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The metabolic cost of two forms of physiotherapy in cystic fibrosis /Williams, Marie T. Unknown Date (has links)
Thesis (PhD)--University of South Australia, 1997
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Survey of adult cystic fibrosis patients and parents of cystic fibrosis patients on nutrition educationDurham, Dixie Lea. January 2009 (has links)
Thesis (M.H.S.)--Boise State University, 2009. / Title from t.p. of PDF file (viewed Apr. 6, 2010). Includes bibliographical references (leaves 35-38).
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Survey of adult cystic fibrosis patients and parents of cystic fibrosis patients on nutrition education /Durham, Dixie Lea. January 2009 (has links)
Thesis (M.H.S.)--Boise State University, 2009. / Includes bibliographical references (leaves 35-38).
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Tenascin expression and distribution in pulmonary and pleural fibrotic disordersKaarteenaho-Wiik, R. (Riitta) 18 June 1999 (has links)
Abstract
Fibrotic pulmonary and pleural disorders represent a group of intrathoracic disorders with different etiologies and prognoses. A prominent part of both pulmonary and pleural fibrotic disorders remains etiologically unknown. An essential feature for all these disorders is an increase and disarray of many extracellular matrix proteins which take part in the remodeling of the fibrotic tissue. Further, the injury in pulmonary as well as in pleural fibrosis occurs often at the border between the epithelial or mesothelial and the mesenchymal cells breaking the epithelial basement membrane. Tenascin is an oligomeric matrix glycoprotein of the extracellular matrix. The best known isoforms are tenascin -C, -X, -R, -Y and -W. Tenascin-C is synthesized during embryonic development, expressed in a variety of tumors, being absent or scantily expressed in most adult tissues. The function of tenascin-C is still unclear. In lung, tenascin-C has been shown to be expressed in fetal lung during branching morphogenesis, benign and malignant lung tumors, idiopathic pulmonary fibrosis, sarcoidosis and asthma.
The aim of the present study was to study tenascin-C (later called tenascin) expression in various types of pulmonary fibrosis such as usual interstitial pneumonia (UIP), desquamative interstitial pneumonia (DIP), nonspecific interstitial pneumonia (NSIP), bronchiolitis obliterans organizing pneumonia (BOOP), sarcoidosis and extrinsic allergic alveolitis as well as in fibrotic and inflammatory disorders of the pleura of different etiologies. Further, the aims were to compare the accumulation of tenascin with the prognosis in UIP, to confirm the immunohistochemical findings in UIP by Western blotting and immunoelectron miscroscopic (immuno-EM) studies, to investigate which cells synthesize tenascin in UIP and in pleural fibrosis by mRNA in situ hybridization, and to determine whether epithelial lining fluid (ELF) and serum tenascin concentration are increased in patients with UIP, sarcoidosis and extrinsic allergic alveolitis.
Tenascin was shown to be increased by immunohistochemical studies in all types of pulmonary and pleural fibrotic disorders included in the study. In UIP, increased tenascin expression was associated with a shortened survival time of the patients. In immuno-EM, labeling for tenascin was seen within type II pneumocytes. UIP cases showed reactivity for a polypeptide of Mr = 200 000 by Western blotting. Myofibroblasts and type II pneumocytes were mainly shown to synthesize tenascin in UIP. Also in pleural fibrosis myofibroblasts, and in addition possibly mesothelial cells, were observed to be responsible for its synthesis. ELF and serum tenascin concentrations were increased in UIP, sarcoidosis and extrinsic allergic alveolitis.
In conclusion, tenascin expression is increased in pulmonary and pleural fibrotic disorders, especially in newly formed fibrosis. In UIP, tenascin is actively synthesized at the sites of recent epithelial injury, suggesting that it plays an important role in the fibrogenesis in the lung.
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The epidemiology of cystic fibrosis related diabetes (CFRD) in cystic fibrosis patients attending the adult cystic fibrosis clinic at the Charlotte Maxeke Johannesburg Academic HospitalRomain, Marc 27 January 2012 (has links)
M.Med. (Internal Medicine), Faculty of Health Sciences, University of the Witwatersrand, 2011. / Background
Cystic fibrosis (CF) is one of the most common fatal autosomal recessive inherited
conditions in the Caucasian population. Survival of patients with cystic fibrosis has
increased due to optimal medical therapy and as a consequence, later complications such
as cystic fibrosis related diabetes (CFRD) may develop.
Materials and methods
A retrospective patient file review was conducted on all the patient files in the Adult
Cystic Fibrosis Unit, Ward 496, Charlotte Maxeke Johannesburg Academic Hospital.
The aim of the review was to determine the prevalence of Cystic fibrosis related diabetes
(CFRD) and to determine the characteristics of patients with CFRD in terms of age,
gender, genotype, lung function, body mass index (BMI), HBA1c, use of corticosteroids
and pancreatic function.
Patients were classified as normal glucose tolerance, impaired glucose tolerance or CFRD
based on the results of oral glucose tolerance testing. For statistical analysis, patients
with impaired glucose tolerance and CFRD were analyzed together under the group
abnormal glucose homeostasis.
50 patient files were reviewed.
Results
12 patients (24%) had normal glucose tolerance, 10 (20%) had impaired glucose
tolerance, 23 (46%) had CFRD without fasting hyperglycaemia and 3 patients (6%) had
CFRD with fasting hyperglycaemia. 2 patients (4%) did not have OGTT done and
therefore could not be categorised. The prevalence of CFRD was 54 % (including all
patients with CFRD without fasting hyperglycaemia and patients with CFRD with fasting
hyperglycaemia). 75 % of patients had abnormal glucose homeostasis. Statistical
analysis failed to demonstrate any significant difference in the characteristics of patients
with and without CFRD. This may be related to the small sample size. HBA1c values
were higher in patients with abnormal glucose homeostasis compared to patients with
normal glucose tolerance.
Conclusions
The prevalence of CFRD in the adult cystic fibrosis population at Charlotte Maxeke
Johannesburg Academic Hospital correlates with the prevalence of CFRD in other cystic
fibrosis centres. There were no statistically significant differences in the characteristics
of patients with and without CFRD.
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Combating fibrosis in mdx mice with a novel antifibrosis drug - HalofuginoneHuebner, Kyla Danielle 26 April 2007 (has links)
The effects of the antifibrotic drug Halofuginone hydrobromide (Halo) on muscle function, regeneration and cardiorespiratory function were studied using mdx mice. It was hypothesized that Halo treatment would resolve pre-established fibrosis and prevent collagen deposits, improving muscle and cardio-respiratory function.
Mice 8-9 mos were treated with saline or Halo for 5 (n = 4/group), 10 (n = 5/group) and 12 weeks (n = 4-5/group). Muscle strength and endurance, respiration and muscle susceptibility to damage were assessed. Tissues were collected from all mice. Additional mice were treated for 10 wks (3-4 wks n = 9-10/group; 8-9 mos n = 8-9/group) for echocardiography.
Halo reduced fibrosis. As a consequence, there was muscle repair and damage was reduced. There were functional improvements and disease progression was slowed. There was resolution of pre-existing fibrosis and reduction of new collagen synthesis. This treatment could improve quality of life and lengthen the lifespan of DMD patients. / May 2007
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Transgenic complementation studies on cystic fibrosis mouse /Oceandy, Delvac. January 2001 (has links) (PDF)
Thesis (Ph. D.)--University of Queensland, 2002. / Includes bibliographical references.
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Psychosocial aspects for teenagers and young adults with cystic fibrosisTolbert, Christopher F. January 2003 (has links) (PDF)
Thesis--PlanB (M.S.)--University of Wisconsin--Stout, 2003. / Includes bibliographical references.
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Molecular Mechanisms of Myocardial Fibrosis DevelopmentRosin, Nicole 14 April 2014 (has links)
The prevention of heart failure in our aging population has become a healthcare
priority. As part of the normal aging process patients have been shown to develop age-related myocardial fibrosis, which is characterized by excess deposition and a lack of
clearance of extracellular matrix (ECM) proteins, contributing to the development of
heart failure. The increase in ECM proteins is caused by an imbalance between the
following: (1) collagen biosynthesis by fibroblasts, (2) post-synthetic collagen processing
(cross-linking) which stabilizes collagen, and (3) collagen degradation.
The focus of this thesis lays mainly on collagen biosynthesis and processing, and
how interrupting these can affect the overall balance of collagen in the myocardium. The
two main objectives of this thesis lay in 1 – characterizing the role of a key pro-fibrotic
signal in the TGFβ pathway, connective tissue growth factor (CTGF), in hypertension
induced myocardial fibrosis development, and 2 – investigating the role of lysyl oxidase
(LOX), an enzyme involved in the crosslinking of collagen that has been implicated in
age-related myocardial fibrosis development.
This thesis provides novel evidence that CTGF regulation is an important step in
the early stages of myocardial fibrosis development through regulating new collagen
synthesis and that CTGF is produced as a downstream mediator of TGFβ after AngII
exposure not directly through AngII-AT1R signaling. Additionally, novel evidence is
presented that LOX inhibition reduces age-related myocardial fibrosis and that the
decrease in collagen protein content observed after LOX inhibition is associated with a
significant decrease in new pro-collagen 1 mRNA synthesis. This suggests that LOX
inhibition also inhibits new collagen synthesis by an unknown mechanism. Overall, these
studies have contributed new mechanistic knowledge about myocardial fibrosis
development and explored potential therapeutic strategies aimed at interrupting collagen
homeostasis, which are key to understanding myocardial fibrosis development.
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Combating fibrosis in mdx mice with a novel antifibrosis drug - HalofuginoneHuebner, Kyla Danielle 26 April 2007 (has links)
The effects of the antifibrotic drug Halofuginone hydrobromide (Halo) on muscle function, regeneration and cardiorespiratory function were studied using mdx mice. It was hypothesized that Halo treatment would resolve pre-established fibrosis and prevent collagen deposits, improving muscle and cardio-respiratory function.
Mice 8-9 mos were treated with saline or Halo for 5 (n = 4/group), 10 (n = 5/group) and 12 weeks (n = 4-5/group). Muscle strength and endurance, respiration and muscle susceptibility to damage were assessed. Tissues were collected from all mice. Additional mice were treated for 10 wks (3-4 wks n = 9-10/group; 8-9 mos n = 8-9/group) for echocardiography.
Halo reduced fibrosis. As a consequence, there was muscle repair and damage was reduced. There were functional improvements and disease progression was slowed. There was resolution of pre-existing fibrosis and reduction of new collagen synthesis. This treatment could improve quality of life and lengthen the lifespan of DMD patients.
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