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Gibson, Hannah Taylor. "Handgrip Strength in Children with Cystic Fibrosis." BYU ScholarsArchive, 2017. https://scholarsarchive.byu.edu/etd/6761.
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Background: Body mass index (BMI) is the primary accepted method to determine nutrition status in children with cystic fibrosis (CF); however, lean body mass (LBM) is more strongly associated with pulmonary function. Handgrip strength (HGS) measures muscle function and is reflective of LBM. The aims of this study were to assess if there was a relationship among HGS, nutrition status, and pulmonary function, to assess if HGS changed after hospitalization, and to assess if there was a relationship between HGS and nutrient intake. Methods: Twenty-three children with CF ages 6-18 years participated. BMI z-scores, nutrition risk scores, and pulmonary function were assessed about five months before, day 5-7 of, and about six weeks after hospitalization. HGS z-scores and arm anthropometrics were measured during and after hospitalization. Nutrient intakes were assessed during hospitalization. Results: Mean dominant HGS z-score was -1.95 ± 0.92 at hospitalization and -1.59 ± 1.06 at follow-up (p=0.007). Mean BMI z-score was -0.09 ± 0.64 at hospitalization and 0.06 ± 0.54 at follow-up (p=0.178). No significant relationship was found between HGS z-scores and BMI z-scores (p=0.892) or HGS z-scores and pulmonary function (p=0.340). Conclusions: HGS z-scores were lower than the standard even though mean BMI z-scores classified participants as normal nutrition status. Further research should be done utilizing a larger sample size in order to better examine HGS's potential as a nutrition assessment tool in this population.
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Bizzell, Laurie. "Maternal Stress and Cystic Fibrosis." Thesis, University of North Texas, 1996. https://digital.library.unt.edu/ark:/67531/metadc278693/.
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The purpose of the current study was to examine the relationship between parent and child factors for mothers of children diagnosed with cystic fibrosis to predict mother's psychological distress. Mothers were surveyed to identify measurement models in areas of Child and Parental characteristics and a Full Causal Model of Maternal distress. Factors related to Child Characteristics include general parental stressors and cystic fibrosis specific parental stressors. Factors related to Parental Characteristics include the mother's sense of parental competence and self-esteem. Additional factors related to the Full Causal Model include social support, major and minor life events, and demographics. Results were analyzed using LISREL IV structural equation modeling. Measurement model analysis found a good fit for the Child Characteristics model (Chi Square = 6.85, df = 4, JD = .144, Goodness of Fit Indices = .972) and Parental Characteristics model (Chi Square = 5.89, df = 3, p = .117, Goodness of Fit Indices = .971), but not for the full causal model of maternal distress (Chi Square = 114.98, df = 66, E = .000, Goodness of Fit Indices = .853)
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Modi, Avani C. "Adherence in children with cystic fibrosis and asthma." [Gainesville, Fla.] : University of Florida, 2004. http://purl.fcla.edu/fcla/etd/UFE0005641.
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Thesis (Ph.D.)--University of Florida, 2004.Typescript. Title from title page of source document. Document formatted into pages; contains 94 pages. Includes Vita. Includes bibliographical references.
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Goodhart, Frances. "What children with cystic fibrosis know about their illness." Thesis, City University London, 2003. http://ethos.bl.uk/OrderDetails.do?uin=uk.bl.ethos.269436.
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Davidson, Joanna K. "Nutrition and Bone Density in Children with Cystic Fibrosis." DigitalCommons@USU, 2004. https://digitalcommons.usu.edu/etd/5521.
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The purpose of these studies was to further research on bone density in children with cystic fibrosis, particularly as it pertains to nutritional parameters and care. The first paper presented a comparison of a group of 50 children with cystic fibrosis to a group of 32 control children. There were no significant differences between the groups in any of the pertinent bone density measurements. Serum 25(0H) vitamin D was positively correlated with spine density z score in the cystic fibrosis group.
The second paper, incorporating all of the information obtained from the first paper, describes an intervention study with the implementation of a fortified milk to determine the effects of additional calcium and vitamin D on bone density in the cystic fibrosis group. A follow-up bone scan was done. The fortified milk did not significantly improve bone density, but the fortified milk group did have significantly higher lung function scores on follow-up.
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Buntain, Helen Mary. "Clinical aspects of bone mass accrual in children and adolescents with cystic fibrosis /." [St. Lucia, Qld.], 2005. http://www.library.uq.edu.au/pdfserve.php?image=thesisabs/absthe19505.pdf.
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Dahné, Tova, and Tatyana Filonova. "Cystic Fibrosis and Physical Activity : Total Energy Expenditure and Physical Activity Levels in Children and Adolescents with Cystic Fibrosis." Thesis, Uppsala universitet, Institutionen för folkhälso- och vårdvetenskap, 2012. http://urn.kb.se/resolve?urn=urn:nbn:se:uu:diva-169166.
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Aim: The aim with the current study was to examine the physical activity levels (PAL) in a group of children and adolescents with cystic fibrosis (CF) and compare PAL-values between boys and girls. Further, the aim was to look at the total energy expenditure (TEE) estimated with an activity diary and measured with the accelerometer and compare values between these two measurement methods. Method: The sample consisted of 29 children and adolescents diagnosed with CF where PAL and TEE was measured during a three-day registration with activity diary and the ActiCal© accelerometer. The data was analyzed with the Mann-Whitney U-test. Results: The results indicated that the girls had a slightly higher median PAL-value than the boys. In addition, the range between the lowest and highest PAL-value was larger among the boys. The lowest PAL-value was lower among the boys than among the girls and the highest value was similar to the girls’ highest PAL-value. Overall, the subjective estimation of the PAL-values were slightly higher than what was objectively measured by the accelerometer. There was a slight difference between TEE estimated with the activity diary compared to the TEE measured by the accelerometer. There was no significant difference in PAL-values between boys and girls. Conclusion: There was no significant difference in PAL-value between boys and girls and that there was a slight difference in TEE estimated with the activity diary and measured with the accelerometer. Both methods of data collection are reliable enough when used together to produce a valid estimation of CF- children’s activity levels and TEE. The fact that both PAL and TEE values were higher in the activity diary may be because the participants perceived a higher level of physical activity then what was objectively measured with the accelerometer.
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Attard, Melanie Jane. "Carriers of responsibility : an existential encounter with parents who know their child is, or could be, a carrier of a mutation in the cystic fibrosis gene." Phd thesis, Faculty of Nursing and Midwifery, 2009. http://hdl.handle.net/2123/7883.
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Watson, Melissa. "Factors affecting the psychological adjustment of children with cystic fibrosis." Thesis, Open University, 2001. http://ethos.bl.uk/OrderDetails.do?uin=uk.bl.ethos.273318.
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Dakin, Carolyn Women's & Children's Health Faculty of Medicine UNSW. "Infection and inflammation in children with cystic fibrosis lung disease." Awarded by:University of New South Wales. Women's & Children's Health, 2009. http://handle.unsw.edu.au/1959.4/44624.
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The purpose of this study was to examine the relationships between inflammation, infection and lung function in cystic fibrosis during the evolution of lung disease in childhood and early adolescence. The developmental stages of childhood and the progression of lung disease together affected the methods and techniques used in the study, with the consequence that the work for this thesis fell naturally into two parts. The first part concerned the study of early lung disease in infants and young children who were unable to expectorate or to cooperate with lung function testing. In the second part, the inflammatory processes in both stable lung disease and during clinical exacerbations in older children and adolescents were studied non-invasively using sputum. The absence of a recognised definition of pulmonary exacerbation lead to further investigation into clinical heterogeneity in the diagnosis and management of an exacerbation. In early lung disease, inflammation was not found to be independent of infection, with pathogens in the lower airways found to correlate with levels of inflammation, respiratory system compliance and degree of air trapping (a relationship not previously shown). This suggested that infection remains the key target to minimizing lung damage in cystic fibrosis. The relationship between sputum markers of inflammation and lung pathology in established disease was found to be less clear, with high inflammation levels in both stability and during exacerbation. Reduction in sputum inflammatory levels following treatment of an exacerbation was found to be greater in those with lower pre-treatment levels. The definition and management of an exacerbation was found to be an area lacking consensus among clinicians, with likely consequent heterogeneity of clinical care and therefore inhomogeneity of hospitalization as a surrogate measure of exacerbation in a research setting. The work from this thesis, and the ensuing publications, has contributed to the understanding of the interactions between the inflammatory and infectious processes involved in CF lungdisease, in both early and more established lung disease in childhood.
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Oke, Carolyn Louise. "Social skills of children with cystic fibrosis and their siblings." Thesis, National Library of Canada = Bibliothèque nationale du Canada, 1997. http://www.collectionscanada.ca/obj/s4/f2/dsk3/ftp05/mq20894.pdf.
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Boesch, Richard Paul. "Outcomes and complications of fundoplication in children with cystic fibrosis." Cincinnati, Ohio : University of Cincinnati, 2008. http://www.ohiolink.edu/etd/view.cgi?acc_num=ucin1204839634.
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Thesis (M.S. of Epidemiology)--University of Cincinnati, 2008.Advisor: Dr. James E Heubi. Title from electronic thesis title page (viewed May 12, 2008). Includes abstract. Keywords: fundoplication; cystic fibrosis; pulmonary function; nutrition. Includes bibliographical references.
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BOESCH, RICHARD PAUL. "Outcomes and complications of fundoplication in children with cystic fibrosis." University of Cincinnati / OhioLINK, 2008. http://rave.ohiolink.edu/etdc/view?acc_num=ucin1204839634.
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Bogdasarova, Karina. "Oral Microbial Community Composition in Young Children with Cystic Fibrosis." The Ohio State University, 2014. http://rave.ohiolink.edu/etdc/view?acc_num=osu1404331049.
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Jaffe, Adam. "Assessment and feasibility of gene therapy for cystic fibrosis in children." Thesis, Imperial College London, 2000. http://ethos.bl.uk/OrderDetails.do?uin=uk.bl.ethos.589769.
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Gathercole, Katie Adele. "The educational experiences of children and young people with cystic fibrosis." Thesis, University of Leeds, 2017. http://etheses.whiterose.ac.uk/18012/.
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Children with medical conditions may experience significant challenges in education, of which there is often low awareness within schools. Yet, there is a dearth of research into cystic fibrosis (CF) and education. Research about CF commonly takes a medicalised or psychological standpoint and therefore the educational experiences of children and young people with condition are largely unknown. Educational research has focused on children with varying medical conditions, although most studies regard them as a homogenous group. CF remains distinctive from other conditions because it is invisible, it has an erratic profile, and a vast regime of treatments are needed in order to manage symptoms on a daily basis. The impact of CF upon the educational experiences of children and young people with the condition is a neglected research area. The study has begun to address this research gap. As an adult who has CF, this cross-disciplinary education and health study benefitted from an ‘insider’ research approach, which exemplifies the value of experiential knowledge. The research employed a two-phased, mixed methods design and drew upon an ecological, biopsychosocial approach to take account of the interacting systems in which children participate. First, questionnaires were administered to 75 school-aged individuals with CF at a large regional paediatric CF centre. Second, online interviews took place with 5 children and young people, and telephone or face-to-face interviews were conducted with parents, teachers and CF nurse specialists. The findings demonstrated that children’s needs are obscured in the school setting and they often experience low teacher awareness and understanding of the condition. Subsequently, children and young people may be subjected to vulnerable educational circumstances. The research informs developments in the education of children and young people with CF through the identification of their specific needs and challenges, and recommendations for educationally supportive practice.
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Riddell, Amy. "The muscle-bone in children and adolescents with and without cystic fibrosis." Thesis, University of Cambridge, 2016. https://www.repository.cam.ac.uk/handle/1810/268034.
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Introduction: Puberty is a crucial period for rapid changes in bone mineral, size, geometry, and microarchitecture. The mechanostat theory postulates that increased mechanical loading will affect bone phenotype and strength during development and in later life. Individuals with cystic fibrosis (CF) have an increased risk of developing osteoporosis and fragility fractures in young adulthood, which may be caused by poor growth. The aim was to investigate whether sex and disease status modified the relationship between: 1) puberty and bone, and 2) muscle and bone. This would contribute to the understanding of how sex (males vs. females) and disease group (CF vs.controls) alters the relationship between bone and muscle in children and adolescents as they transition through puberty and who, on a population level, differ in the prevalence of osteoporosis and risk of fracture in later life. Methods and Analyses: This observational study used novel imaging and muscle assessment techniques to measure bone and muscle parameters in White Caucasian children and adolescents, aged 8 to 16 years, living in the UK, with children with CF (n=65) and controls (n=151). Anthropometry and pubertal status were assessed. Dual energy X-ray absorptiometry, peripheral quantitative computed tomography (pQCT), high-resolution pQCT, and jumping mechanography were used to measure bone and muscle outcomes. ANCOVA with Scheffé post hoc and multiple linear regression tests were performed. Data were adjusted according to the research aims and included covariates; sex, disease group, pubertal stage, age, quadratic age, height, weight, maximum force (Fmax), and maximum power (Pmax). Data are presented as beta-coefficient (%) and p-value, with the significance level set to p < 0.05. Results: In height adjusted analyses, among healthy participants, females had smaller bones and lower bone density compared to males. With pubertal maturation, females had lower apparent gains in the distal and proximal total area (Tt.Ar and CSA), distal cortical porosity (Ct.Po) and proximal bone strength (SSI) but higher apparent gains in distal and proximal cortical bone density(Ct.BMD, Ct.TMD, vBMD). Females had consistently lower distal total area (total CSA) and density (total vBMD), distal trabecular density(BV/TV) and number(Tb.N), and proximal cortical area(CSA) compared to males, across all stages of puberty. With increasing muscle force (Fmax), females had higher apparent gains in total body less head bone mineral (TBLH BMC) and bone area(BA), distal total and trabecular density (total and trab vBMD) compared to males. In contrast, with increasing muscle power (Pmax), females had higher apparent gains in distal total and cortical densities (D100, Ct.BMD and Ct.TMD), and distal trabecular thickness (Tb.Th), and proximal cortical density (cortical vBMD) but lower apparent gains in distal cortical porosity (Ct.Po) and trabecular number (Tb.N) compared to males. In height adjusted analyses, participants with CF had smaller bones and lower bone density compared to controls. With increasing pubertal maturation, participants with CF had lower apparent gains in total body less head bone mineral and bone area, and in distal trabecular density, cortical porosity, and trabecular thickness compared to controls. Participants with CF had consistently lower distal total and cortical area, distal total and trabecular densities and proximal bone strength compared to controls, across all stages of puberty. With increasing muscle force, participants with CF had lower apparent gains in total body less head bone mineral and bone area, distal total density, trabecular density, and trabecular number. In contrast, with increasing muscle power (Pmax), participants with CF had higher apparent gains in distal trabecular density (BV/TV) and trabecular number (Tb.N) compared to controls. Conclusion: These findings suggests that sex and disease status do modify the relationships between puberty and bone, and between muscle function and bone. Skeletal adaptation to muscle differs between sexes and in populations with chronic disease, which may explain sex and disease group differences in risks of osteoporosis and fracture. Bone adaptation to muscle in children with CF is altered, which may lead to narrow, under-mineralised bones, with lower bone strength in later life. Understanding better impairments in muscle functions may provide targets for intervention to improve skeletal health in later life.
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Keller, Bigna Magdalena. "Growth in prepubertal children with cystic fibrosis, homozygous for the \223DF508 mutation /." [S.l.] : [s.n.], 2001. http://www.ub.unibe.ch/content/bibliotheken_sammlungen/sondersammlungen/dissen_bestellformular/index_ger.html.
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Gangell, Catherine Louise. "Evaluation of the forced oscillation technique for clinical assessment of young children with cystic fibrosis." University of Western Australia. School of Paediatrics and Child Health, 2008. http://theses.library.uwa.edu.au/adt-WU2008.0213.
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Background: Measurements of lung function are routinely used in patients with cystic fibrosis (CF) to provide information that may be clinically relevant. Spirometry is the conventional lung function measurement used, however young children find spirometry difficult to perform and often cannot achieve the strict acceptability criteria for the test. The forced oscillation technique (FOT) is a lung function measurement that only requires tidal breathing and is easy for young children to perform. However, there is limited information about the utility of this technique in the clinical assessment of young children with CF who are unable to perform spirometry. Aims: The aim of this project was to evaluate the FOT for clinical assessment in 2 to 7 year old children with CF. Specifically this involved: 1. Technical assessment of the FOT in children with CF; 2. Comparisons of lung function using the FOT in children with CF and healthy children; 3. Evaluation of associations with factors known to be associated with lung disease including: i) inflammation ii) infection and iii) structural damage. Methods Lung function was measured in a cohort of 59 children between the ages of 2 and 7 years with CF at the time of quarterly clinic visits. Resistance and reactance at 6, 8 and 10Hz (Rrs6, Rrs8, Rrs10, Xrs6, Xrs8, Xrs10, respectively) were reported and expressed as Z scores. Children were classified as asymptomatic or symptomatic based on a respiratory questionnaire and physical examination at the time of testing. Bronchoalveolar lavage and high resolution computed tomography (HRCT) were performed annually under general anaesthesia annually. BAL fluid was assessed for the presence of micro-organisms and quantification of a range of inflammatory markers and HRCT used to determine the extent of structural abnormalities. Results: The between test repeatability (n=25) for lung function was within limits previously described in healthy children. No systematic bias was observed and repeatability was not affected by the presence of respiratory symptoms. Children with CF (n=57) had significantly increased Rrs6-10 (p<0.0001) and decreased Xrs6-10 (p<0.004) compared to healthy children. Rrs6 and Xrs6-10 were significantly worse in the presence of respiratory symptoms, and Rrs6-10 progressively worsened from an asymptomatic to a symptomatic clinic visit. Children with CF (n=48) had no greater bronchodilator response (BDR) compared to healthy children. BDR was not influenced by the presence of an infection or respiratory symptoms. No relationships between inflammatory markers and lung function (n=39) were identified when the presence of an infection was adjusted for. Children with a current infection (n=20) had increased Rrs6-10 (p<0.01) and decreased Xrs6-10 (p<0.04) compared to children who were uninfected (n=23). These relationships were most marked for children infected with Pseudomonas aeruginosa, with children having a reduced lung function between 0.95 and 1.47 of a Z score. No relationships with the presence or absence of mild structural abnormalities (bronchiectasis, bronchial wall thickening and air trapping) and lung function at the time of HRCT were identified (n=34). Conclusion: The FOT is a repeatable measurement of lung function in children with CF and reliable results can be obtained in children as young as 2 years old. Young children with CF exhibit altered respiratory function which was affected by the presence of factors known to be associated with lung disease. The FOT has the potential to provide useful information about changes in clinical status in young children with CF and may be used to direct management of patient lung disease.
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Drury, Donna. "Vitamin D and K status and bone health in pediatric cystic fibrosis patients." Thesis, McGill University, 2006. http://digitool.Library.McGill.CA:80/R/?func=dbin-jump-full&object_id=101116.
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The objective of this study was to investigate the extent to which vitamin D and K are associated with bone health in pediatric cystic fibrosis (CF) patients. We hypothesized that: (1) the prevalence of vitamin D and K deficiencies would be high despite routine vitamin therapy, (2) bone health would be reduced and (3) vitamin K and D status would be associated with bone health.Our results showed poor bone mineral mass in these CF children despite mild disease and good nutritional status. Neither vitamin K nor D was a predictor of bone health but weight and height Z-scores, fat-free mass, physical activity and lung function were all consistent predictors.
These results indicate that nutritional status as well as physical activity are key determinants of bone health in CF children and offer a unique opportunity in the prevention of CF-related bone disease. Further vitamin intervention research needs to be done in this population.
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Cotter, Breda. "The journey through school for children with cystic fibrosis : an interpretive phenomenological analysis." Thesis, University of Newcastle upon Tyne, 2016. http://hdl.handle.net/10443/3302.
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This study set out to explore the experiences of children making their way through school with a diagnosis of Cystic Fibrosis. Cystic Fibrosis is a chronic illness which requires a rigorous treatment regimen and for which there is no cure. It is a recessively inherited disorder which affects multiple organ systems and involves a life expectancy of approximately 40 years. It is the most common life-threatening disorder in the Caucasian race (Wennstrom et al., 2011) and the incidence in Ireland is the world’s highest (Farrell, 2008). The aim of the study was to explore identity construction among these children with a view to establishing whether or not they consider themselves to be disabled and to have a disability. This involved accessing their self-theories in relation to a series of issues, the main focus being each child’s fundamental sense of self. Such a focus was considered an appropriate goal in view of the fact that learning who one is and where one fits into the world is a core preoccupation among school-age children (Erikson, 1968, 1982). In addition, the construct of identity has particular relevance in the context of a chronic illness. Issues relating to identity and to disability were ascertained by drawing on the relevant literatures in psychology and sociology. Given that the focus was on the children’s life-worlds, the methodology chosen for this study was Interpretative Phenomenological Analysis. Using a set of questions devised on foot of the literature review and covering five domains of enquiry, four children, recruited through the Cystic Fibrosis Association of Ireland and living in different parts of the country, comprised the research sample. Analysis of their responses revealed that the identity construction process is complex and that the issue of disability remains an empirical question requiring additional investigation.
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Kwarteng, Owusu Sandra. "Cystic fibrosis in black African children in South Africa: a case control study." Master's thesis, Faculty of Health Sciences, 2020. http://hdl.handle.net/11427/32272.
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Background Cystic fibrosis (CF) is described more commonly in Caucasian populations in whom p.Phe508del is the most common mutation. There is a paucity of data of CF in black African children. The aim of this study was to describe and compare the presentation and outcomes of black African children with CF to those with p.Phe508del genotype. Methods A retrospective case-controlled study was conducted from January 2000 – March 2018 of children with CF attending two CF centres in South Africa. Presentation, genotype, nutrition and pulmonary function outcomes of black African children were compared to matched controls with the p.Phe508del mutation. Results Thirty-four black African children (cases) with median age of diagnosis (5.5 months, IQR 2.0- 15.0) were matched to 34 controls. Among cases, 3120+1G->A CFTR mutation was most commonly identified; homozygous n=22 (64.7%) and heterozygous=7(20.5%). Compared to controls, cases at diagnosis were more malnourished and fewer presented with neonatal bowel obstruction [cases n=2 (5.9%) vs. controls n=10 (29.4%); p = 0.03]. Nutrition and pulmonary function (FEV1 in children ≥ 6 years) outcomes and changes over time from ages 3-16 years were similar in both groups; median FEV1 z-score at age 6,10 and 14 years was -0.9 (±1.5), -1.8 (±2.0) and -1.8 (±1.9) respectively for all patients. Deaths were recorded in three cases (8.8%) and one control (2.9%) (p = 0.6). Conclusion Black African children with CF were more malnourished at diagnosis, and fewer presented with neonatal bowel obstruction. Cases and controls had comparable nutritional, pulmonary function and early mortality outcomes.
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Gayer, Debra A. "Family functioning, marital status, and coparental cooperation as predictors of mother's adherence to prescribed CF treatments /." free to MU campus, to others for purchase, 1997. http://wwwlib.umi.com/cr/mo/fullcit?p9841288.
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Slater, Shalonda K. "The relationship between stress and depression in primary caregivers of children with cystic fibrosis." [Gainesville, Fla.]: University of Florida, 2003. http://purl.fcla.edu/fcla/etd/UFE0000792.
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Tran, Therese Marie Pascale Thi My Dung. "Steatorrhea and nutritional condition in cystic fibrosis children effects of a proton-pump inhibitor /." [Maastricht : Maastricht : Unigraphic, Rijksuniversiteit Limburg] ; University Library, Maastricht University [Host], 1996. http://arno.unimaas.nl/show.cgi?fid=6273.
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Westwood, Anthony Thomas Read. "Cystic fibrosis in children and adolescents in the Western Cape : epidemiological and clinical aspects." Master's thesis, University of Cape Town, 2005. http://hdl.handle.net/11427/10745.
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Includes bibliographical references (leaves 284-314).Cystic fibrosis (CF) is an autosomal recessive genetic disease caused by mutations on chromosome 7 in the gene for the CFTR protein. This gene encodes for a chloride channel on the apical surface of certain epithelial cells. The clinical manifestations of CF largely arise out of the resultant defect in water and electrolyte secretions in exocrine glands and epithelia such as are found in the pancreas, respiratory, gastrointestinal and genital tracts and sweat glands. First delineated as a clinical entity in the mid-20th century, CF was shown to be identifiable through the demonstration of elevated electrolyte levels in sweat - the sweat test. After many false starts, the underlying genetic defect was identified in the 1980s, culminating in the identification of the defective gene in 1989. This opened up possibilities of more accurate diagnosis and targeted treatments. Treatment of CF with pancreatic enzyme replacement therapy and antibiotics in the 1950s proved successful in controlling some of the severe and often fatal aspects of the disease. Further refinements to nutritional and antimicrobial therapies in the 1970s and 1980s produced rapid increases in longevity in many patients with CF. In SA, CF' has been identified since the 1950s. Clinical and research activities developed in the 1980s, mainly focused on the epidemiological and genetic aspects. Two clinical studies described features in children in Cape Town and adults in Johannesburg. My own clinical involvement in the RCCH's CF Service in Cape Town since 1992 led to the research activities that make up the bulk of this thesis. The thesis describes a number of aspects of CF as it affects patients in SA. The study population (described in Chapter 2) for most of the projects consists of 181 CF patients born between October 1974 and September 2003 who were identified by a combination of clinical features, positive sweat or genetic tests and/or post-mortem findings. All were resident in the Western Cape Province and received at least part of their health care at the RCCH. One hundred and sixty (88%) were born in the province and 21 settled in the province from elsewhere. Cape Town is unique in SA for its population demographics and the CF patients reflect this. CF has mainly been identified in coloured and white patients. Four black cases have been diagnosed. Compared with the CF population as described in the early 1980s, the CF population in the 21st century is larger (100 versus 64), older and there is a greater proportion of coloured patients. Nearly 3 in every 4 patients live in Cape Town.
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Hourigan, Shannon Estelle. "Improving Nutrition in Toddlers and Preschool Children with Cystic Fibrosis: Behavioral Parent Training Intervention." VCU Scholars Compass, 2012. http://scholarscompass.vcu.edu/etd/2818.
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The purpose of this single-case study was to pilot a developmentally sensitive adaptation of an evidence-based intervention aimed at improving nutrition in children with cystic fibrosis (CF). Children with CF must adhere to a high-calorie diet to prevent malnutrition and maintain health. Toddler and preschool age children present a unique feeding challenge to parents as they begin to exert independence and exhibit a variety of challenging behaviors. Parents trying to adhere to CF nutrition guidelines often use ineffective strategies that inadvertently encourage children not to eat. This six-week group parent-training intervention combined nutrition and behavior elements to provide parents with the nutrition and child behavior management strategies necessary to improve children’s intake and ensure adequate nutrition. Parents of four children (one girl) between the ages of 21 and 30 months of age participated in two groups; all children were Caucasian, and all parents were married. Two children were malnourished and had primary goals of increasing intake and weight; two were adequately nourished and had primary goals of improving diet quality. Primary and secondary treatment outcomes were established individually for each child. Families completed three-day diet diaries and video recorded mealtime interactions across two baseline weeks, six weeks of intervention, and a twelve-week post-intervention follow-up. Children’s weights were measured at baseline, post-treatment, and follow-up. Caloric intake was calculated for all meals, and video taped meals were coded using a behavioral coding system. Treatment fidelity was also assessed. The two malnourished children increased caloric intake throughout the intervention and demonstrated clinically significant weight gains at post-treatment. Further, these gains were maintained at follow-up. One of the two adequately nourished children demonstrated improved diet quality. Findings support the efficacy of this developmentally-sensitive adaptation to increase weight in toddler children with CF, and findings provide partial support for the efficacy of this intervention in improving diet quality in adequately nourished children. A parent group intervention provides training in CF-specific child management skills to multiple families and may provide significant benefits to parents who often struggle with the demands of nutrition requirements and toddler behavior.
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McClellan, Catherine B. "Evaluation of time out based discipline strategy to manage children's noncompliance with cystic fibrosis treatment." Morgantown, W. Va. : [West Virginia University Libraries], 2004. https://etd.wvu.edu/etd/controller.jsp?moduleName=documentdata&jsp%5FetdId=3518.
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Thesis (Ph. D.)--West Virginia University, 2004.Title from document title page. Document formatted into pages; contains v, 111 p. : ill. Includes abstract. Includes bibliographical references (p. 67-76).
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Whyte, Dorothy A. "The experience of families caring for a child with cystic fibrosis : a nursing response." Thesis, University of Edinburgh, 1989. http://hdl.handle.net/1842/8370.
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This thesis is concerned with families and chronic illness in childhood. The specific focus of the study is the nursing contribution to support of the family. A longitudinal study of the experience of four families caring for a child with cystic fibrosis was carried out using the ethnographic approach. Analysis of the four case studies provides insight to the effect of cystic fibrosis on each family member and on family interaction. The psychosocial transition by which the families moved from seeing themselves as healthy families to accepting the reality of a long-term health problem is described. The complexity of the effect of the genetic aspects and the grim prognosis is explored. The importance of finding meaning in suffering, the experiences of crisis and the chronic burden of care during the long-term adaptive stage of the illness are described. The support networks used by the families, and the nurse's contribution to that support are analysed. The research arose from practice and raises issues for nursing - issues relating to the level of interpersonal skill and the emotional investment required. The theoretical underpinning of nursing interaction is elaborated and the utility of systems theory in the understanding of the nursing situation is discussed. The importance of synchrony in the parents' adaptation to the child's illness is an emergent theme. The implications of the findings for nursing practice, management and research are discussed. The case for the development of a concept of family nursing to meet contemporary health care needs is argued.
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Babyar, Heather Michelle. "The Role of Social Media in the Relationship Between Social Support and Adherence in Children with Cystic Fibrosis." Kent State University / OhioLINK, 2016. http://rave.ohiolink.edu/etdc/view?acc_num=kent1469545679.
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Dawson, Kenneth P., and mikewood@deakin edu au. "Cystic fibrosis in children of the Eastern Arabian Peninsula : A clinical, spatial and genetic study." Deakin University. School of biological and chemical sciences, 2003. http://tux.lib.deakin.edu.au./adt-VDU/public/adt-VDU20050728.091141.
Full textAbstract:
Aim:
The aim of this thesis is to describe the process by which the inherited disease, cystic fibrosis, (CF) was recognised as an important clinical entity in the United Arab Emirates (UAE) and the Sultanate of Oman (Oman). It examines the clinical presentation of the first patients and assesses their degree of severity. Further, it describes the first studies carried out to determine the underlying CF mutations associated with the disease in the UAE and Oman. An estimate is offered of the birth frequency of the condition. Overall, the cultural, geographical and historical aspect of the societies in which the disease occurs is stressed.
Methods:
An initial literature search was carried out using Medline of any literature pertaining to the
Arab World and CF. this was read and classified into the relevance to Arabs in general, the
Middle East and then specifically the Arab (Persian) Gulf societies.
Thereafter, a clinic was established at Tawam Hospital, Al Ain, UAE, for children presenting
With chronic respiratory disease that could serve as a national referral centre. It was run by the
Author as a service of the Paediatric Department of the UAE University Medical School. I sent
a letter to every Paediatrician working in the UAE informing them of our clinic and offering
our services for the diagnosis and management of chronic respiratory disease in children. This
was based on the author's experience as a respiratory paediatrician in Australia and New
Zealand and as the Professor of Paediatrics in the UAE. No such service then existed in the UAE.
Funding was sought to establish a research programme and develop a molecular genetics laboratory in the UAE Medical School. A series of successful research applications provided the grants to commence the investigations. Once a small number of children had been identified as having CF from those referred to the respiratory clinic, the initial project was to assess and report their clinical presentation. Following this an early start was made on the identification of the mutations responsible. Once these were established an attempt was made to estimate the frequency of the condition at birth.
Additional clinical studies revolved around assessing the severity of the condition that was associated with the main mutations that were identified. A clinical comparison was made with those with the mutation AF508 and the other main mutation, despite the obvious limitation of small numbers then available. Radiological assessment was made to evaluate the progression of the disease.
The final aspect of the study was to assess patients from Oman and compare their findings and mutations with the neighbouring UAE. Based on information gained hypotheses are proposed regarding the spread of the gene mutation by population drift.
Thesis outline:
A literature review is presented in the form of a critique on the disease and a resume of the relevant aspects of the genetics of CF. Additionally, facts about the two countries' geography and history are presented. Finally, knowledge about CF mutations and population origins from other areas is presented.
The second main section deals with the clinical features of the disorder as it presents in the UAE. Molecular findings are then presented and details of the common mutation found in Bedouin Arabs. Hypotheses are then presented based on the information gathered.
Results:
CF is not a rare disease in the Arab children of the UAE and Oman. These findings refute previous reports of CF being a rare or non-existent disease in Arabs. The condition presents with a severe clinical picture, with early colonisation of the respiratory tract with staphylococcus, haemophilus and pseudomonas organisms, even with conventional CF management practices in place.
The CF mutation S549R is prevalent in Arabs of Bedouin stock, while AF508 is found in those of Baluch origin. The former may be descendants of Arabs who left southern Arabia and travelled to the Trucial Coast at the time of the destruction of the great dam at Marib. The origins of this mutation may lie in the area that corresponds to the modern Republic of Yemen. The latter groups are descendants of those who came originally from Baluchistan. It is hypothesised also that the ancestral home of the AF508 mutation may be in the geographical area now known as Baluchistan, that spans three separate modern political territories. The evidence presented supports the concept that the S549R mutation may be associated with a severe, if not the severest, clinical pattern recognised. It equates with that seen with the homozygous AF508 genotype. The absence of an additional mutation in the promoter region accounts for the different clinical pattern seen in previously described patients.
Conclusions:
There needs to be a major awareness of the presence of CF as a severe clinical disease in the children of the Gulf States. The clinical presentation and findings support the concept of under recognition of the disease. Climatic conditions put the children at special risk of hyponatraemia and electrolyte imbalance. The absence of surviving adults with the disease suggests premature deaths have occurred, but the high fertility rates have maintained the gene pool for this recessive disorder.
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Stapleton, Denise R. "Development, implementation and evaluation of a nutrition education and behaviour program for children with cystc fibrosis." Thesis, Curtin University, 2001. http://hdl.handle.net/20.500.11937/784.
Full textAbstract:
Background: Cystic fibrosis (CF) is a genetically inherited disease which adversely affects the respiratory and gastrointestinal systems. Malnutrition is a major clinical problem in individuals with the disease. Nutritional interventions are warranted as improvements in nutritional status could improve the rates of morbidity and mortality associated with the disease. The review of the literature indicated the need to develop a behavioural-based nutrition prevention program in order for children to achieve CF dietary requirements and appropriate pancreatic enzyme replacement therapy.Methods: The intervention program, Go and Grow with CF, and nutrition and pancreatic enzyme knowledge and self-management questionnaires were developed for children with CF and their carers as part of this thesis. Social learning theory constructs which particularly assist children in achieving desirable behaviours were applied during the development of the Go and Grow with CF program. The program consisted of workshops and a home-based course.Fifty eight children with cystic fibrosis, aged 2 to 11 years, and their carers participated in a clinical trial that was designed to assess the effects of the Go and Grow with CF pilot program on knowledge, self-management, behaviour, dietary intake and body composition, using anthropometry. Process evaluation was conducted on the pilot program and on the clinic-wide implementation of the revised Go and Grow with CF program. The revised program included the Australian Pancreatic Enzyme Replacement Therapy Guidelines and the effects of fat-based dosing were assessed with a cohort of 29 children with CF-related pancreatic insufficiency aged 1 to 13 years.Results: Similar to the process evaluation of the pilot program, 100% of carers who completed the revised home-based course indicated that they would recommend Go and Grow with CF to other families with a child who has CF. The 'objective assessment of knowledge indicated a significant m improvement in' children's knowledge in the short-term. There were no statistically significant improvements in any of the other parameters assessed. The lack of significant improvements in self-management, behaviour, dietary intake and anthropometry may have been because the program had no effect, the parameters assessed or the instruments used (particularly the questionnaires) were not sufficiently sensitive, the sample size (which was determined by the CF population available) was too small or the duration of the intervention and follow-up was too short.Conclusion: Carers' unanimous recommendation of Go and Grow with CF, together with high levels of perceived learning, reported increase in confidence and improvement in children's knowledge in the short-term, indicate the benefits of the program.Although there was no statistically significant improvement in the anthropometric measurements after the intervention, 'the extensive data obtained during this study suggest that measurements of height and weight may underestimate the presence of poor nutritional status. It is likely that comprehensive assessments of body composition of children with CF would be useful in detecting mild degrees of malnutrition and in providing information about the effects of nutritional status on morbidity and mortality associated with the disease.Fat-based pancreatic enzyme replacement therapy dosing warrants further investigation given that parents had a strong preference for this method and that fat absorption remains abnormal in the majority of individuals who have pancreatic insufficiency. Evaluation of all pancreatic enzyme replacement therapy dosing methods are needed and this research suggests that dose should be assessed on a meal and snack basis, rather than just on daily intake, in order for levels of adherence to be examined.The apparent absence of a long-term effect of a single exposure to the program on knowledge suggests that regular, ongoing education and counselling is required by families to reinforce aspects related to the child's current stage of development and disease status.
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Saynor, Zoe Louise. "Assessment and interpretation of aerobic exercise (dys)function in paediatric patients with cystic fibrosis." Thesis, University of Exeter, 2016. http://hdl.handle.net/10871/21875.
Full textAbstract:
The purpose of this thesis was to extend our understanding of the assessment and interpretation of aerobic exercise function of paediatric patients with cystic fibrosis (CF). The first investigation sought to establish (1) the validity of traditional criteria to verify maximal oxygen (V ̇O2max) during a maximal cardiopulmonary exercise test (CPET); and (2) the utility of supramaximal verification (Smax) to confirm V ̇O2max. Traditional criteria significantly underreported V ̇O2max, whilst Smax was shown to provide a valid measurement in this patient group. The reproducibility of this CPET protocol, over the short- (48 h) and medium- (4-6 weeks) term, was then established in study two. V ̇O2max was repeatedly determined with no learning effect over 48 h (typical error (TE): ∆150 mL; ∆9.3%) and 4-6 weeks (TE: ∆160 mL; ∆13.3%). Supplementary maximal and submaximal CPET parameters should be incorporated for a comprehensive evaluation of a patient, however they are characterised by greater variability over time. The influence of mild-to-moderate CF on aerobic exercise function and the matching of muscle O2 delivery-to-O2 utilisation during ramp incremental exercise to exhaustion were then examined in study three. Aerobic function was impaired in CF, indicated by very likely reduced fat-free mass normalised V ̇O2max (mean difference, ±90% CI: -7.9 mL∙kg-1∙min-1, ±6.1), very likely lower V ̇O2 gain (-1.44 mL∙min-1∙W-1, ±1.12) and a likely slower V ̇O2 mean response time (MRT) (11 s, ±13). Arterial oxygen saturation was lower in CF, supporting the notion that centrally mediated O2 delivery may be impaired during ramp incremental exercise. Although a faster rate of fractional O2 extraction would be expected in the face of reduced O2 delivery, this was not observed, suggesting additional impairment in O2 extraction and utilisation at the periphery in CF. The fourth study then demonstrated the clinical utility of CPET to assess the response to 12 weeks treatment with Ivacaftor, using a case-based design. Whilst one patient with relatively mild disease demonstrated no meaningful change in V ̇O2max, the second demonstrated a 30% improvement in V ̇O2max, due to increased O2 delivery and extraction. Furthermore, changes in aerobic function were detected earlier than spirometric indices of pulmonary function. This study demonstrated that CPET represents an important and comprehensive clinical assessment tool and its use as an outcome measure in the functional assessment of patients is encouraged. Study five investigated the V ̇O2 kinetics in this patient group. During moderate intensity cycling, the phase II V ̇O2 time constant (τ) (p = 0.84, effect size (ES) = 0.11) and overall MRT (p = 0.52, ES=0.33) were not slower in CF. However, both were slowed during very heavy intensity cycling (p = 0.02, ES = 1.28 and p = 0.01, ES = 1.40, respectively) in CF. Cardiac output and muscle deoxygenation dynamics were unaltered in CF, however, the arterial-venous O2 content difference (C(a-v ̅)O2) was reduced (p=0.03) during VH and ∆C(a-v ̅)O2 correlated with the phase II τ (r= -0.85; p=0.02) and MRT (r = -0.79; p=0.03) in CF. This study showed that impaired oxidative muscle metabolism in this group is exercise intensity-dependent and mechanistically linked to an intrinsic intramuscular impairment, which limits O2 extraction and utilisation. In conclusion, this thesis has provided guidelines for a valid and reproducible CPET protocol for children and adolescents with mild-to-moderate CF, demonstrated the utility of CPET as clinical outcome measure and furthered our understanding of the factors responsible for impaired aerobic exercise function in this patient group.
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Luna, Ruth Ann. "The Clinical Utility of Molecular Typing of Multiply-resistant Pseudomonas aeruginosa in Children with Cystic Fibrosis." VCU Scholars Compass, 2010. http://scholarscompass.vcu.edu/etd/2060.
Full textAbstract:
Chronic infection with P. aeruginosa is expected in patients with cystic fibrosis (CF), but the ability to delay, prevent, or better manage infection with multiply-resistant P. aeruginosa (MRPA) can potentially increase quality of life and extend survival. The Texas Children’s Hospital CF Care Center has identified an endemic MRPA strain (dominant clone), and this study aimed to identify risk factors for acquisition of the clone as well as determine differences in patient outcome associated with subsequent infection with the clone. The study included 71 patients with CF with documented MRPA infection. Designation of patients as members of the dominant clone or a non-dominant clone group was based on molecular typing by rep-PCR of MRPA isolates from respiratory cultures. Patient data was collected from Port CF, the national patient registry of the CF Foundation. Patient demographic information and clinical parameters prior to MRPA infection were analyzed by logistic regression as potential risk factors. Differences in patient outcome including change in BMI, change in FEV1, and hospitalization rate were evaluated by MANOVA. Recent hospitalization (< 90 days) was a statistically significant (p = 0.035) risk factor for acquisition of the dominant clone. Patients hospitalized < 90 days prior to MRPA diagnosis were four times more likely to be infected with the dominant clone, and patients hospitalized 91-180 days prior were almost three times more likely. Increased hospitalization rates were seen in the dominant clone group both pre- (11 more days/year) and post-infection (14 more days/year) as compared to the non-dominant clone group. Patients infected with the endemic strain exhibited poorer outcomes in terms of nutritional status (3.73% decrease/year in BMI %ile) and lung function (3.7% decrease/year in FEV1 %ile). Significant overlap in hospitalization episodes of patients known to be infected with the dominant clone and patients subsequently infected with the dominant clone was observed. Recent hospitalization was a significant risk factor for infection with the dominant MRPA clone, and following infection, patients infected with the endemic strain exhibited declines in nutritional status and lung function and increased hospitalization rates. The results suggest potentially increased virulence and transmissibility of the endemic MRPA strain.
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Jenkins, Bradlee A. "Effect of Asthma and Cystic Fibrosis on Health Utilization and Education Progress in Children and Adolescents." Digital Commons @ East Tennessee State University, 2013. https://dc.etsu.edu/honors/108.
Full textAbstract:
Aims. To determining the effect of asthma, cystic fibrosis (CF), and both on healthcare utilization, absences from school, and rate of progress in school.
Background. Previous studies on academic progression and health care utilization in children with the respiratory diseases of asthma and CF have been limited or conflicting.
Design. Non experimental, cross-sectional, secondary data analysis using a multilevel probability sample.
Methods. The response of all persons under 18 years of age in the U.S. 1997-2012 National Health Interview Survey to interviewer questions on demographic, educational, and health care utilization items were analyzed. The mean and 95% confidence intervals for these variables were compared for children with asthma, CF, and controls.
Results/Findings. Children with asthma, CF, or both had a higher utilization of all major types of healthcare services than children with neither of these conditions. Children with asthma or CF were more likely to have not only poor health, but more rapid declines in recent health with the greatest decline in those with both conditions. The number of missed school days was also higher, and highest in children with both CF and asthma. Despite the many missed school days, children with asthma paradoxically had a greater rate of progression in school than those without asthma. Those with CF or CF with asthma had a slower rate.
Conclusion. Asthma in CF has a negative impact on attendance and progress in school largely opposite that of asthma alone.
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Russell, Jeremy. "Innovative psychological strategies for the management of distress amongst young people with cystic fibrosis." Thesis, Queensland University of Technology, 2021. https://eprints.qut.edu.au/207787/2/Jeremy_Russell_Thesis.pdf.
Full textAbstract:
Cystic Fibrosis (CF) and its treatment pose challenges to young people and their families. This research identified the psychological needs of young people with CF, assessed the feasibility of a brief treatment, and examined whether aspects could be delivered by a social robot. Thoughts young people had about their distress were an important target. The new treatment helped the young people develop a positive story of life they shared with their family, and resulted in reduced distress. Use of a social robot was feasible, and aided engagement. The research provides a foundation for further work in the area.
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Stapleton, Denise R. "Development, implementation and evaluation of a nutrition education and behaviour program for children with cystc fibrosis." Curtin University of Technology, School of Public Health, 2001. http://espace.library.curtin.edu.au:80/R/?func=dbin-jump-full&object_id=13047.
Full textAbstract:
Background: Cystic fibrosis (CF) is a genetically inherited disease which adversely affects the respiratory and gastrointestinal systems. Malnutrition is a major clinical problem in individuals with the disease. Nutritional interventions are warranted as improvements in nutritional status could improve the rates of morbidity and mortality associated with the disease. The review of the literature indicated the need to develop a behavioural-based nutrition prevention program in order for children to achieve CF dietary requirements and appropriate pancreatic enzyme replacement therapy.Methods: The intervention program, Go and Grow with CF, and nutrition and pancreatic enzyme knowledge and self-management questionnaires were developed for children with CF and their carers as part of this thesis. Social learning theory constructs which particularly assist children in achieving desirable behaviours were applied during the development of the Go and Grow with CF program. The program consisted of workshops and a home-based course.Fifty eight children with cystic fibrosis, aged 2 to 11 years, and their carers participated in a clinical trial that was designed to assess the effects of the Go and Grow with CF pilot program on knowledge, self-management, behaviour, dietary intake and body composition, using anthropometry. Process evaluation was conducted on the pilot program and on the clinic-wide implementation of the revised Go and Grow with CF program. The revised program included the Australian Pancreatic Enzyme Replacement Therapy Guidelines and the effects of fat-based dosing were assessed with a cohort of 29 children with CF-related pancreatic insufficiency aged 1 to 13 years.Results: Similar to the process evaluation of the pilot program, 100% of carers who completed the revised home-based course indicated that they would recommend Go and Grow with CF to other families ++with a child who has CF. The 'objective assessment of knowledge indicated a significant m improvement in' children's knowledge in the short-term. There were no statistically significant improvements in any of the other parameters assessed. The lack of significant improvements in self-management, behaviour, dietary intake and anthropometry may have been because the program had no effect, the parameters assessed or the instruments used (particularly the questionnaires) were not sufficiently sensitive, the sample size (which was determined by the CF population available) was too small or the duration of the intervention and follow-up was too short.Conclusion: Carers' unanimous recommendation of Go and Grow with CF, together with high levels of perceived learning, reported increase in confidence and improvement in children's knowledge in the short-term, indicate the benefits of the program.Although there was no statistically significant improvement in the anthropometric measurements after the intervention, 'the extensive data obtained during this study suggest that measurements of height and weight may underestimate the presence of poor nutritional status. It is likely that comprehensive assessments of body composition of children with CF would be useful in detecting mild degrees of malnutrition and in providing information about the effects of nutritional status on morbidity and mortality associated with the disease.Fat-based pancreatic enzyme replacement therapy dosing warrants further investigation given that parents had a strong preference for this method and that fat absorption remains abnormal in the majority of individuals who have pancreatic insufficiency. Evaluation of all pancreatic enzyme replacement therapy dosing methods are needed and this research suggests that dose should be assessed on a meal and snack basis, rather than just on daily intake, in order ++
for levels of adherence to be examined.The apparent absence of a long-term effect of a single exposure to the program on knowledge suggests that regular, ongoing education and counselling is required by families to reinforce aspects related to the child's current stage of development and disease status.
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Schurer, Geertruida Wilhelmina. "Changes in body composition, anaerobic muscle power, and pulmonary function with age in children with cystic fibrosis." Thesis, National Library of Canada = Bibliothèque nationale du Canada, 1997. http://www.collectionscanada.ca/obj/s4/f2/dsk2/ftp04/mq24505.pdf.
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Aurora, Paul. "Multiple-breath inert gas washout to detect inhomogeneity of ventilation distribution in preschool children with cystic fibrosis." Thesis, University College London (University of London), 2005. http://discovery.ucl.ac.uk/1445299/.
Full textAbstract:
Measurement of lung function in preschool children (those aged two to five years) is notoriously difficult. The aims of this thesis were to determine whether multiple- breath inert gas washout (MBW) could be performed successfully in preschool children, and whether MBW indices were more sensitive for detecting cystic fibrosis (CF) lung disease in preschool children than spirometry indices. First, quality control criteria for data collection and interpretation in children with and without CF were examined. 75% of preschool children successfully performed spirometry at first attempt, but adult criteria for start and end of test and reproducibility were inappropriate. Similarly, 79% of preschool children completed MBW at first attempt. The primary outcome measure from MBW (Lung Clearance Index LCI ) was independent of subject characteristics in healthy preschool and school-age children. Analysis of the progression of the phase III slope through MBW allowed derivation of indices representing conducting zone (Scond) and acinar zone (Sacin) inhomogeneity. Volume correction of these indices facilitated comparison between groups. MBW and spirometry results were compared in 55 school-age and 60 preschool children. Whilst group differences were seen for both tests, 11/22 (50%) of school- age children with CF had normal spirometry, whilst only one (5%) had normal LCI. 26/30 (87%) of preschool children with CF had normal spirometry, whilst only eight (27%) had normal LCI. Virtually all children with CF had raised Scond, with no age relationship seen. Sacin was normal in most younger children with CF, and raised in most aged 10 years and older. MBW detected lung disease in preschool children with CF more frequently than spirometry. Most young children with CF had evidence of conducting airway disease, whilst acinar zone involvement was predominantly seen in older school-age children. These findings support the hypothesis that MBW will have value as a clinical measure in this patient group.
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Vandenbroucke, Natalie Joëlle. "Lung function determinants and mortality of children and adolescents with cystic fibrosis in South Africa 2007-2016." Master's thesis, Faculty of Health Sciences, 2019. https://hdl.handle.net/11427/31782.
Full textAbstract:
Objectives: Cystic fibrosis (CF) is one of the commonest inherited disorders in South Africa, affecting all population groups. Progressive pulmonary disease with declining forced expiratory volume in one second (FEV1) is the main predictor of morbidity and mortality in individuals with CF. This study aimed to describe the change in lung function, nutritional status and mortality of children and adolescents with CF, attending the Red Cross War Memorial Children’s Hospital (RCWMCH) CF Clinic and to identify factors associated with poor pulmonary function outcomes and mortality. Methods: A retrospective study was conducted of the clinical records and annual pulmonary function tests, with matched body mass index (BMI), of children between 5 and 18 completed years of age attending the RCWMCH CF clinic in Cape Town, South Africa, between January 2007 and December 2016. Results: A total of 143 study participants (51.4% male; median age at diagnosis 5.5 months) were included. Population mean FEV1 and body mass index (BMI) Z scores improved from -2.5 1.70 to -1.9 1.70 (p = 0.1) and from -0.7 1.2 to -0.4 1.2 (p = 0.3) respectively from 2007 to 2016. FEV1 Z score declined by an average of 0.17 per year of age and this was mirrored by an average decline in BMI Z scores of 0.07 for each year of advancing age. FEV1 decline was greater in patients who died compared to those who survived (p = 0.03). Of the factors postulated to influence lung function decline, there was no significant correlation between FEV1 at any age and age of diagnosis, sex, ethnicity, genotype, geographical location, pancreatic status, or Methicillinresistant S. aureus or Aspergillus spp. infection. Participants who were ever infected or colonised with P. Aeruginosa had consistently lower FEV1, however this difference only became significant at certain ages. On multiple stepwise regression analysis, only FEV1 at age 6 was found to be a significant independent predictor of mortality (adjusted odds ratio (95% CI) 0.5 (0.3 – 0.8); p = 0.005). Conclusion: Pulmonary function of children with cystic fibrosis improved non-significantly over the 10-year study period. FEV1 at age 6 was identified as an independent predictor for CF-related mortality. Early diagnosis and measurement of pulmonary function in young children with CF is essential to identify children at risk of poor outcomes.
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Cox, Madison Allen. "Self-Reports of Hearing and Tinnitus Related to Audiometry in Children and Young Adults with Cystic Fibrosis." Walsh University Honors Theses / OhioLINK, 2020. http://rave.ohiolink.edu/etdc/view?acc_num=walshhonors1588691309804014.
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Passweg, Jakob. "Salivary caffeine clearance for measurement of liver function in children with liver disease, cystic fibrosis and epilepsy /." [S.l : s.n.], 1987. http://www.ub.unibe.ch/content/bibliotheken_sammlungen/sondersammlungen/dissen_bestellformular/index_ger.html.
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Booth, Erin B. "Influence of Contextual Factors and Self Efficacy on Self- Management in Parents of Children with Cystic Fibrosis." VCU Scholars Compass, 2017. https://scholarscompass.vcu.edu/etd/5152.
Full textAbstract:
Cystic Fibrosis, a life threatening autosomal recessive genetic disease, is characterized by a defective gene resulting in the production of thick mucus that obstructs the lungs and pancreas. CF requires intensive management performed at the home. An initial pilot study was performed to describe knowledge of CF related diabetes (CFRD) in adults with CF. The findings of this study, which demonstrated that adults with CF lacked sufficient knowledge about CFRD confirmed the need to explore additional factors of self-management guided by a theoretical framework. The second study presented in this dissertation used the Individual and Family Self-Management Theory (IFSMT) to describe context (condition-specific and individual and family factors) and process (self-efficacy and knowledge) and outcome (family self-management) variables for caregivers of children with CF. It also compared differences in context, process, and outcomes in caregivers based on socioeconomic status (Medicaid vs. private insurance), and explored correlations among context, process, and outcomes. Participants for this cross-sectional descriptive study were caregivers of individuals with CF who were under the age of 18 and diagnosed with CF for at least 9 months. Participants completed a demographic survey and questionnaires that included measures of perceived disease severity (VAS), depression (Patient Health Questionnaire), self-efficacy (Perceived Health Competence Scale, Mountain West Cystic Fibrosis Consortium Questionnaire), knowledge (CF Knowledge and Attitudes Questionnaire), and self management behaviors (Self-Management Behaviors Questionnaire) Additional information was collected on the children with CF and included demographic information as well as height/weight/BMI, pulmonary function test results, medication profile, and insurance status.
Participants in this study were primarily female caregivers with high self-efficacy, and average knowledge. The children with CF in this study had moderate treatment complexity and normal/mild impairment in lung function. Deficits were noted in the areas of caregivers’ reproductive and genetic knowledge. This study found differences between Medicaid and private insurance groups related to knowledge. There were significant relationships between disease severity and CF specific self-efficacy and nutritional surveillance as well as general self-efficacy and respiratory surveillance.
These findings confirmed that the IFMST would provide a consistent framework to guide future studies aimed at identifying factors that influence self-management behaviors of CF in patients and their caregivers.
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Santos, José Manuel Mendes Pereira dos. "Intervenção da fisioterapia em crianças e jovens com fibrose quística: uma revisão sistemática." Bachelor's thesis, [s.n.], 2020. http://hdl.handle.net/10284/9183.
Full textAbstract:
Projeto de Graduação apresentado à Universidade Fernando Pessoa como parte dos requisitos para obtenção do grau de Licenciado em FisioterapiaObjetivo: Determinar a intervenção da fisioterapia em crianças e jovens com Fibrose Quística (FQ). Metodologia: Pesquisa computorizada nas bases de dados Scopus, Web of Science e Pubmed com o objetivo de obter artigos randomizados controlados que avaliassem os efeitos da intervenção da fisioterapia em crianças e jovens com FQ. A pesquisa foi realizada entre Julho e Agosto 2019. A qualidade metodológica foi avaliada através da Physiotherapy Evidence Database scoring scale (PEDro) Resultados: Foram incluídos 7 artigos com um total de 317 participantes com score total de 7 pontos de acordo com a PEDro. Dos estudos incluídos nesta revisão, todos avaliaram os efeitos do exercício terapêutico em crianças e jovens. Dois avaliaram os efeitos de um programa de exercícios num suporte digital comparando com um treino tradicional, 2 avaliaram a combinação de um programa de exercícios com um treino dos músculos inspiratórios, 1 avaliou os efeitos do exercício nas alterações posturais e barapométricas, 1 verificou os efeitos do treino aeróbio na função pulmonar e outro avaliou os efeitos da associação entre o treino aeróbio e o treino de força na função pulmonar. Conclusão: A fisioterapia tem um papel indispensável no tratamento de crianças e jovens com FQ.
Objetive: To determine a physiotherapy intervention in children and young cystic fibrosis (CF). Methodology: Computerized research was carried out in the Scopus, Web of Science and Pubmed databases with the objective of obtaining randomized articles controlled by assessing the effects of the physiotherapy intervention in children and young people with CF. Research was carried out between July and August 2019. Methodological quality was assessed using the Physiotherapy Evidence Database scoring scale (PEDro). Results: Were included 7 articles, with a total of 317 participants with a total score of 7 on the PEDro. The studies in this review, all of them evaluated the effects of therapeutic exercise on children and young. Two assessed the effects of an exercise program on a digital medium compared to a traditional workout, 2 the combination of an exercise program with inspiratory muscle training, 1 assessed the effects of exercise on postural and barapometric changes, 1 verified the effects of aerobic training on lung function and another evaluated the effects of the association between aerobic training and strength training have on lung function. Conclusion: Physiotherapy has an indispensable role in the treatment of CF in children and young people.
N/A
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O'Haver, Judith. "Parental Perceptions of Risk and Protective Factors Associated with the Adaptation of Siblings of Children with Cystic Fibrosis." Diss., The University of Arizona, 2007. http://hdl.handle.net/10150/194222.
Full textAbstract:
When a child is diagnosed with a chronic life threatening illness there is a significant impact on the entire family. Siblings are at risk for psychological adaptation problems because of their unique relationship with the ill child and the effect of that illness on family functioning. Few studies have been reported which examine the impact of chronic life-threatening illnesses in children on healthy siblings.The purpose of this study was to investigate the predisposing risk and protective factors that affect the psychological adaptation of healthy siblings of a child with Cystic Fibrosis (CF). A descriptive study was conducted using a convenience sample from two CF centers. The relationship between several variables was explored using non- parametric correlations.In this sample, significant negative correlations were found between parental stress and their reported financial well being and emotional or behavioral problems in the well siblings prior to the diagnosis of CF was made in the sick sibling and perceived parental support.For adolescent siblings, The Behavioral Symptoms Index (BSI) was correlated to reported stress in their parents and negatively correlated to the parental perceived support. The Emotional Symptoms Index (ESI) was correlated to the BSI. A significant negative correlation was also noted between age and Internalizing Behaviors. These relationships were not significant for the child siblings in this sample.There were no significant relationships among gender, maternal education, and caretaker for the well sibling when the child with CF was hospitalized and their Internalizing and Externalizing Behaviors. The health care teams in these clinics seldom discussed CF with the well sibling. However, for the children in this study, there was a significant negative correlation with this discussion and their Externalizing Behaviors.Findings from this study suggest that the family environment, especially parental stress and perceived social support, may affect the adaptation of the well sibling. Adolescent siblings were more at risk for this environmental influence than their younger counterparts.
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Urquhart, D. S. "Exploration of the relationship between hypoxia and measures of clinical status and inflammation in children with cystic fibrosis." Thesis, University College London (University of London), 2010. http://discovery.ucl.ac.uk/19232/.
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Hypoxia in cystic fibrosis (CF) may occur during sleep, and also during exercise, chest exacerbations and air travel. No standardised definition of nocturnal hypoxia in CF exists. Theoretical evidence suggests hypoxia may have a deleterious impact on clinical status in CF, due to effects on upregulation of pro-inflammatory cytokines, changes in Pseudomonas aeruginosa growth patterns, and causation of pulmonary hypertension. It was hypothesised that hypoxia, and resultant inflammation would adversely affect clinical phenotype in CF. Forty-one children with CF were studied, each undergoing home oximetry before attending for a day of clinical testing (exercise testing, lung function, respiratory and skeletal muscle testing, echocardiography, and quality of life assessment). In vitro work was undertaken to assess the effects of hypoxia on cell growth and interleukin-8 (IL-8) secretion in wild-type and CF airway epithelial cells. The effects of hypoxia were compared to a known proinflammatory stimulus - lipopolysaccharide (LPS) from Pseudomonas aeruginosa. ROC statistics were used to derive the most sensitive and specific definition of sleep hypoxia in the detection of elevated levels of inflammation (WBC, CRP, neutrophil counts and IL-8 levels). This definition (SpO2 <93% for>10% sleep) was used to dichotomise the study population. Hypoxic CF subjects (n=9) had, when compared to normoxic controls (n=32): lower exercise capacity, lower BMI, lower FEV1 and FVC, elevated RV/TLC ratio, and higher Chrispin-Norman scores. Hypoxic subjects also had reduced quality of life, bone density, and increased RV thickness on echocardiogram. Hypoxic cell culture was suggested to be pro-inflammatory, with increased IL-8 production, and synergistically increased IL-8 secretion when cells were co-incubated with LPS under hypoxic conditions. Hypoxia is associated with reduced clinical well-being and increased inflammation in childhood CF. The paradigm exists of whether hypoxia is merely an endpoint of severe CF lung disease; or whether hypoxia may be a causative factor (as suggested by the in vitro work), as well as an effect of CF lung inflammation. A trial of restoration of normoxia in children with CF, with careful re-evaluation of clinically-relevant outcomes is suggested from this preliminary work.
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Deeley, Linda G. "Children with siblings with cystic fibrosis : impact of the illness and strategies used to cope, from the child's perspective." Thesis, University of Southampton, 1996. http://ethos.bl.uk/OrderDetails.do?uin=uk.bl.ethos.243145.
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Santos, Camila Isabel da Silva. "Efeito da antibioticoterapia e da fisioterapia respiratoria na função pulmonar de pacientes com fibrose cistica em exacerbação pulmonar aguda." [s.n.], 2008. http://repositorio.unicamp.br/jspui/handle/REPOSIP/309972.
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Orientador: Jose Dirceu RibeiroTese (doutorado) - Universidade Estadual de Campinas, Faculdade de Ciencias Medicas
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Doutorado
Saude da Criança e do Adolescente
Doutor em Saude da Criança e do Adolescente
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Sheridan, Helen Sarah. "The practical use of the Multiple Breath Washout test in children : biological variability in health and disease." Thesis, University of Edinburgh, 2017. http://hdl.handle.net/1842/29543.
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The Multiple Breath Washout (MBW) test is increasingly being recognised as a sensitive method of detecting early small airways lung disease. Indices of MBW include lung clearance index (LCI), Scond and Sacin. Factors that affect MBW variability have not been fully established. This thesis presents five studies which examine MBW repeatability in children with and without cystic fibrosis (CF) or asthma. MBW was performed using 0.2% sulphur hexafluoride and the modified Innocor (Innovision). Testing was performed at the Clinical Research Facility of the Royal Hospital for Sick Children in Edinburgh. (1) MBW and spirometry were performed in children with and without CF (n=20 in each group), initially while sitting and then 30 minutes after assuming a supine posture. LCI was found to significantly rise on lying supine in healthy children (p < 0.01) and children with CF (p=0.03). (2) Thirty two children with CF performed MBW and spirometry on four study visits, results were correlated with findings from high resolution chest computed tomography scans taken on the first visit. LCI showed the strongest correlation with extent and severity of bronchiectasis (r=0.66, p < 0.01 and r=0.69, p < 0.01 respectively). Variability of LCI was similar to FEV1 over the 4 visits. (3) MBW and spirometry of 66 healthy children were compared to 63 children with stable asthma; lung function of asthmatic children was related to symptoms and medication use. LCI was higher in the asthmatic group (6.7 vs 6.3, p < 0.01); within the asthmatic group LCI was significantly higher if asthma was less well controlled (p=0.02). (4) Children with and without asthma (n=21 in each group) performed MBW and spirometry before and after exercise and again after salbutamol, symptom data was collected from asthmatic children. Baseline LCI was abnormal in the asthmatic group who had severe exercise induced bronchospasm during testing. (5) Asthmatic children admitted to hospital due to exacerbation performed MBW and spirometry. Mean (SD) LCI was abnormally high at 8.5 (1.7) in the nine patients recruited and returned to normal 6.7 (0.6) in three patients who attended follow up. I have presented evidence that LCI is repeatable and sensitive to early disease in CF and asthma. I have described for the first time the effects of exercise and exacerbation on MBW indices in asthmatic children. MBW is potentially a very useful tool in paediatrics; standardisation of testing and equipment may enable clinical use.
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Minasian, Caro. "A randomised, cross-over, comparative study of inhaled mannitol, daily rhDNase and a combination of both in children with cystic fibrosis." Thesis, Imperial College London, 2010. http://ethos.bl.uk/OrderDetails.do?uin=uk.bl.ethos.528305.
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