Introdução: A Agamaglobulinemia, a Imunodeficiência Comum Variável (IDCV) e a Síndrome Hiper IgM (SHIGM) são imunodeficiência primárias predominantemente humorais que se beneficiam da reposição de imunoglobulina humana, com redução da morbimortalidade. No entanto, apesar da reposição adequada de imunoglobulina, complicações pulmonares podem ocorrer, influenciando o prognóstico destes pacientes. Objetivo: O objetivo do estudo foi descrever as alterações morfológicas e funcionais pulmonares em pacientes com hipogamaglobulinemia primária em tratamento com reposição de imunoglobulina humana. Métodos: Foram avaliados 30 pacientes (agamaglobulinemia n=14; IDCV n=9; SHIGM n=7) que receberam imunoglobulina e antibioticoterapia profilática regularmente. A avaliação utilizou dados dos prontuários sobre o início e a evolução da doença, bem como dados de espirometria e tomografia computadorizada de tórax. O escore de Bhalla foi aplicado à tomografia mais recente de cada um dos pacientes para correlacionar as alterações tomográficas pulmonares com os dados clínicos, resultados das espirometrias e ocorrência de processos infecciosos sino-pulmonares após o início da reposição de imunoglobulina. Para as análises estatísticas utilizou-se o programa SPSS 13.0, e valores de p<0,05 foram considerados estatisticamente significantes. As variáveis nominais foram comparadas através do teste de Fisher e as contínuas, através de testes não paramétricos (Mann-Whitney, Kruskal- Wallis e Wilcoxon). Para as correlações do escore de Bhalla com as demais variáveis foi utilizado o coeficiente de Spearman. Resultados: Houve diminuição na frequência de pneumonias (p<0,001) e aumento na frequência de sinusites (p<0,001) após o início da reposição de imunoglobulina. Distúrbios ventilatórios foram evidenciados em 14 dos 23 pacientes que puderam realizaram espirometria (7 obstrutivos, 5 restritivos e 2 inconclusivos). Pacientes com bronquiectasias ao diagnóstico e aqueles à primeira avaliação tomográfica apresentaram mediana de idade mais elevada ao diagnóstico (p=0,015 e p=0,001, respectivamente) e tempo mais prolongado entre o início dos sintomas e o diagnóstico que aqueles sem bronquiectasias (p=0,010 e p=0,001, respectivamente). Sete pacientes desenvolveram bronquiectasias durante o tratamento. Pacientes com bronquiectasias à avaliação final apresentaram maior frequência de sinusites antes do início da reposição de imunoglobulina que aqueles sem bronquiectasias (p=0,010). Houve correlação estatisticamente significante do escore de Bhalla com VEF1 pré e pós-broncodilatador (r= -0,778 e r= -0,837, respectivamente), CVF (r= -0,773), FEF25-75% (r= -0,571) e com a frequência de pneumonias após o início do tratamento (r= 0,561). Conclusões: O tratamento com reposição regular de imunoglobulina e antibioticoterapia profilática reduziu a frequência e gravidade das infecções pulmonares, porém não evitou a ocorrência de sinusites, o aparecimento de bronquiectasias nem de outras alterações morfológicas e funcionais pulmonares / Introduction: Agammaglobulinemia, Common Variable Immunodeficiency (CVID) and Hyper IgM Syndrome (HIGM) are predominantly antibody deficiencies that benefit from immunoglobulin replacement therapy, with reduction of their morbidity and mortality. Despite regular immunoglobulin replacement, pulmonary complications may occur in those patients, affecting their prognosis. Objective: The aim of this study was to describe tomographic and functional pulmonary abnormalities in patients with primary hypogammaglobulinemia receiving immunoglobulin replacement therapy. Methods: Thirty patients (agammaglobulinemia n=14, CVID n=9, HIGM n=7) receiving antimicrobial prophylaxis and regular immunoglobulin infusions were evaluated. Clinical records were reviewed to obtain data concerning the onset and evolution of the disease and the results of spirometry and computed tomography of the chest. Bhalla score was applied to the most recent tomography of each patient to correlate tomographic pulmonary abnormalities with clinical data, spirometry results and the occurrence of sinusal and pulmonary infections after the onset of the immunoglobulin replacement. Statistical analysis was performed using the software SPSS 13.0 and p values < 0.05 were interpreted as statistically significant. Nominal variables were tested using Fisher´s exact test and continuous variables were tested using non-parametric tests (Mann-Whitney, Kruskal-Wallis e Wilcoxon). Spearman coefficient was used to correlate Bhalla score with the other variables. Results: The frequency of pneumonias decreased (p<0.001) and the frequency of sinusitis increased (p<0.001) after the onset of immunoglobulin replacement. Pulmonary function was abnormal in 14 of 23 patients (7 obstructive, 5 restrictive, 2 inconclusive). Patients with bronchiectasis at diagnosis and those with bronchiectasis at the first tomographic evaluation presented higher median age at diagnosis (p=0.015 and p=0.001, respectively) and longer duration between the onset of symptoms and diagnosis than those without bronchiectasis (p=0.010 e p=0.001, respectively). Seven patients developed bronchiectasis during treatment. Patients with bronchiectasis at the last tomographic evaluation presented a higher frequency of sinusitis before therapy onset than those without bronchiectasis (p=0.001). Statistically significant correlation was found between Bhalla score and pre and post bronchodilator FEV1 (r= -0.778 and r= -0.837, respectively), FVC (r= -0.773) and FEF25-75% (r= -0.571) and between Bhalla score and the frequency of pneumonias after the onset of immunoglobulin replacement therapy (r=0.561). Conclusions: Immunoglobulin replacement therapy and antimicrobial prophylaxis reduced the frequency and severity of pulmonary infections but did not prevent the occurrence of sinusitis, the development of bronchiectasis or other morphological and functional pulmonary abnormalities

<p>Bakgrund: Kronisk obstruktiv lungsjukdom (KOL) innebär svårigheter med andningsflödet genom luftvägarna. Sjukdomen utvecklas smygande och lungkapaciteten avtar gradvis. Sjuksköterskan bör se över patientens egenvårdsförmåga och därefter anpassa information/utbildning och stöd. Syfte: Att belysa omvårdnadsåtgärder som sjuksköterskan kan ta till hjälp för att främja egenvård hos patienter med KOL. Metod: 15 artiklar från PubMed och Cinahl analyserades med innehållsanalys och låg till grund för litteraturstudien. Även manuell sökning gjordes. Resultat: Två kategorier framträdde tydligt i resultatet; information/utbildning och stöd. Sjuksköterskans arbete med att informera/utbilda patienter samt att ge individuellt stöd kunde underlätta egenvårdsförmågan hos personer med KOL. Diskussion: För att uppnå egenvård fanns vissa centrala begrepp som sjuksköterskan kan följa; att få patienten delaktig, att situations-och individanpassa och att ha en helhetssyn. Konklusion: Mer kunskap och träning i att informera/utbilda samt att ge stöd till personer med KOL behövdes för att deras behov skulle tillgodoses.</p>

<p>Att drabbas av KOL är något som påverkar vardagen på många sätt. Sjukdomen har ökat i omfattning och visar inga tecken på att minska. Det innebär att vårdpersonal alltmer kommer att möta dessa personer. Behandlingen är oftast inriktad mot de fysiska behoven utan att ta hänsyn till helheten. Syftet var att belysa dessa personers upplevelser av att leva med KOL för att kunna möta deras behov och ge det stöd de själva vill ha. Litteraturstudie valdes som metod och systematiska sökningar gjordes i databaserna Cinahl, Pubmed och PsycINFO. Resultatet bygger på 14 artiklar och visade att andningsproblemen var den dominerade upplevelsen som påverkade vardagen negativt. Andningsproblemen gjorde att personerna med KOL upplevde att de tappade orken, upplevde beroende, men även så småningom en acceptans över att leva med den kroniska sjukdomen. Det visade sig hur dessa upplevelser påverkade varandra och kunde bli som en ond cirkel. Det är därför viktigt att i fortsatt forskning undersöka hur vårdpersonalen kan stödja dessa personers egenvårdsstrategier där peer-support är en viktig del.</p>

Background: Air pollution is associated with negative health effects. Exposure to combustion-derived particulate matter (PM) air pollution has been related to increased incidence of cardiovascular and respiratory morbidity and mortality, specifically in susceptible populations. Ambient particles, with a diameter of less than 2.5 mm, have been suggested to be the strongest contributor to these health effects. Diesel exhaust (DE) is a major source of small combustion-derived PM air pollution world wide.  In healthy volunteers, exposure to DE, has been associated with airway inflammation and impaired vasomotor function and endogenous fibrinolysis. The aims of this thesis were to further elucidate the underlying mechanisms to the reported cardiovascular effects following exposure to DE, with specific focus on endothelin-1 (ET-1). Additionally, the vascular effects of the major gaseous component of DE, nitrogen dioxide (NO2), were assessed together with the impact of an exhaust particle trap to reduce the observed negative vascular effects after DE exposure. Methods: In all studies healthy, non-smoking male volunteers were included and exposed for one hour during intermittent exercise in a randomised double-blind crossover fashion. In studies I-III, subjects were exposed to DE at a particulate matter concentration of approximately 300 μg/m3 and filtered air, on two different occasions. In study V an additional exposure was employed, during which DE was filtered through an exhaust particle trap. In study IV subjects were exposed to nitrogen dioxide (NO2) at 4 ppm or filtered air. In study I, thrombus formation and platelet activation were assessed using the Badimon ex vivo perfusion chamber and flow cytometry. Study II comprised the determination of arterial stiffness including pulse wave analysis and velocity. In studies III-V, vascular assessment was performed using venous occlusion plethysmography. In studies IV and V, the vascular responses to intra-arterially infused endothelial-dependent and endothelial-independent vasodilatators were registered. In study III, vascular responses to intra-arterial infusion of Endothelin-1 (ET-1) and ET-1-receptor antagonists were assessed. Venous occlusion phlethysmography was in all cases performed 4-6 hours following exposures. Blood samples for markers of inflammation, coagulation and platelet activation were collected before and throughout the study periods in studies III and V. Results: Exposure to DE increased ex vivo thrombus formation and arterial stiffness, in terms of augmentation index. DE inhalation impaired vasomotor function and endogenous fibrinolysis. The exhaust particle trap reduced the particle concentration by 98% and abolished the effects on vasomotor function, endogenous fibrinolysis and ex vivo thrombus formation. Plasma concentrations of ET-1 and its precursor big-ET-1 were unchanged following exposure. Dual endothelial receptor antagonism caused similar vasodilatation after both exposures, although vasodilatation to the endothelin-A receptor alone was blunted after DE exposure. ET-1 infusion induced vasoconstriction only following DE exposure. Exposure to nitrogen dioxide did not affect vascular function. Conclusion: Inhalation of diesel exhaust in young healthy men impaired important and complementary aspects of vascular function in humans; regulation of vascular tone and endogenous fibrinolysis as well as increased ex vivo thrombus formation. The use of an exhaust particle trap significantly reduced particle emissions and abolished the DE-induced vascular and prothrombotic effects. The adverse vascular effects following DE exposure do not appear to be directly mediated through the endothelin system. Neither is NO2 suggested to be a major arbiter of the DE-induced cardiovascular responses. Arterial stiffness is a non-invasive and easily accessible method and could thus be employed to address vascular function in larger field studies. Taken together, this thesis has given further knowledge about the mechanisms underlying the DE-induced vascular effects.

It is well established that air pollution has detrimental effects on both human health as well as the environment. Exposure to ozone and particulate matter pollution, is associated with an increase in cardiopulmonary mortality and morbidity. Asthmatics, elderly and children have been indicated as especially sensitive groups. With a global increase in use of vehicles and industry, ambient air pollution represents a crucial health concern as well as a political, economical and environmental dilemma. Both ozone (O3) and diesel exhaust (DE) trigger oxidative stress and inflammation in the airways, causing symptoms such as wheezing, coughing and reduced lung function. The aim of this thesis was to further examine which pro-inflammatory signaling pathways that are initiated in the airways by ozone, as compared to diesel exhaust. Furthermore, to study the effects of these two ambient air pollutants in a sequential exposure, thus mimicking an urban profile. In order to investigate this in healthy as well as asthmatic subjects, walk-in exposure chambers were utilized and various airway compartments were studied by obtaining induced sputum, endobronchial biopsies, or airway lavage fluids. In asthmatic subjects, exposure to 0.2 ppm of O3 induced an increase in the cytokines IL 5, GM-CSF and ENA-78 in the bronchial epithelium six hours post-exposure. The healthy subjects, however, displayed no elevations of bronchial epithelial cytokine expression in response to the ozone exposure. The heightened levels of neutrophil chemoattractants and Th2 cytokines in the asthmatic airway epithelium may contribute to symptom exacerbations following air pollution exposure. When examining an earlier time point post O3 exposure (1½ hours), healthy subjects exhibited a suppression of IL-8 as well as of the transcription factors NFκB and c-jun in the bronchial epithelium, as opposed to after filtered air exposure. This inhibition of early signal transduction in the bronchial epithelium after O3 differs from the response detected after exposure to DE. Since both O3 and DE are associated with generating airway neutrophilia as well as causing direct oxidative damage, it raises the query of whether daily exposure to these two air pollutants creates a synergistic or additive effect. Induced sputum attained from healthy subjects exposed in sequence to 0.2 ppm of O3 five hours following DE at a PM concentration of 300 µg/m3, demonstrated significantly increased neutrophils, and elevated MPO levels, as compared to the sequential DE and filtered air exposure. O3 and DE interactions were further investigated by analyses of bronchoalveolar lavage and bronchial wash. It was demonstrated that pre-exposure to DE, as compared to filtered air, enhances the O3-induced airway inflammation, in terms of an increase in neutrophil and macrophage numbers in BW and higher EPX expression in BAL. In conclusion, this thesis has aspired to expand the knowledge of O3-induced inflammatory pathways in humans, observing a divergence to the previously described DE initiated responses. Moreover, a potentially adverse airway inflammation augmentation has been revealed after exposure to a relevant ambient combination of these air pollutants. This provides a foundation towards an understanding of the cumulative airway effects when exposed to a combination of ambient air pollutants and may have implications regarding future regulations of exposure limits.

The overall aim of this work was to examine the effects of depleted nutritional status on some aspects of Chronic Obstructive Pulmonary Disease (COPD). Morbidity. In paper І, we found that energy intake was lower than the calculated energy demand for all patients. A low body mass index (BMI) at inclusion and weight loss, during the one year follow-up period were independent risk factors for having a new exacerbation (p = 0.003 and 0.006, respectively). Mortality. Nineteen percent of the patients in paper ІІ, where underweight (BMI&lt;20). A significant positive correlation was found between BMI and FEV1, and this correlation remained significant after adjustment for age, sex and pack years (p&lt;0.0001). Being underweight was related to increased overall mortality and respiratory mortality but not to mortality of other causes, 19% of the patients had died within 2 years. The lowest mortality was found among the overweight patients (BMI 25-30 kg/m). Physical capacity and effect of training. In paper ІІІ we investigated baseline characteristics of patients that were starting physical training. We found that peak working capacity was positively related to BMI (r=0.35, p=0.02) and fat free mass index (FFMI) (r=0.49, p=0.004) and negatively related to S-Fibrinogen and serum C reactive protein (S-CRP). BMI and FFMI were significantly related to the 12 minutes walking distance when adjusted for body weight. Fifty to 76% of the variation in physical capacity was accounted for when age, gender, FEV1, FFMI and CRP were combined in a multiple regression model. In Paper ІV the median change in fat free mass (FFM), after 4 months of physical training was 0.5 kg. Old age, low FEV1 and high level of dyspnoea were independent negative predictors of FFM increase after the training period. In conclusion nutritional status is an important determinant of morbidity, mortality and physical capacity in COPD. Low FEV1 and high level of dyspnea are negative predictors for increased FFM after physical training.

Bakgrund: Kronisk obstruktiv lungsjukdom (KOL) innebär svårigheter med andningsflödet genom luftvägarna. Sjukdomen utvecklas smygande och lungkapaciteten avtar gradvis. Sjuksköterskan bör se över patientens egenvårdsförmåga och därefter anpassa information/utbildning och stöd. Syfte: Att belysa omvårdnadsåtgärder som sjuksköterskan kan ta till hjälp för att främja egenvård hos patienter med KOL. Metod: 15 artiklar från PubMed och Cinahl analyserades med innehållsanalys och låg till grund för litteraturstudien. Även manuell sökning gjordes. Resultat: Två kategorier framträdde tydligt i resultatet; information/utbildning och stöd. Sjuksköterskans arbete med att informera/utbilda patienter samt att ge individuellt stöd kunde underlätta egenvårdsförmågan hos personer med KOL. Diskussion: För att uppnå egenvård fanns vissa centrala begrepp som sjuksköterskan kan följa; att få patienten delaktig, att situations-och individanpassa och att ha en helhetssyn. Konklusion: Mer kunskap och träning i att informera/utbilda samt att ge stöd till personer med KOL behövdes för att deras behov skulle tillgodoses.

Att drabbas av KOL är något som påverkar vardagen på många sätt. Sjukdomen har ökat i omfattning och visar inga tecken på att minska. Det innebär att vårdpersonal alltmer kommer att möta dessa personer. Behandlingen är oftast inriktad mot de fysiska behoven utan att ta hänsyn till helheten. Syftet var att belysa dessa personers upplevelser av att leva med KOL för att kunna möta deras behov och ge det stöd de själva vill ha. Litteraturstudie valdes som metod och systematiska sökningar gjordes i databaserna Cinahl, Pubmed och PsycINFO. Resultatet bygger på 14 artiklar och visade att andningsproblemen var den dominerade upplevelsen som påverkade vardagen negativt. Andningsproblemen gjorde att personerna med KOL upplevde att de tappade orken, upplevde beroende, men även så småningom en acceptans över att leva med den kroniska sjukdomen. Det visade sig hur dessa upplevelser påverkade varandra och kunde bli som en ond cirkel. Det är därför viktigt att i fortsatt forskning undersöka hur vårdpersonalen kan stödja dessa personers egenvårdsstrategier där peer-support är en viktig del.

Syftet med studien var att jämföra livskvalitet, kunskap om KOL och antal patienter som inlett ett rökstopp där en grupp erhöll standard vård och en annan grupp förutom standard vård även fick ett strukturerat omhändertagande på en distriktssköterskeledd KOL- mottagning med undervisning i egenvård.Studien genomfördes med kvasiexperimentell design. Urvalet var 52 patienter från primärvården med diagnosen KOL. Patienterna matchades utifrån kön, sjukdomens svårighetsgrad och slumpades sen till två grupper, en interventionsgrupp och en jämförandegrupp. Jämförandegruppen erhöll standardvård medan interventionsgruppen utöver standardvård erhöll två utbildningsbesök i egenvård hos astma KOL-sjuksköterskan på en vårdcentral i mellansverige.Som datainsamlingsmetod har två enkäter använts. En enkät som innehöll frågor om kön, ålder, civilstånd, utbildning, upplevd kunskap om KOL och rökstatus samt en enkät som hade till syfte att ge information om hur andningsbesvären påverkar patientens livskvalitet. Båda grupperna har svarat på enkäterna vid två tillfällen, vid det första besöket och vid det sista besöket efter tre månader. Interventionsgruppen har däremellan fått två utbildningsbesök.Resultaten visade statistiskt säkerställda skillnader mellan grupperna gällande livskvalitet, rökstopp och kunskaper om KOL. Interventionsgruppen hade fått minskade andningsbesvär, hade ökat sin fysiska aktivitet och fått en bättre psykosocial hälsa. Medan jämförande gruppen hade försämrats något i alla dessa avseenden. I interventionsgruppen hade sex av sexton rökande patienter slutat att röka, medan ingen hade slutat av de fjorton rökande patienterna i jämförandegruppen. Patienterna i interventions- gruppen hade också väsentligen större kunskaper om sin sjukdom jämfört med patienterna i jämförandegruppen.

Thesis (Ph. D.)--West Virginia University, 2002. / Title from document title page. Document formatted into pages; contains x, 98 p. : ill. (some col.). Vita. Includes abstract. Includes bibliographical references (p. 76-88).