Although medical advances have improved prognosis for children with cystic fibrosis (CF) and congenital heart disease (CHD), these youth experience poor psycho - social health. Embedded within the Medical Research Council’s framework, the purpose of this Dissertation was to a) examine how CF and CHD children experience physical activity, b) explore parents’ perceptions toward their child’s activity, c) develop a theory of physical activity in childhood chronic diseases, d) develop a physical activity counselling program for youth with CF and their parents, and e) evaluate the program impact on quality of life and physical activity. Study One explored how 14 CF youth experience physical activity. Active and inactive youth were characterized by different experiences, such as a sense of hope or despair. Given the burden of treatment and the fatal nature of the disease, youth negotiated temporal barriers to activity. Study Two explored perceptions toward activity among 29 CF and CHD parents. Parents discussed the benefits and barriers associated with physical activity for both child and self, and underscored the importance of role modeling. By adopting a Grounded Theory approach, the theoretical constructs from Study One and Two were crystallized to develop a theory of physical activity in youth with CF and CHD. This theory was used to develop “CF Chatters:” A Six Week Physical Activity Counselling Program for Youth with CF and their Parents, and the intervention employed behavioural self regulation ii skills. In Study Three, four case families in the CF clinic at the Hospital for Sick Children participated in CF Chatters. Improvements were noted in quality of life and physical activity, and participants described the program as convenient and relevant to their activity concerns. CF Chatters afforded therapeutic benefits to participants. By employing an eclectic qualitative approach, this Doctoral Program has made theoretical and practical contributions toward our understanding of how physical activity is experienced among children living with CF/CHD. The findings support the use of behavioural counselling as an effective and feasible modality for enhancing quality of life and physical activity. This Dissertation calls on clinicians to attend to the activity needs of chronically ill Canadian youth.

published_or_final_version / Paediatrics and Adolescent Medicine / Master / Master of Medical Sciences

The work described in this thesis has been carried out over the period 1958-1965, having been begun during the tenure of a residency at the University of Colorado, Denver, U.S.A. At this time, surgery for the closure of septal defects was being actively carried out, and interest was aroused, in two clinical features which were apparent from close contact with this programme. The first was the nature of the chest deformities associated with septal defects; the second, the striking alleviation of symptoms which followed successful closure of these defects. The reasons for these phenomena were by no means clear and the subsequent work has been concerned with an attempt to understand them.

published_or_final_version / Medical Sciences / Master / Master of Medical Sciences

Darbo aktualumas: Vaikų su įgimta širdies yda gyvenimo kokybės tyrimas atskleidžia ir padeda suvokti pagrindines vaikų ir jų tėvelių, šeimos problemas, poreikius, vertybes, nuostatas. Šių vaikučių gyvenimo kokybės vertinimas yra papildomas sveikatos priežiūros paslaugos veiksmingumo kriterijus. Juo remdamiesi galime stebėti sveikatos priežiūros veiksmingumą, esant reikalui, jį koreguoti, tobulinti. Tyrimo tikslas: Įvertinti 2–18 metų amžiaus vaikų, sergančių įgimta širdies yda, ir jų tėvų gyvenimo kokybę. Tyrimo uždaviniai: 1. Apžvelgti vaikų su įgimta širdies yda ir jų tėvų socialines-demografines charakteristikas. 2. Įvertinti vaikų, sergančių įgimta širdies yda, gyvenimo kokybę vaikų požiūriu. 3. Įvertinti vaikų, sergančių įgimta širdies yda, gyvenimo kokybę tėvų požiūriu. 4. Palyginti vaikų su įgimta širdies yda ir jų tėvų gyvenimo kokybės vertinimo požiūrio skirtumus. 5. Pateikti praktines rekomendacijas, šeimoms auginančioms vaikus su širdies yda, siekiant pagerinti tėvų ir vaikų gyvenimo kokybę. Tyrimo populiacija. Vaikai su ĮŠY ir jų tėveliai–gydyti Vilniaus Universiteto ligoninės Santariškių klinikose II širdies chirurgijos skyriuje bei atvykę konsultuotis į klinikos konsultacininę polikliniką, dėl širdies ydos. Tyrimo metodai:. Tyrimas atliktas anketinės apklausos metodu. Tyrimo instrumentai – anketa: PedsQLTM (Pediatric Quality of Life Inventory ™) širdies modulis versija 3.0, PedsQLTM įtakos šeimai modulis versija 2.0 bei papildoma anketa, skirta šeimos... [toliau žr. visą tekstą] / Urgency of the problem: research of quality of life of children with CHD uncovers and helps to understand the main children‘s and their parents‘, families‘ problems, demands, valuables, attitudes. Evaluation of quality of life of such children is an additional criterion to effectiveness of public health service. It can be used as a base for monitoring of effectiveness of public health service, to improve or adjust it, if needed. The aim of the research work was to evaluate quality of life of 2-18 years old children with CHD and their parents. Tasks of the research: 1. To review social–demographic characteristics of children with CHD as well as their parents. 2. To evaluate quality of life of children with CHD in children‘s point of view. 3. To evaluate quality of life of children with CHD in parent‘s point of view. 4. To compare evaluation approaches to quality of life of children with CHD and their parents. 5. To provide practical recommendations for families with CHD in order to improve quality of life of children and their parents. Sampling. Children with CHD–cured at II Surgery Department of Vilnius University hospital Santariškių klinikos as well as consulted at Consultation Clinic of the same hospital as well as their parents. Methods: The research was performed by questioning. Used tools were questionnaires PedsQLTM Cardiac module 3.0, PedsQLTM Family impact module 2.0 and additional questionnaire to evaluate family demographic data. The data was processed using... [to full text]

Congenital heart disease (CHD) is the major cause of death in the first year of life, the estimated incidence being 0.5-5% of live births; therefore it is important to understand the genetic causes underlying the complex process of heart formation to help prophylaxis, diagnosis and treatment of affected patients. CHD is the commonest phenotype associated with left-right (LR) disorders. LR asymmetry is determined during embryonic development. The three major body axes ? antero-posterior, dorso-ventral and left-right ? are patterned at gastrulation. LR asymmetry is established shortly after the two other major axes are patterned. The process of LR determination can be sub-divided into four integrated steps: 1. breaking of molecular symmetry in the gastrulation organizer; 2. transfer or relay of this asymmetric information to the lateral plate mesoderm (LPM), from which most internal organs will be formed; 3. reinforcement and propagation of asymmetric cues throughout the LPM and 4. conversion of asymmetric molecular information into proper organ morphogenesis. The goal of this work is to investigate mechanisms involved at two specific points in the laterality pathway: the initial generation/maintenance of asymmetric gene expression in the LPM and the morphogenetic translation of these early events into correct heart formation in the mouse. My emphasis has been on the characterization of laterality targeted cells via careful analysis of Pitx2c expression using a Pitx2c-lacZ reporter transgene, the role of BMP signalling, via Smad1, in generation/maintenance of early asymmetric signalling in the LPM, and the later involvement of both Smad1 and Pitx2 in cardiac morphogenesis through analyses of knockout mice.

Thesis (M.S.)--University of Michigan, 1987.

Thesis (M. Phil.)--University of Hong Kong, 1999. / Includes bibliographical references (leaves 111-127).

Thesis (M.S.)--University of Michigan, 1987.

Background Adults with congenital heart disease (CHD) is a growing population and related to advances in surgical and medical treatment, they now outnumber the children with corresponding lesions. Since a congenital heart lesion often results in reduced exercise capacity, this population is a potential target for physiotherapy. To what extent this reduction in exercise capacity is caused by abnormal cardiovascular anatomy and physiology or to what degree insufficient physical activity contributes is not known. To support the advancements in paediatric cardiac care, increased knowledge regarding physical performance, physical activity level, body composition and the effects of exercise training among adults with CHD is required. Methods In a cross-sectional study skeletal- and respiratory muscle function, physical activity level and exercise self-efficacy was investigated among 85 adults with various forms of CHD and 42 control subjects. A second study was conducted to analyse height, weight and body mass index (BMI) in 538 adults with complex CHD and 1886 adults with simple CHD. Data were extracted from the Swedish registry on congenital heart disease (SWEDCON) and compared to data from a national population survey. In a third study, factors associated with self-reported quality of life (QoL) were analysed using SWEDCON data on 315 adults with congenital aortic valve disease. Finally, a randomised controlled trial was conducted to investigate the effects of interval exercise training among adults with complex CHD. Results Adults with complex CHD showed impaired muscle function compared to both patients with simple CHD and controls. In addition, patients with complex CHD had a lower exercise self-efficacy compared to controls. Patients with CHD were equally active at moderate-to-vigorous level as the controls. However, approximately 50% of both patients and controls failed to reach the recommended physical activity level. In general patients with CHD had the same height, weight and BMI, as the general population. However, compared to the general population, men with CHD were more commonly underweight and less commonly overweight/obese. Additionally, especially male patients with complex CHD were shorter compared to the general population. Among adults with congenital aortic valve disease, a higher physical activity level was associated with better QoL. Furthermore, interval training increased exercise capacity and endurance among adults with complex CHD. Conclusion A higher physical activity level was associated with better self-reported QoL in patients with congenital aortic valve disease which implies that QoL might be possible to improve, by adopting a physically active life-style. Adults with CHD were equally active as controls at a moderate-to-vigorous physical activity level. However, approximately half of both groups were insufficiently physically active based on current recommendations. This indicates that low physical activity, on group level, does not explain the lower exercise capacity commonly found among patients with CHD. In addition, this is consistent the finding that the majority of patients followed the same pattern regarding BMI as seen in the general population. However, impaired muscle function in combination with the shorter stature and higher prevalence of underweight found in men, especially with complex CHD, implies an altered body composition in this group. The findings of the present thesis suggests an indication for physiotherapy targeting increased physical activity level and individualized exercise training in this patient population. Moreover, regular evaluation of muscle function, exercise self-efficacy and QoL, in addition to exercise capacity, might be useful for monitoring disease development over time.