A intersubjetividade no contexto da família de pessoas com fibrose cística
Trata-se de uma investigação qualitativa, com aproximação fenomenológica, que objetivou desvelar os processos intersubjetivos no contexto da família de pessoas com Fibrose Cística (FC).
Geisa Santos Luz +5 more
doaj +1 more source
Implementation of Model-Based Dose Adjustment of Tobramycin in Adult Patients with Cystic Fibrosis
Therapeutic drug monitoring (TDM) of tobramycin is widely performed in patients with cystic fibrosis (CF), but little is known about the value of model-informed precision dosing (MIPD) in this setting.
Jérémy Reverchon +12 more
doaj +1 more source
Les enjeux du dépistage néonatal systématique de la mucoviscidose : reconfigurations organisationnelles et professionnelles autour d’une maladie incurable [PDF]
En France, la mucoviscidose, maladie génétique rare et incurable, fait l’objet depuis 2002, d’un Dépistage Néonatal Systématique mis en place par les pouvoirs publics.
C. Langeard
core +1 more source
Surfactant protein a in cystic fibrosis: supratrimeric structure and pulmonary outcome. [PDF]
The state of oligomerization of surfactant associated protein-A (SP-A) monomers differs between individuals. This likely affects SP-A's functional properties and could thereby influence clinical status in patients with lung diseases.
Ballmann, Manfred +7 more
core +2 more sources
Phenotype‐Genotype Correlations in ABCA3 Patients—The RespiRare Cohort
ABSTRACT Background ATP‐binding cassette transporter A3 (ABCA3) deficiency is one of the most severe causes of childhood interstitial lung diseases (chILD). This study aims to report the RespiRare ABCA3 cohort and to establish phenotype‐genotype correlations. Methods Phenotypic and genotypic data of patients under 18 years were retrospectively included
Manon Fleury +34 more
wiley +1 more source
Prática fisioterapêutica no tratamento da fibrose cística
A fibrose cística (FC), denominada também de mucoviscidose, é uma doença genética do tipo autossômica recessiva que acomete principalmente crianças e indivíduos de raça branca.
Carolina Lazzarim de Conto +6 more
doaj +1 more source
La transition des soins des adolescents atteints de mucoviscidose passant d'un centre pédiatrique à un service de soins pour adultes: interventions infirmières : une revue de littérature étoffée [PDF]
L’espérance de vie des enfants atteint de mucoviscidose a considérablement augmenté ces dernières années. Afin d’offrir des soins adaptés à cette nouvelle population atteinte de mucoviscidose, des centres pour adultes ont dû être développés.
Lerch, Naïma +2 more
core
Phagocytosis depends on TRPV2-mediated calcium influx and requires TRPV2 in lipids rafts: alteration in macrophages from patients with cystic fibrosis. [PDF]
Whereas many phagocytosis steps involve ionic fluxes, the underlying ion channels remain poorly defined. As reported in mice, the calcium conducting TRPV2 channel impacts the phagocytic process.
A Abu-Arish +47 more
core +4 more sources
One year of ETI reduces lung bacterial colonisation in adults with cystic fibrosis
The triple combination elexacaftor-tezacaftor-ivacaftor (ETI) has provided unprecedented clinical benefits for people with cystic fibrosis (pwCF) and drastically transformed the outcome of this disease.
Lucile Mianowski +7 more
doaj +1 more source
Assessment of the efficacy of an antimicrobial peptide in the context of cystic fibrosis airways
Antimicrobial peptides (AMPs) offer a promising alternative to control airway infections with multi-resistant bacteria, such as methicillin-resistant Staphylococcus aureus (MRSA), which commonly infects patients with cystic fibrosis (CF).
Albane Jouault +3 more
doaj +1 more source

