Cystic Fibrosis
Mostrando 1-12 de 1648 artigos, teses e dissertações.
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1. O que é considerado constipação no lactente e como abordar?
Constipação é definida como dificuldade para evacuar ou a evacuação de fezes duras, de largo calibre e/ou ressecadas, que necessitam muito esforço, com frequência evacuatória de duas ou menos vezes por semana e/ou associação à dor. Quando os sintomas estão presentes por menos de quatro semanas é tida como constipação aguda e além deste per
Núcleo de Telessaúde Rio Grande do Sul. Publicado em: 12/06/2023
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2. Systematic review on fecal calprotectin in cystic fibrosis
Abstract Objectives: Fecal calprotectin is an inflammatory marker used for monitoring intestinal diseases. It has been studied as a marker of intestinal inflammation in cystic fibrosis (CF), a multi-systemic genetic disease caused by alterations to the CFTR gene. Manifestations of the disease favor a systemic inflammation not limited to the respiratory trac
Jornal de Pediatria. Publicado em: 2023
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3. Five-year performance analysis of a cystic fibrosis newborn screening program in northeastern Brazil
Abstract Objective: To analyze the performance of the cystic fibrosis (CF) newborn screening (NBS) program over its first five years in a Brazilian northeastern state. Methods: A population-based study using a screening algorithm based on immunoreactive trypsinogen (IRT)/IRT. Data were retrieved from the state referral screening center registry. The progra
Jornal de Pediatria. Publicado em: 2023
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4. Pediatric population with cystic fibrosis in the centre of Portugal: candidates for new therapies
Abstract Objectives: Cystic fibrosis (CF) is a severe autosomal recessive disease that results from mutations in a gene encoding the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) protein, a chloride channel. This study aims to characterize the clinical and genetic features of a cohort of pediatric people with CF (PwCF) in the center of Portugal
Jornal de Pediatria. Publicado em: 2022
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5. Predictive factors for premature birth and respiratory exacerbation in pregnancies of women with cystic fibrosis
Abstract Objective: In this present study, the authors evaluated the predictive factors for adverse maternal-fetal outcomes in pregnancies of women with cystic fibrosis (CF). Patients were followed up by a referral center for adults in southern Brazil. Methods: This is a retrospective cohort study that used data from electronic medical records regarding pr
Jornal de Pediatria. Publicado em: 2022
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6. Clinical complications in children with false-negative results in cystic fibrosis newborn screening
Abstract Objective: To present signs and symptoms and clinical course in cystic fibrosis patients with false-negative newborn screening (CF NBS). Materials and methods: All children presented in this paper were covered by CF NBS. The group of 1.869.246 newborns was screened in the Institute of Mother and Child in Warsaw within a period of 01.01.1999 – 31
Jornal de Pediatria. Publicado em: 2022
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7. Approach and anesthetic management for kidney transplantation in a patient with bilateral lung transplantation: case report
Abstract Lung transplantation is the last resort for end-stage lung disease treatment. Due to increased survival, lung recipients present an increased likelihood to be submitted to anesthesia and surgery. This case report describes a 23-year-old female patient with history of lung transplantation for cystic fibrosis, with multiple complications, and chronic
Brazilian Journal of Anesthesiology. Publicado em: 2022
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8. Upper Airway Findings and Markers of Lung Disease Progression in Patients with Cystic Fibrosis
Abstract Introduction Cystic fibrosis (CF) is a genetic disease that limits the quality of life mainly due to respiratory symptoms. The relationship between findings of the upper airways and CF lung disease is not yet completely understood. Objective The aim of the present study is to describe the most frequent nasal findings and pathogens in patients with
Int. Arch. Otorhinolaryngol.. Publicado em: 2020-12
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9. Association between lung function, thoracoabdominal mobility and posture in cystic fibrosis: pilot study
Resumo Introdução: A deterioração da função pulmonar é a principal causa de mortalidade na fibrose cística (FC), portanto é fundamental estudar diferentes fatores que se relacionam com esta variável. Objetivo: Verificar em indivíduos FC, a associação da função pulmonar com a mobilidade toracoabdominal e a postura. Método: Estudo piloto tra
Fisioter. mov.. Publicado em: 13/01/2020
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10. Lung ultrasound assessment of response to antibiotic therapy in cystic fibrosis exacerbations: a study of two cases
J. bras. pneumol.. Publicado em: 25/11/2019
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11. Adesão de adolescentes com fibrose cística a terapia de reposição enzimática: fatores associados
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 d
Ciênc. saúde coletiva. Publicado em: 25/11/2019
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12. Determining the pathogenicity of CFTR missense variants: Multiple comparisons of in silico predictors and variant annotation databases
Abstract Pathogenic variants in the Cystic Fibrosis Transmembrane Conductance Regulator gene (CFTR) are responsible for cystic fibrosis (CF), the commonest monogenic autosomal recessive disease, and CFTR-related disorders in infants and youth. Diagnosis of such diseases relies on clinical, functional, and molecular studies. To date, over 2,000 variants have
Genet. Mol. Biol.. Publicado em: 14/11/2019