Cystic Fibrosis in Bangladesh – Diagnosis by Pilocarpine Iontophoresis Sweat Chloride Test and Phenotypic Spectrum of the Disease: An Observational Study

2021 ◽  
Author(s):  
M. S. Khaled ◽  
Firoza Akter ◽  
Jahangir Khan ◽  
Sayedul Islam ◽  
Md. Golam Dostogir Harun
Keyword(s):  
Cystic Fibrosis ◽  
Observational Study ◽  
Sweat Chloride ◽  
Phenotypic Spectrum ◽  
Pilocarpine Iontophoresis

scholarly journals Bacteraemia and fungaemia in cystic fibrosis patients with febrile pulmonary exacerbation: a prospective observational study

2017 ◽  
Vol 17 (1) ◽  
Author(s):  
Joerg Grosse-Onnebrink ◽  
Florian Stehling ◽  
Eva Tschiedel ◽  
Margarete Olivier ◽  
Uwe Mellies ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Observational Study ◽  
Prospective Observational Study ◽  
Pulmonary Exacerbation

Factors Associated With Clinical Progression to Severe COVID-19 in People With Cystic Fibrosis: A Global Observational Study

2021 ◽  
Author(s):  
Siobhán B. Carr ◽  
Elliot McClenaghan ◽  
Alexander Elbert ◽  
Albert Faro ◽  
Rebecca Cosgriff ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Observational Study ◽  
Clinical Progression ◽  
Factors Associated

ΔF508 Genotype Does Not Predict Disease Severity in an Ethnically Diverse Cystic Fibrosis Population

PEDIATRICS ◽  
1994 ◽  
Vol 93 (1) ◽  
pp. 114-118
Author(s):  
Lucille A. Lester ◽  
Jerome Kraut ◽  
John Lloyd-Still ◽  
Theodore Karrison ◽  
Carol Mott ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Disease Severity ◽  
Ethnically Diverse ◽  
Clinical Parameter ◽  
Population Based ◽  
Age At Diagnosis ◽  
Chest Roentgenogram ◽  
Sweat Chloride ◽  
Cystic Fibrosis Population ◽  
Cftr Mutations

Objective. As part of a study to determine population-based frequencies of CFTR mutations in an ethnically diverse, midwestern cystic fibrosis (CF) population, clinical histories were studied in 119 CF patients. Methodology. We sought to examine the association between genotype as characterized by the ΔF508 and 11 other commonly occurring mutations and clinical parameters including age at diagnosis, clinical presentation, sweat chloride level, chest roentgenogram score, clinical scores, pulmonary function test results, percent weight for height, and presence of associated CF complications. Results. Age at diagnosis of CF was significantly associated with homozygosity for ΔF508 (mean age at diagnosis ± SE: 1.7 ± 0.3 years for ΔF508/ΔF508 vs 3.9 ± 0.9 years for ΔF508/other and other/other; P = .03). No other age-adjusted clinical parameter was significantly associated with ΔF508 or any other genotype. Conclusion. These data suggest that in this sample of CF patients, ΔF508 genotype is not predictive of disease severity. The lack of association between disease severity and genotype in this ethnically diverse sample may reflect the presence of more severe undetected mutations in our sample, or the effects of modifying genes at other, non-CF loci.

Rectal organoid morphology analysis (ROMA) as a promising diagnostic tool in cystic fibrosis

Thorax ◽  
2021 ◽  
pp. thoraxjnl-2020-216368
Author(s):  
Senne Cuyx ◽  
Anabela Santo Ramalho ◽  
Nikky Corthout ◽  
Steffen Fieuws ◽  
Eva Fürstová ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Image Analysis ◽  
Diagnostic Tool ◽  
Automated Image Analysis ◽  
Sweat Chloride ◽  
Morphology Analysis ◽  
Morphological Differences ◽  
Two Parameters ◽  
Analysis Protocol

Diagnosing cystic fibrosis (CF) when sweat chloride is not in the CF range and less than 2 disease-causing CFTR mutations are found requires physiological CFTR assays, which are not always feasible or available. We developed a new physiological CFTR assay based on the morphological differences between rectal organoids from subjects with and without CF. In organoids from 167 subjects with and 22 without CF, two parameters derived from a semi-automated image analysis protocol (rectal organoid morphology analysis, ROMA) fully discriminated CF subjects with two disease-causing mutations from non-CF subjects (p<0.001). ROMA, feasible at all ages, can be centralised to improve standardisation.

Physical activity participation by adults with cystic fibrosis: An observational study

Respirology ◽  
2015 ◽  
Vol 21 (3) ◽  
pp. 511-518 ◽  
Author(s):  
Narelle S. Cox ◽  
Jennifer A. Alison ◽  
Brenda M. Button ◽  
John W. Wilson ◽  
Judith M. Morton ◽  
...  
Keyword(s):  
Physical Activity ◽  
Cystic Fibrosis ◽  
Observational Study ◽  
Physical Activity Participation ◽  
Activity Participation
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