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.     

Objawowy uchyłek osierdzia leczony torakoskopowo u 16-letniego chłopca

A pericardial diverticulum was diagnosed incidentally on a chest x-ray in a 10-year-old boy. The computed tomography and echocardiogram showed a round, unilocular cyst in the left cardiophrenic angle. The lesion was asymptomatic and its treatment was temporarily postponed. At the age of 16, the boy started to complain of left parasternal chest pain and epigastric fullness on physical exertion. During video-assisted thoracoscopic resection, the lesion was diagnosed as a pericardial diverticulum due to its communication with the pericardial cavity. The symptoms were relieved by surgery. Histological examination revealed a thin cyst with a fibrous wall lined by mesothelium.     

Wpływ zanieczyszczenia powietrza ozonem na układ oddechowy u dzieci

Ozone is an important component of air pollution. This review presents the negative impact of ozone air pollution on the respiratory system in children, including the induction and maintenance of inflammation in the airways and exacerbation of respiratory diseases. The relationship between the occurrence of exposure to elevated ozone concentrations and the prevalence of asthma in the context of selected genetic polymorphisms also are described. The possibilities of interventions to reduce exposure to ozone are discussed.     

Wykładniki metabolizmu tlenowego u dzieci i młodzieży z refluksem żołądkowo-przełykowym

Gastroesophageal reflux disease is a consequence of backward movement of the stomach content to the esophagus. The symptoms are caused by irritation of esophageal mucosa by acidic ingestant. Various factors cause disorders of cellular defense mechanism that lead to significant increase of oxygen-derived free radicals concentration and as a consequence to pathological damage of tissues and development of inflammation.     

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.     

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.     

Zespół hemofagocytarny u dzieci

Hemophagocytic lymphohistiocytosis (HLH) also known as hemophagocytic syndrome is a severe, life threatening inammation, caused by ineffective, prolonged immune response. It may occur as a primary (genetic) or secondary (acquired) disease. Primary HLH is divided into familiar HLH (FHLH) and other genetically determined conditions. In familiar form, HLH is the only manifestation of the disease. In other genetic forms, HLH is one of clinical manifestations. Secondary HLH may arise in a course of severe infections, autoimmune diseases, neoplasms, metabolic diseases, immunosuppressive therapy and after organ transplantation. The prognosis in HLH is inferior. Lack of proper treatment leads to death in primary HLH and in most cases of secondary HLH.     

Stała stymulacja serca u dzieci – wskazania,techniki implantacji oraz powikłania

The electrotherapy in pediatric patients dates from the early 60s of the twentieth century. Cardiac conduction abnormalities and arrhythmias may be associated with inflammation, congenital heart disease, cardiac surgery in the past, and autoimmune disease in mother (antibodies SS-A, SS-B). In past years, electrodes have been implanted epicardial manner, almost exclusively. Nowadays, this method is rather preferred in the youngest children with slight body size, complicated cardiac deformities, right-to-left intracardiac shunting or in the post-operative absence of venous access to the right heart. Increasing number of transvenous implantations is performed because of technological progress, miniaturizaion and more advanced operator experience. However, development of the young organism, intensive physical activity of the children and sometimes improper implantation technique may lead to serious complications. In our article we would like to summarize the present knowledge of indications, implantation techniques and complications management of electrotherapy in children.     

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.     

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.     

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.     

Przebieg kliniczny i odległe następstwa zespołu Guillaina-Barrégo u dzieci

IntroductionGuillain-Barré syndrome (GBS) is an autoimmune disorder of peripheral nervous system causing progressive weakness and areflexia. The process leads to acute flaccid paralysis of the two or four limbs and cranial nerves dysfunction. Epidemiologic studies have reported an annual incidence of 1:100000 children under 15.AimThe aim of study was to analyse the clinical course and long-term evaluation of GBS in children.Material and methodsEighteen children with classic form of Guillain-Barré syndrome were enrolled. In the first part of study we retrospectively reviewed the clinical course of disorder. In the second part we analysed the children's recovery, basing on the inquiry studies.ResultsIn the analysed group all children developed the paralysis of lower limbs, 38.9% – sphincter and bladder dysfunction, 72% – tetraparesis, 22.2% – respiratory tract insufficiency. Upper respiratory tract infections were the most common preceding GBS. Under performed treatment all children started to walk in first 6 months after GBS but they learned to run and climb the stairs more than one year. The efficiency of upper limbs was coming back faster than the lower limbs, 83% children could dress themselves and carry the heavier objects in half of the year. More than the half of group suffered from the subjective symptoms: limbs pains, hyperesthesia, and fatigability. These signs disappeared in the year after GBS. We reported the late effects of GBS, which were detected in analysed group after 12 month from GBS. There were: slowly running, fatigability, inability of dressing, climbing the stairs, hyperesthesia, tingling hands.     

Molekularne podłoże keratynopatii

Keratin disorders are a group of rare bullous skin diseases. They are caused by mutations in genes encoding keratins – the main structural proteins of intermediate filament cytoskeleton of epithelial cells and also one of the major components of hair and nails. Different pairs of keratins are the expressed depending on the skin layer, thus variability in the course of keratinopathies is observed. This disorders are inherited in autosomal dominant pattern mostly. Mutations occur in different regions of the keratin genes. In most of these diseases correlation between the location of mutations, and the severity of clinical symptoms can be observed. The most common keratin disorder is epidermolysis bullosa simplex (EBS), which is caused by mutations in KRT5 and KRT14. These genes are expressed in basal layer of epidermal cells. Depending on the effect of the molecular defect in the protein, EB simplex can phenotype of EBS may be highly different - from mild and localized forms, to severe and generalized. Another disease from this group is epidermolytic ichthyosis, which is caused by mutations in KRT1 and KRT10, expressed in epidermal suprabasal cell layer. This paper contains an overview of selected keratin disorders in terms of their molecular pathology. Beside those mentioned above, ichthyosis bullosa of Siemiens, pachyonychia congenita, and Dowling-Degos disease are also described.     

Słoiczkowe dania z żółtkiem dla niemowląt – przyczółek zdrowej diety

Introduction

Numerous investigations have demonstrated that the fatty acids composition of egg yolk is readily modified by dietary fat. Eggs are the most excellent vehicles to incorporate several health-promoting componenets, like docosahexaenoic acid (DHA), in it.In Poland, processing industries have been successfully manufacturing and marketing jarred baby foods.

Aim

We aimed to survey manufacturer claims concerning added eggs yolk in jarred infant foods supplied to the Polish market.

Methods

In August 2013, a total of 194 jarred so-called dinners in seven markets located in Warsaw were analyzed. Only three types of ready-to-eat jars contained yolk. The reduction of DHA intake may be due to increased intake of DHA-poor foods.

Conclusion

Better information on nutritional value of yolk and counselling of parents is desirable.     

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.     

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.     

Powikłania ospy wietrznej u dzieci w województwie łódzkim w latach 2001–2012

Varicella may cause serious, even life-threatening complications.     

Uchyłek Meckla u dzieci – ocena trudności diagnostycznych na podstawie doświadczeń własnych

Introduction

Abdominal pain is a common symptom encountered in medical practice. In some situations a diagnosis of abdominal pain requires close collaboration of a pediatrician and a pediatric surgeon, especially when the Meckel's diverticulum is suspected.

Aim of study

The aim of the study is to present diagnostic difficulties in patients suffering from the Meckel's diverticulum on the basis of own experience.

Materials and methods

An analysis was conducted on the basis of medical documentation of patients hospitalized at the Paediatric Surgery Department of the Provincial Specialist Hospital in Radom in the period from 2004 to 2011. The analysis included clinical, laboratory, imaging and endoscopic examination.

Results

The Meckel's diverticulum was diagnosed in 61 children out of 1021 patients who underwent laparotomy. It did not show any symptoms in 39 patients, but it caused abdominal pain in 22 of them. Within the group of 22 patients subject to the analysis, abdominal pain accompanied diverticulitis in 11 patients. In 9 children, Meckel's diverticulum caused mechanical occlusion of the alimentary tract whereas in 2 patients it co-existed with intensive bleeding from the lower alimentary tract.

Conclusions

A considerable changeability of clinical manifestation in patients with diagnosed Meckel's diverticulum can be observed. Interview and physical examination of a patient was of great significance in differential diagnosis of acute inflammation occurring within the abdominal cavity, which might be caused by Meckel's diverticulum. However, the final diagnosis was determined during an operation.     

Autoimmunizacyjne zespoły gruczołowe u dzieci – przebieg kliniczny i klasyfikacja na podstawie analizy wybranych przypadków

Autoimmune polyglandular syndrome, APS, is characterised by the presence of two or more chronic autoimmune organ-specific diseases in one individual. There are four types of APS; however, variable clinical course often makes the proper APS-type diagnosis difficult.In this paper, we would like to present 3 patients with APS, whose diagnosis has changed over time. The first patient was a girl with alopecia and normocytic anaemia, both of which occurred when she was 9 years old. At the age of 12, an autoimmune thyroiditis was diagnosed, and antibodies against adrenal cortex were found when she turned 17. The second patient was a 10-year-old boy admitted to the hospital because of vomiting, stomach ache and stomatitis/candidiasis. He also suffered from polydypsia and decreased appetite for several days. Type 1 diabetes and Addison's disease had been diagnosed. The third patient was a 16-year-old female with acute thrombocytopenia and anaemia accompanied by Graves’ disease. Additionally, during the course of the disease, she developed symptoms of lupus-like syndrome and central facial nerve palsy.The described cases indicate that the course of the disease as well as the diagnosis of the patient with APS may change over time. Atypical development of an autoimmune disease or atypical response for standard treatment should lead to extensive diagnosis towards new elements of the syndrome. Every organ-specific autoimmune disease might be the first prodrome of APS.