This study was designed to characterize the cardiac output (Q$ sb{ rm c}$) response to progressive submaximal upright (U) exercise in CF patients. Secondly, the Q$ sb{ rm c}$ adjustments were compared to those of similar supine (S) exercise, in an attempt to assess myocardial accommodation to the enhanced ventricular preload in the S posture. Q$ sb{ rm c}$ generally increased with exercise intensity in both U and S positions, although gr.IV plateaued at 50% VO$ sb2$max (S). Maximal stroke volume index (SI) was achieved at 50% VO$ sb2$max (U) in all groups, except gr.IV and at 30% VO$ sb2$max (S) in all groups. The change from U to S posture resulted in a significant (p $ le$.05) increase in SI at rest and for every submaximal exercise in gr.I, but not in CF patients, independent of disease severity eg(Rest:gr.I:27 $ pm$ 7(U) vs 39 $ pm$ 8(S); gr.II:24 $ pm$ 5vs28 $ pm$ 10; gr.III:18 $ pm$ 4 vs 22 $ pm$ 5; gr.IV:17 $ pm$ 4 vs 20 $ pm$ 6 ml/bt/m$ sp2$). These observations suggest a limitation in ventricular volume accommodation in CF patients which becomes apparent under the S exercise conditions.

Thesis (M.D.)--University of Adelaide, 1987. / Includes bibliographical references (leaves x-xxii).

Thesis (M.S.) in Microbiology--University of Maine, 2008. / Includes vita. Includes bibliographical references (leaves 52-58).

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.

No description available.

Epidural fibrosis following spinal surgery is common, and subsequent reoperations are more technically challenging with higher complication rates. A safe and effective therapeutic solution to this difficult clinical problem has yet to be realized. Previous research has demonstrated the effectiveness of alanyl-glutamine in reduction of peritoneal adhesions in a rat abdominal sepsis model. I hypothesized that alanyl-glutamine may be similarly efficacious in minimizing epidural fibrosis in a rat laminectomy model. Rats were randomized into three groups: no surgery, laminectomy/normal saline and laminectomy/alanyl-glutamine (1g/kg). The surgical groups underwent a lumbar laminectomy with instillation of either normal saline or alanyl-glutamine into the peridural space. Thirty days after surgery, the rats were euthanized and the spinal columns prepared for histological evaluation. A blinded veterinary pathologist and a less experienced student independently graded the extent and maturity of epidural fibrosis. The laminectomy model was an effective model for epidural fibrosis formation. Rats that underwent laminectomy demonstrated significant fibrosis compared to control animals (p<0.001). However, there was no significant difference in histological grade of fibrosis between normal saline and alanyl-glutamine treatment groups (p=0.83). Based on this study, alanyl-glutamine does not appear to have an effect in reducing epidural fibrosis at a histological level. It is possible that alanyl-glutamine may have an effect that is not detectable using this model, in which case further studies with a more sensitive model may be indicated. Resources may be better used elucidating the mechanism by which glutamine acts to reduce adhesions in the peritoneal model and further studies exploiting those mechanisms can be designed.

This thesis reviews the current literature on idiopathic pulmonary fibrosis (IPF), a progressive, scarring lung condition largely affecting older adults that is experiencing an increasing incidence in the U.S. and abroad. Two troubling clinical aspects of IPF are the difficulty of timely diagnosis and uncertain progression once diagnosed. The need for early detection is driven by the condition’s median survival rate post-diagnosis of about 3 years. Environmental and familial risk factors are important predictors of IPF, but cannot alone determine who is at risk for the condition. High-resolution computed tomography is currently the best non-invasive diagnostic tool, but many efforts are now underway to identify biological markers, which may aid not only in diagnosis, but illuminate both susceptibility and progression of the disease. Although the pathogenesis of IPF remains unclear, a compelling correlation has surfaced between the mechanics of IPF and herpes virus infection, which also may lead to a biological marker for the condition. Likewise, some genetic factors have shown promise in revealing pathogenesis and possible diagnosis. The only treatment currently available to ameliorate IPF is lung transplantation, but it is a last resort effort. In terms of pharmaceutical treatment, the most significant development has been the recent approval and use of two anti-fibrotic drugs, pirfenidone and nintedanib, that appear to slow the progression of the disease, but do not eliminate the fibrotic condition that impairs patients’ breathing. As efforts progress in addressing affirmative treatments for IPF, there is consensus that not enough is being done to address palliative and psychological needs of IPF. In sum, a review of the current literature suggests tremendous accomplishments have made in treating what remains a fatal condition, but much work remains to truly understand how and why IPF occurs, and whether, short of lung transplantation, there are treatments that can improve, not just maintain, patients’ health.

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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