Navegando por Assunto "Fibrose cística"

Agora exibindo 1 - 5 de 5

Acesso aberto (Open Access)
Aspectos clínicos e epidemiológicos da infecção por pseudomonas aeruginosa em pacientes portadores de fibrose cística no Estado do Pará
(Universidade Federal do Pará, 2003-12-23) MARTINS, Valéria de Carvalho; VIEIRA, José Luiz Fernandes; http://lattes.cnpq.br/2739079559531098
Pseudomonas aeruginosa is a gram-negative motile rod; they are important pathogens in neutropenic patients, burn and artificially ventilated patients in Intensive Care Units, where they may cause nosocomial infections. At this condition, the infection seems to be serious and sometimes lethal. However, in Cystic Fibrosis patients the course of infection by P. aeruginosa, has become severe chronic lung infection, because this bacteria produce many toxins and others virulence factors with potential effect on the lungs of this patients that are thought to play a role during establishment of the initial persistent colonization of the Cystic Fibrosis respiratory tract. The most characteristic feature of persistent P. aeruginosa infection is the production of mucoid alginate and the formation of the micro colonies in the lungs of the patients that is the survival strategy of environrnent bacteria. P. aeruginosa growing in alginate biofilms is highly resistant to antibiotics and is usually associated with progressive loss of pulmonary function. This research wants to study Cystic Fibrosis patients chronically colonized by P. aeruginosa who has been taken care on University Hospital João de Barros Barreto, Belém (Pará), in 2003 year, at about epidemiological and clinicalaspects. The sputum of these patients, have been collected by expectoration and swab of throat, to microbiological study realized on microbiology laboratory of this hospital. Thirty two patients with Cystic Fibrosis have been evaluated and divided according microbiological aspect of their sputum in three groups: patients who have never been infected by P. aeruginosa (Gl), patients that have been infected by this bacterial but not colonized (G2), patients chronically colonized by this microorganism. Patients who concern to G3 group have been more serious respiratory complications than patients that belong to the others groups. The pattern mucoid of P. aeruginosa has been significant on patients' sputum of G3. The incident of non- mucoid strains in most patients of groups G2 and G3 seems to be similar. Respiratory symptoms have been important during diagnosis of Cystic Fibrosis. The median age for fibrosis cystic diagnosis was about 7,69 years. Certainly several events contribute to support P. aeruginosa respiratory colonization on Cystic Fibrosis patients of G3, like: low social and economic conditions, lateness diagnosis, malnutrition and pattern Cystic Fibrosis gene mutation.
Acesso aberto (Open Access)
Avaliação da adesão ao tratamento com alfadornase em pacientes com fibrose cística
(Universidade Federal do Pará, 2017) FEITOSA, Keith Brabo Tavares; MARTINS, Valéria de Carvalho; http://lattes.cnpq.br/1904927472781784; ANDRADE, Marcieni Ataíde de; http://lattes.cnpq.br/8514584872100128
Cystic Fibrosis is an inherited disease, clinically manifested by digestive and respiratory symptoms. The early diagnosis offers the possibility of a better therapeutic control of the disease’s symptoms in favor of the prognosis and contributing to increasing quality of life. The aim of this study was to demonstrate the patient's behavior towards the disease and to evaluate the adherence to the treatment with the alfadornase enzyme. A cross-sectional study was carried out using epidemiological data collected through direct interviews with patients and/or their caregivers and by medical records analysis. Morisky-Green and Batalla Martinez tests were employed in order to evaluate the patient’s adhesion and knowledge of the disease, respectively. In addition, the clinical profile was assayed by Shwachman-Kulczyki score. Fifty-one (51) patients with cystic fibrosis in treatment with alfadornase were recruited at the outpatient clinic of the Hospital Universitário João de Barros Barreto. At final, forty-seven (47) patients participated in the study. Most of the patients (53.2%) were under 18 years of age, male (56.6%) and were from countryside (40.4%). Patients with long term use of alfadornase (> 5 years) show possess greater adherence than the others with a shorter treatment period. The male sex was associated with higher adherence, as well as greater education level and family income. The Batalla Martinez test demonstrates that only 40.42% of patients had some knowledgement about their disease. Thus, the factors associated with low adherence were: use of more than five medications, low education and gender. On the other way, these results shows that adherence to the medication was associated with the best clinical score.
Acesso aberto (Open Access)
Função pulmonar dos pacientes com fibrose cística colonizados por Pseudomonas aeruginosa no Pará
(Universidade Federal do Pará, 2008) FALCÃO, Edilene do Socorro Nascimento; CARNEIRO, Irna Carla do Rosário Souza; http://lattes.cnpq.br/4389330944043163; SOUSA, Rita Catarina Medeiros; http://lattes.cnpq.br/3560941703812539
Pseudomonas aeruginosa is an opportunistic pathogen in cystic fibrosis becoming the most frequent cause of lung infection; it is responsible for high mortality rates in patients who present this disease. The goal of this study is to showing spirometric and clinic characteristics of patients who present cystic fibrosis and who are attended at João de Barros Barreto University Hospital (HUJBB), in the State of Pará. A retrospective study was performed with 44 patients´ notes record who were attended at HUJBB during the 1997 to 2007 time frame; these patients fit into inclusion criteria, in which 14 presented P. aeruginosa bacteria colony, nine patients died. The significance rate presented was of 5%. Within the group who was colonized by P. aeruginosa, ten patients were women and the median age for their initial symptoms was of 0.3 ± 0.6 year which was significantly different when compared with patients who didn´t present the bacteria colony (p<0.05). The median age for diagnosis was of 13.1 ± 10.8 in colonized patients and all of them presented respiratory symptoms pertaining to the diagnosis. The median of the predicted percentage values of spirometries for initial and final evaluation of the colonized group was VEF1 (60.0 ± 25.0%) and (47,82 ± 16.1%) and FEF25-75% (42.5 ± 22.9%) and (26.5 ± 17.9%), and in the non-colonized group, the median was VEF1 (79.2 ± 21.0%) and (79,6 ± 18.0%) and FEF25-75% (69.2 ± 26.7%) and (68.9 ± 26.8%), respectively (p<0.005). The median initial Shwachman score in the colonized patients was 42.9 ± 13.5 and in the non-colonized patients it was 68.4 ± 15.1 (p<0.0001), and at the final evaluation the median was 36.6 ± 18.7 and 73.6 ± 12.3 (p<0.0001), respectively, showing important difference between the two groups. Factors related to deaths found in the study were related to P. aeruginosa colonization, inadequate nutritional status and reduced VEF1. In the studied casuistry, a larger damage to respiratory function and older median age for diagnosis were found in the State of Pará when compared to other Brazilian States. These data reinforce the need for action towards precocious diagnosis in the State of Pará so that an efficient and guided therapeutic approach is put into practice guiding to survival increase and quality of life improvement for these individuals, which is the main goal of professionals involved in the assistance practice area.
Acesso aberto (Open Access)
O luto pela perda da saúde: vivências de ser mãe de uma criança com Fibrose Cística
(Universidade Federal do Pará, 2012-12-14) ALMEIDA, Nancy Limeira de; SOUZA, Airle Miranda de; http://lattes.cnpq.br/5311796283730540
The objective of this study is to understand the way mothers of children suffering from Cystic Fibrosis cope with the loss of the health of their child, considering that any evolution of the disease poses a death threat to the life of the child, when the absence of treatment adherence. Cystic Fibrosis is an incurable and lethal chronic genetic disease, with guarded prognosis, requiring high impact treatment and intensive care. The methodology is based on a qualitative clinical approach, with a particular focus on content analysis. Eleven mothers of children diagnosed with Cystic Fibrosis and benefiting from the "Cystic Fibrosis Support Programme" of João de Barros Barreto University Hospital took part in this study. Data were collected through a semi-structured interview with the mother and by realizing two drawings aimed at understanding the mourning of these mothers in relation to the disease of their child, the losses and the significance associated with the illness of the health of the child. The results indicate that the mothers experience the mourning for the health loss of their child by redefining the concepts associated with death and end of life. This is confirmed by the hypothesis that the mother of a child suffering from cystic fibrosis knows the disease, is fully aware of the lethal risks associated and considers that the symptomatic treatment undergone can improve the quality of life of her child. For them, the proximity of the existence of an imminent death leads to a reorganization as well as to internal, external, personal and family changes contributing to redefining the meaning of their life, since the moment when they face the disease.
Acesso aberto (Open Access)
Prevalence of ∆F508, G551D, G542X, and R553X mutations among cystic fibrosis patients in the North of Brazil
(2005-01) ARAÚJO, Flávia Gonçalves de; NOVAES, Fabrício Chaves; SANTOS, Ney Pereira Carneiro dos; MARTINS, Valéria de Carvalho; SOUZA, Sheila Maria Ferraz Mendonça de; SANTOS, Sidney Emanuel Batista dos; SANTOS, Ândrea Kely Campos Ribeiro dos
Cystic fibrosis (CF) is the most common genetic disease among Caucasians and is rare among sub-Saharan Africans. The Brazilian population is not ethnically homogeneous but it is the result of three-way ethnic admixture of Europeans, Africans and Amerindians in varying proportions, depending on the region. In the present study, we investigated 33 patients who had been diagnosed and are currently under treatment for CF at the University Hospital João de Barros Barreto, Belém, Pará State. The molecular analysis for G542X, G551D and R553X mutations was performed by PCR followed by RFLP using BstNI, HincII and MboI, respectively, in polyacrylamide gel eletrophoresis and stained with AgNO3. The DF508 mutation (a deletion of 3 bp) was only analyzed by polyacrylamide gel electrophoresis and stained with AgNO3. Each sample was analyzed for regions of interest in the CFTR gene using amplified by PCR and specific primers. The DF508 and G551D mutations presented frequencies of 22.7 and 3%, respectively. In 74.3% of the remaining patients, none of the mutations investigated was found. The present study characterized in a sample of patients with an established clinical diagnosis of CF (asthma, repeated bronchopneumonia, disorders of nutritional status, etc.) the most frequent mutation ( DF508) in the North region of Brazil and is also the first report of the G551D mutation. In spite of the wide spectrum of CF mutations and the heterogeneous ethnic origin of the Amazon population, the molecular diagnosis is a helpful additional tool for the diagnosis and treatment of CF patients.