Parents' Knowledge of the Impact of Cystic Fibrosis on the Quality of Life of Children and Adolescents Suffering from This Disease as an Element of Patient Safety.

Parental perspective on the health, safety, and quality of life in children and adolescents with cystic fibrosis (CF). AIM OF THE STUDY: Assessment of the impact of a chronic disease such as cystic fibrosis (CF) on the quality of life and safety of children and adolescents as perceived by parents/caretakers. METHODS: The study was conducted at the Department of Lung Diseases of the Institute of Mother and Child, a branch of the Cystic Fibrosis Centre Children of Warsaw SZPZOZ in Dziekanów Lesny, the largest pediatric CF center in Poland, and in the Rodzinamuko group on Facebook. A total of 139 parents participated in the study. The study was conducted using the diagnostic survey method with the use of the Kid- & Kiddo-KINDLR questionnaire for examining the quality of life of children and adolescents and a demographic questionnaire. RESULTS: The perception of cystic fibrosis (CF) as a chronic disease varies based on parental residence and professional status. The well-being of children and adolescents with CF is tied to their parents' employment, particularly regarding schooling. Social interactions are influenced by the level of parental education. The quality of life in children and adolescents with CF is age-dependent, with younger children exhibiting higher quality of life. This age-quality of life relationship extends to physical well-being, emotional well-being, and school-related aspects. Furthermore, the emotional dimension of quality of life is affected by the child's age at the time of diagnosis. CONCLUSIONS: The Kid- & Kiddo-KINDLR QoL Questionnaire for children with cystic fibrosis is a good tool to measure parental knowledge. The study shows the need for the whole family to understand and be aware of the impact of CF on family life. Parents may be tired or may misunderstand or miscommunicate the medical team's instructions, which may affect both family life and patient safety. To ensure patient safety, parents should work with healthcare professionals at hospitals or clinics but also at home. They should also account for the family as a whole, not just for the problems of the child with CF.

Off pump hybrid extra-anatomic techniques for aortic arch repair-own experience.

BACKGROUND: Treatment of the aortic arch pathologies is technically challenging. In this study we assess early and late outcomes of hybrid aortic arch repairs that comprise extra-anatomic surgical procedures completed by thoracic endovascular interventions [thoracic endovascular aortic repair (TEVAR)]. METHODS: Since 2007, 21 patients (8 women and 13 men) with a median age of 48 years have undergone hybrid procedures for aortic arch pathologies. All of them were treated without cardio-pulmonary bypass. All survivors were followed up regularly and imaging examination were performed. A technical success, procedural complications as well as the early and late mortality and morbidity rates were evaluated. RESULTS: All patients survived surgery and TEVAR was technically successful in all of them. However, 2 individuals died (in-hospital mortality rate 9.5%) during in-hospital stay, both due to multi-organ failure (MOF). Additionally, one patient developed symptoms of cerebral stroke, another one of spinal cord ischemia. During the follow-up that ranged from 6 to 118 months and was completed by 100% of the survivors, one patient died 3 years after procedure because of sepsis (aorto-oesophageal fistula prior to intervention) and late vascular graft occlusions were noted in three cases. CONCLUSIONS: Hybrid procedures on the aortic arch that comprise surgical and endovascular interventions has become an attractive and safe therapeutic option with acceptable mortality and morbidity rate. They may be considered as a method of choice in treatment of the elderly and high-risk patients.

Analytical approach for the determination of steroid profile of humans by gas chromatography isotope ratio mass spectrometry aimed at distinguishing between endogenous and exogenous steroids.

The contamination of commonly used supplements by unknown steroids as well as their metabolites (parent compounds) become a challenge for the analytical laboratories. Although the determination of steroids profile is not trivial because of the complex matrix and low concentration of single compound, one of the most difficult current problem is to distinguish, during analytical procedure, endogenous androgens such as testosterone, dehydrotestosterone or dehydroepiandrosterone from their synthetic equivalents. The aim of this work was to develop and validate an analytical procedure for determination of the steroid profile in human urine by gas chromatography-combustion-isotope ratio mass spectrometry (GC/C/IRMS) toward distinguishing between endogenous and exogenous steroids. Beside the optimization of the experimental parameters for gas chromatography separation and mass spectrometry, attention was focused on urine sample preparation. Using an optimized sample preparation protocol it was possible to achieve better chromatographic resolutions and better sensitivity enabling the determination of 5 steroids, androsterone, etiocholanolone, testosterone, 5-androstandiol, 11-hydroxyandrdostane, pregnandiol, with the expanded uncertainty (k=2) below 1‰. This enable to evaluate the significant shift of the δ(13)C/(12)C [‰] values for each of examined steroids (excluding ERC). The analytical protocol described in this work was successfully used for the confirmation of positive founding urine by evaluation T/E ratio after GC/C/IRMS analysis.