolescents’ health (direct and indirect relationship) and life satisfaction (indirect relationship). In both cases, support and communication within the family were the mediators.

Conclusion

Promoting family meals should be an important method of improving the quality of life of young people.     

Nowe techniki kariotypowania molekularnego przydatne w rozpoznawaniu mikroaberracji chromosomów u dzieci z zespołami wad,opóźnieniem psychoruchowym i niepełnosprawnością intelektualną

Precise cytogenetic diagnostics (e.g. FISH) in patients harboring chromosomal microaberrations is difficult, mostly due to a manifestation of similar clinical symptoms. Possible cost of the diagnosis could be increased to an unacceptable extent which results in resignation from further analyses. However these patients might account for the large number of people in genetic counselors’ offices; therefore they demand precisely anomaly-oriented further diagnostics. These problems could be usually solved by introduction of molecular karyotyping techniques (e.g. Real-time PCR, multiplex ligation-dependent probe amplification, BACs-on-Beads™), which allow detection of different chromosomal microaberrations in a single DNA sample required for final diagnosis of genetic syndrome, and thus seem to be very useful in patients suspected of harboring such an aberration.     

Udział małoletniego pacjenta,który ukończył 16. rok życia,w procesie diagnostyczno-terapeutycznym – wybrane aspekty prawne

The article presents chosen legal aspects linked with the participation of a juvenile, who is over 16-years-old, in diagnostic and therapeutic process. Furthermore, an issue of juvenile's consent to have a health benefit has been raised. In this range, legal basis has been shown allowing the doctor to take a medical intervention when the juvenile does not give consent to it. What is more, the article discusses doctor's responsibilities while handing over the information about health condition to the juvenile. A problem of the patient's right to intimacy has been also taken up.     

Ocena występowania powikłań miejscowych w wyniku zastosowania różnych technik nieinwazyjnej wentylacji u noworodków

Objectives

The assessment of the appearance of local complications associated with the use of different non-invasive ventilation techniques in neonates.

Materials and Methods

The study was conducted in a group of 103 neonates, in whom non-invasive ventilation was administered using different techniques. In these subjects non-invasive respiratory CPAP (conventional system) support was used with single lumen nasopharyngeal cannula, double lumen Hudson type short cannula as well as the Baby Flow and the Infant Flow systems for administration of all modes of ventilation. Data were collected based on own observations and on medical records of the neonates treated in the Neonatal Intensive Care Department of the Medical University of Warsaw between 21.01.2008 and 06.05.2009.

Results

The average duration of the different non-invasive ventilation techniques was 7.77 days (min. 1 day, max. 32 days). Skin complications were mainly concerned with the nasal structures and were of varying intensity: from transient ischemia, erythema, dermal abrasion, bleeding, necrosis and narrowing of the lumen of the nasal passages (edema of the nasal mucosa). Complications associated with the use of the Baby Flow and Infant Flow systems were dependent upon the type of nasal tip. In cases when a mask was used, the most frequent type of complication concerned lesions of the base of the nose (34 cases) and lesions of the upper labial sulcus (13 cases). Complications involving the nasal septum (8 cases) were associated with the use of short double lumen nasal cannulae with the Baby Flow and Infant Flow systems as well as with Hudson type cannulae (2 cases). Necrosis of the nasal septum was associated with the use of Hudson type cannulae in two neonates.

Conclusions

The number of complications affecting the skin associated with the use of non-invasive ventilation is dependent on the maturity of the neonate, the body mass and the duration of ventilation. Complications affecting the skin are markedly more frequent in those cases when masks are used compared with the use of short double lumen cannulae, and they mainly affect the base of the nose. Undoubtedly, the majority of complications are transient in nature.     

Heterogenność zespołu Guillaina-Barrégo na przykładzie opisu przypadków

Guillain-Barré syndrome (GBS) is an autoimmune disorder of peripheral nervous system, heterogeneous in clinical manifestation. Basing on clinical materials, in that article authors present the following forms of GBS: Miller-Fisher syndrome, Bickerstaff's brainstem encephalitis and the pharyngeal-cervical-brachial variant.     

Kwalifikacja chorych na mukowiscydozę do transplantacji płuc – doświadczenia własne

Background

Lung transplantation is a recognized treatment option for selected patients with cystic fibrosis in the end stage of bronchopulmonary disease. This is, however, a major challenge associated with the right choice of recipients proceedings before, around and after surgery, many complications and psychosocial factors.

Materials and methods

This article presents six patients aged 14–19 years who have been referred for LT from the Institute of Mother and Child. The eligibility criteria, the risk factors of death, which must be taken into account in the care of patients in the end stage of bronchopulmonary disease, were analyzed.

Results

Six patients out of 350 treated in the Outpatient Clinic of Cystic Fibrosis were qualified for the lung transplantation from 2008 to 2012. One patient died 22 months after LT, the remaining survival time is between 2 and 5 years. They are in good general condition under the strict control of transplantation centers.

Conclusions

The exact determination of the time to put a CF patient on the waiting list for transplantation is very difficult. But it is also of paramount importance to minimize mortality before and maximize survival after LT. Multidisciplinary team experienced in the treatment of CF, caring for a patient with transplant centre should make such difficult decisions. The principle of treatment of patients with CF before LT is to maximize all available and relevant aspects of care. The procedure should be tailored to the patient's needs and capabilities of the center.     

Rola interleukiny 17 w chorobach układu oddechowego u dzieci

Chronic or recurrent symptoms of respiratory diseases are the most common cause of visits to the pediatrician and hospitalization of children. Several studies in adults and children have shown that the profile of cytokines, including interleukin-17 (IL-17) in body fluids, is associated with the etiology of the symptoms of bronchopulmonary diseases. Synthesis of IL-17 strongly stimulates inflammation, autoimmune diseases, and respond to infections (viruses, bacteria, and fungi) and it is also involved in allergic reactions (asthma and allergic rhinitis). The production of IL-17 in the airway is stimulated by virus products and bacterial polypeptides, leading to an influx of neutrophils and release by a number of substances (such as metalloproteinases, elastase, or free radicals), and this may have an adverse effect on the pathomechanism of chronic lung diseases including asthma or cystic fibrosis. IL-17 is associated with airway hyperresponsiveness – existing studies indicate a compound Th2 responses and eosinophilia with Th17 response and neutrophilia. The article discusses the possible role of IL-17 in the development of chronic or recurrent symptoms of respiratory diseases in children.