Thesis (Ph. D.)--University of Wisconsin--Madison, 1967. / Typescript. Vita. eContent provider-neutral record in process. Description based on print version record. Includes bibliographical references.

Within the Cystic Fibrosis (CF) literature it is acknowledged that parents play a significant role in supporting children with treatment procedures. Furthermore, a number of parenting variables have been associated with treatment adherence within the paediatric CF population. Interventions that target parenting practices may therefore have the potential to improve CF treatment adherence. Paper one presents a systematic literature review of parenting interventions targeting treatment adherence in children and adolescents with CF. The majority of studies focussed on dietary adherence and overall findings from these studies suggested that combined behavioural and nutritional counselling parenting interventions led to improvements in calorie intake and positive parenting practices. Interventions specifically targeting exercise adherence and interventions targeting multiple aspects of the CF treatment regimen were also shown to improve treatment adherence. The review highlighted that interventions targeting some of the more laborious treatments (i.e. chest physiotherapy) were lacking, as were interventions specifically tailored to the needs of adolescents and their parents. Over recent years, CF life expectancy has increased substantially due to medical advances. As a result, more children are living into adulthood, therefore needing to adhere to an increasingly complex treatment regime in order to manage increasing symptoms. Adolescence is a particularly challenging time for treatment adherence as children increase their independence and parents begin to allow the child to manage their own disease management. The study described in Paper 2 aimed to explore the acceptability and feasibility of the Self-Directed Teen Triple P parenting intervention within the adolescent CF population. It also explored whether parent-reported treatment adherence, positive parenting practices, parent wellbeing, and child emotional and behavioural functioning were increased as a result of this intervention. Whilst data from two cases indicated increasing trends in treatment adherence and positive parenting practices following the onset of the parenting intervention, uptake and retention to the intervention was poor. Interviews with parents and CF nurses indicated low acceptability and feasibility of the intervention in its current form and a number of adaptations were reported. The study concludes that researchers need to include parents within the design of tailored parenting interventions within this population in order to increase acceptability. Following this, larger scale studies are required to increase the reliability and rigor of research findings in this area. Paper 3 is a critical reflection and considers both Paper 1 and Paper 2. Within this paper the approaches used, the challenges encountered, and future research are considered.

Orientador: Carmen Silvia Bertuzzo / Tese (doutorado) - Universidade Estadual de Campinas, Faculdade de Ciencias Medicas / Made available in DSpace on 2018-08-13T02:33:23Z (GMT). No. of bitstreams: 1 Correia_CyntiaArivabenideAraujo_D.pdf: 1775265 bytes, checksum: 99ecfece81f00d7833282ae41bae5731 (MD5) Previous issue date: 2009 / Resumo: A Fibrose Cística (FC) possui uma grande variabilidade de expressão fenotípica, o que significa que crianças com o mesmo genótipo podem diferir quanto à sua apresentação. A proteína defeituosa formada é chamada CFTR (proteína reguladora da conductância iônica), causa transporte anormal de sódio e cloro através da membrana apical das células epiteliais das vias aéreas, pâncreas, intestino e aparelho reprodutor. Essa proteína é codificada por um único gene que recebe o mesmo nome da proteína, CFTR, e localiza-se no braço longo do cromossomo 7, região 7q3.1. Gêmeos monozigóticos apresentam maior concordância em relação à gravidade da doença pulmonar que os dizigóticos, sugerindo que a FC seja modulada por fatores genéticos secundários - genes modificadores - além do gene CFTR. A característica mais importante na FC é a sobrevida que é influenciada pela doença pulmonar. Portanto, genes que estejam envolvidos na imunidade, inflamação, reparação do epitélio e produção de muco são candidatos a genes modificadores da doença. Os objetivos foram: 1) determinar a prevalência dos polimorfismos -308G/A e -238G/A do gene TNF a entre portadores de FC e verificar existência de associação entre esses polimorfismos e a gravidade do quadro pulmonar, 2) identificar alterações de sequencia nos exons e junções exon/ intron dos genes ADIPOQ e STATH e verificar existência de associação entre possíveis variações nesses genes e a gravidade da FC. Foi realizada PCR seguida por digestão enzimática para o polimorfismo -308G/A do gene TNF a, reação em cadeia da polimerase ARMS para o polimorfismo -238G/A do gene TNF a, e para os genes ADIPOQ e STATH foi feita a triagem de mutações através de cromatografia líquida de alta resolução por desnaturação - DHPLC com posterior sequenciamento da região onde foi encontrada alteração. Foram analisados 49 pacientes com FC em seguimento no Ambulatório de Mucoviscidose do HC/UNICAMP, homozigotos para a mutação F508 ou heterozigotos compostos para mutações de classe I ou II ou homozigotos para mutações de classe II, que são alterações que não levam à formação de proteína funcional. Além disso, foram selecionados indivíduos que apresentem alteração de eletrólitos no suor. Para o polimorfismo -308G/A do gene TNFa os genótipos GG, AA e GA foram encontrados com as seguintes frequencias: 14,28, 67,35 e 18,36% respectivamente. Estes dados se opõem ao relatado na literatura. Tal diferença deve ocorrer pelas características populacionais da população brasileira. Para o polimorfismo -238G/A do gene TNFa, os genótipos GG e AG tiveram as seguintes frequencias: 79,59 e 20,41% respectivamente. O genótipo AA não foi encontrado na amostra analisada. A alta frequencia do genótipo GG comparado com o AA, concorda com a literatura. Não foi encontrada alteração na sequencia dos genes STATH e ADIPOQ. Não foi possível estabelecer uma associação entre a gravidade da FC e os genes TNFa, STATH e ADIPOQ, nas regiões analisadas. / Abstract: Cystic Fibrosis (CF) has a great variety expression, which means that the seriousness of the disease can vary a lot among people who have it. The defective protein, called CFTR (Cystic Fibrosis Transmenbrane Regulator), causes abnormal transportation of chloride and sodium through the apical membrane of the epithelial cells of the airway, liver, intestine and masculine reproductive tract. This protein is encoded by a single gene which has the same name, CFTR, and is located within the long arm of chromosome 7, region 7q3.1. CF is a disease which expressivity is much variable, with different degrees of damage and the age when the symptoms begins is also much variable, even within individuals of the same family, like twins. Because of it, it is been said that others genetic factors besides CFTR, can be modulating the clinical presentation. As the pulmonary state is the great responsible for the mortality of the disease genes that are involved in host defense, inflammation, epithelial repair, mucin production, and airway reponsiveness are of great interest. Base on this the objectives of this work were: determine the prevalence of the polymorphisms -308G/A e -238G/A from the TNF a gene and verify if there is an association between these polymorphisms pulmonary disease severity, and identify alterations on ADIPOQ and STATH genes and verify if there is an association between these polymorphisms and CF severity. PCR followed by restriction enzyme digestion was performed to detect the polymorphism -308G/A from the TNF a gene, ARMS PCR to the polymorphism -238G/A from the TNF a gene the DHPLC method associated to the sequencing to analyze ADIPOQ and STATH genes, were used. We performed analyses of 49 cystic fibrosis patients that are followed in a Cystic Fibrosis center from HC/UNICAMP, that are \F508 homozygous or compound heterozygous to mutations from class I or II, or that are homozygous to class II mutations, which are alterations that do not produce functional protein. Besides this, were selected individuals that have sweat test altered. To the polymorphism 308G/A from TNFa gene the genotypes GG, AA e AG were in the following frequencies: 14,28, 67,35 e 18,36%. This data is contradictory to the literature and may occur because of the racial admixture of the Brazilian population. To the polymorphism -238G/A from TNFa gene, the genotypes GG AG were in the following frequencies 79,59 e 20,41%. The genotype AA was not found in the analyzed group. The high frequency of the genotype GG is in agreement of the data. It was not possible to find any alteration on ADIPOQ and STATH genes. And also it was not possible to make any correlation between the severity of the CF disease and the genes TNFa, STATH and ADIPOQ between the analyzed regions. / Doutorado / Ciencias Biomedicas / Doutor em Ciências Médicas

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Thesis (Ph. D.)--Illinois State University, 1973. / Title from title page screen, viewed Oct. 14, 2004. Dissertation Committee: William L. Daniel (chair), Herman E. Brockman, David F. Weber, Arlan Richardson, Howard Hetzel. Includes bibliographical references (leaves 53-66) and abstract. Also available in print.

Class of 2015 Abstract / Objectives: The primary objective of the study was to evaluate for change in vitamin D levels and regimens in cystic fibrosis (CF) patients following implementation of the 2012 Cystic Fibrosis Foundation (CFF) vitamin D guidelines. Secondary endpoints included clinician adherence to guideline recommendations for treatment and management of vitamin D deficiency. Methods: This retrospective chart review included CF patients with 25-hydroxy vitamin D (25(OH)D) levels from University of Arizona Medical Center (UAMC) between April 1, 2011-March 31, 2012 and July 1, 2012-June 30, 2013. Total 25(OH)D levels and vitamin D regimens were collected along with data on respiratory cultures, pulmonary function, and hospitalizations. Data were analyzed by Student’s T-tests and chi square analyses. Results: A total of 62 patients were included in the study. Mean 25(OH)D levels did not significantly differ between the study periods (28.9±10.5 ng/mL pre-guideline and 27.0±9.1 ng/mL post-guideline, p=0.158). Cholecalciferol use increased post-guideline (57.1%) versus pre-guideline (75.8%, p=0.027). Post-guideline cholecalciferol doses increased to 2836.5±2669.4 international units [IU] daily compared to 1518.0±912.0 IU daily pre-guideline (p<0.001). Clinician adherence to dose titration recommendations resulted in significant 25(OH)D level elevations (28.3±8.9 ng/mL versus 24.7±9.0, p=0.047). Conclusions: The prescribing pattern of clinicians significantly changed to reflect vitamin D regimens suggested by CFF guidelines. This finding suggests that had sufficient time been allowed following guideline implementation, a significant difference in 25(OH)D levels would have resulted. Additional research is needed concerning the effect of the guidelines on vitamin D status, clinical outcomes, and comorbidities.

Following reports by Shapira et al. that α₂Macro-globulin (α₂M) is abnormal in cystic fibrosis (CF), the author set out to examine the properties of α₂M isolated from the plasma of children with CF and from the plasma of age/sex matched controls. To do so, a technique capable of isolating pure, physiologically "active" α₂M from small plasma samples had to be developed. By a two-step chromatographic technique, involving Cibacron Blue Sepharose chromatography and immuno-adsorption, the author was able to isolate "active" CF and control α₂M of at least 98 percent purity from 5 ml of plasma, regardless of plasma haptoglobin type. Having accomplished this, comparative studies of CF and control α₂M were undertaken. Four parameters were investigated: (1) the molar protease binding of α₂M (2) the interaction of α₂M -bovine cationic trypsin (BCT) complexes with the low molecular weight substrate BAEE, (3) the stability of formed α₂M-BCT complexes, and (4) the subunit structure of α₂M. Contrary to the reports of Shapira and his colleagues, this author found no differences between the subunit structure of CF and control α₂M nor between the abilities of CF and control α₂M to interact with BCT. Based upon these findings, the author believes that no firm evidence exists to implicate an α₂M defect in the pathogenesis of cystic fibrosis. / Medicine, Faculty of / Pathology and Laboratory Medicine, Department of / Graduate

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