Assessment of Quality of Life in Children With Pulmonary Hypertension Using Parent and Self-report Questionnaires.

Pulmonary arterial hypertension (PAH) is a progressive disease characterized by elevation of pulmonary vascular resistance and right ventricular failure. By using advanced therapies to reduce mortality, clinicians focus on improving functional status and quality of life (QOL). The aim of our study was to assess health-related QOL of pediatric patients with PAH. Parents of all children (aged 2-18 years) and patients aged 5-18 years with an appropriate level of intellectual development completed general and cardiac-specific validated surveys (Pediatric Quality of Life Inventory 4.0 and Pediatric Quality of Life Inventory 3.0, respectively). Demographic and clinical information was collected to grade disease severity. Twenty-five children were enrolled, yielding 25 parent reports and 15 patient self-reports. The PAH group had significantly lower scores than healthy children in all domains. Patients with World Health Organization Functional Class I had significantly higher parent proxy scores in School Functioning (P = .029) and in Heart Problems and Symptoms domain (P = .014) Patients with tricuspid annular plane systolic excursion below -2 z score showed impairment in each parent proxy general domain and in the Cognitive Problems score of the Cardiac module (P = .006). In conclusion the QOL of patients with PAH was impaired in every domain compared with healthy children. Patients with reduced right ventricle systolic function showed significantly lower QOL in all core domains. These results point to the need for psychosocial rehabilitation in addition to somatic care to improve the QOL in this severely ill population.

Use of Hemadsorption in a Case of Pediatric Toxic Shock Syndrome.

BACKGROUND: Toxic shock syndrome is a potentially fatal toxin-mediated disease. The role of toxins in this clinical entity made us hypothesize that extracorporeal blood purification with CytoSorb® could play a beneficial role in the clinical management of toxic shock syndrome. This case report describes the successful treatment of toxic shock syndrome using a combination of renal replacement therapy and hemadsorption in a pediatric patient. CASE PRESENTATION: A 5-year-old girl with Down's syndrome presented with an inflamed area surrounding an insect bite, signs of systemic inflammation, and multiple organ failure. As previous attempts of immune modulation therapy were unsuccessful, renal replacement therapy was supplemented by the cytokine absorber CytoSorb. Treatment using this combination was associated with a rapid and significant stabilization in the hemodynamic situation and a decrease in inflammatory mediators within hours after the initiation of therapy. The application of CytoSorb therapy was simple and safe. CONCLUSION: The use of extracorporeal blood purification with CytoSorb proved potentially beneficial by removing toxins and inflammatory mediators in this case and could therefore play a role in the clinical management of toxic shock syndrome. Whether CytoSorb has the potential to even positively influence mortality in patients with toxic shock syndrome still needs to be confirmed.

Pediatric myocarditis: A sentinel of non-cardiac chronic diseases?

INTRODUCTION: Although long-term outcome studies in large pediatric myocarditis/cardiomyopathy populations have been reported in literature, none of them focused on comorbidities. METHODS: All children and adolescents (age <18 years) treated with myocarditis at the Department of Pediatrics, University of Debrecen, Hungary were followed. Patients suffering from myocarditis during the period 1996-2011 were enrolled. RESULTS: Over the 16-year period, a diagnosis of myocarditis was established in nine children. Their median age was 1.11 (0.03-8.71) years. Three of the nine patients died. Left ventricular dilatation and ejection fraction normalized within 1-21 months in the survivors. None of the cases progressed to dilated cardiomyopathy. Regarding non-cardiac comorbidities, myocarditis or recurrent peri-myocarditis preceded the manifestation of celiac disease in two patients, while cystic fibrosis was diagnosed after the improvement of cardiac function in another, and Alström syndrome was diagnosed several years after complete recovery from myocarditis in yet another patient. CONCLUSION: These results suggest that manifestations of other chronic pediatric diseases may be more frequent among survivors of pediatric myocarditis. Prolonged follow-up of patients who survive myocarditis is therefore recommended not only to detect possible progression to cardiomyopathy but also to identify non-cardiac comorbidities.

Recycling of bowel content: the importance of the right timing.

INTRODUCTION: Extracorporeal stool transport (recycling of chyme discharged from the proximal stoma end to the distal end of a high jejunostomy or ileostomy) is thought to be beneficial in preventing malabsoprtion, sodium loss, cholestasis and atrophy of the distal intestine until restoration of the intestinal continuity becomes possible. However little is known about its adverse effects. Our aim was to investigate the microbiological safety of recycling. MATERIAL AND METHOD: Native samples were taken from the proximal stoma in 5 premature neonates who underwent an ileostomy or a jejunostomy due to necrotising enterocolitis, for qualitative culture. The first sample was drawn immediately after the change of the stoma bag, further samples were sent from the stoma bag at 30, 60, 90, 120, 150, and 180min later. The samples were inoculated by calibrated (10 µl) loops onto blood agar (5% sheep blood), eosin-methylene blue agar and anaerobic blood agar, respectively (Oxoid). The aerobic plates were incubated for 18-20 h at 5% CO2, whereas the anaerobic plates were incubated for 24-48 h in an anaerobic chamber (Concept 400). The bacterial strains were identified to species level by specific biochemical reactions, RapID-ANA II system (Oxoid) and ID32E, Rapid ID 32 Strep ATB automatic system cards (bioMérieux). RESULTS: The number of colony forming unit (CFU) of Gram-negative bacteria (mainly E. coli) exponentially increased after 30 min and reached 10(5)/ml after 120 min. Gram-positive strains (primarily E. faecalis) were detected after 60 min and CFU increased to 10(5)/ml after 120 min. The number of anaerobic (principally Bacteroides fragilis) CFU started to increase after 120 min. In two cases coagulase negative Staphylococcus strains were isolated the earliest in the chyme. The average of total CFU approached 10(5)/ml after 90 min and exceeded 10(5)/ml after 120 min. CONCLUSION: The chyme in the stoma bag is colonized by commensal facultative pathogenic enteral/colonic as well as skin flora species after 120 min. Recycling of stoma bag content may be dangerous after 90 min.

Measuring health-related quality of life in Hungarian children with heart disease: psychometric properties of the Hungarian version of the Pediatric Quality of Life Inventory 4.0 Generic Core Scales and the Cardiac Module.

OBJECTIVES: The aim of the study was to investigate the psychometric properties of the Hungarian version of the Pediatric Quality of Life Inventory (PedsQL) Generic Core Scales and Cardiac Module. METHODS: The PedsQL 4.0 Generic Core Scales and the PedsQL 3.0 Cardiac Module was administered to 254 caregivers of children (aged 2-18 years) and to 195 children (aged 5-18 years) at a pediatric cardiology outpatient unit. A postal survey on a demographically group-matched sample of the general population with 525 caregivers of children (aged 2-18 years) and 373 children (aged 5-18 years) was conducted with the PedsQL 4.0 Generic Core Scale. Responses were described, compared over subgroups of subjects, and were used to assess practical utility, distributional coverage, construct validity, internal consistency, and inter-reporter agreement of the instrument. RESULTS: The moderate scale-level mean percentage of missing item responses (range 1.8-2.3%) supported the feasibility of the Generic Core Scales for general Hungarian children. Minimal to moderate ceiling effects and no floor effects were found on the Generic Core Scales. We observed stronger ceiling than floor effects in the Cardiac Module. Most of the scales showed satisfactory reliability with Cronbach's alpha estimates exceeding 0.70. Generally, moderate to good agreement was found between self- and parent proxy-reports in the patient and in the comparison group (intraclass correlation coefficient range 0.52-0.77), but remarkably low agreement in the perceived physical appearance subscale in the age group 5-7 years (0.18) and for the treatment II scale (problems on taking heart medicine) scale of the Cardiac Module in children aged 8-12 years (0.39). Assessing the construct validity of the questionnaires, statistically significant difference was found between the patient group and the comparison group only in the Physical Functioning Scale scores (p = 0.003) of the child self-report component, and in Physical (p = 0.022), Emotional, (p = 0.017), Psychosocial Summary (p = 0.019) scores and in the total HRQoL (health-related quality of life) scale score (p = 0.034) for parent proxy-report. CONCLUSION: The findings generally support the feasibility, reliability and validity of the Hungarian translation of the PedsQL 4.0 Generic Core Scales and the PedsQL 3.0 Cardiac Module in Hungarian children with heart disease.

Measuring health-related quality of life in Hungarian children attending a cardiology clinic with the Pediatric Quality of Life Inventory.

The aim of the study was to evaluate health-related quality of life (HRQoL) of Hungarian children attending a pediatric cardiology outpatient unit with the Pediatric Quality of Life Inventory (PedsQL) questionnaire. The PedsQL Generic Core Scales and Cardiac Module were administered to 254 families including 195 children during a pediatric cardiology outpatient visit, and 525 families including 373 children from the general population were examined by the PedsQL Generic Core Scale by a postal survey. The relationships between PedsQL scores and patient characteristics were analyzed. Hungarian children attending a cardiology outpatient unit as an entire group and patients with severe heart diseases report significantly lower physical functioning than the general population, while more HRQoL dimensions are negatively affected according to the parents' opinion. Children with congenital heart disease of mild and great complexity also report impaired psychosocial functioning. HRQoL impairment is concentrated to the age of 5-7 years. Comparing our results with previous ones on U.S. pediatric cardiologic samples, we found significantly lower scores mainly for the physical functioning and heart symptoms subscales. This HRQoL study with an internationally well-validated instrument on children with heart disease in a Central European country highlights certain aspects of the health-care system and brings the possibility for the assessment of pediatric cardiology outcomes in a more comprehensive way.

[Hungarian validation of the cardiac module of the Pediatric Quality of Life Inventory]. / A Pediatric Quality of Life Inventory (PedsQL) gyermekkori életminoség-méro kérdoív kardiológiai moduljának magyarországi validálása.

UNLABELLED: The authors report the validation process of the cardiac module of the Pediatric Quality of Life Inventory (PedsQL ) into Hungarian. BACKGROUND: The PedsQL which is a modular instrument to evaluate health-related quality of life (HRQL ) in children and adolescents of ages 2-18 years, comes up to the current professional requirements at a high level. There is no report on health-related quality of life measure in Hungary which was performed among children with heart disease. OBJECTIVE: To adapt and to test a pediatric quality of life questionnaire for measuring HRQL in children with heart disease. METHODS: Sequential validation process which follows international guidelines. Pilot-study on 105 children with heart disease. RESULTS: According to the results of the pilot-study the psychic domains have a negative influence on general HRQL index in both child and parent-proxy reports in all age groups. On the cardiac module parents of children of all age groups but only children of ages 5-7 years reported marked treatment anxiety. Cognitive and communication problems are mainly important for children of ages 8-18 years. Parent-child concordance is depending on the age of the child, there was expressed difference in the psychosocial domains. We have found no negative effect of heart operation on HRQL by itself but taking medicine may impair it. CONCLUSION: Further methodologic research should evaluate the psychometric properties of the newly validated Hungarian version of the PedsQL cardiac module. The detailed report about the validation process can help in the spreading of patient reported outcome measures in Hungary.

[Quality-of-life measures in pediatric cardiology]. / Eletminôség-mérések a gyermekkardiológiában.

UNLABELLED: The need of outcome measures from the patients' aspect emerges reasonably in the field of rapidly developing pediatric cardiac surgery and pediatric cardiology. The professional standards and the possibilities of applicability of quality of life measures are often unknown for medical doctors. This is particularly true in pediatrics due to the numerous difficulties of pediatric measures and--in consequence--to the lower number of correct investigations. AIM: To summarize the basic professional expectations and results of previous quality of life studies performed in pediatric cardiology from a clinical point of view. METHOD: Survey of the literature of quality of life measures performed on mixed or specified adult and child population with congenital heart disease with a presentation of the main results and the basic characteristics of methodology. CONCLUSION: According to previous results, quality of life measures have a role in pediatric cardiology. Adequate measures can improve the level of care.

[Principles of quality of life measurement and its application in childhood]. / Az életminôség-mérés alapjai és annak gyermekkori alkalmazása.

BACKGROUND: The evaluative and critical analysis of articles on the quality of life which appear in growing number in the medical literature, and the adaptation of the measures to clinical practice can be realised only through knowledge of the professional standards of the measuring methods. OBJECTIVE: To outline the most widely accepted definitions of life- and health-related quality of life, to define the application possibilities as well as the parameters of the measuring methods. To demonstrate the most common problems as well as the possibilities of implementing pediatric measures. To give concrete guidance on evaluating previous measures and designing new ones. METHOD: Through a review of literature on methodology, cultural equivalence and paediatric measures, the authors outline the most frequent problems and give examples of some methods corresponding to the current professional standards. CONCLUSION: The quality of health care can be improved by using patient-reported outcome measures such as health-related quality of life measures. To assess different patient groups, modular approaches with general and disease-specific modules are advisable. The linguistic and cultural equivalence and the international comparability of the results can be achieved by adhering to professional guidelines and by performing psychometric tests to control the parameters of the methodology. In paediatric measures efforts should be made to conduct the simultaneous questioning of children and their parents as early as possible.

[Botulism in infancy - survey of literature based on a case report]. / Botulizmus csecsemokorban - irodalmi áttekintés egy eset kapcsán.

The authors have drawn attention to a rare disease called infant botulism, which raises some difficulties with regard to differential diagnosis. In the case of the six-month-old infant portrayed, infant botulism and food-born botulism appeared together. As a result of the severity of the disease and due to its rapid course observed in the presented case as well, the diagnosis has to be raised relying on the accurate patient's history and on the clinical examination, then it should be confirmed by appropriate examinations. In relation to presenting a case, the authors examine the microbiological background of the disease, its symptomatology, its epidemiological characteristics, the appropriate methods of electrophysiological examination and laboratory analysis as recommended in current literature, together with the difficulties that arise in differential diagnosis and also the possibilities of treatment. They pay special attention to the particular characteristics of the infant form of the disease, to the difficulties of making a diagnosis due to the characteristics of the age group and also to the questions in relation to the guiding principles of therapy. The rapid and fatal process observed in the presented case warns us that serious complications can occur even when up-to-date therapy is used. Therefore, despite the improvement in prognosis we have to strive to prevent the disease by spreading the proper regulations of nutritional hygiene.