Academic literature on the topic 'Cystic fibrosis Patients Victoria Nutrition'

Cystic fibrosis (CF) is a reversed autosomal genetic disease that originates from some white or caucasian races. This condition is caused by mutations in the CFTR gene, especially the CFTR Phe508del. If CFTR damage or error occurs then normal function will be disrupted and have a clinical impact on other organs or multiorgan. Complications such as the pancreas, liver, digestive tract, bone, genital, and respiratory tract that can cause most deaths in patients with cystic fibrosis. Malabsorption, inflammation, infection and lung obstruction are signs of cystic fibrosis. Therapy for cystic fibrosis is limited to the improvement of the airway mokus, recovery of infections maintained with antibiotics, improve physical health and nutrition of patients

AbstractOptimal nutrition support has been integral in the management of cystic fibrosis (CF) since the disease was initially described. Nutritional status has a clear relationship with disease outcomes, and malnutrition in CF is typically a result of chronic negative energy balance secondary to malabsorption. As the mechanisms underlying the pathology of CF and its implications on nutrient absorption and energy expenditure have been elucidated, nutrition support has become increasingly sophisticated. Comprehensive nutrition monitoring and treatment guidelines from professional and advocacy organizations have unified the approach to nutrition optimization around the world. Newborn screening allows for early nutrition intervention and improvement in short- and long-term growth and other clinical outcomes. The nutrition support goal in CF care includes achieving optimal nutritional status to support growth and pubertal development in children, maintenance of optimal nutritional status in adult life, and optimizing fat soluble vitamin and essential fatty acid status. The mainstay of this approach is a high calorie, high-fat diet, exceeding age, and sex energy intake recommendations for healthy individuals. For patients with exocrine pancreatic insufficiency, enzyme replacement therapy is required to improve fat and calorie absorption. Enzyme dosing varies by age and dietary fat intake. Multiple potential impediments to absorption, including decreased motility, altered gut luminal bile salt and microbiota composition, and enteric inflammation must be considered. Fat soluble vitamin supplementation is required in patients with pancreatic insufficiency. In this report, nutrition support across the age and disease spectrum is discussed, with a focus on the relationships among nutritional status, growth, and disease outcomes.

Approximately 10% of cystic fibrosis (CF) cases remain undiagnosed until age 18 or later and medical professionals are still unclear about the needs of these individuals. Adults with CF iii must either adapt to life with a chronic disease or transition into adulthood with CF. The purpose of this study was to address the impact of age-at-diagnosis on quality of life in a CF adult population. The psychological functioning of 21 adult CF patients (seven adult-diagnosed (AD) and 14 pediatric-diagnosed (PD)) was assessed using the Beck Depression Index (BDI)-II. A descriptive review of demographic and clinical data, which included an assessment of the financial impact of active outpatient medications, was also conducted among patients in both groups. The incidence of depression among participants was relatively low and appeared independent of age-at-diagnosis. Differences between AD and PD patients with CF in terms of demographic and clinical parameters were also minimal. (96 pages).

BACKGROUND: Cystic fibrosis (CF) patients grow poorly and tend to be malnourished. They frequently suffer from lung infections necessitating the repeated use of antibiotics. AIM: This study was conducted to determine whether supplementation with a probiotic Lactobacillus reuteri (L. reuteri) could reduce the incidence and duration of lung infections, and whether this would impact on their anthropometric data. The secondary purpose was to compare the nutritional status of the CF patients attending CF clinics in Kwazulu-Natal (KZN) with CF patients attending CF clinics in Cape Town (CT). METHODS: Twenty three CF patients 6-31 years of age from 2 CF clinics in Kwazulu-Natal started the study although only 16 patients completed it. The study was a randomized, double blind, placebo controlled crossover trial with six months on placebo and six months on probiotic. Weight, height, mid arm circumference (MAC), triceps skin fold thickness (TSF), forced expiratory volume in 1 second (FEV1) and forced vital capacity (FVC) were measured, sputum collected and a symptom diary completed over the 12 month period. Anthropometric data of CF patients attending CF clinics in CT was obtained from the publication by Westwood & Saitowitz (1999). RESULTS: Compliance with taking the L. reuteri was poor. Most took only 50% of the required daily dose. Probiotic supplementation showed a slight (non significant) trend to improve FEV1 and FVC, while no significant difference could be seen in the number and duration of the lung infections. Sputum analysis showed a non significant trend towards the probiotic reducing the number of bacteria in the sputum. There was a significant reduction of symptoms for fever, running nose, sore throat and ear ache while on placebo. There was a significant increase in weight gained off probiotic compared to the probiotic period. The changes in height, weight for age (WFA) percentiles, height for age (HFA) percentiles, WFA and HFA Z-scores, percentage expected weight for age and percentage expected height for age all showed no difference whether on or off probiotic. Over half the CF children in the KZN clinics were underweight for their actual height compared to one third in the CT clinics with a higher number of subjects below the 5th percentile for MAC and TSF readings compared to CT. CONCLUSION: Due to a small sample size and poor compliance no firm conclusions could be drawn. However a slight (non significant) improvement could be seen in favour of the probiotic for FEV1, FVC, and sputum analysis. Although all other findings were not significantly different it would be of benefit to carry out further investigation with improved compliance with the probiotic to see if the parameters set out above could be improved. The KZN and CT CF groups were comparable and the nutritional status of CF patients on KZN was well below that of the CT CF clinics and further monitoring would need to be carried out.

Introduction : La fibrose kystique (FK) est une maladie génétique qui atteint plusieurs organes dont le pancréas, le foie et les poumons. Elle s’exprime par une accumulation de mucus visqueux qui va entraîner une altération des fonctions de ces organes. Les progrès scientifiques ont permis d’améliorer la condition de vie et d’augmenter considérablement l’espérance de vie des patients atteints de FK. L’amélioration de l’espérance de vie des patients FK est associée à l’apparition d’anomalies de la tolérance au glucose précédant l’apparition du diabète associé à la FK (DAFK). Le DAFK présente des similitudes avec le diabète de type 1 [DT1] (faible poids, faible sécrétion d’insuline) et le diabète de type 2 [DT2] (intolérance au glucose, anomalies de la sensibilité à l’insuline), mais il est spécifique pour ses causes et ses conséquences. Le DAFK est associé à un risque accru de perte de poids, de réduction de la fonction pulmonaire et de mortalité précoce et touche 50 % des patients adultes. La principale cause de ce diabète est décrite par une sécrétion d'insuline réduite et retardée. Les facteurs de risque, menant au développement du DAFK et les conséquences de son apparition ne sont pas encore bien comprises. La diète (riche en lipides et en énergie) recommandée en FK visant à maintenir un poids adéquat pourrait être responsable de l’accumulation de graisse ectopique, de résistance à l’insuline, de stéatose hépatique et d’anomalies du bilan lipidique rapportés en FK. Pour les patients sans FK, ces anomalies sont associées au développement du DT2. Objectif : Le but de ce travail de thèse visait à l’identification de nouveaux facteurs de risque d’anomalies de la tolérance au glucose dans une population d’adultes atteints de FK. Méthode : Pour cela nous avons i) observé l’évolution de la sécrétion d’insuline chez les patients FK âgés ; ii) identifié l’association entre les enzymes hépatiques et la prévalence du DAFK ; iii) identifié la prévalence de dyslipidémie chez les patients FK adultes et l’association avec le risque de développement du DAFK. Résultats : Nos résultats ont montré que les patients FK adultes présentent une sécrétion d’insuline altérée, mais qu’elle ne se dégrade pas davantage sur une décennie. Par contre, sur la même période, les patients deviennent plus résistants à l’insuline. Nous avons mis en évidence l’existence d’une relation entre le niveau d’enzymes alanine aminotransférase (ALT) élevé et la prévalence de DAFK. Enfin, nous avons montré l’existence d’une forte prévalence de dyslipidémie en FK, mais ces anomalies ne sont pas associées à la survenue du DAFK. Conclusion : Ces travaux ont permis de mieux comprendre l’association entre différents facteurs de risque en lien avec les anomalies de la tolérance au glucose chez des patients adultes FK. Nous avons identifié un mécanisme et un possible biomarqueur du DAFK, les enzymes hépatiques ALT, chez les patients FK adultes. Ces données peuvent fournir un rationnel pertinent pour la poursuite d’autres études cliniques dans le but d’améliorer la qualité de vie des patients atteints de FK.
Introduction: Cystic fibrosis (CF) is a genetic disorder that affects several organs including the pancreas, liver, and lungs. It is expressed by an accumulation of viscous mucus which will cause an impairment of the functions of these organs. Scientific advances have improved the condition of life and significantly increased the life expectancy of patients with CF. This improved life expectancy of CF patients is associated with the onset of glucose tolerance abnormalities before the onset of CF-associated diabetes (CFRD). CFRD has similarities with type 1 diabetes [T1D] (low body weight, low insulin secretion) and type 2 diabetes [T2D] (glucose intolerance, abnormal insulin sensitivity), but it is specific for its causes and consequences. CFRD is associated with an increased risk of weight loss, reduced lung function and early mortality and affects 50% of adult patients. The main cause of this diabetes is described as a reduced and delayed insulin secretion. The risk factors leading to the development of CFRD and the consequences of its appearance are not well understood. The diet (rich in lipids and energy) recommended in CF, that aims at maintaining an adequate body weight, could be responsible for the accumulation of ectopic fat, insulin resistance, fatty liver and abnormalities of the lipid balance reported in FK. For patients without CF these anomalies are associated with the development of T2D. Objective: The aim of this thesis work was to identify new risk factors for abnormal glucose tolerance in a population of adults with CF. Method: For this we have i) observed the evolution of insulin secretion in elderly CF patients; ii) identified the association between liver enzymes and the prevalence of CFRD; iii) studied the prevalence of dyslipidemia in adult CF patients and the association with the risk of developing CFRD. Results: Our results have shown that adult CF patients have impaired insulin secretion, but it has not degraded further over a decade. However, over the same period, patients become more resistant to insulin. We have highlighted a relationship between the high alanine aminotransferase (ALT) enzyme level and the prevalence of CFRD. Finally, we have shown the existence of a high prevalence of dyslipidemia in CF but these anomalies are not associated with the occurrence of CFRD. Conclusion: This work has made possible to better understand the association between different risk factors linked to glucose tolerance abnormalities in adult CF patients. We have identified a mechanism and a possible biomarker, ALT hepatic enzyme, of CFRD in adult CF patients. These data may provide a relevant rationale for the pursuit of other clinical studies in order to improve the quality of life of patients with CF.

Cystic Fibrosis (CF) is a multi-system disorder, requiring not just respiratory expertise but also management of nutrition, diabetes, musculoskeletal and psychosocial issues. This online resource is a concise companion for all health care professionals who manage patients with CF, and it covers all aspects of care, including both paediatric and adult-specific issues, and summarizes up-to-date literature in a concise and focussed style. There is an emphasis on the practical aspects of management with separate chapters covering the effects of CF in the lung, the microbiology of pulmonary CF, and management of exacerbations. Psychosocial aspects of CF care, end of life care and lung transplantation are also covered, and potential future therapies reviewed. This second edition has been extensively updated to reflect the UK CF Trust Standards of Care, treatment guidelines and Cochrane reviews, and it also includes updates on emerging organisms, an expanded section on physiotherapy, and a new pharmacopeia that covers all common CF medications.

Cystic fibrosis lung disease is characterized by chronic infection, inflammation and a progressive loss of lung function. Patients are also affected by recurrent episodes of increased respiratory symptoms, called exacerbations which have a detrimental effect on quality of life, the rate of lung function decline, and mortality. Early diagnosis and treatment is vital. Diagnosis relies on a combination of symptoms, examination findings, the results of laboratory tests, and lung function. Antibiotics are the mainstay of treatment but airway clearance, nutrition, and glucose homeostasis must also be optimized. Mild exacerbations are usually treated with oral antibiotics and more severe exacerbations with intravenous antibiotics. The choice of antibiotic is guided by the patient’s chronic pulmonary infections, the in-vitro antibiotic sensitivities, known antibiotic allergies, and the previous response to treatment. In patients with chronic Pseudomonas aeruginosa infection, antibiotic monotherapy is thought to increase the risk of resistance and treatment with 2 antibiotics is therefore suggested (usually a β‎-lactam and an aminoglycoside). Although there is a lack of evidence on the duration of treatment, most patients receive around 14 days. This can be altered according to the time taken for symptoms and lung function to return to pre-exacerbation levels. If patients are carefully selected and receive appropriate monitoring, home intravenous antibiotics can be as effective as in-patient treatment. They are also associated with decreased disruption to patients / family life, decreased risk of cross infection and decreased costs.

The liver is affected in many systemic diseases, with important examples being cardiovascular diseases—raised venous pressure can lead to hepatic congestion. Hepatomegaly is frequent in moderately severe heart failure. Cardiac cirrhosis is a rare complication. Pulmonary diseases—conditions that involve the liver as well as the lungs include cystic fibrosis, sarcoidosis, and α‎₁-antitrypsin deficiency. Gastrointestinal diseases—inflammatory bowel disease is associated with a range of hepatic pathology including fatty change, pericholangitis, sclerosing cholangitis, autoimmune hepatitis, cirrhosis, and (rarely) amyloidosis. Hepatobiliary disease associated with total parenteral nutrition varies from a mild, asymptomatic disease to jaundice, cirrhosis, and liver failure. Coeliac disease may rarely present with abnormal liver tests. Obesity, especially in association with the metabolic syndrome, may be associated with nonalcoholic hepatitis and steatohepatitis. Endocrine diseases—autoimmune hepatitis and primary biliary cholangitis may be associated with autoimmune endocrine disorders. Both hypothyroidism and hyperthyroidism can cause abnormalities of liver function, which are usually mild. Haematological diseases—conditions associated with abnormal blood clotting, such as protein C or S deficiency and paroxysmal nocturnal haemoglobinuria, may lead to Budd–Chiari syndrome (hepatic vein thrombosis). The liver may be involved in both non-Hodgkin’s lymphoma and leukaemia. Infectious diseases—agents that particularly affect the liver (e.g. viral hepatitis) are discussed elsewhere although many systemic infections also infect the liver. Abnormal liver function may occur during many systemic infections, but it is rare for patients with sepsis to present primarily with liver symptoms, although jaundice, abnormal liver function tests, or (very rarely) fulminant hepatic failure may be the principal presenting feature. Rheumatological diseases—hepatic disease may either be a consequence of treatment or occur in association with other autoimmune diseases.