Jornal de Pediatria, 1998 OBJECTIVE: Due to the great advances recently achieved in the treatment of Cystic Fibrosis as well as to the fact that pediatricians need to have a better understanding of this disease, the authors propose an extensive review of the subject. METHODS: We selected the most outstanding publications on Cystic Fibrosis in the international literature of the
F J, Reis, N, Damaceno
openaire +4 more sources
miR-17 overexpression in cystic fibrosis airway epithelial cells decreases interleukin-8 production. [PDF]
, 2015 Interleukin (IL)-8 levels are higher than normal in cystic fibrosis (CF) airways, causing neutrophil infiltration and non-resolving inflammation.
Agrawal, Raman +9 more
core +2 more sources
The impact of 12 months treatment with ivacaftor on Scottish paediatric patients with cystic fibrosis with the G551D mutation : a review [PDF]
, 2016 We reviewed the impact of ivacaftor on Scottish paediatric cystic fibrosis (CF) patients ≥ 6 years of age after 12 months of treatment. Statistically significant improvements in FEV1 and BMI and a reduction in sweat chloride, all comparable with ...
Scottish Paediatric Cystic Fibrosis Managed Clinical Network (SPCFMCN)
core +1 more source
Cystic Fibrosis Transport Regulator and its mRNA are Expressed in Human Epidermis [PDF]
, 2002 Cystic fibrosis transport regulator is a cAMP-dependent chloride channel protein. Normal (non cystic fibrosis) human epidermis stained positive for cystic fibrosis transport regulator as densely as did the eccrine sweat gland when three monoclonal ...
Sato, Fusako +3 more
core +11 more sources
Interventions for the eradication of meticillin-resistant Staphylococcus aureus (MRSA) in people with cystic fibrosis [PDF]
, 2015 Background: Cystic fibrosis is an inherited recessive disorder of chloride transport that is characterised by recurrent and persistent pulmonary infections from resistant organisms that result in lung function deterioration and early mortality in ...
Smyth, Alan R.
core +4 more sources
Cystic fibrosis mice carrying the missense mutation G551D replicate human genotype phenotype correlations [PDF]
, 1996 We have generated a mouse carrying the human G551D mutation in the cystic fibrosis transmembrane conductance regulator gene (CFTR) by a one-step gene targeting procedure.
Alton, Ewfw +11 more
core +2 more sources
Elevated sweat chloride test: is it always cystic fibrosis?
Italian Journal of Pediatrics, 2021 Background The sweat chloride test (ST) is the gold standard for cystic fibrosis (CF) diagnosis in symptomatic patients, within the newborn screening and in the follow-up of CF patients during molecular therapies.
C. Cimbalo +6 more
doaj +1 more source
Early respiratory viral infections in infants with cystic fibrosis [PDF]
, 2015 This article is made available for unrestricted research re-use and secondary analysis in any form or by any means with acknowledgement of the original source.
A Peshkin +41 more
core +2 more sources
Longitudinal impact of demographic and clinical variables on Health-Related Quality of Life in Cystic Fibrosis [PDF]
, 2015 Objectives: The insights that people with cystic fibrosis have concerning their health are important given that aspects of health-related quality of life (HRQoL) are independent predictors of survival and a decrease in lung function is associated with a ...
A-Rahman +33 more
core +2 more sources
Cystic Fibrosis Foundation and European Cystic Fibrosis Society Survey of cystic fibrosis mental health care delivery [PDF]
, 2015 Background: Psychological morbidity in individuals with cystic fibrosis (CF) and their caregivers is common. The Cystic Fibrosis Foundation (CFF) and European Cystic Fibrosis Society (ECFS) Guidelines Committee on Mental Health sought the views of CF ...
A.L. Quittner +33 more
core +1 more source