Results 51 to 60 of about 5,413 (194)

Proteomic profiling of sweat in patients with cystic fibrosis provides new insights into epidermal homoeostasis

Skin Health and Disease, 2023
Background A high proportion of patients with Cystic Fibrosis (CF) also present the rare skin disease aquagenic palmoplantar keratoderma. A possible link between this condition and absence of a functional CF Transmembrane conductance Regulator protein in
Matthieu Cornet, Thao Nguyen‐Khoa, Mairead Kelly‐Aubert, Vincent Jung, Frédérique Chedevergne, Muriel Le Bourgeois, Laura Aoust, Kévin Roger, Chiara Ida Guerrera, Isabelle Sermet‐Gaudelus   +9 more
doaj   +1 more source

A intersubjetividade no contexto da família de pessoas com fibrose cística

Revista Brasileira de Enfermagem, 2012
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, Silvana Sidney Costa Santos, Valéria Lerch Lunardi, Ellen Dayane Cargnin Pimentel, Sandra Marisa Pelloso, Maria Dalva de Barros Carvalho   +5 more
doaj   +1 more source

Implementation of Model-Based Dose Adjustment of Tobramycin in Adult Patients with Cystic Fibrosis

Pharmaceutics, 2022
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, Vianney Tuloup, Romain Garreau, Viviane Nave, Sabine Cohen, Philippe Reix, Stéphane Durupt, Raphaele Nove-Josserand, Isabelle Durieu, Quitterie Reynaud, Laurent Bourguignon, Sandrine Charles, Sylvain Goutelle   +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]

, 2012
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

Phenotype‐Genotype Correlations in ABCA3 Patients—The RespiRare Cohort

Pediatric Pulmonology, Volume 60, Issue 10, October 2025.
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, Céline Delestrain, Alice Hadchouel, Julie Mazenq, Myriam Benhamida, Anne‐Sophie Bernard, Raphaël Borie, Jacques Brouard, Harriet Corvol, Pierrick Cros, Christophe Delacourt, Tifenn Desroziers, Jean‐Christophe Dubus, Carole Egron, Ralph Epaud, Michael Fayon, Aude Forgeron, Lisa Giovannini‐Chami, Christophe Marguet, Alexandra Masson‐Rouchaud, Hortense Petat, Marie‐Catherine Renoux, Léa Roditis, Caroline Thumerelle, Clémentine Vigier, Aurore Coulomb L'Herminé, Hubert Ducou le Pointe, Pascale Fanen, Camille Fletcher, Chiara Sileo, Laureline Berteloot, Camille Louvrier, Alix de Becdelièvre, Marie Legendre, Nadia Nathan   +34 more
wiley   +1 more source

Prática fisioterapêutica no tratamento da fibrose cística

ABCS Health Sciences, 2014
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, Cintia Teixeira Vieira, Kelen Nunes Fernandes, Laís Machado Jorge, Gabriela da Silva Cândido, Rafael Inácio Barbosa, Rafael Cypriano Dutra   +6 more
doaj   +1 more source

De l'animal expérimental au modèle en recherche biomédicale. [PDF]

, 2008
Cet article est destiné à la Revue PhilosophiqueSince the beginning of experimentation in biology, animals have been used to study processes that cannot be approached on human beings.
Gachelin, Gabriel
core   +3 more sources

Linguistic validation of cystic fibrosis quality of life questionnaires [PDF]

, 2006
OBJECTIVE: The purpose of this study was to validate the Portuguese translations of four cystic fibrosis quality of life questionnaires (CFQ). The first three were developed for patients with cystic fibrosis aged from 6 to 11 years, from 12 to 13 years ...
Cunha, Maristela T., Jardim, José Roberto, Nascimento, Oliver Augusto, Quittner, Alexandra L., Rozov, Tatiana   +4 more
core   +2 more sources

Expression and Roles of Antimicrobial Peptides in Innate Defense of Airway Mucosa: Potential Implication in Cystic Fibrosis

Frontiers in Immunology, 2020
The treatment of respiratory infections is associated with the dissemination of antibiotic resistance in the community and clinical settings. Development of new antibiotics is notoriously costly and slow; therefore, alternative strategies are needed ...
Regina Geitani, Carole Ayoub Moubareck, Carole Ayoub Moubareck, Zhengzhong Xu, Zhengzhong Xu, Zhengzhong Xu, Dolla Karam Sarkis, Lhousseine Touqui, Lhousseine Touqui   +8 more
doaj   +1 more source

Phagocytosis depends on TRPV2-mediated calcium influx and requires TRPV2 in lipids rafts: alteration in macrophages from patients with cystic fibrosis. [PDF]

, 2018
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, A Aderem, A Di, A Magenau, A Oláh, A Perez, A Perálvarez-Marín, B Svobodova, D Wang, DJ Hackam, E Hisanaga, E Pernet, F Antigny, F Antigny, G Santoni, H Balghi, H Grassmé, H Matsui, JD Young, JH Guo, Jvan Grevenynghe, K Abe, K Aoyagi, K Simonin-Le Jeune, K-Y Jih, L Ambrus, L Nie, L Petrocellis De, L Wei, LM Carrithers, LV Deriy, M Lévêque, M Nabissi, M Nagasawa, M Nagasawa, M Saito, MD Carrithers, MJ Caterina, MP Kowalski, N Qin, NE Alexis, P Schatzlmaier, P Zhang, T Hishikawa, T Ma, TM Link, Y Iwata, É Sághy   +47 more
core   +4 more sources