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1.
Article in Portuguese | LILACS | ID: biblio-1368463

ABSTRACT

RESUMO: Objetivo: avaliar o comportamento de parâmetros do sistema respiratório durante internação para antibioticoterapia intravenosa (AIV) como tratamento da exacerbação pulmonar aguda (EPA) em escolares com fibrose cística (FC). Métodos: estudo do tipo analítico observacional, before-after, realizado no Hospital Infantil Joana de Gusmão, Florianópolis ­ Santa Catarina. Foram incluídas crianças com diagnóstico de FC, entre seis e 15 anos, em internação para tratamento da exacerbação pulmonar aguda, no início (T1), durante (T2) e ao final (T3) da internação foi conduzida avaliação de escores específicos de EPA, dados antropométricos e realizada avaliação dos parâmetros do sistema respiratório pelo sistema de oscilometria de impulso (IOS) e espirometria. Foram obtidos, em prontuário, dados de colonização bacteriana, genótipo, gravidade da doença (Escore de Schwachman-Doershuk-ESD) e espirometria mais recente em estabilidade clínica. Aplicou-se o teste Shapiro-Wilk para análise da distribuição dos dados e os testes ANOVA de medidas repetidas, teste de Friedman, teste T pareado e Wilcoxon, com nível de significância de 5%. Resultados: participaram 16 crianças/adolescentes (68.8% meninas, 12.88±1.67anos). Houve aumento dos parâmetros da espirometria e dados antropométricos (p<0.005) no T3, bem como redução dos escores de EPA e do X5 (p<0.005) no T3. Conclusão: os dados apresentados nesse trabalho mostram melhora dos escores de EPA, dados antropométricos, parâmetros da espirometria e do parâmetro de recolhimento elástico do IOS (X5).RESUMODescritores: Fibrose cística, Exacerbação dos sintomas, Testes de função pulmonar, Mecânica respiratória. (AU)


ABSTRACT: Objective: Evaluate the respiratory system parameters of children with cystic fibrosis (CF) during hospitalization for acute pulmonary exacerbation (APE) treatment. Methods: observational study before-after that occurred at the CF reference center. There were included children with cystic fibrosis (CF) between six to 15 years old hospitalized due to APE. The registration of the APE clinical scores, anthropometric data, and respiratory system (IOS and spirometry) evaluation occurred at the beginning (T1), during (T2), and at the end (T3) of the hospitalization. There were registered pathogens, genetic mutation, disease severity (Schwachman-Doershuk Score), and the most recent spirometry when they were clinically stable. The Shapiro-Wilk test was applied to analyze data distribution, and the repeated measure ANOVA, Friedman test, Tpaired test, and Wilcoxon test were performed to compare data, with a significance level set at 5%. Results: sixteen children/adolescents participated in the study (68.8% girls, 12.88±1.67 years old). The spirometric parameters, X5 parameter, and anthropometric data increased (p<0.005) and the APE scores decreased (p<0.005) at T3. Conclusion: APE scores, anthropometric data, spirometric parameters, and IOS elastic recoil parameter (X5) improved at the end of hospitalization.ABSTRACTKeywords: Cystic fibrosis, Symptom flare up, Respiratory function tests, Respiratory mechanics.1. Universidade do Estado de Santa Catarina ­ UDESC ­ Florianópolis, (SC) ­ Brasil https://doi.org/10.11606/issn.2176-7262.rmrp.2022.183755Tayná Castilho1, Renata Maba Gonçalves Wamosy1, Camila Isabel Santos Schivinski1Este é um artigo publicado em acesso aberto (Open Access) sob a licença Creative Commons Attribution, que permite uso, distribuição e reprodução em qualquer meio, sem restrições, desde que o trabalho original seja corretamente citado. (AU)


Subject(s)
Humans , Male , Female , Child , Adolescent , Oscillometry , Respiratory Function Tests , Spirometry , Respiratory Mechanics , Cystic Fibrosis/therapy , Symptom Flare Up
2.
Einstein (Säo Paulo) ; 20: eRW5686, 2022. tab
Article in English | LILACS | ID: biblio-1364796

ABSTRACT

ABSTRACT Objective To develop a scientific consensus on nutrition in cystic fibrosis. Methods Sixteen coordinators elaborated relevant questions on nutritional therapy in cystic fibrosis, which were divided into six sections: nutritional assessment, nutritional recommendations, nutritional intervention, dietary counseling, special situations and enzyme replacement, and gastrointestinal manifestations. Two to three specialists in the field were responsible for each section and obtaining answers formulated based on standardized bibliographic searches. The available literature was searched in the PubMed®/MEDLINE database, after training and standardization of search strategies, to write the best level of evidence for the questions elaborated. Issues related to disagreement were discussed until a consensus was reached among specialists, based on the current scientific literature. Results Forty-two questions were prepared and objectively answered, resulting in a consensus of nutritional therapy in cystic fibrosis. Conclusion This work enabled establishing a scientific consensus for nutritional treatment of cystic fibrosis patients.


Subject(s)
Humans , Cystic Fibrosis/complications , Cystic Fibrosis/therapy , Brazil , Nutrition Assessment , Nutritional Status , Nutritional Support
3.
Arch. argent. pediatr ; 119(1): S17-S35, feb. 2021. tab, ilus
Article in Spanish | LILACS, BINACIS | ID: biblio-1147358

ABSTRACT

La presente revisión de la "Guía de diagnóstico y tratamiento de pacientes con fibrosis quística"publicada en el año 2014 tiene como objetivo actualizar el conocimiento acerca de varios aspectos relacionados con el manejo clínico de la enfermedad, según los avances científicos publicados y en desarrollo en los últimos años. Solo se tratarán aquí tópicos nuevos y aquellos que, a la luz de la investigación, requieren ser modificados, por lo que el contenido de la anterior guía permanece vigente en el resto de las cuestiones.Los aspectos actualizados se dan en el diagnóstico de la enfermedad por los cambios realizados en el test del sudor, la pesquisa neonatal y la biología molecular, la actualización de estudios de seguimiento, como el índice de aclaramiento pulmonar y la resonancia magnética nuclear, y modificaciones con respecto al área nutricional (diabetes secundaria a fibrosis quística) y el tratamiento kinésico y de rehabilitación pulmonar


The present revision of the Guide for the diagnosis and treatment of patients with cystic fibrosis published in 2014 aims to update the knowledge about various aspects related to the clinical management of the disease, according to the scientific advances published and in development in recent years. Only new topics will be discussed here and those that, in light of the research, require modification, so the content of the previous guide remains valid in the rest of the aspects.The updated aspects range over the diagnosis of the disease due to the changes made in the sweat test, the neonatal screening and molecular biology, the update of follow-up studies, such as the Lung Clearance Index and the Nuclear Magnetic Resonance, and modifications regarding the nutritional area (diabetes secondary to cystic fibrosis) and the physiotherapy treatment and pulmonary rehabilitation.


Subject(s)
Humans , Child , Cystic Fibrosis/diagnosis , Cystic Fibrosis/therapy , Respiratory Tract Infections/therapy
4.
J. bras. pneumol ; 47(4): e20200557, 2021. tab, graf
Article in English | LILACS | ID: biblio-1286951

ABSTRACT

ABSTRACT Objective: Massive hemoptysis is one of the most serious complications in patients with cystic fibrosis (CF). This study aimed to evaluate the hemoptysis-free period following bronchial and non-bronchial artery embolization (BAE/non-BAE) in CF patients and to investigate predictors of recurrent bleeding and mortality by any cause. Methods: This was a retrospective cohort study of CF patients ≥ 16 years of age undergoing BAE/non-BAE for hemoptysis between 2000 and 2017. Results: We analyzed 39 hemoptysis episodes treated with BAE/non-BAE in 17 CF patients. Hemoptysis recurrence rate was 56.4%. Of the sample as a whole, 3 (17.6%) were hemoptysis-free during the study period, 2 (11.8%) underwent lung transplantation, and 3 (17.6%) died. The median hemoptysis-free period was 17 months. The median hemoptysis-free period was longer in patients with chronic infection with Pseudomonas aeruginosa (31 months; 95% CI: 0.00-68.5) than in those without that type of infection (4 months; 95% CI: 1.8-6.2; p = 0.017). However, this association was considered weak, and its clinical significance was uncertain due to the small number of patients without that infection. Conclusions: BAE appears to be effective in the treatment of hemoptysis in patients with CF.


RESUMO Objetivo: A hemoptise maciça é uma das complicações mais graves em pacientes com fibrose cística (FC). O objetivo deste estudo foi avaliar o período livre de hemoptise após a embolização arterial brônquica/não brônquica (EAB/não EAB) em pacientes com FC e investigar preditores de sangramento recorrente e mortalidade por qualquer causa. Métodos: Trata-se de um estudo retrospectivo de coorte de pacientes com FC com idade ≥ 16 anos submetidos a EAB/não EAB para o tratamento de hemoptise entre 2000 e 2017. Resultados: Foram analisados 39 episódios de hemoptise tratada por meio de EAB/não EAB em 17 pacientes com FC. A taxa de recidiva da hemoptise foi de 56,4%. Do total de pacientes, 3 (17,6%) permaneceram sem hemoptise durante o estudo, 2 (11,8%) foram submetidos a transplante de pulmão e 3 (17,6%) morreram. A mediana do período sem hemoptise foi de 17 meses. A mediana do período sem hemoptise foi maior em pacientes com infecção crônica por Pseudomonas aeruginosa (31 meses; IC95%: 0,00-68,5) do que naqueles sem esse tipo de infecção (4 meses; IC95%: 1,8-6,2; p = 0,017). No entanto, essa associação foi considerada fraca, e sua importância clínica foi considerada incerta em virtude do pequeno número de pacientes sem essa infecção. Conclusões: A EAB parece ser eficaz no tratamento de hemoptise em pacientes com FC.


Subject(s)
Humans , Cystic Fibrosis/complications , Cystic Fibrosis/therapy , Embolization, Therapeutic , Bronchial Arteries , Retrospective Studies , Treatment Outcome , Hemoptysis/etiology , Hemoptysis/therapy
5.
Rev. chil. endocrinol. diabetes ; 14(4): 171-177, 2021. tab
Article in Spanish | LILACS | ID: biblio-1348218

ABSTRACT

Los avances en el campo de la fibrosis quística han aumentado la esperanza de vida de estos pacientes, por lo que cada vez es más prevalente la Diabetes Relacionada con la Fibrosis Quística (DRFQ) y sus complicaciones. La DRFQ se asocia a mayor morbimortalidad, deterioro de la función pulmonar y del estado nutricional. Por lo mismo, el manejo óptimo de esta patología depende de un diagnóstico precoz, tratamiento individualizado y vigilancia de las complicaciones diabéticas. El screening de DRFQ debe realizarse anualmente a partir de los 10 años, mediante una Prueba de Tolerancia a la Glucosa Oral (PTGO), lo cual permite el diagnóstico. El manejo de esta patología tiene por objetivo estabilizar y mejorar la función pulmonar y el estado nutricional y metabólico de los pacientes. Actualmente, la insulina es el tratamiento farmacológico de elección para controlar la hiperglicemia y el esquema de uso debe ser individualizado para cada persona. En caso de enfermedades agudas pueden existir mayores requerimientos de insulina. Además, se deben tener consideraciones especiales en cuanto a la dieta y la insuficiencia pancreática exocrina que presentan estos pacientes. Para la vigilancia de complicaciones microvasculares se debe realizar una monitorización anual a partir de los 5 años desde el diagnóstico de DRFQ. Debido a la complejidad de estos pacientes, para alcanzar el mejor cuidado posible se necesita un enfoque multidisciplinario con distintos profesionales de la salud coordinados, incluyendo en la toma de decisiones al paciente y su familia.


Advances made in the field of cystic fibrosis have increased the life expectancy of these patients, which is why Cystic Fibrosis-Related Diabetes (CFRD) and its complications are becoming more and more prevalent. CFRD is associated with increased morbidity and mortality, lower lung function and inadequate weight maintenance. Therefore, the optimal management of this pathology depends on an early diagnosis, individualized treatment and monitoring of diabetic complications. For CFRD, routine screening with an Oral Glucose Tolerance Test (OGTT) should be carried out yearly from the age of 10, which allows to diagnose it. The treatment goals in CFRD are to stabilize and improve lung function and obtain adequate weight gain. Currently, insulin is the pharmacological treatment of choice to control hyperglycemia and the insulin regimen must be personalized for each person. In acute illnesses, there may be higher insulin requirements. In addition, special considerations must be taken regarding diet and exocrine pancreatic insufficiency that these patients present. For the surveillance of microvascular complications, annual monitoring should be carried out 5 years after the diagnosis of CFRD. Due to the complexity of these patients, in order to achieve the best possible care, a multidisciplinary approach is needed with different coordinated health professionals, including the patients and their family in the decision-making process.


Subject(s)
Humans , Cystic Fibrosis/complications , Cystic Fibrosis/diagnosis , Cystic Fibrosis/therapy , Diabetes Mellitus/etiology , Diabetes Mellitus/therapy , Patient Care Team , Mass Screening , Cystic Fibrosis/physiopathology , Nutrition Therapy , Diabetes Mellitus/diagnosis , Diabetes Mellitus/physiopathology , Insulins/therapeutic use , Glucose Tolerance Test , Hypoglycemic Agents/therapeutic use
6.
Cad. Saúde Pública (Online) ; 36(12): e00115720, 2020.
Article in Portuguese | SES-SP, LILACS, SES-SP | ID: biblio-1142635

ABSTRACT

Resumo: O presente artigo se propõe a discutir as muitas complexidades envolvidas na incorporação de novas tecnologias em saúde para doenças raras, tomando como foco central da discussão o caso da fibrose cística. Tal escolha acontece por ser essa uma doença rara, genética, autossômica recessiva, considerada a mais comum entre as doenças raras. Também por ser uma doença que tem se beneficiado imensamente dos investimentos em pesquisa no campo da biologia molecular, feitos principalmente nos Estados Unidos, mas também em grupos de pesquisa europeus que resultaram no registro e comercialização de quatro novos medicamentos. Esses novos fármacos atuam, pela primeira vez, no defeito básico da fibrose cística. Numa perspectiva que entende as doenças raras como um campo de pesquisa tecido entre muitos outros, o presente texto tenta problematizar, com base em uma perspectiva mais centrada nas pessoas com fibrose cística, a dualidade de testemunhar de longe a molecularizaçao da vida, o surgimento de medicamentos de última geração que interrompem, em nível molecular, a cascata de erros e portanto sintomas e evolução de sua doença. O texto busca trazer para o debate os vários elementos que atravessam a complexa realidade local dos pacientes brasileiros com fibrose cística, num contexto global de inovação tecnológica e de quebra de paradigma em seus tratamentos. Baseando-se no campo das doenças raras, passando pela apresentação da fibrose cística em tempos da medicina de precisão, aliado a discussões sobre biopolíticas num contexto de inovação em saúde e medicamentos de alto custo, o artigo tenta dar visibilidade aos desafios e possibilidades do tempo presente.


Abstract: The article proposes to discuss the many complexities involved in the incorporation of new health technologies for rare diseases, with a central focus on the case of cystic fibrosis. Cystic fibrosis was chosen because it is a autosomal recessive genetic disorder, considered the most common of the rare diseases. The disease has also benefited greatly from investments in research in the field of molecular biology, mainly in the United States, but also among European research groups, which resulted in the registration and marketing of four new drugs. These new drugs act for the first time on the basic defect in cystic fibrosis. From a perspective that views rare diseases as a field of research woven among many others, the article aims to problematize cystic fibrosis from a more person-centered approach, the duality of witnessing from afar the molecularization of life, the emergence of last-generation drugs that interrupt, at the molecular level, the cascade of errors and thus the symptoms and evolution of the disease. The article aims to bring various elements to the debate that traverse the complex local reality of Brazilian cystic fibrosis patients in a global context of technological innovation and with a break in the treatment paradigm. Based on the field of rare diseases, including the presentation of cystic fibrosis in the age of precision medicine, alongside discussions on biopolitics in a context of health innovation and high-cost drugs, the article aims to shed light on the current challenges and possibilities.


Resumen: El presente artículo se propone discutir las diversas complejidades implicadas en la incorporación de nuevas tecnologías en salud para enfermedades raras, centrando la discusión en el caso de la fibrosis quística. Tal elección se produce por ser esta una enfermedad rara, genética, autosómica recesiva, considerada la más común entre las enfermedades raras. También por ser una enfermedad que se ha beneficiado inmensamente de las inversiones en investigación en el campo de la biología molecular, realizadas no sólo en los Estados Unidos, sino también en grupos de investigación europeos que resultaron en el registro y comercialización de cuatro nuevos medicamentos. Estos nuevos fármacos actúan, por la primera vez, en el defecto básico de la fibrosis quística. Desde una perspectiva que entiende las enfermedades raras, como un campo de investigación tejido entre muchos otros, el presente texto intenta problematizar, a partir de una perspectiva más centrada en las personas con fibrosis quística, la dualidad de testimoniar de lejos la molecularización de la vida, el surgimiento de medicamentos de última generación que interrumpen, a nivel molecular, la cascada de errores y por tanto síntomas y evolución de su enfermedad. El texto busca presentar en el debate los diferentes elementos que atraviesan la compleja realidad local de los pacientes brasileños con fibrosis quística, en un contexto global de innovación tecnológica y de quiebra de paradigma en sus tratamientos. A partir del campo de las enfermedades raras, pasando por la presentación de la fibrosis quística en tiempos de la medicina de precisión, junto a discusiones sobre biopolíticas en un contexto de innovación en salud y medicamentos de alto coste, el artículo intenta dar visibilidad a los desafíos y posibilidades del tiempo presente.


Subject(s)
Humans , Cystic Fibrosis/therapy , Brazil , Cystic Fibrosis Transmembrane Conductance Regulator , Rare Diseases/therapy , Precision Medicine
9.
Clin. biomed. res ; 40(2): 105-110, 2020.
Article in English | LILACS | ID: biblio-1148366

ABSTRACT

Introduction: Inhalation therapy is a crucial part of the cystic fibrosis (CF) treatment regimen. Drugs that assist in mucociliary clearance and inhaled antibiotics are used by most patients. Methods: This is a cross-sectional study where patients with CF and their caregivers answered questionnaires regarding their adherence to inhalation therapy and QoL. Demographic, spirometric, and bacteriological data, as well as S-K scores and hospitalization frequencies were also collected. Results: We included 66 patients in this study; participants had a mean age of 12.3 years and Z-scores of -1.4 for forced expiratory volume in 1 second and 48.6 for body mass index. Patients were divided into 2 groups according to their self-reported adherence to inhalation therapy: high adherence (n = 46) and moderate/low adherence (n = 20). When comparing both groups, there was no statistically significant differences in age, sex, family income, and S-K score (p > 0.05). The high-adherence group had had shorter hospitalization periods in the previous year (p = 0.016) and presented better scores in the following domains of the QoL questionnaire: emotion (p = 0.006), eating (p = 0.041), treatment burden (p = 0.001), health perception (p = 0.001), and social (p = 0.046). Conclusions: A low self-reported adherence to inhalation therapy recommendations was associated with longer hospitalizations in the previous year and with a decrease in QoL in pediatric patients with CF. (AU)


Subject(s)
Humans , Male , Female , Child , Adolescent , Quality of Life , Respiratory Therapy , Cystic Fibrosis/therapy , Treatment Adherence and Compliance , Cross-Sectional Studies
10.
Neumol. pediátr. (En línea) ; 15(4): 429-483, 2020. ilus, tab
Article in Spanish | LILACS | ID: biblio-1146394

ABSTRACT

Cystic Fibrosis (CF) is the most frequent hereditary disease in whites, with a reserved prognosis. Since 2003, Chile began a comprehensive National Cystic Fibrosis Program, directed by the Respiratory Health Unit of the Ministry of Health. To date, the main results of the Program record a significantly longer survival (average 27 years) and a significant reduction in the age of diagnosis of patients admitted from 2006 onwards. Access to Chilean Explicit Health Guarantees, the implementation of neonatal screening in some regions of the country, the organization and setting up of CF-trained teams of various specialties, has contributed to improving results. Although the main manifestations are of the respiratory and digestive system, the multisystemic nature of CF makes it necessary to know the different aspects involved in its management, in order to optimize the results of the treatment and the resources invested, both in the public and private sectors. This document is a review and an update on the main aspects of the diagnosis, monitoring and treatment of the respiratory and non-respiratory manifestations of CF.


La Fibrosis Quística (FQ) es la enfermedad hereditaria de pronóstico reservado más frecuente en raza blanca. Desde el año 2003, Chile inicia un Programa Nacional de Fibrosis Quística, de carácter integral, dirigido por la Unidad de Salud Respiratoria del Ministerio de Salud. Hasta la fecha, los principales resultados del Programa registran una significativa mayor sobrevida (promedio 27 años) y una significativa reducción en la edad de diagnóstico de los pacientes ingresados desde 2006 en adelante. El acceso a la canasta GES (Garantías Explícitas en Salud), la implementación del tamizaje neonatal en algunas regiones del país, la organización y la constitución de equipos entrenados en FQ de diversas especialidades, ha contribuido a mejorar los resultados. Si bien las principales manifestaciones son del aparato respiratorio y digestivo, el carácter multisistémico de la FQ obliga a conocer los distintos aspectos involucrados en su manejo, a fin de optimizar los resultados del tratamiento y los recursos invertidos, tanto en el sector público como privado. Este documento es una revisión y actualización sobre los principales aspectos del diagnóstico, seguimiento y tratamiento de las manifestaciones respiratorias y no respiratorias de la FQ.


Subject(s)
Humans , Child , Adult , Delivery of Health Care, Integrated , Cystic Fibrosis/diagnosis , Cystic Fibrosis/therapy , Chile , Nutritional Status , Cystic Fibrosis/rehabilitation , Consensus , Health Resources
11.
Ciênc. Saúde Colet ; 24(12): 4717-4726, dez. 2019. tab
Article in Portuguese | LILACS | ID: biblio-1055745

ABSTRACT

Resumo O objetivo deste artigo é avaliar a prevalência e os fatores associados à adesão a terapia de reposição enzimática em adolescentes com Fibrose cística. Estudo transversal, descritivo e observacional. Foram coletados dados sociodemográficos e clínicos. Os instrumentos utilizados para avaliar adesão foram: questionário de Morisky-Green e a dispensação de medicação na farmácia e para os fatores associados, entrevista com questionário estruturado. Foram entrevistados 44 adolescentes. Segundo o método de análise de dispensação da farmácia e o questionário de Morisky-Green, encontramos uma adesão de 45,5% e 11,4%, respectivamente. A maior adesão foi observada naqueles com diagnóstico precoce e a menor nos adolescentes mais velhos e nas meninas. Os fatores com a maior prevalência de não adesão foram: não levar as enzimas ao comer fora de casa, tomar enzimas somente nas grandes refeições, função pulmonar normal, com obstrução grave e muito grave. A prevalência de adesão a enzima foi pequena. Informações relacionadas à doença e ao tratamento devem ser aprimoradas, principalmente em adolescentes mais velhos e com comprometimento da função pulmonar, com criação de estratégias e estudos longitudinais para identificar fatores que interferem na adesão.


Abstract This article sets out to evaluate the prevalence and factors associated with adherence to enzyme replacement therapy among adolescents with cystic fibrosis. It is a cross-sectional, descriptive and observational study. Sociodemographic and clinical data were collected. The instruments used to assess adherence were: the Morisky-Green questionnaire and medication dispensation at the pharmacy, and interviews with structured questionnaires for the associated factors. Forty-four adolescents were interviewed. According to the method of the pharmacy medication dispensation analysis and the Morisky-Green questionnaire, the adherence of 45.5% and 11.4% was found, respectively. The higher adherence was observed in those with early diagnosis and the lowest in older adolescents and girls. The factors with the highest prevalence of non-adherence were: not taking enzymes when eating out of the home; only taking enzymes with major meals; normal lung function; with severe and very severe obstruction. The prevalence of adhesion to enzymes was low. Information related to the disease and treatment should be improved, especially among older adolescents and with impairment of lung function, with the creation of strategies and longitudinal studies to identify factors that interfere with adherence.


Subject(s)
Humans , Male , Female , Child , Adolescent , Young Adult , Cystic Fibrosis/therapy , Medication Adherence/statistics & numerical data , Enzyme Replacement Therapy/statistics & numerical data , Cross-Sectional Studies , Surveys and Questionnaires , Cystic Fibrosis/diagnosis , Early Diagnosis
12.
Neumol. pediátr. (En línea) ; 14(2): 86-91, jul. 2019. ilus, tab
Article in Spanish | LILACS | ID: biblio-1015004

ABSTRACT

Bronchiectasis is a suppurative lung disease with heterogeneous phenotypic characteristics. It is defined as abnormal dilation of the bronchi, losing the existing relationship between bronchial sizes and accompanying artery. According to their form, they can be cylindrical, varicose, saccular or cystic. According to its location, they could be diffuse or localized. The diagnosis of bronchiectasis is usually suspected in patients with chronic cough, mucopurulent bronchorrea, and recurrent respiratory infections. The etiology can be varied, being able to classify in cystic fibrosis bronchiectasis, when there is cystic fibrosis transmembrane regulator (CFTR) gene mutation and not cystic fibrosis, being post infectious the most frequent. Its relationship with childhood is unknown. Severe respiratory infections can predispose in a susceptible subject the so-called theory of the "vicious circle" and the development of these. Persistent bacterial bronchitis in children has been described as a probable cause of not cystic fibrosis bronchiectasis in adults. The treatment is based on the management of symptoms and the prevention of exacerbations. The evidence is poor and many treatments are extrapolated from cystic fibrosis bronchiectasis. We are going to describe the diagnostic and therapeutic approach of non-cystic fibrosis bronchiectasis in adults.


La bronquiectasia es una enfermedad pulmonar supurativa con características fenotípicas heterogéneas. Se define como la dilatación anormal de los bronquios, perdiendo la relación existente entre tamaño bronquial y arteria que acompaña. Según su forma, pueden ser clasificadas en cilíndricas, varicosas, saculares o quísticas y según su etiología presentarse de forma difusa o localizada. El diagnóstico de bronquiectasias se sospecha generalmente en pacientes con tos crónica, broncorrea mucosa, mucupurulenta e infecciones respiratorias recurrentes. La etiología es variada, pudiendo clasificarse en bronquiectasias fibrosis quística, aquellas que se encuentran en el contexto de la mutación del gen regulador transmembrana de fibrosis quística (CFTR) y no fibrosis quística, de etiologías diversas, siendo post infecciosas la gran mayoría. No se conoce con certeza su relación con la infancia, es sabido que infecciones respiratorias severas pueden predisponer en un sujeto susceptible, a la llamada teoría del "circulo vicioso" y el desarrollo de estas. La bronquitis bacteriana persistente en niños se ha descrito como una causa probable del desarrollo de bronquiectasias no fibrosis quística en adultos. El tratamiento se basa en el manejo de los síntomas y la prevención de las exacerbaciones. La evidencia es escasa y la mayoría de las terapias se han investigado en las bronquiectasias tipo fibrosis quística. En este trabajo se explicará el enfrentamiento diagnóstico y terapéutico de los adultos portadores de bronquiectasias no fibrosis quística.


Subject(s)
Humans , Male , Child , Adult , Aspergillosis, Allergic Bronchopulmonary/diagnosis , Aspergillosis, Allergic Bronchopulmonary/therapy , Bronchiectasis/physiopathology , Bronchiectasis/therapy , Cystic Fibrosis/diagnosis , Aspergillosis, Allergic Bronchopulmonary/diagnostic imaging , Bronchiectasis/diagnosis , Bronchiectasis/etiology , Bronchiectasis/epidemiology , Radiography, Thoracic , Macrolides/therapeutic use , Cystic Fibrosis/therapy , Cystic Fibrosis/epidemiology , Anti-Bacterial Agents/therapeutic use
13.
Rev. cuba. pediatr ; 91(1): e460, ene.-mar. 2019. tab, graf
Article in Spanish | LILACS | ID: biblio-985589

ABSTRACT

Introducción: Las investigaciones sobre los cuidadores han tomado importancia por los efectos negativos que provoca en las esferas físicas, psicológicas y sociales de estas personas, debido a su dedicación prolongada a la atención de enfermos cuyas dolencias les impiden totalmente su autocuidado. Objetivos: Referir las características de los cuidadores principales de pacientes pediátricos con fibrosis quística. Métodos: Estudio descriptivo de corte transversal realizado en el segundo semestre del 2016, con 54 cuidadoras principales de pacientes menores de 19 años, atendidos en los hospitales Pediátrico de Centro Habana, Juan Manuel Márquez, de Marianao, y el William Soler, de Boyeros. Resultados: Las cuidadoras son mujeres, madres en su mayoría, sin vínculo laboral, con nivel educativo medio superior, dedicadas al cuidado de los enfermos a tiempo completo, lo que interfiere su vida personal y social. Ante estas situaciones responden con mediana capacidad de afrontamiento y adaptación ante las situaciones que les generan estrés. Conclusiones: Las estrategias de afrontamiento utilizadas por las cuidadoras están afectadas pues no generan respuestas efectivas que les permita adaptarse y responder a las situaciones de forma óptima, por lo que se requiere reforzar los recursos que están utilizando. La meta de enfermería como ciencia y como disciplina es un factor muy importante para obtener esos recursos necesarios para mejorar la calidad de vida de los niños, adolescentes y sus cuidadores(AU)


Introduction: Research on caregivers has become important because of the negative effects this activity causes in the physical, psychological and social aspects of these people due to their prolonged dedication to the care of patients, whose illnesses prevent them from fully looking for themselves. Objective: To describe the characteristics of the main caregivers of pediatric patients with cystic fibrosis. Methods: A cross-sectional descriptive study conducted in the second semester of 2016, with 54 main caregivers of patients under 19 years old that were attended in Centro Habana Pediatric Hospital, Juan Manuel Márquez (Marianao´s municipality Pediatric Hospital), and William Soler (Boyeros municipality´s Pediatric Hospital). Results: Caregivers are women, mostly mothers, with no employment, with a medium superior level of education, who are devoted to caring for the sick patients full-time, and this interferes with their personal and social life. Given these situations, they respond with a medium capacity for coping and adapting to situations that generate stress. Conclusions: Coping strategies used by caregivers are affected because they do not generate effective responses that allow them to adapt and respond to situations in an optimal way, so it is necessary to reinforce the resources they are using. The goal of nursing as a science and as a discipline is a very important factor in obtaining those necessary resources to improve the quality of life of children, adolescents and their caregivers(AU)


Subject(s)
Humans , Male , Female , Child , Adolescent , Caregivers/psychology , Cystic Fibrosis/therapy , Adaptation, Psychological/ethics , Epidemiology, Descriptive , Cross-Sectional Studies
14.
J. bras. pneumol ; 45(3): e20170351, 2019. tab
Article in English | LILACS | ID: biblio-1012549

ABSTRACT

ABSTRACT Objective: Home nebulizers are routinely used in the treatment of patients with cystic fibrosis (CF). This study aims to evaluate the contamination of nebulizers used for CF patients, that are chronically colonized by Pseudomonas aeruginosa, and the association of nebulizer contamination with cleaning, decontamination and drying practices. Methods: A cross-sectional, observational, multicenter study was conducted in seven CF reference centers in Brazil to obtain data from medical records, structured interviews with patients/caregivers were performed, and nebulizer's parts (interface and cup) were collected for microbiological culture. Results: overall, 77 CF patients were included. The frequency of nebulizer contamination was 71.6%. Candida spp. (52.9%), Stenotrophomonas maltophilia (11.9%), non-mucoid P. aeruginosa (4.8%), Staphylococcus aureus (4.8%) and Burkholderia cepacia complex (2.4%) were the most common isolated pathogens. The frequency of nebulizers' hygiene was 97.4%, and 70.3% of patients reported cleaning, disinfection and drying the nebulizers. The use of tap water in cleaning method and outdoor drying of the parts significantly increased (9.10 times) the chance of nebulizers' contamination. Conclusion: Despite the high frequency hygiene of the nebulizers reported, the cleaning and disinfection methods used were often inadequate. A significant proportion of nebulizers was contaminated with potentially pathogenic microorganisms for CF patients. These findings support the need to include patients/caregivers in educational programs and / or new strategies for delivering inhaled antibiotics.


RESUMO Objetivo: Nebulizadores caseiros são usados rotineiramente no tratamento de pacientes com fibrose cística (FC). Este estudo objetiva avaliar a contaminação de nebulizadores utilizados por pacientes de FC que estão cronicamente colonizados por Pseudomonas aeruginosa e a associação da contaminação do nebulizador com a higienização, esterilização e método de secagem. Métodos: Um estudo transversal, observacional, multicêntrico foi conduzido em sete centros de referência de FC no Brasil para obter dados de registros médicos; foram feitas entrevistas estruturadas com os pacientes/cuidadores e partes de nebulizadores (máscara e copo) foram coletados para cultura microbiológica. Resultados: No geral, 77 pacientes com FC foram incluídos. A frequência da contaminação do nebulizador foi de 71,6%. Candida spp. (52,9%), Stenotrophomonas maltophilia (11,9%), P. aeruginosa não mucoide (4,8%), Staphylococcus aureus (4,8%) e complexo Burkholderia cepacia (2.4%) foram os patógenos isolados mais comuns. A frequência de higienização dos nebulizadores foi de 97,4%, e 70,3% dos pacientes relata higienização, esterilização e secagem dos aparelhos. A lavagem com água da torneira e secagem das partes no tempo, em espaço aberto, aumentou significativamente (9 a 10 vezes) a chance de contaminação dos nebulizadores. Conclusões: Apesar dos relatos de frequente higienização dos nebulizadores, os métodos de limpeza e esterilização usados eram inadequados. Uma proporção significativa de nebulizadores foi contaminada com microrganismos potencialmente patogênicos para pacientes com FC. Estes resultados apoiam a necessidade de inclusão dos pacientes/cuidadores em programas educacionais e/ou novas estratégias para fornecimento de antibióticos inalatórios.


Subject(s)
Humans , Male , Female , Child , Adolescent , Adult , Young Adult , Pseudomonas aeruginosa/isolation & purification , Nebulizers and Vaporizers/microbiology , Equipment Contamination/statistics & numerical data , Cystic Fibrosis/therapy , Reference Values , Brazil , Colony Count, Microbial , Logistic Models , Decontamination/methods , Decontamination/statistics & numerical data , Disinfection/methods , Disinfection/statistics & numerical data , Equipment Contamination/prevention & control , Cross-Sectional Studies
15.
Rev. chil. pediatr ; 89(3): 332-338, jun. 2018. tab, graf
Article in Spanish | LILACS | ID: biblio-959530

ABSTRACT

INTRODUCCIÓN: El pronóstico de los pacientes con fibrosis quística (FQ) ha mejorado en forma notable. La evaluación de la progresión de la enfermedad se basa en la medición del Volumen Espirado al primer segundo (VEF1). OBJETIVOS: 1. Describir volúmenes y flujos espiratorios forzados y comparar su interpretación según diferentes patrones de referencia (Knudson, multiétnicas Global Lung Initiative, Gutiérrez); 2. Comparar evolución de VEF1 según diferentes patrones de referencia; 3. Describir respuesta a broncodilatador. PACIENTES Y MÉTODO: Estudio retrospectivo de fichas clínicas y espirometrías de pacientes con FQ controlados en Hospital Dr. Sótero del Río. Se obtuvo antecedentes demográficos, resultados de prueba de sudor, estudio genético, estudio bacteriológico. Se evaluó respuesta a broncodilatador (salbutamol 400 ugr), considerando significativo un aumento en 12% en el VEF1. El valor de cloro en sudor se obtuvo mediante método de Gibson y Cooke. Se registraron: Capacidad Vital Forzada (CVF), Volumen Espirado al primer segundo (VEF1) y relación VEF1/CVF. Para graficar la progresión del VEF1 en el tiempo y las curvas teóricas de GLI, Knudson y Gutiérrez, se utilizó el software de libre distribución R versión 3.3.1. RESULTADOS: Se incluyeron 14 pacientes, 7 varones, edad entre 6 y 24 años, mediana 15 años, me diana índice de masa corporal (IMC) 18,15 (rango 14,6-23,3), mediana cloro en sudor 76 mEq/l (rango 50,2- 119), 7 pacientes con al menos 1 mutación F508del. Al utilizar fórmulas predictivas multiétnicas y de Gutiérrez, el compromiso de la función pulmonar ocurría con anterioridad en relación al uso de ecuaciones de Knudson. Ninguno de los pacientes presentó respuesta significativa a broncodilatador. CONCLUSIÓN: El grupo de pacientes descritos presenta en su mayoría compromiso funcional respiratorio y no tiene respuesta a broncodilatador. La interpretación del compromiso funcional respiratorio varía según los valores teóricos utilizados.


INTRODUCTION: The prognosis of patients with cystic fibrosis (CF) has remarkably improved. The as sessment of the disease progression is based on the measurement of the FEV1 (Forced Expiratory Volume in one second). OBJECTIVES: 1. To describe forced expiratory flows and volumes and com pare their interpretation according to different reference standards (Knudson, Gutiérrez, and multi ethnic GLI); 2. To describe bronchodilator response. Patients and Method: The medical records and spirometries of all patients with CF controlled at the Dr. Sotero del Rio Hospital were reviewed. Demographic background, sweat test results, genetic study , and bacteriological study were obtained. In addition, Forced Vital Capacity (FVC) was recorded as well as FEV1 and FEV1/FVC ratio. RESULTS: Data from 14 patients, were analyzed, seven males, aged 6-24 years, median 15 years, median BMI 18.15 (range 14.6-23.3), median sweat chloride test 76 mEq/l (range 50,2-119 mEq/l), seven patients with at least one F508del mutation. Using multi-ethnic and Gutierrez predictive formulas, lung function involvement occurred previously in relation to the use of Knudson equations. None of the patients had a significant bronchodilator response. CONCLUSION: The group of patients descri bed mostly presents functional respiratory involvement and had no bronchodilator response. The interpretation of functional respiratory involvement varies according to the theoretical values used.


Subject(s)
Humans , Male , Female , Child , Adolescent , Young Adult , Spirometry/standards , Bronchodilator Agents/therapeutic use , Cystic Fibrosis/diagnosis , Cystic Fibrosis/therapy , Reference Standards , Vital Capacity , Forced Expiratory Volume , Retrospective Studies , Follow-Up Studies , Treatment Outcome
16.
Ciênc. Saúde Colet ; 23(5): 1451-1457, Mai. 2018. tab
Article in Portuguese | LILACS | ID: biblio-890595

ABSTRACT

Resumo O objetivo desse estudo foi identificar o perfil do cuidador principal de pacientes pediátricos com Fibrose Cística (FC). Estudo transversal, descritivo e prospectivo, no qual foi realizado, durante consulta farmacêutica, entrevista com cuidadores de pacientes fibrocísticos acompanhados em um centro de referência de um Hospital Universitário do Sul do Brasil. Foram obtidas informações gerais sobre os cuidadores, sobre o entendimento da doença, os medicamentos em uso do paciente e a dinâmica do tratamento em domicílio e na escola. Foram entrevistados 75 cuidadores. Maioria do sexo feminino, com média de idade de 37,3 anos, mãe do paciente e que não trabalhava fora do domicílio. Sessenta e um cuidadores referiram dificuldade de aquisição de medicamentos e as associações de apoio ao paciente foram a principal alternativa para a não interrupção do tratamento. A sobrecarga do processo do cuidar que recai em um único cuidador, gerando impacto social e econômico e alterando o cotidiano familiar foi outro dado observado. Foi constatada a necessidade da intervenção de equipe multidisciplinar capacitada na identificação das dificuldades apresentadas, investindo nas relações interpessoais e efetivando a assistência.


Abstract The scope of this study was to establish the profile of caregivers of pediatric patients diagnosed with Cystic Fibrosis (CF). It was a cross-sectional, descriptive and prospective study in which the caregivers of fibrocystic patients were interviewed during pharmaceutical consultation in a reference center of a University Hospital in southern Brazil. General information was obtained about the caregivers and about their understanding of the disease, drug consumption and dynamics of treatment at home and at school. Seventy-five caregivers were interviewed. Most of them were female, 37.3 years old on average, mothers of the patients who did not work outside the home. Seventy-one caregivers declared difficulties in drug acquisition and patient support associations were highlighted as the main alternative to avoid the interruption of treatment. Another fact observed was the overload of the caregiving process on the shoulders of only one caregiver resulting in social and economic impacts and changes to the family's daily routine. This fact emphasizes the need of intervention by a qualified multidisciplinary team to identify and alleviate difficulties, investing in interpersonal relations and administering care.


Subject(s)
Humans , Male , Female , Child, Preschool , Child , Adolescent , Adult , Young Adult , Health Knowledge, Attitudes, Practice , Caregivers/statistics & numerical data , Cystic Fibrosis/therapy , Interpersonal Relations , Patient Care Team/organization & administration , Brazil , Cross-Sectional Studies , Prospective Studies , Middle Aged
17.
Neumol. pediátr. (En línea) ; 12(4): 182-186, oct. 2017. tab
Article in Spanish | LILACS | ID: biblio-999180

ABSTRACT

Chest physiotherapy is an essential component of cystic fibrosis treatment. Its aim is to delay lung deterioration and preserve physical function, improving quality of life and long-term results. It is possible to proceed early, even when the child is asymptomatic.The therapist must be a competent professional, able to participate in a health care team and to make therapeutic decisions. Currently, the therapist is responsible for optimizing mucociliary clearance, but in, addition, their work has extended to education, aerosol therapy, physical activity recommendations, non-invasive ventilatory support, oxygen therapy.This article provides a general description of the chest therapist proceedings related to the integral management of cystic fibrosis


La kinesioterapia respiratoria es parte del tratamiento integral de la Fibrosis Quística. Con ella, apuntamos a retrasar el deterioro pulmonar y preservar la función física, mejorando la calidad de vida y los resultados a largo plazo. Es posible actuar de manera precoz, incluso cuando el niño es asintomático.Es de importancia que el kinesiólogo que trate a pacientes con Fibrosis Quística sea un profesional competente, con capacidad de formar parte de un equipo de salud y participar activamente en las decisiones terapéuticas requeridas, ya que en la actualidad, el kinesiólogo ha ampliado su labor, ya no solo circunscribiéndose a las técnicas manuales e instrumentales para optimizar el drenaje bronquial. Este articulo entrega una descripción general del actuar del kinesiólogo en el manejo integral del paciente con Fibrosis Quística


Subject(s)
Humans , Infant , Child, Preschool , Physical Therapy Modalities , Cystic Fibrosis/rehabilitation , Respiratory Therapy , Breathing Exercises , Bronchodilator Agents/administration & dosage , Drainage, Postural , Positive-Pressure Respiration , Cystic Fibrosis/therapy , Physical Therapy Specialty/methods , Exercise Therapy
18.
J. bras. pneumol ; 43(3): 219-245, May-June 2017. tab, graf
Article in English | LILACS | ID: biblio-893829

ABSTRACT

ABSTRACT Cystic fibrosis (CF) is an autosomal recessive genetic disorder characterized by dysfunction of the CFTR gene. It is a multisystem disease that most often affects White individuals. In recent decades, various advances in the diagnosis and treatment of CF have drastically changed the scenario, resulting in a significant increase in survival and quality of life. In Brazil, the current neonatal screening program for CF has broad coverage, and most of the Brazilian states have referral centers for the follow-up of individuals with the disease. Previously, CF was limited to the pediatric age group. However, an increase in the number of adult CF patients has been observed, because of the greater number of individuals being diagnosed with atypical forms (with milder phenotypic expression) and because of the increase in life expectancy provided by the new treatments. However, there is still great heterogeneity among the different regions of Brazil in terms of the access of CF patients to diagnostic and therapeutic methods. The objective of these guidelines was to aggregate the main scientific evidence to guide the management of these patients. A group of 18 CF specialists devised 82 relevant clinical questions, divided into five categories: characteristics of a referral center; diagnosis; treatment of respiratory disease; gastrointestinal and nutritional treatment; and other aspects. Various professionals working in the area of CF in Brazil were invited to answer the questions devised by the coordinators. We used the PubMed database to search the available literature based on keywords, in order to find the best answers to these questions.


RESUMO A fibrose cística (FC) é uma doença genética autossômica recessiva caracterizada pela disfunção do gene CFTR. Trata-se de uma doença multissistêmica que ocorre mais frequentemente em populações descendentes de caucasianos. Nas últimas décadas, diversos avanços no diagnóstico e tratamento da FC mudaram drasticamente o cenário dessa doença, com aumento expressivo da sobrevida e qualidade de vida. Atualmente, o Brasil dispõe de um programa de ampla cobertura para a triagem neonatal de FC e centros de referência distribuídos na maior parte desses estados para seguimento dos indivíduos. Antigamente confinada à faixa etária pediátrica, tem-se observado um aumento de pacientes adultos com FC tanto pelo maior número de diagnósticos de formas atípicas, de expressão fenotípica mais leve, assim como pelo aumento da expectativa de vida com os novos tratamentos. Entretanto, ainda se observa uma grande heterogeneidade no acesso aos métodos diagnósticos e terapêuticos para FC entre as diferentes regiões brasileiras. O objetivo dessas diretrizes foi reunir as principais evidências científicas que norteiam o manejo desses pacientes. Um grupo de 18 especialistas em FC elaborou 82 perguntas clínicas relevantes que foram divididas em cinco categorias: características de um centro de referência; diagnóstico; tratamento da doença respiratória; tratamento gastrointestinal e nutricional; e outros aspectos. Diversos profissionais brasileiros atuantes na área da FC foram convidados a responder as perguntas formuladas pelos coordenadores. A literatura disponível foi pesquisada na base de dados PubMed com palavras-chave, buscando-se as melhores respostas às perguntas dos autores.


Subject(s)
Humans , Male , Female , Cystic Fibrosis/diagnosis , Cystic Fibrosis/therapy , Practice Guidelines as Topic , Age Factors , Brazil , Evidence-Based Medicine , Nutritional Status , Physical Therapy Modalities , Quality of Life
19.
Brasília; CONITEC; out. 2016. ilus, graf.
Monography in Portuguese | LILACS, BRISA | ID: biblio-837307

ABSTRACT

Contexto: Os pacientes com fibrose cística apresentam infecções respiratórias recorrentes, também chamadas de exacerbações, causadas por bactérias, em especial a Pseudomonas aeruginosa. As infecções geram piora da função pulmonar e reduzem sua qualidade de vida. A proposta avaliada é o uso da tobramicina em nebulização para reduzir as exacerbações respiratórias. Pergunta: Nos pacientes com fibrose cística o uso de tobramicina inalatória reduz infecções respiratórias ou impacta em outros desfechos importantes para o paciente? Evidências científicas: Foi realizado monitoramento do horizonte tecnológico na base Cortellis e revisão da literatura priorizando revisões sistemáticas nas bases PubMed e biblioteca Cochrane. Não foram localizadas evidências de impacto em desfechos importantes para o paciente. O uso inalatório do antibiótico pode induzir broncoespasmos. Decisão: Incorporar a tobramicina inalatória para o tratamento da infecção crônica por Pseudomonas aeruginosa das vias aéreas em pacientes com fibrose cística, conforme Protocolo do Ministério da Saúde, no âmbito do Sistema Único de Saúde - SUS, dada pela Portaria SCTIE-MS nº 36 publicada no Diário Oficial da União (DOU) nº 207, de 27 de outubro de 2016.


Subject(s)
Humans , Cystic Fibrosis/therapy , Pseudomonas aeruginosa , Pseudomonas Infections , Tobramycin/therapeutic use , Brazil , Cost-Benefit Analysis , Respiratory Tract Infections , Technology Assessment, Biomedical , Unified Health System
20.
J. bras. pneumol ; 42(1): 29-34, Jan.-Feb. 2016. tab
Article in Portuguese | LILACS | ID: lil-776477

ABSTRACT

Objective : To evaluate the level of self-reported adherence to physical therapy recommendations in pediatric patients (6-17 years) with cystic fibrosis (CF) and to ascertain whether the different levels of adherence correlate with pulmonary function, clinical aspects, and quality of life. Methods : This was a cross-sectional study. The patients and their legal guardians completed a questionnaire regarding adherence to physical therapy recommendations and a CF quality of life questionnaire. We collected demographic, spirometric, and bacteriological data, as well as recording the frequency of hospitalizations and Shwachman-Kulczycki (S-K) clinical scores. Results : We included 66 patients in the study. Mean age, FEV1 (% of predicted), and BMI were 12.2 ± 3.2 years, 90 ± 24%, and 18.3 ± 2.5 kg/m2, respectively. The patients were divided into two groups: high-adherence (n = 39) and moderate/poor-adherence (n = 27). No statistically significant differences were found between the groups regarding age, gender, family income, and total S-K clinical scores. There were statistically significant differences between the high-adherence group and the moderate/poor-adherence group, the latter showing lower scores for the "radiological findings" domain of the S-K clinical score (p = 0.030), a greater number of hospitalizations (p = 0.004), and more days of hospitalization in the last year (p = 0.012), as well as lower scores for the quality of life questionnaire domains emotion (p = 0.002), physical (p = 0.019), treatment burden (p < 0.001), health perceptions (p = 0.036), social (p = 0.039), and respiratory (p = 0.048). Conclusions : Low self-reported adherence to physical therapy recommendations was associated with worse radiological findings, a greater number of hospitalizations, and decreased quality of life in pediatric CF patients.


Objetivo : Avaliar o grau de adesão autorrelatada às recomendações fisioterapêuticas em pacientes pediátricos (6-17 anos) com fibrose cística (FC) e determinar se os diferentes níveis de adesão se correlacionam com a função pulmonar, aspectos clínicos e qualidade de vida. Métodos : Estudo transversal no qual os pacientes e responsáveis responderam um questionário sobre a adesão à fisioterapia recomendada e um questionário da qualidade de vida em FC. Foram coletados dados demográficos, espirométricos e bacteriológicos, assim como a frequência de internações e resultados do escore clínico de Shwachman-Kulczycki (S-K). Resultados : Participaram 66 pacientes. As médias de idade, VEF1 (em % do previsto) e IMC foram, respectivamente, 12,2 ± 3,2 anos, 90 ± 24% e 18,3 ± 2,5 kg/m2. Os pacientes foram divididos em dois grupos: alta adesão (n = 39) e moderada/baixa adesão (n = 27). Não houve diferenças estatisticamente significativas para idade, sexo, renda familiar e escore clínico de S-K total na comparação dos dois grupos. Houve diferenças estatisticamente significativas entre os grupos alta adesão e moderada/baixa adesão, este último mostrando valores significativamente menores para o domínio "achados radiológicos" do escore clínico de S-K apresentou (p = 0,030), um maior número de hospitalizações (p = 0,004) e de dias de internação no último ano (p = 0,012), assim como menores escores para os seguintes domínios do questionário de qualidade de vida: emocional (p = 0,002), físico (p = 0,019), tratamento (p < 0,001), saúde (p = 0,036), social (p = 0,039) e respiratório (p = 0,048). Conclusões : A baixa adesão autorrelatada às recomendações fisioterapêuticas associou-se com piores achados radiológicos, maior número de hospitalizações e diminuição da qualidade de vida em pacientes pediátricos com FC.


Subject(s)
Humans , Male , Female , Child , Adolescent , Cystic Fibrosis/therapy , Patient Compliance/statistics & numerical data , Respiratory Therapy/statistics & numerical data , Cross-Sectional Studies , Hospitalization/statistics & numerical data , Quality of Life , Respiratory Therapy/methods , Self Report , Spirometry , Statistics, Nonparametric , Treatment Outcome
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